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JOURNAL
OF
THE MOUNT SINAI HOSPITAL
VOLUME XII 1945-1946
Waverly Press, Inc. Baltimore, Md.
EDITORIAL BOARD
Joseph H. Globus, I Ira Cohen, M.D. (ex-officio) Leo Edelman, M.D. Joseph Harkavy, M.D. Eli Moschcowitz, M.D. Albert Cornell, M.D. Solon S. Bernstein, M.D.
[.D., Editor-in-chief Harold Neuhof, M.D.
WlLLI.\M M. HiTZIG, M.D.
IsiDOR C. Rubin, M.D. Samuel Silbert, M.D. Seymour Wimpfheimer, M.D. Frederic D. Zeman, M.D.
Copyright, 1945, The Mount Sinai Hospital
CONTENTS OF VOLUME XII
Number 1, May-June, 1945
PAGE
Foreword. George Baehr, M.D xi
Eli Moschcowitz, on the Doorstep of the Hospital. Howard Lilienthal, M.D. 1 Dr. Eli Moschcowitz — His Contributions to the Science and Art of Medicine
and Related Topics 2
Stigmata of Infertility. Robert T. Frank, M.D 5
Congenital Atresia of the Esophagus With Tracheoesophageal Fistula. Er- nest E. Arnheim, M.D ■ 9
Hernia Repair. Hugh Auchincloss, M.D 27
The Diagnosis of Uncomplicated Syphilitic Aortitis With a Note on Roent-
genkymography of the Aorta. Samuel H. Averbuck, M.D 41
Continuous Immobilization of Both Lungs by Air Pressure and its Application
to the Treatment by Pulmonary Tuberculosis. Alvan L. Barach, M.D. 53 Multiple Purulent Arthritis Due to Meningococcus in Very Early Infancy.
Murray H. Bass, M.D., and Gerhard Nothman, M.D 60
Hyperinsulinism. Solon S. Bernstein, M.D 66
Arcus Senilis and Arterio.'^clerosis. Ernst P. Boas, M.D 79
Familial Hypercholesterolemia (Xanthomatosis) and Atherosclerosis. Ernst
P. Boas, M.D., and David Adlersberg, M.D 84
Twenty -Five Years of Physiological Chemistry at the Mount Sinai Hospital
(1902-1927). Samuel Bookman, M.D 87
The Modified Insulin Technique in the Treatment of Ambulant Psychiatric
Patients. Richard M. Brickner, M.D 91
Changes in the Precordial Electrocardiogram Due to the Position of the Ex- ploring Electrode. Clough Turrill Burnett, M.D., F. A. C. P 97
The Reliability of Serologic Tests for Syphilis. Louis Chargin, M.D., and
Charles R. Rein, M.\jor, M.C., A.U.S Ill
Spinal Extradural Arachnoid Cyst Associated with Extradural Malignancy.
Ira Cohen, M.D 116
The Surgery of Gastric, Duodenal and Jejunal Ulcer. Ralph Colp, M.D 119
Gastrp-Enterology at the Mount Sinai Hospital. Burrill B. Crohn, M.D 129
Unilateral Adrenal Apoplexy. Report of a Case. Leo Edelman, M.D_ 137
The Anatomy and Surgery of the Edwin Smith Surgical Papyrus. Charles A.
Elsberg, M.D 141
Metabolic Aspects of the Shock Problem. Frank L. Engel, M.D 152
Mechanisms of Fainting. George L. Engel, M.D 170
On the Relation of the Thyroid to Allergic States. Albert A. Epstein, M.D. 191 An Evaluation of the Kenny Treatment of Poliomyelitis. Alfred E. Fischer,
M.D 200
Compression Fracture of the Spine in the Carotid Sinus Syndrome. Arthur
M. Fishberg, M.D., and Robert K. Lippmann,M.D 206
Ulcerative Colitis: A Cooperative Hospital Problem. John H. Garlock, M.D. 210 Significance of Gastric-Juice Depletion on the Effect of Novasurol Diuresis.
S, Glaubach, M.D., Ph.D., and Ernest P. Pick M.D 214
Craniopharyngeoma and Suprasellar Adamantinoma. Joseph H. Globus, M.D.,
AND Kenneth M. Gang, M.D 220
Asthma Syndrome Associated with Anoxemia, Hepatic and Renal Involvement.
Joseph Harkavy, M.D 277
iii
9-0 3 G)
iv
CONTENTS OF VOLUME XII
Complement Titrations in Human Sera. II. Michael Heidelberger, M. D.,
Otto G. Bier, Manfred M. Mayer, and Graciela Leyton 285
Pneumothorax Therapy in Chronic Bronchiectasis. Herman Hennell, M.D. 286
A Case of Subacute Bacterial Endocarditis Caused by an Unusual Micro- organism Related to the "Pleuropnei'monia-like" or Grahamella Group. C. Herschberger, D. Dantes, and Gregory Shwartzman, M.D 295
Venoi;s Pressure Curves in Normal and .\bnormal Circulatory States. I. Nor- mal Venous Pressure Curves and the Negatix b "Hepato-Jugular Reflux Phenomenon." William M. Hitzig, M.D 309
Essential Hypertension Associated with Unilater.vl Dl^ease of the Kidneys. A Report of two Cases with Apparent Relief Following Nephrectomy. A. Hyman, M.D., and H. E. Leiter, M.D 335
Generalized Secondary Amyloidosis. A Clinico-Pathologic Study of 84 Cases.
Mendel Jacobi, M.D., and Harold Grayzel, M.D 339
Fibrous Dysplasia of Bone; A Disease Entity and Specifically not an Expres- sion OF Neurofibromatosis. Henry L. Jaffe, M.D 364
The Pathogenesis of Atherosclerosis. Louis N. Katz, M.D., and D. V. Dauber,
M.D 382
The Effect of High Oxygen Tension- on the Respiratory System. Julius Kau-
NiTZ, M.D 411
Recent Advances in Cellul.\r Pathology. Paul Klemperer, M.D 416
The Clinical Significance of P.vin in .Vcute Coronary Occlusion with Myo- cardial Infarction. M. A. Kucel, M.D 422
Acute Toxic Nephrosis in Carbon Tetrachloride Poisoning. Victor Kugel,
Captain, M.C 440
The Contemporary Treatment of Chronic Si.mple (JIlai coma. Robert K. Lam- bert, M.D., and Sylvan Bloomfiei.d, ^LD 448
A Distinctive but Frequently Unrecognized \'ariety of' Ringworm of the Skin.
Oscar L. Levin, M.D., and Howard T. Behrman, M.D 457
Recent Adv.vnces in Cancer Research. Richard Lew isohn, M.D 464
Tibial Re-Implantation for Osteomybliti.s. Robert K. Lippmann, M.D... 498
Pulmonary Emphysema. Edgar Mayer, M l) , and Israel Rappaport, M.D. ... 505
Cerebral Anuerysm as a Cause op Hemipleoia and Massin e Intracerebral Hem- orrhage. Sylvan E. Moolten, Ma.iok, .M.( '., A. U S 516
Plasma Vitamin A and Carotene in I)iabei i';s .Mi;i.i in s. Herman O. Mosenthal,
M.D., and Winifred C. Loughlin, M l) 523
Massive Pulmonary Embolism. Harold Neuhof, M.D., and Samuel Klein,
Ma,ior, M.C, A.U.S 527
Toxemias of Pregnancy. Joseph Nox ak, M.D 534
.Vrrest by Penicillin of two Cases of SrB.\( tte B.\cteriai. I^ni-o. vhiuhs; di e Respectively to an .Vnaerobic STAPiivLococcrs, and i'o S i HEi roi oi ( i s \ihi- dans. B. S. Oppenheimer, M.D., and .\ Leonard Li hby, M.D 541
Immunity to Tetanus Induced by a Tiiiud I )osk of Toxoid Three Months to Four Years after Basic Immunization on a Sudv of 159 .Vllergic Children. M. Murray Peshkin, M.D 555
Dermoid Cyst of Ovary Containing a Stritcture Similar to a Hi:man Body
(HoMUNCULUs). Alfred Plaut, M.D 567
The Heart in Experimental Shock. Myron Prinzmetal, M.D., and H. C. Berg- man, Ph.D 579
A Description of an E^pidemic caused by Salmonella Typhimurium and the Bac- teriology OF THIS Organism. David II. Ross, M.D 584
Hyperparathyroidism. Report of a Case. Herman S. Roth, M.D 598
Early Cerntcal Carcinom.v in Three Clinically Unsuspected Cases Incidental
TO Pla.stic Operations. Isidor C. Rubin, M.D 607
CONTENTS OF VOLUME XII V
Plasma Cell Invasion ok Pkripheral Blood in Multiple Myeloma. Michael A.
Rubinstein, M.I) 616
The Dissemination and Control of Meningococcal Infections. Emanuel B.
ScHOENBACH, Ma.ior, M.C., A. U.S., and John J. Phair 624
Staphylococcic Pulmonary Abscess Due to Trauma of the Skin. Jacob Schwartz,
M.D 637
Notes on Poliomyelitis and Its Phevai-ence in New York City during the Last
Forty Years. Herman Schwarz, M.D 647
The Talcum Powder Problkm. N. G. Seelig, M.D., and D. J. Verda,M.D 655
An Evaluation of the Clinical Applications of the Yaginal Smear Method.
Ephraim Shorr, M.D 667
Moenckeberg's Sclerosis: A Clinical Entity. Samuel Silbert, M.D., and Heinz
I. LippMANN, M.D 689
The Bi.ooi) IdinN i; in thk Period After Thyroidectomy. Solomon Silver, Major,
M.C., .\ r.S , AND Mary Catherine Tyson, M.D 701
Possible Postwar Malaria Outbreaks in the United St.\tes. Facts and Fal- lacies. I. Snapper, M.D 708
The Roentgen Diagnosis of Dilatation of the Pulmonary Artery. Marcy L.
SussMAN, M.D., and Arthur Grishman, M.D 716
Preclinical and Symptomless Pulmonary Tuberculosis. Max Taschman, M.D. 724 The Preoperative and Postoperative Care of the Diabetic Patient Requiring
Surgery. Edward Tolstoi, M.D 742
Psychosomatic Disorders of the Gastro-Intestinal Tract. Edward Weiss, M.D. 750
Man Himself the Chief Problem in Cardiology. Paul Dudley White, M.D 763
Theca Cell Tumor of the Ovary in a 72 Year Old Woman. Seymour Wimpf-
heimer, M.D 768
Peptic Ulcer in Adolescence: Its Relation to Pituitary Dysfunction. Asher
Winkelstein, M.D 773
Thrombo-Angiitis Obliterans and Diabetes Mellitus in the Same Patient.
Herman A. Zazeela, M.D., and Leonard A. Weinroth, M.D 776
Life's Later Years. Part 7. The Medieval Period. Frederic D. Zeman, M.D. 783 Abstracts 793
Number 2, July-August, 1945
The Edward (Jamaliel Janeway Lectures. Aspects of the Transmissions of the Nervous Impulse. I. Mediation in the Peripheral and Central Nervoi's System. Otto Loewi, M.D., ScD 803
Cystadenoma of the Pancreas. A Case Successfully Treated by Surgical Ex- tirpation. Jerome S. Beloff, M.D 817
PULMONOPLEURAL FIBROSIS SECONDARY TO PuLMONARY INFARCTION. OPERATIVE
Relief. Maurice S. Harte, M.D 821
Penicillin Therapy in a Case of Chronic Suppurative Bronchiectasis. Louis E.
Siltzbach, M.D 825
Essays on the Biology of Disease. Chapter 9. The Biology of Graves' Dis- ease. Eli Moschcowitz, M.D 828
Life's Later Years. Studies in the Medical History of Old Age. Part 8. The Revival of Learning. Frederic D. Zeman, M.D 833
Abstracts 847
Number 3, September-October, 1945
The Edward Gamaliel Janeway Lectures. Aspects of the Transmission of the Nervous Impulse. II. Theoretical and Clinical Implications. Otto Loewi, M.D., ScD 851
vi
CONTENTS OF VOLUME XII
Studies on Extractable Factors in the Spleen and Other Organic Sources which Influence the Blood Platelet Count. Sylvan E. Moolten, Major, M.C., A.U.S 866
Correlations Between the Electroencephalogram and the Histological Struc- ture of Gliogenous and Metastatic Brain Tumors. L. Greenstein, M.D., AND Hans Strauss, M.D 874
Reminlscences 1900-1937. Robert T. Frank, M.D 878
Life's Later Years. Studies in the Medical History of Old Age. Part 9. The Seventeenth Century. Frederic D. Zeman, M.D 890
Abstracts 902
Number 4, November-December, 1945
The Role of Castration as Auxiliary Treatment in Carcinoma of the Breast. Report of a Case. Bernard S. Wolf, M.D 907
The Role of Infection in Thyroid Crisis. Emanuel S. Cohen, M.D 917
A Hitherto Undescribed Anomaly of the Extra-Hepatic Bile Ducts and Blood
Vessels and its Surgical Implications. Stanley J. Sarnoff, M.D 921
A Case of Chronic Abscess of the Costal Cartilage Due to the Bacillus Pyo- cyaneus. Daniel Burdick, M.D 925
Essays on the Biology of Disease. Chapter 10. Recent Advances in Psychoso- matic Medicine. Eli Moschcowitz, M.D 928
Life's Later Years. Studies in the Medical History of Old Age. Part 10. The Eighteenth Century. Frederic D. Zeman, M.D 939
Obituary. Hiram N. Vineberg, M.D 954
Ab.stracts 957
Number 5, January-February, 1946
The Edward Gamaliel Janeway Lectures. I. Vitamin K, its Discovery, Bio- chemistry AND Application in Medicine. Henrik Dam, Sc.D 961
The VlsceralManifestations OF Kaposi's Sarcoma. Daniel Stats, M.D 971
Effect of Weight Reduction on Course of Arterial Hypertension. David Ad-
lersberg, M.D., H. Robert Coler, M.D., and Joseph Laval, M.D 984
Cancer of the Lung in the Presence of Pulmonary Tuberculosis. Report of
Two Cases. H. Hennell, M.D 993
Anterior Mediastinal Neoplasm With Unusual X-ray Appearance. C. B. Rabin, M.D 996
Cylindroma of Bronchus. Pneumonectomy. Peripheral Carico.ma of Lung.
Lobectomy and Chest Wall Resection. H. Neuhof, M.D 1000
Essays ON THE Biology OF Disease. Chapter 11. .\rteriosclerosis. Eli Mosch- cowitz, M.D 1003
Obituaries. Herman Schwarz 1011
Isidor Friesner 1014
Abstracts 1017
Number 6, March-April, 1946
The Edward Gamaliel Janeway Lectures. II. Role of Fats and Certain Other
Dietary Components in Vitamin E Deficiency. Henrik Dam, Sc. D 1021
Observations on Two Cases of Human Filariasis (Wuchereria Bancroft: and
Mansonelia Ozzardi). I. Snapper, M.D., and R. Merliss. M.D 1032
Xon-Specific Localized Granulomatous I'Lcer of the Jejunit.m. Gordon D.
Oppenheimer, M.D 1039
Chronic P^mpyema Associated with Osteomyelitis of Spine and Ribs. A. H.
AuFSES, M.D 1042
CONTENTS OF VOLUME XII vii
The Miliary Form of Pulmonary Sarcoidosis. Solon S. Bernstein, M.D 1045
Staphylococcus Aureus Empyema in an Infant, Aged Six Weeks. Streptococcus Viridans Empyema in an Infant, Aged Twenty-One Months. E. E. Arnheim
M.D 1050
Essays on the Biology of Disease. Chapter 12. Periarteritis Nodosa. Eli
MoscHcowiTZ, M.D 1054
Abstracts 1064
Index of Volume Twelve 1065
JOURNAL
OF
THE MOUNT SINAI HOSPITAL
NEW YORK
VOLUME XII o NUMBER 1 MAY-JUNE, 1945
CONTENTS
PAGE
Foreword. George Baehr, M.D xi
Eli Moschcowitz, ox the Doorstep of the Hospital. Howard
Lilienthal, M.D 1
Dr. Eli Moschcowitz — His Contributioxs to the Sciexce and
Art of Medicixe axd Related Topics 2
Stigmata of Ixfertility. Robert T. Frank, M.D 5
Congenital Atresia of the Esophagus with Tracheoesophageal
Fistula. Ernest E. Arnheim, M.D 9
Hernia Repair. Hugh Aucliincloss, M .D 27
The Diagnosis of Uncomplicated Syphilitic Aortitis with a Note
ON ROENTGENKYMOGRAPHY OF THE AORTA. Samuel H. Avcr-
buck, M.D 41
Continuous Immobilization of Both Lungs by Air Pressure and Its Application to the Treatment by Pulmonary Tubercu- losis. Alvan L. Barach, M.D 53
Multiple Purulent Arthritis Due to Meningococcus in Very Early Infancy. Murray H. Bass, M.D., and Gerhard Noth-
man, M.D 60
Hyperinsulinism. Solon S. Bernstein, M.D 66
Arcus Senilis and Arteriosclerosis. Ernst P. Boas, M.D 79
Familial Hypercholesterolemia (Xanthomatosis) and Athero- sclerosis. Ernst P. Boas, M.D., and David Adlersberg, M.D. ... 84 Taventy-Five Years of Physiological Chemistry at the Mount
Sinai Hospital (1902-1907). Samuel Bookman, M.D 87
The Modified Insulin Technique in the Treatment of Ambu- lant Psychiatric Patients. Richard M. Brickner, M.D 91
ii
CONTENTS
PAGE
Changes in the Precordial Electrocardiogram Due to the Position of the Exploring Electrode. Clough Turrill Bur-
nett, M.D., F.A.C.P 97
The Reliability of Serologic Tests for Syphilis. Louis Chargin,
M. D., and Cliarlrs R. Rrin, Major, M.C, A.U.S Ill
Spinal Extradural .Vrachxoid Cyst Associated with Extradural
Malignancy. Ira Cohen, M.D 116
The Surgery of Gastric, Duodenal and Jejunal Ulcer. Ralph
Colp,M.D 119
Gastro-Enterology at the Mount Sinai Hospital. Burrill B.
Crohn, M.D 129
Unilateral Adrenal Apoplexy. Report of a Case. Leo Edel-
nion, M.D 137
The Axato.my and Surgery of the Edwin Smith Surgical Papyrus.
Chnrb.^ A. Elsbcrg, M.D Ul
^Metabolic Aspects of the Shock Problem. Frank L. Engel, M.D. . 152
[Mechanisms of Fainting. George L. Engel, M.D 170
On the Relation of the Thyroid to Allergic St.vtes. Albert A.
Epstein, M.D 191
An Evaluatiox of the Kenny Tre.\tment of Poliomyelitis.
Alfred E. Fischer, M.D 200
Compression FitvcTruE of the Spine in the Carotid Sinus Syn- drome. Arthur M. Fishberg, M.D., and Robert K. Lippmann, M.D 206
Ulcerative Colitis: A Cooperative Hospital Problem. John
//. Gar lark, M.D 210
Significance of Gastrk -Ji ici; Dkpletion on the Effect of Nova- surol Diuresis. .S. Glanlxieh. M.I)., Ph.D., and Ernest P. Pick, M.D 214
Craniophar^ xgeo:\ia axd Si praskllar Adamaxtixoma. Joseph
II. (Holms. M.D.. and Kmnrth M. Gang. .\LD 220
Asthma Syndrome Associated with Axoxemia, Hepatic and Renal
Involvement. Joseph Ilarkavy, ALD 277
Complement Titrations in Human Sera. IL Michael Heidel-
berejer, M.D., Otto G. Bier, Manfred M. Mager, and Graciela Leyton . 285
Pneumothorax Therapy in Chronic Bhox( hiki i asis. Herman
Hennell, M.D 286
A Case of Subacute I^acteuial I']ndocarditis Caused by ax Un- usual Microorgaxis.m Hklated to the "Pleuropxeumonia- like" or (Jrahamella Gkoup. C. Herschberger, D. Dantes, and Gregory Shwartznian , M .1) 295
Venous Pressuke Cuhves ix Normal and Abnormal Ciuculatory States. L Normal N'enous Pressure Curves and the Negative "Hepato-Jugular Reflux Phenomenon." William M . ffit:i<i. M.D. 309
Essential Hypertension Associated with Uxii, a tlkai. Disease of the Kidxeys a Report of two Cases witli Apparent KeUef Following Nephrectomv. .4. Ilynian, M.D., and IL L. Ldter, M.D ■, 335
Generalized Secondary Amyloidosis. A Clinico-P.\thologic Study of 8-1 Cases. Mendel Jacobi, i\LD., and Harold Grayzel, M.D 339
Fibrous Dysplasia of Bone: A Disease Entity and Specifically not an Expression of Neurofibromatosis. Henry L. Jaffe, M.D 364
CONTENTS lU
PAGE
The Pathogenesis of Atherosclerosis. Louis N . Katz, M.D., and
D. V. Dauber, M.I) 382
The Effect of High Oxygen Tension on the Respiratory System.
Julius Kauniiz, M.I) 411
Recent Advancks in ( "iolltlar Pathology. Paul Kh mpcrcr, M.D. . -IIG
The Climc.vl Si(;mfi< am i-: of Pain in Accti; Couon akv Occlusion
with Myocardial Infarction. M. A. Kugd, M.D 422
Acute Toxic Nephrosis in Carbon Tetrachloride Poisoning.
Victor Kugd, Captain, M.C 440
The Contemporary Tri: \t_mkn r or ( iiiioxk Simplk Glaucoma.
Robert K. Lambert, }Ll).. an,! Si/lmn lUnntni,, 1,1 , M.D 448
A Distinctive but FiiEQUENTLY In recognized \'ariety of Ring- worm of the Skin. Oscar L. Levin, M.D., ami Howard T. Behr- man, M.D 457
Recent Advances IN Cancer Research. Rielmrd L( u i.sohn, M.D. . . 464
Tibial Re-Implantation for Osteomyelitis, h'ohcrt K. Lippmann,
M.D 498
Pulmonary Emphysema. Edgar Mayer, M.D., and Israel Rappa-
port, M.D ' 505
Cerebral Aneurysm as a Cause of Hemiplegia and Massive In- tracerebral Hemorrhage. Sylvan E. Moolten, Major, M.C, A.U.S 516
Plasma Vitamin A and CAiuyn;NE in Diaisetes Mellitus. Herman
0. Mo.^< nll,(il, }Ll)., and Winifred ('. Loughlin, M.D 523
Massive Pulmonaiiv I^mhoi.ism. VI. Harold Neuhof, M.D., and
Samud Klein. Major, .l/.r., A.U.S ." 527
ToxEApAS OF Pregnancy . ./ o.srph Novak, .}LD 534
Arrest by Penicillin of two Cases of Si hai r i i; I')Actehial Endo- carditis; DUE Respectively to an ANAi.iioiiic Staphylo- coccT S, AND to STitKi'TOi occus V'iridans. B. S. Oppenhcimcr, M.U., and A. Lumnrd Liiliinj, M.D 541
Immunity to Tetanus Indi i i:d hy a Third Dose of Toxoid Three Months to Four Years after Basic hrMt nization on a Study of 159 Allergic Children. .1/. .1/ iirnuj J'( shkin, M.D 555
Dermoid Cyst of Ovary Containing a Sti;i ( i thk Similar to a
Human Body (HoMUNcuLus). Alfred Pin nl, M.D 567
The Heart in Experimental Shock. M if ran 1' n n~ metal, M.D.,
and H. C. Bergman, Ph.D ' 579
A De.scription of an Epidemic caused by Salmonella typhi
MURIUM and the BACTERIOLOGY OF THIS OrGANTSM. David H.
Ross, M.D 584
Hyperpar.vthyroidism. Report of a Case. Herman S. Roth,
M.D 598
Early Cervical Carcinoma in Three Clinically Unsuspected
Cases Incidental to Plastic Operations. Isidor C. Rubin,
M.D 607
Plasma Cell Invasion of Peripheral Blood in Multiple
Myeloma. Mid,ad A. Ridnnstrin. }[ .1) 616
The Dissemination and ('on i imil ok M kningococoal Infections.
EmanudB. Sehumhaeh, Major, M.C, .[.CS., and. John J. Phair . . 624 Staphylococcic Pulmonary Abscess Due to Trauma of the Skin.
Jacob Schwartz, M.D 637
Notes on Poliomyelitis and Its Prevalence in New York City
during the Last Forty Years. Herman Schwarz, AI.D 647
IV
CONTENTS
PAGE
The Talcum Powder Problem. M. G. Seelig, M.D., and D. J.
Verda,M.D 655
An Evaluation of the Clinical Applications of the Vaginal
Smear Method. Ephraim Shorr, M.D 667
Moenckeberg's Sclerosis: A Clinical Entity. Samuel Silbert,
M.D., and Heinz I. Lippmann, M.D 689
The Blood Iodine in the Period after Thyroidectomy. Solomon
Silver, Major, M.(\, A. U.S., and Mart/ Catherine Tyson, M.D 701
Possible Postwar Malaria Outbreaks in the United St.\tes.
Facts and Fallacies. /. Snapper, M.D 708
The Roentgen Diagnosis of Dilatation of the Pulmonary
Artery. Marcy L. Sussman, M.D., and Arthur Grishman, M.D. . 716 Preclinical and Symptomless Pulmonary Tuberculosis. Max
Taschman, M.D 724
The Preoperative and Postoperative Care of the Diabetic
P.\TiENT Requiring Surgery. Edward Tolstoi, M.D 742
Psychosomatic Disorders of the Gastro-Intestinal Tract.
Edward Weiss, M.D 750
M.\N Himself the Chief Problem in Cardiology. Paul Dudley
White, M.D 763
Theca Cell Tumor of the Ovary in a 72 Ye.\r Old Woman. Sey- mour Wimpfheimer, M.D 768
Peptic Ulcer in Adolescence: Its Relation to Pituitary Dys- function. Asher Winkelstein, M.D 773
Thrombo-Angiitis Obliterans and Diabetes Mellitus in the
Same Patient. Herman A. Zazeela, M.D., and Leonard A.
Weinroth, M.D 776
Life's Later Years. Part 7. The Medieval Period. Fredsric
D. Zeman, M.D 783
Abstracts 793
EDITORIAL BOARD
Joseph H. Globus, Ira Cohen, M.D. (ex-officio) Leo Edelman, M.D. Joseph Harkavy, M.D. Eli Moschcowitz, M.D. Albert Cornell, M.D. Solon S. Bernstein, M.D.
M.D., Editor-in-chief
Harold Neuhof, M.D. William M. Hitzig, M.D. IsiDOR C. Rubin, M.D. Samuel Silbert, M.D. Seymour Wimpfheimer, M.D. Frederic D. Zeman, M.D.
Manuscripts, abstracts of articles, and correspondence relating to the editorial management should be sent to Dr. Joseph H. Globus, Editor of the Journal of The Mount Sinai Hospital, 1 East 100th Street, New York 29, N. Y.
Changes of address must be received at least two weeks prior to the date of issue, and should be addressed to the Journal of the Mount Sinai Hospital, Mt. Royal and Guilford Avenues, Baltimore 2, Maryland, or 1 East 100th Street, New York 29, N. Y.
TO
DR. ELI MOSCHCOWITZ
THIS VOLUME IS DEDICATED ON HIS SIXTY-FIFTH BIRTHDAY BY HIS FRIEXDS, ASSOCIATES .^JNTD PUPILS
*******
Dr. David Aldeisberg
Mr. Leonard Arkin
Dr. Ernest E. Arnheim
Mr. & Mrs. Max Ascoli
Dr. Hugh Auchincloss
Dr. Arthur H. Aufses
Dr. Samuel H. Averbuck
Dr. George Baehr
Dr. Alvan L. Barach
Dr. Murray H. Bass
Mr. Louis Baumgold
Dr. David Beck
Dr. Howard T. Behrman
Dr. A. A. Berg
Dr. H. C. Bergman
Mr. Louis Bernstein
Dr. Solon S. Bernstein
Dr. Otto G. Bier
Dr. William Bierman
Mr. Nathan Bijur
Mr. Herbert Bleyer
Mr. Da,vid Block
Dr. Svlvan Bloomfield
Dr. Ernst P. Boa.s
Mr. Carl Bomeisler
Dr. Samuel Bookman
Miss Lucrezia Bori
Mrs. F. Brentano
Dr. Richard M. Brickncr
Mrs. N. E. Brill
Mr. William Brill
Dr. & Mrs. M. R. Buchman
Dr. Clough Turrill Burnett
Dr. Louis Cliargin
Mr. I. Chanin
Mr. & Mrs. S. Cliotzinoff
Dr. Ira Cohen
Dr. Ralph Colp
Dr. Burrill B. Crohn
Dr. D. Dantes
Dr. D. V. Dauber
Mrs. J. C. Davies
Mrs. Leopold DeMuth
Mr. & Mrs. Leonard Dickson
Mr. George Diehl
Dr. Henry Dolger
Dr. Leo Edelman
Miss Nanette Ehrman
Dr. Bernard H. EUasbterg
Dr. Benjamin Eliasoph
Dr. Charles A. Elsberg
Dr. Frank L. Engel
Dr. George L. Engel
Dr. Albert A. Epstein
Mrs. Blanche Erdman
Mr. & Mrs. Herbert Erdman
Mr. Fred Fechheimer
Mr. Laurel Fellman
Mr. & Mrs. Leon Fergenson
Di . Alfred E. Fischer
Di. Arthur M. Fishberg
Dr. Robert T. Frank
Mrs. Lee K, Fiankel
Mi \- Mrs .\;uon Freydberg
Ml Mi-, l;al|ih Freydberg
Mi, .\ithiii 1-nend
JIi. l orcv .M. l iowenfeld
Mr, .folin C, Gallagher
Dr. Kenneth M. Gang
Dr. .John H. Garlock
Dr. Sol W. Ginsburg
Dr. 8. Glaubaoh
Dr. .Joseph H. Globus
Dr. .Morris A. Goldbcrger
Mr. & Mrs. I. E. Goldwasser
Dr. Harold Grayzel
Dr. Benjamin B. Greenberg Dr. .\rthur Grishman Dr. .Jcseph Harkavv Dr. William Haiiis Mr. Ilaii', Haixrv
Mrs, I.,:- 11,1/. .11
Dr. Mi. l,,i..| 11, i.lrli.erger Mr, cV Ml- It 11, iiu.man
,11 II,'
Mr, .\ II. :ii-li, ini
Mr. cV- -\li- >i-\ nii,iii Hevman
Mrs. Heniamin Hirschfield
Dr. William M. Hitzig
Mr. Walter Hort
Dr. .\braharn Hvman
Mrs. Edward H>me3
Dr. Mendel .Jacobi
Dr. Henry L. .Jaffe
Miss A. Ji, .Johnson
Mrs. Harris Jonas
Dr. Louis N. Katz
Dr. Julius Kaunitz
Dr. Albert Kean
Mr. & Mrs. Ralph Kempner
Dr. Frederick H. King
Major Samuel H. Ivlein, M.C.
Dr. Paul Klemperer
Dr. Emanuel Klempner
Mr. & -Mrs. J. Klingenstein
Dr. Percy Klingenstein
Mrs. Hazel Kolman
Dr. Rudolph Kramer
Mr. & -Mrs. Herman Kudlich
Dr. M. A. Kugel
Captain Victor Kugel, M.C.
Mrs. Harold D. Lachman
Dr. Robert K. Lambert
Mr. & Mrs. J. Lasky
Dr. .Joseph Laval
Dr. Hyman Leiter
Mrs. Florence L. Levi
Dr. Oscar L. Levin
Dr. & Mrs. David Levy
Dr. Richard Lewisohn
Dr. Graciela Leyton
Dr. Emanuel Libman
Dr. Howard Lilicntlial
Dr. Heinz L Lipfirnarm
Dr, Riihi'ii K I.ippmann
Mr. & Mis E,li;ai l.oew
Dr, W iiuli,-i I ■ l.,,iiyhlin
Mr, UaiM ,1, L,.,n^
Mr, \" \ l.i.xvn,-
Dr, .\ l,.,,n:.nl l,i,l,hy
Dr, S\ h an I ) \1:, iiheim
Dr, I i.-,l,.M, L .\I .\Iarek
Dr. Aril. ,11 .M. .Master
Dr. E.lnai .Mavei
Mr. & Mrs. F. S .Mayer
Dr. Manfred M. Ma,\er
Mr. & Mrs. Walter Mendelsohn
Dr. Alfred Meyer
Mr. Charles H. Meyer
Mr. & Mrs. Robert Misch
Major Sylvan E. Moolten, M.C.
Mrs. A. V. Moschcowitz
Dr. Herman O. Mosenlhal
Mr. & Mrs. A. Munves
Mr. Alfred Naumberg
Dr. Harold Neuhof
Miss Amy Nordlinger
Dr. Gerhard Nothman
Dr. Joseph Novak
Mr. & .Mrs. William Oestreicher
Mr. & Mrs. Robert Oppenheim
Dr. B. S. Oppenheimer
Mr. Walter .J. Oppenheimer
Dr. Reuben Ottenberg
Dr. M. .Murray Peshkin
Mr. .John J. Phair
Dr. Ernest P. Pick
Dr. Alfred Plant
Dr, -M,\ion Prinzmetal
Mr. & Mrs. J. Proskauer
Dr. Coleman B. Rabin
Dr. Howard G. Rappaport
Dr. Israel Rappaport
Dr. Leonard Rau
Major Charles R. Rein, M.C.
Mr. Siegfried Rindskopf
Mr. & .Mrs. Manuel Robbins
Mr. Me\er Robbins
Dr, Alfred Romanoff
Dr, Samuel Ro.sen
Miss EveU n Rosenfeld
Mr. Milton A. Rosenfeld
Dr. Nathan Rosenthal
Dr. Hari,\- Rosenwasser
Dr. David H. Ross
Dr. Herman S. Roth
Dr. Irving Roth
Mr. & Mrs. Philip Arthur Roth
Mr. & Mrs. Victor Roudin
Dr, Isidor C, Rubin
Dr, Michael .\, Rubinstein
.Mr, A- Mis. A. Samuels
Mis, Walter SchilTer
Dr. Arthur Schifrin
Majoi ICinanuel B. Sclioenbach, M.C.
Dr. .Martin Schreiber
Mr. Mrs. Nathan Schulraan
Dr, .lacob .^^-li^vartz
Di , Herman .'^chwaiz
Ml- Hclle S, luvarz.schild
Mr .V Mis, C.oigeSeaton
Di , M ( ; .-^..flig
.Mi ,I ,i,i,'- >, -libman
I >! ' 'li L„'; Slnvartzman
1)1 ,salii,l, l Mll.prt
.\Iai,.i ,Sol.,mon Silver, M.C.
.Mrs. Delta Sklarck
Dr. Isidore Snapper
Dr. Harry Sobotka
Dr. Louis J. Soffer
Dr. Arthur R. Sohval
Dr. Max Som
Mr. & Mrs. Edgar Stern
Mis. Fred Stern
Mr, ct Mrs, M. K. Stern
l)i .Maii\ I, Sussman
Di Max Taschman
Dr. Ivlward Tolstoi
Dr. Joseph Turner
Dr, Mary Catherine Tyson
Mr. & Mrs. Nathan UUraan
Mr. L. Valenstein
Dr. D. J. Verda
Dr. Peter Vogel
Mrs. Albert Weiler
Dr. Leonard A. Weinroth
Mr. & Mis. Karl Weisheit
Dr. Harry Weiss
Dr. Edward Weiss
Dr. Paul Dudley White
Mr. & Mrs. I. Wile
Dr. Se\'mour U'impfheimer
Dr. .\sher Winkelstein
Dr. Herman A. Zazeela
Dr. Frederic D. Zeman
THE .\NNIVERSARY VOLUME COMMITTEE WISHES TO EXPRESS ITS APPRECIATION TO THE MOUNT SINAI HOSPITAL FOR MAKING POSSIBLE THE DEDICATION OF THIS SPECIAL ISSUE OF THE JOURNAL TO
DR. ELI MOSCHCOWITZ
AN ESTEEMED COLLEAGUE, WHO HAS GIVEN TWENTY -SEVEN YEARS OF DEVOTED SERVICE TO OUR HOSPITAL
B. B. OPPENHEIMER SAMUEL H. AVERBUCK, M.D. GEORGE BAEHR, M.D. DAVID BECK, M.D. SOLON S. BERNSTEIN, M.D. RALPH COLP, M.D. ARTHUR M. FISHBERG, M.D. ROBERT T. FRANK, M.D. JOHN H. GARLOCK, M.D. WILLIAM M. HITZIG, M.D.
JOSEPH H. GLOBU
, M.D., Chairman
ABRAHAM HYMAN, M.D.
PAUL KLEMPERER, M.D.
PERCY KLINGENSTEIN, M.D.
ROBERT K. LAMBERT, M.D.
RICHARD LEWISOHN, M.D.
HAROLD NEUHOF, M.D.
MARTIN SCHREIBER, M.D.
ISIDORE SNAPPER, M.D.
JOSEPH TURNER, M.D. S, M.D., Editor
FOREWORD
This volume is dedicated to Dr. Eli Moschcowitz, physician, scientist, scholar, traveller, epicure, bibliophile, magician, and lover of music and the arts. The contributions published in this anniversary volume in honor of his sixty-fifth birthday have been submitted by the authors as evidences of their esteem and affection. No member of the staff of The Mount Sinai Hospital in this genera- tion has commanded the respect and the devotion of his associates in quite the same degree.
Dr. Moschcowitz's association with The Mount Sinai Hospital began as intern, following graduation from the College of Physicians and Surgeons of Columbia rniv(Msity in 1900. Upon completion of his internship in 1903, he spent the follow ing year in Berlin in the pathological laboratory of his friend and teacher, Professor Ludwig Pick. This training led to his appointment as pathologist at the Beth Israel Hospital, New York, where he acquired the fundamental back- ground which determined his subsequent career in clinical medicine. During this period came his early publications in the field of pathology.
In 1920, he joined the staff of the medical service of The Mount Sinai Hospital, and from that time date a series of original contributions to clinical medicine which reflect an increasing awareness of the fact that manj' disea.se processes are a reflection of disturbances of the psycho-biologic unit as a whole. These publi- cations atti-acted wide attention and were frequently quoted, for they are the contributions of a scholarly physician, skilled in the art as well as the science of medicine.
It is difficult for a friend and colleague to assay the relative worth of the great variety of his talents. To manj', his capacity for friendship and his worldly philosophy seem outstanding. To others, the freshness of his outlook upon life and his enjoyment of music and of books are most attractive. Undoubtedly, the combination of these qualities is responsible for the fact that the fertility of his mind and the youthfulness of his spirit continue undiminished, and give increasing promise for the future.
George Baehr
JOURNAL OF THE MOUNT SINAI H OSPI T A L * V OL. XII » NO. 1
ELI MOSCHCOWITZ On the Doorstep of the Hospital HOWARD LILIENTHAL, M.D.
Competitive tests for positions on the House Staff of The Mount Sinai Hos- pital were being held and I was one of the investigators. My part was to read the manuscripts and to form my own opinion of the general character as well as of the medical education of the examinee. (That's a correct English word — Century Dictionary.)
As I recollect it, there were about forty candidates and it was no small job to go over the papers.
Chirography has always interested me and even now a manuscript conveys an impression of character, perhaps psychic in type, which could not be given by a type-written page.
Questions answered by Yes or No do not reveal more than the simple knowl- edge of a fact and, therefor, fuller methods of expression are more informative.
One of the papers, the' handwriting atypical, I was inclined to reject — but for my conscience. However, I had not proceeded far when I was struck by the writer's method of revealing not only information but a technical accuracy and mode of expression which would not have been considered an attribute of a mere -Student. As I recollect, none of the manuscripts was signed, only the number of the candidate being subscribed. The farther I went in this paper the more was I impressed by its maturity and I even admit that I received specific sci- entific knowledge. I could not do otherwise than place the writer's number at the top of the list.
At my first meeting with Eli Moschcowitz his youthful appearance might well have matched his handwriting; his manner was humorous and inviting. It was then that I learned he was the brother of my friend, Alexis, a surgeon of well recognized importance.
Dr. Eli did not follow his brother's example but became a pathologist and, finally, a general practitioner of medicine and was appointed to the Attending Staff of the hospital which he served for many years, later becoming an active consultant. His knowledge and skill in the diagnosis of unusual cases and in advising therapeutic measures have placed him high in the ranks of his pro- fession.
1
DR. ELI ^lOSCHCOWITZ— HIS COXTRIBUTIOXS TO THE SCIENCE AND ART OF MEDICINE AND RELATED TOPICS
Xon-parasitic Cysts of the Liver with a Study of Aberrant Bi,le Ducts.
Am. J. .M." Sc., April, 1906. Zur Appendicitis Frage. (Co-author A. V. Moschcowitz.)
Arch. f. klin. Chir.. vol. S2, 1907. 500 "Surgical Suggestions." (Co-author Dr. Walter Brickner.)
Surger\- Publishing Co.. Xew York, 1907. Eosinophilia and .\naphylaxis.
X. Y. Med. J., January, 1911. Thrombosis of Aorta and Both Iliac Arteries following Streptococcus sepsis.
Proc. X. Y. Path. Soc, vol. 14, January, 1914. \'egetative Endocarditis of the Pulmonary \'alve.
Proc. X. Y. Path. Soc, January, 1914. Carcinomatous Invasion of the Xerves.
Virchows Arch. f. path. Anat. 118: 351, 1914. Histopathology of Calcification of Spinatus Tendons as Associated with Subacromial Bursitis.
Am. J. .M. S., 150: 115, 1915. William Withering.
Med. Pickwick, 1: 72, 1915. The Relation of Angiogenesis to Ossification.
Johns Hopkins Med. Bull., vol, 28, March, 1916. The Pathological Diagnosis of Diseases of the Appendix Based on the Study of 1500 Speci- mens.
Ann. Surg. p. 697, June 1916. Ectopic and Latent Chorionepithelioma.
Proc. X. Y. Path. Soc, 17: 164, 1917. A Critifiuf of Banti's Disease.
J. .\. .M. .v., 60: 1045, 1917. Focal Xecrosis of the Adrenal Gland.
Am. J. M. Sc., 156: 313, 1918. Congenital Xeuroblastoma of the X'ose.
Proc X. Y. Path. Soc, IS: 57, 1918. John Hall: Shakespeare's Son in La\\-.
Johns Hopkins Mod. Hull . June, 1918. Hyperteiusioii : Its Siiinificancc. Relation to Arteriosclerosis and X'ephritis and Etiology.
Am. .1. .\1, Sc.. l.iS: liGS. Kilii. Clinical ami .Vnatomical l>elations in Chronic X'ephritis.
Arch. Int. -Med.. 26: 259, 1920. The Treatment of Ilyiicrtension.
Am. J. M. Sc. 159: 317, 1920, Hypertension with Minimal Renal Lesions.
J. A. M. A., 77: 1075, 1921. Xanthoma of the Tongue,
Proc. X. Y. Path. Soc, 22: 135, 1922, The Pathology of Hypertension,
J, A, M, A,, 79: 1922. X'on-Specific Granulomata of the Intestine.
Am. J. M. Sc., 166: 48, 1923. A Case of Gaucher's Disease.
Proc. X. Y. Path. Soc, 24: 18, 1924.
2
CONTRIBUTIONS TO SCIENCE AND ART OF MEDICINE
3
Congenitai Peripheral Resistance: Its Causative Relation to the Precocious Hypertensive States.
Arch. Int. Med.. 33: 5m. 1024. The First Editions of Sir TIiuiikir Browne.
Ann. Med. Hist., 0: 363, 1024. An Unpublished Letter of Sir Thomas Browne.
Ann. i\Ied. Hist., 6: 287, 1924. An Acute Pleiochromic Anemia with Hyaline Thrombosis of the Capillaries.
Arch. Int. Med., 36: 89, 1925. Problems on Renal Pathology.
Am. J. M. Sc., 172: 469, 1926. Pains of Parietal Origin Stimulating Viceral Disease.
J. A. M. A., 88: 897, 1927. Hypertension of the Pulmonary Circulation.
Am. J. M. Sc., 174: 1927. Pseudo-, or Transient Arteriosclerosis.
J. A. M. A., 20: 1526, 1928. Tobacco Angina Pectoris.
J. A. M. A., 90: 733, 1928. The Cause of Hypertension of the Greater Circulation. •
J. A. M. A., 93: 347, 1929. The Cause of Arteriosclerosis.
Am. J. M. Sc., 178: 244, 1929. The Natural History of Glomerulonephritis.
New England J. Med., 202: 320, 1930. The Nature of Grave's Syndrome.
Arch. Int. Med., 46: 610, 1930. The Pathogenesis of Brown Induration of the Lung.
Am. Heart J., 6: 171, 1930. The Relation of Achlorhydria to Pernicious Anemia.
Arch. Int. Med., 48: 171, 1931. Phlebosclerosis of the Hepatic Veins.
Emanuel Libman Anniversary Volumes, 1932. Der Intravasculare Druck als Ursache der Arteriosklerose.
Virchows Arch. f. path. Anat. 283: 282, 1932. A Biologic Concept of Disease.
J. A. M. A., 99: 714, 1932. A New Sign of Pericardial Effusion.
J. A. M. A., 100: 1663, 1933. Hypoproteinemia.
J. A. M. A., 100, 1086, 1933. A Case of Pseudo or Transient Arteriosclerosis.
J. Mt. Sinai Hosp., 1: 11, 1934. A Psychoanalytic Interpretation of the Constitution in Graves' Syndrome. (Co-author
Sandor Lorand) J. Nerv. & Ment. Dis., 79: 136, 1934. Psychogenic Origin of Organic Disease.
New England J. Med., 212: 603, 1935. Allergy to Life: An Interpretation of the Neurotic Constitution.
New England J. Med., 213: 617, 1935. Arteriosclerosis.
Cyclopedia of Medicine (Piersol), 211, 1935. Primary Amyloidosis.
J. Mt. Sinai Hosp., 2: 14, 1935. The Clinical .\spects of Amyloidosis.
Ann. Clin. Med., 10: 73, 1936.
4
ELI MOSCHCOWITZ
Hiatus Hernia. ,
J. Mt. Sinai Hosp., 2: 263, 1936. Bursitis of the Sartorius Bursa.
J. A. M. A., 109: 1362, 1937. Superior Sulcus Tumor.
J. Mt. Sinai Hosp., 3; 198, 1937. The Simultaneous Association of Hiatus Hernia and Coronary Disease.
J. Mt. Sinai Hosp., 4: 272, 1937. Anorexia Nervosa.
Festschrift dedicated to Dr. Robert T. Frank, June, 1937. The Latent or .\lbuminuric Phase of Glomerulonephritis.
J. Mt. Sinai Hosp., 4: 993, 193S. The Xosolosical Status of Periarteritis Nodosa.
J. Mt. Sinai Hosp., 5: .337, 193S. The Hemato-fJncephalic Barrier. (Co-author Drs. Kessler and Savitsky).
J. Nerv. & Ment. Dis., 90: 594, 1939. Terminal Ileitis.
J. Mt. Sinai Hosp., 7: 77, 1940. On EtioloKv.
.1. .Ml . Sinai Hosp., 7: 471, 1941. Causes of Painless ( Jast ro-DTiotlenal Hemorrhage.
Am. J. M. Sc., 2i)2: 52, 1941. Effect of Edonia and Integumentary Infiltrations on Basal Metabolism Electrocardiogram and Blood Cholesterol.
Anil. Int. Med., 67: 828, 1941. The Validity- of Nephrosis as a Nosological Concept.
J. Mt. Sinai Hosp., 8: 878, 1942. Potential or Latent Congestive Failure in the Aged.
J. Mt. Sinai Hosp., 9: 663, 1942. Vascular Sclerosis.
Oxford Press, New York, 1942. The Hyperkinetic Diseases.
Am. .J. M. Sc., 206; 576, 1943. The Relation of Neurocirculatory Asthenia to Graves' Disease.
Am. Heart J., 28: 177, 1944. The F^lectrocardiogram in Uncomplicated Disease of the Gall Bladder and the Changes Induced by Operation.
J. Mt. Sinai Hosp., 10: 632, 1944. The Biology of Hypertension of the Pulmonary Circulation.
J. Mt. Sinai Hosp., 10: 475, 1944. The Biology of Hypertension of the Greater Circulation.
J. Mt. Sinai Hosp., 10: 747. 1944. The Biology of .Achlorhydria in Relation to .\nemia.
J. Mt. Sinai Hosp., 10: 796, 1944. The Biological Evolution of Toxic Hepatitis.
J. Mt. Sinai Hosp., 11: 29, 1944. The Biology of Glomerulonephritis.
J. Mt. Sinai Hosp., 11: 83, 1944. The Biology of Polycythemia ^'era.
J. Mt. Sinai Hosp., 11: 232, 1944. The Biology of Follicular Lymphoblastoma.
J. Mt. Sinai Hosp., 11: 298, 1945. The Biology of Obesity.
J. Mt. Sinai Hosp., 11: 357, 1945.
STIGMATA OF INFERTILITY
ROBERT T. FRANK, M.D.
The gynecologist and endocrinologist are consulted by childless couples who seek help. Previous in\'estigation has demonstrated that the hushiuul's semen is normal, his genito-urinary tract is uninfected and that he is \[y\\(\
The wife, likewise, has been shown to have a permeable uterus and tubes, her cervix is uninflamed, her menstruation regular. Study of vaginal smears, endometrial biopsy, excretion of pregnandiol, have given as conclusive evidence as our present methods permit, that her cycles are o\-ulatory. Her basal metab- olism is normal. Semen insufflation has been tried and yet no pregnancy oc- curs.
These are trying and puzzling problems. The well informed specialist searches, and too often searches in vain for some explanation. He knows and recognizes well known constitutional stigmata such as eunuchoid and infantile build, marked obesity, hirsuties, a persistently infantile uterus, etc., which occur with frequency in the sterile group, but he is aware that the significance of these findings is not absolute, that accentuation of familial traits may minimize their importance.
I desire to call attention to several stigmata, which, during the course of many years, I have noted in the sterile group under discussion. Again it should be emphasized that these are not absolute criteria of sterility, rather they are con- stitutional marks of the low fertility group.
The Levi-Lorraine type of infantilism is well known and recognized. There are some women, however, who do not fall into this group, but are contiguous to it. By anthropometric evaluation they prove normal. However, their ap- pearance of over-youthfulne.ss, their mincing voice, their small-pored delicate skin, their fine silky hair, their thin bones and weak musculature, their small hands and feet unmask them to the trained observer. As a group these women fall into the least fertile class.
Minor deviations in the breast development are of freciuent occurrence and of little significance. They include large, small, assymmotrical, accessory breasts, retracted nipples, etc. There is one breast chant^c, howcM i-, to which I ascribe importance, particularly as it may be detected immediately after puberty. In default of a better name, I call it the "cup shaped areola." Usu- ally these areolae are large. Instead of being flat, as is normal, the entire areola bulges upward above the breast and on its summit is the nipple. It gives the impression of a weakening of the fascial sheath which envelops the breast, with consequent symmetrical extrusion of the breast tissue at the site of the ring. In my experience this sign appears with significant frequency in the infertile group (figs. 1 and 2).
In performing pelvic examinations, I have noted a sinistro-position of the uterus in a number of nulliparous patients, including virgins. No history of antecedent inflammation can be elicited. Nor is there any sign of inflammation
5
Fig. 2. Schematic antero-posterior section of breast. 1. Normal. 2. "Cup shaped
areola"
STIGMATA OF INFERTILITY
7
present. By vaginal or rectal examination, both cervix and fundus are situated close to the left pelvic wall. The fundus, usually is in anteflexion, sometimes retroflexed. The left parametrium is short but not infiltrated, the right cor- respondingly elongated and thin. The left adnexae are high, close to the pelvic wall and above the fundus, the right adnexae are in a normal position and there-
FiG. 3. Schematic transverse section of pelvis showing congenital sinistro-position of uterus. Note consequent elongation of right cardinal ligament
Fig. 4. Congenital cervical erosion, Xornial iiulliparous external os. Uniform involve- ment of entire portio vaginalis
fore elongated. These findings are permanent and uninfluenced by the fullness or emptyness of the sigmoid flexure (fig. 3).
The condition has been noted in early adolescence and persists. It appears to be congenital. I have not had the opportunity of \iewing it when the ab- domen is opened. Howe\'er, I feel certain that the uterus is sjinmetrical and the adnexae are equally developed on both sides. If this were not the case,
8
ROBERT T. FRANK
one might be tempted to ascribe the condition to partial aplesia of one Miillerian duct with a minor degree of uterus unicornis. Under the circumstances, I am unable to offer any explanation of the causation. The frequency of sterility in these patients has become apparent to me and I consider it of major prognostic significance.
Finally, I desire to call attention to a condition of the cervix which, too often, is diagnosed incorrectly. It is the congenital erosion, a non-inflammatory le- sion (fig. 4). Instead of a smooth, pale normal portio, the entire area is velvety, deep red and vascular. However, the absence of an infected mucopurulent cervical plug is striking. Characteristic is the uniform involvement of the entire portio. Biopsy shows a surface consisting of shallow, closely approximated folds covered by a single laj^er of high cylindrical (cervical) epithelium with basal nucleus. The condition does not respond to astringent applications or to cauteri- zation. In one patient whom I had the opportunity of observing for many years and in whom X-ray castration was induced for other reasons, this erosion was uninfluenced by induction of the artificial menopau.se.
The congenital erosion, as its name implies, dates from the early fetal stage. Normally, the cylindrical epithelium (Miillerian derivation), which covers the portio, is replaced hy the multilayered squamous epithelium (urogenital sinus derivation) Avhich clothes the vagina when Miillerian duct and sinus coalesce at the third month of fetal life. In these patients the fetal state persists in- definitely. Per se the erosion is of no significance. However, it appears most often in patients who have never been able to conceive.
The stigmata, to which I have called attention, should be taken into account in evaluating the fertility or infertility of a patient, in the same way that the profession generally considers an infantile uterus (long, narrow, with cervix- fundus ratio 1:1 instead of 1:4) of significance. This will counterbalance the purely mechanistic viewpoint (permeability of cervix and tubes) as well as the exaggerated "endocrine" concept of anovulatory cycle or the still vaguer diag- nosis of "ovariopituitary dysfunction" which so often is used to hide our ig- norance. Some patients are so constituted that they conceive readily. Others — fortunately for the race, few in number — conceive with difficulty and then only at rare intervals. Others never become pregnant. At best, the size up of these patients, even after all available data have been evaluated, is not conclusive and therefore the prognosis given never should be dogmatic or hopeless.
CONGENITAL ATRESIA OF THE ESOPHAGUS WITH TRACHEOESOPHAGEAL FISTULA'
ERNEST E. ARNHEIM, M.D.
Congenital atresia of the esophagus with or without a fistula communicating with the trachea comprises a difficult problem in pediatric surgery. Surgeons of the past had foimd the problem insoluble and it is only within the past five years that a successful solution has been fovmd, but it is still the feeling of many sur- geons and pediatricians alike that this condition is incompatible with life and that it is useless to attempt operation.
Although atresia of the esophagus with or without tracheoesophageal fistula is not a common condition, about 450 cases have been reported in the literature. The outstanding communications have been those of Plass (17), Vogt (23), Rosenthal (19), O'Hare (16), Ashley (1), Leven (12, 13), Shaw (21), Lanman (11), Haight and Towsley (7), Ladd (9), Humphreys (8), and Haight (4). Plass and Rosenthal have considered the embryolcgic asjiects in detail.
There is a variety of classifications of c( ngenilal aiH inalies of the esophagus, but for simplicity and usefulness I employ the one used at the Children's Hos- pital, Boston, described by Ladd and originally suggested by Vogt. These anomalies are divided into four types. In type I, the upper blind pouch of the esophagus ends at about the level of the first or second dorsal vertebra, and the lower end of the esophagus begins again as a blind pouch at the level of the fourth or fifth dorsal vertebra. In type II, the upper pouch of the esophagus ends in a fistulous tract entering the trachea just above its bifurcation, the lower segment being blind. In type III, the upper segment ends blindly, and the lower segment of the esophagus communicates with the trachea just above its bifurcation; this is by far the most common type. The communication with the trachea may be at the carina instead of above the bifurcation. In type IV, both the upper and lower segments communicate with the trachea. These types can be accurately diagnosed before operation, and the knowledge ol 1 Ik sc \ ariations has clinical application in the surgical management of these anomalies.
CLINICAL FEATTIKES
The symptoms of congenital atresia of the esophagus are noted soon after birth. The infant is seen to have an excess of sali\'a in the mouth with a result- ing choking and, usually, some ( yancisis. When a feeding is given there is imme- diate regurgitation with asi)irati()ii of the fluid into the air passages and an in- crease in the choking and cyanosis. Examination of the chest often reveals moist rales in the lungs, usually in the right upper lobe. The findings on ab- dominal examination vary depending up(!n whether there is or is not a fistulous commmiication of the lower pouch of the esophagus with the trachea. In type I and II, previously described, air cannot enter the intestinal tract and the ab-
1 From the Pediatric Services of Dr. Bela Schick and Dr. Murray H. Bass and the Surgical Service of Dr. Harold Neuhof, The Mount Sinai Hospital, New York, N. Y.
9
10
ERNEST E. AENHEIM
domen is scaphoid. In types III and IV, air enters the stomach through the fistulous communication of the lower pouch of the esophagus with the trachea and the abdomen is more or less distended.
Roentgenologic examination is of great aid in diagnosis. In this connection the use of a contrast medium, particularly barium, is not advisable. If any such medium is used, lipoidol is more safe, but any information gained by its use can bemoresafely andeasily obtained without itsuse. Aspiration into the air passages on a contrast medium adds to the dangers of pneumonia, already usually present. The technic of roentgenologic examination is as follows: A soft rui)ber catheter, usually al)Out size 12F., is passed down the esophagus as far as it will go. If it meets an obstruction about 10 to 12 cm. from the mouth, the diagnosis of con- genital atresia of the c'Si)plidgU8 is confirmed. A roentgenogram with the cathe- ter in place \\ ill then tlemonstrate the site of obstruction. The roentgen examin- ation should include the abdomen to differentiate the type of malformation. In types I and II, there is no air in the stomach or intestinal tract; in types III and IV, there is air in these \'iscera.
Congenital anomalies of the esophagus are frequently associated with anomalies of othei- organs of the l)()dy , and some of these abnormalities in themselves may be incompatible with life unless corrected surgically. In the recent report by Ladd of the experiences of the Children's Hospital, Boston (9), there were 63 con- genital anomalies in 72 cases of congenital atresia of the esophagus; the most frequent being anomalies of \\\v heart and aorta and urinary tract, Meckel's diverticulum, and impert\)rate anus.
TREATMENr
There are two plans of surgical treatment of congenital atresia of the esophagus, which, for purposes of simplicity, may be termed the indirect and the direct pro- cedures. The former utilizes the principle of staged operations, and has been used by Richter (1913) (18), Seolt (1928) (20), Mixtcr (1929) (15), Leven (1936 and 1941) (12, 13), Cage and Ochsuer (1936) (3), Carter (1941) (2), and Ladd (1944) (9). Th(>se indirect proeiMhnes have been variously modified, but, in general, follow the follow ins principl(\s: (1) extrapleural ligation of the tracheo- esophaiieal fistula; (2! exieriorization of the upper esophageal pouch; (3) gas- trostomy; (4) constiuetion of an anterior thoracic esophagus.
The direct plan consists of an extrapleural ligation of the tracheoesophageal fistula and restoration of the continuity of the esophagus by an anastomosis of the proximal and distal ends. This procedure has been performed by Shaw (1939) (21), Lanman (1940) (11), Haight and Towsley (1943) (7), Humphreys (1944) (8), Ladd (1944) (9), and Ilaight (1944) (4).
The main disadvantage of the indirect plan is that it in^•olves a number of sur- gical procedures, the most difficult of which is the construction of an anterior thoracic esophagus by a skin-lined tube. It is, however, the procechu-e of neces- sity when the distance between the esophageal segments is too great to allow a primary ana.stomosis. The direct anastomosis is the operation of choice, but it presents manj'- technical difficulties due to the small size of the distal segment of
CONGENITAL ATRESIA OF ESOPHAGUS
11
the esophagus, the distance between the two ends, and the structure of the eso- phageal wall. That these difficulties, however, can be overcome, is showTi by the experience of Haight (4), who was able to perform an anastomosis in 16, or 66.7 per cent, of 24 patients for Axhom an exploration was performed. A sup- plementary gastrostomy is often necessary because of leakage from the anasto- mosis. The details of the operative procediu'cs are fully described in the publi- cations of Leven (12, 13), Carter (2), Lanman (11), Humphreys (8), Ladd (9), and Haight (4), and in the case rf'])oi ts to follow in this communication.
The problem of pneumonia is a serious one, for it is the chief cause of death, and, in many instances, is present before operation. It is hoped that with the advent of penicillin this serious complication can be successfully tieated. Haight (5) states that "in subsequent cases admitted with extensive pneumonia, the plan would be to give penicillin and hope that the pneumonia could be overcome in a period of several days, and then proceed with operation."
It is only within recent years that these operative procedures have been suc- cessfully performed. The largest number of operative recoveries have been re- ported by Ladd (9) and Haight (4) . In a series of 34 cases of atresia of the esoph- agus operated upon by Ladd and Gross from 1940 to 1944, 6 patients had direct anastomoses with 2 recoveries, and 28 patients had the staged procedures with 9 recoveries. The oldest of these patients is -i^ years of age, and 8 pa- tients are under 1 year of age. In 2 of the 9 recoveries with staged procedures the anterior thoracic esophagus had been completed. In a subsequent report Ladd (10) stated that 13 patients were operated upon in 1944, of whom 8 are living. In 5 of the.se patients a primary anastomosis was performed, and 2 are living. In the other 8 patients the fistula was tied off, and esophagostomy and gastrostomy performed, and 6 are living. Haight and Towsley (7) reportedthe first successful case of anastomosis for congenital atresia of the esophagus with tracheoesophageal fistula in 1943, the operation having been performed in 1941. Haight (4) has had the greatest experience with primary anastomosis, and was able to perform anastomoses in 16, or 66.7 per cent, of 24 patients explored. Six, or 37.5 per cent, of the 16 patients for whom an anastomosis was done are living from 7 months to 3rj years after operation. The reconstructed esophagus was patent in all instances. In a recent communication Haight (6) stated that he had performed 5 additional anastomoses with 1 survival. Leven (13) re- reported 1 patient who was living 1 year after the first operation by the staged indirect method, but the anterior thoracic esophagus had not been constructed. In a subsequent communication Leven (14) stated that the child was 4^ years of age, and that he has had 4 additional survivals by the staged indirect method awaiting construction of the anterior thoracic esophagus. Humphreys (8) re- ported 3 survivals in 6 patients operated upon by the indirect procedures, but in these the anterior thoracic esophagus had still to be constructed. The same author reported 1 successful direct anastomosis of the esophagus 122 days after birth, in a group of 6 patients operated upon by this method. In a recent com- munication Shaw (22) stated that a patient, operated upon by direct anastomo- sis, was alive and well 11 months after operation.
12
ERNEST E. ARXHEIM
Prior to these successful results, other surgeons had been successful in perform- ing direct anastomosis of the esophagus, but unfortunate circumstances con- tributed to the death of the patients. Lanman (11) was the first to utilize the procedure of direct anastomosis of the esophagus and closure of the ti-acheoeso- phageal fistula, and reported 4 ])atients operated upon by this teclmic in 193G and 1937. In one of these i):iti(>nts who lived 9 days, death was due to overhydration with intravenous fluids. The first reported immediately successful direct anastomosis was dcsciihcd by Shaw (21) in 1939, and death was attributed to a transfusion reaction on the twelfth postoperative day.
In this discu.ssion of operative treatment the inipnrtancc of adequate pre- and postoperative management l)y well-tranied pediatric and nursinii staffs is stressed. Mucus should be aspirated from the jiliaiynx and oxygen tlierapy instituted. The care of pulmonary complications by suUonaniiiles or penicillin is essential. As a preoperative mea.sure, which shoidd be continued during and after opera- tion, the administration of parenteral fluids is of great importance. This is sup- plemented by transfusions of whole blood or plasma as indicated. Ascorbic acid and vitamin K arc routinely administered before operation.
C.\SE 11EPORT.S
Case 1. History (\dm. 497752) : .J. .!.,:> male infant, a^cd 77 hours, was admitted to the Mount Sitiai Hospital on Xovemlier 10, 11)42. Theiutanl was a first child, horn at term with the aid of forcci).s. A left facial i)arcsis was noted at hirth. hut no other al)normalitie.s were found at this time. The hirth weight was .") pounds and 7 oiuiccs. Diu-ing the second day of life an ahsence of the arms was noted, hut there were no ex idiMiccs of intestinal obstruc- tion. A small amount of water had been !i,i\'en liy numth durin<; the second day, followed by vomiting. The perineum was explored at another hos])ital on tli(> third day of life, but the rectum was not fouml. Slight cyanosis was notetl after operatit)n. .\ hypodermoclysis of 150 cc. of gluc-.Kse had Ih-.mi :h hni insi ercd .
Exmtii nnl tun : The ( enipi ra I ui e w as 1)7 '1'"., and the weight 5 pounds and 5 ounces. The infant, although small, w.as normall.\- de\'(>loped. The abnormal lindings were a left facial paresis, cyanosis, icterus, ami an imperforate anus with a fresh operative wound in the perineum. The abdomen was not distended.
Laboraitinj Diila: I'rine: no alinoi-nialil ies. Blood: lieinoglohin, 112 per cent; redcells, 6,270,000; leucocytes, 1L>,1.')0, of w hich t hei-e were 1!) per cent segnuMltcd polymorphonuclear leucocytes, 44 per cent nonseginented ]H)l> niorph(inucle.ir leucocytes, 21 per cent lympho- cytes, 14 jx'r cent monoc\ tes, and I percent m\<4(ic}tes.
.\ ro(Mitgenogiani of the aluloinen iwithout the use of .a contrast medium) (fig. 1), taken on the day of admission, re\'ealei| an ahsence of g.as in the intestinal tract; a catheter was passed into the esoi)hagus which was obstructed at the le\-el of the se\ enth cervical vertebra. The lungs shovvcd i-<ientKen evidence oF a consolidation of the up|)er third of the right lung.
The infant was observed lor ;i period of twelve hours during which time he had frequent episodes of cj'anosis a in I \dmit ing of mucus which were relieved b>' catheter suction through the pharynx. The (•hild was placetl in an oxygen cubicle, and a hypodermoclysis of 40 cc. of normal saline was administered. A continuous int r,i\ enous infusion of 5 per cent dex- trose in physiologic solution of sodiun\ chloride was started, and operation was performed.
Operation: Through a 5 cm. left upper lectus incision (under local anesthesia), the stom- ach was exposed and appeared to be of normal size. A Witzel gastrostomy, using a no. 12 F. open-end catheter, was performed.
Course: The infant withstood the (iperativ(^ i)roc(><lui'e well. Oxygen therapy in a cubi- cle, pharyngeal suction, and intravenous Huitls were continued, lipisotles of cyanosis were
COXGENITAL ATRESIA OF ESOPHAGUS
13
Fig 1 Roentgenogram of the t licst and alKlomen (without the use of contrast medium) on admission showing an absence ol air m tlie gastrointestinal tract, a catheter m the esophagus obstructed at the level of the seventh cervical vertebra, and a consolidation ot the upper third of the right lung.
14
ERNEST E. ARNHEIM
relie\(Mi by suction. A transfusion of 70 cc. of citrated blood was administered during the first p()sto|)prative day. Tho temperature remained normal, but the respiratory rate was between 60 and 75 per minute. Feeding by gastrostomy was started on the second post- operative day.
Operutid/i: A sigmoid colostomy was i)crform(Mi two days after the gastrostomy, on the fifth day lA Hfe. rndcr Incal anesthesia a 3 cm. left rectus incision was made. Bile- stained fluid was fiiuiid in tiic |)(>ritoneal ca\'ity. Tii(> small intestines were completely col- lapsed, m(>asuring ahout I-! nun. in diameter. Tiic sigmoid colon contained air and was bluish in coloi-; i( mcasuicd aljout 1 .5 cm. in diamctci-. The wall of the sigmoid was sutured to the i)aiictai pci-itoncuni, leaving an area of colonic wall e.xposed. Small packings were inserted around the bowel.
Course: The same therapeutic measures outlined in the previous postoperative course were cuntinucd. On the third day after the colostomy, it was noted that the gastrostomy feediii;:;^ "I (•% apoiatcMl milk and water took a proli}iigcd time to run in by gravity. The tempeial uic remained nurmid, hut th(> respiratory rate continued to be rapid. Examina- tion of the chest re\-eale(l coaise i-honclii over t lie riiilil .interiui' lutig field. The colostomy was opened on the tluid ila>- after exleiiorizalion ol ilie siL^moid, and thick,' sticky meco- nium was evaeualeil, 1 1 oent gen e\a mi na t inn ( )f I lie on t he lift h day of hosi)italization revealed .a (■(iiisolidal inn i n \ i )l\-i nji I Ik- U|iper port inn of I he right lung and, lo a lesser ex- tent, the lowci- h.alf <it the right lung. Sodium suliadiazine was administered pareuterally on the sixth hos])ital day. The gastrnslumy feedings continued to require long periods to gravitate through the tube. Addition:d fluiils were administered daily by hypodermo- clyses. The condition of the infant remained pom- and he died on the eighth hospital day.
Xccrop.si/ Fi itili Nijs: The body was thai ol' an II da>- old, well tleveloped and fairly well nourisheil male infant. Thei-e was a moderate .amount of yellowish fluid in the peritoneal cavity. Fi l>ri nous exudate was present around the stomach, on the anterior surfaces of the spleen and livei-, and in the lesser sac. Tla-re wcmc Hbrinous adhesions around the stomach, duodenum, and gall bladder. The area around the colostomy wound was clean.
The proximal portion of the e.soph.agus was moderately dilated and ended blindly about 2 em. above the bifurcation of the tiaehe.a Theic was a thin, fibrous cord, without a lu- men, extending from this point, along the posterior surface of the pericardial sac, to the cardia ut the stomach. The distal end of I he esophagus was represented by a blind sac, 0.5 cm. long. It was located under IIk^ dia phi .i tini , and openetl into the stomach at the cardia.
The stomaidi was markedly dilated, its wall i lei ately t liicken(>d, and it contained a large
amount (jf i-eddish brown fluid. The gasi losl i)m\- tube was in situ. The pyloric ring was markedly dilated. The proximal portion of the duodenum was also (listended, measuring about :; em in diameter, and ended blindly (atresia) at the junction of its first and second parts. The second part of the (luod(>nuin was of normal width and thickness, the common duct o])ening into il at the p.apill.a of X'ater. Tli(> remainder of the small intestine was nor- mal, containing giiaai meconium. The colon presented no abnormalities except for the sigmoid c(jlostoiny. The icelum was moderately tlilated and ended as a blind pouch 3 cm. above the cutaneous surface of I he perineum.
The other abnormal findings were hypertrophy of the right kidney; hypoplasia and cystic degeneration of the left kidney; atresia of the left ureter; bronchopneumonia of both lower lobes.
Comment: This infant presented the most unusual combination of three atresias of the esophagointestinal tract, involving the esophagus, duodenum, and rectum. The only atresia obx itms at Iiii tli \v:is that of the rectum. An atresia of the esophagus was suspected iKa ausr there weie no chnical evidences of intes- tinal obstruction, present after the first day of life in infants with atresia of the rectum. In fact, there was no air visible in the gastrointestinal tract by roentgen examination. The atresia of tho esophagus prevented the air from entering the
CONGENITAL ATRESIA OF ESOPHAGUS
15
stomach and intestines.- The frequent episodes of cyanosis and vomiting of mucus were also indicative of an atresia of the esophagus. The roentgen examin- ation indicated that the anomaly of the esophagus was of types I or II, described above. The necropsy findings revealed the unusual nature of this anomaly — the atresia extending to the diaphragm. It was safe to perform a gastrostomy, since there was no clinical evidence of a tracheoesophageal fistula. Once this was performed, it was necessary to proceed with a colostomy to obviate an intes- tinal obstruction. The atresia of the duodenum was not diagnosed, although it had been noted that the gastrostomy feedings took a prolonged period of time to run in by gravity after the fifth day following gastrostomy. The atresia of the duodenum was also unusual, in that it w as located above the ampulla of Vater. There were also anomalies of the urinary tract, and the usual bronchopneumonia.
rV/.s-r :?. HiMarii (Adm. 502177): I. K., a male infant, aged 2 days was admitted to the Mount Smai Hospital on February 27, 1(M3. The infant wa.s a first child, born pieinat urely after '\ months of pregnancy. The birth weight was 4 pounds and 12 ounces. He had re- gurgitated all feedings since birth, and had passed a small amount of meconium by rectum. A large amount of mucus was noted in the pharynx. A few hours before admission the chest was examined by fluoroscopy and a small amount of barium mixture was given hy mouth, which appeared to meet an obstruction at about the level of the second dorsal vertebra.
Examinaiion: The temperature was 97°F., and the weight 4 pounds and 10 ounces. The infant, although small, was normally developed. The abnormal findings were icterus, coarse rhonchi throughout both lungs and slight dullness to percussion over the right upper lobe of the lung anteriorly.
Laboratory Data: Urine: no abnormalities except for a few white and red blood cells on microscopic examination. Blood: hemoglobin, 110 per cent; hematocrit, 46 per cent; pro- teins, 7.18 per cent.
A roentgenogram of the abdomen (without the use of a contrast medium) (fig. 2), taken on the day of admission, revealed a moderate amount of air in the stomach and intestines; a small amount of opaque material was in the stoinach (barium administered prior to ad- mission). A catheter was passed into the csophafiu.s, meeting an obstruction at the level of the superior thoracic apoilur<>; the rso|)liatj:us up Id this point appeared to be displaced to the left. The lunjis k x calrd l ocni ucn c, iilcucc oi a ( uii^-nlidation of the right upper lobe and a few areas of greater <lciisil_\-, pvobaMN ii'pi csciiii iin li.ii-ium.
The infant was observed for a period of sixteen houis during which time he was placed in an oxygen cubicle, and a hypodermoclysis of ] s() cc. of normal saline, and sodium sulfadia- zine parenterally, were administered.
Operation: Through a .5 < m li li upper oblitiuc muscle-splitting incision (under local anesthesia), the stomai h was exposed and appeared to be of small size. A Witzel gastros- tomy, using a no. 12 F. open-end catheter, was performed.
Course: The infant withstood the operative procedure well. The therapeutic measures outlined in the preoperative course were continued with the addition of 40 cc. of plasma in- travenously six hours after operation. During the first postoperative day feedings through the gastrostomy tube resulted in regurgitation of fluid through the nose and mouth. The temperature rose to 1()4°F., but drojipod to 101. 4°F. on the same day. Associated with the rise in temperature, the respirations were very rapid and rales were heard in both lungs.
Operation: The second operation was performed on the day after the gastrostomy. Under cyclopropane anesthesia, an incision was started at about the level of the first and second ribs to the right of the spine and continued downward and parallel to the spine to about the level of the fifth rib, where it was curved laterally below the scapula and contin- ued to the axilla. The fifth (?) rib was resected subperiosteally for a distance of about 4 cm., starting close to the transverse process. In order to obtain more adequate exposure, a
ERNEST E. ARiVHEIM
section of the same amount of the sixtli (?) rib was leniovccl. The intervening intercostal muscle was. excised from the utulerlyiiis pleura, siaiiiii; closi' to tlic spine. The pleura was dissected laterally so as to expose tlie |)i>slerii)i- meiliast i luuu. The azyogs vein was
Fig. 2. RoentgenoKram of tluMliesi and alxlonieu (without t he use of a contrast medium) on admission showing air in the ;ias( rointest inal I fact, ati<l eatlieter in the esophagus ob- structed at the superior thoraeie a|)erlure, and a consolidation of the right upper lobe in which a small amount of barium is visible.
exposed crossing the operative field horizontally. Exploration of the posterior medias- tinum revealed, what appeared to be, the lower segment of the esophagus, lying behind the azygos vein. This structure was about 5 nun. in diameter. Two no. 5 silk sutures were
CON'GEXITAL ATRESIA OF ESOPHAGUS
17
passed around it and tied. The defect in the thoracic wall was partially closed with two pericostal silk sutures. The musculature of the thoracic wall and the skin were closed with interrupted silk sutures. An intravenous infusion of 40 cc. of plasma was administered.
Course: The infant withstood the operative procedure well. Upon returning to the ward the infant was dj'spneic, and about four hours after operation marked edmea of the eyelids was noted. The respirations became irregular and more infrequent, and the edema more generalized. The infant died fifteen hours after operation, at the age of 6 days.
Necropsy Findings: The pleural cavities contained a moderate amount of yellow fluid. The parietal pleura showed a few hemorrhages near the mediastinum. The posterior me- diastinum was clean, X ligature was found about the aorta just below the arch. The esophagus was funnel-shaped being wide at its upper end and continuing into a cord-like stricture at the level of the thyroid; the esophagus could not be probed below this level. The lower end of the esophagus communicated with the trachea through a fistulous com- munication about 8 mm. above the bifurcation of the trachea.
The other abnormal findings were bilateral pneumonia and focal areas of atelectasis; patent foramen ovale; patent ductus arteriosus; congestion of abdominal viscera; gener- alized edema.
Comment: The histor,y and examination of this premature infant were typical of congenital atresia of the esophagus, and the roentgen examination indicated a type III anomaly. The dextraposition of the descending thoracic aorta led to the error of mistaking this structure for the esophagus ; pulsations were not noted, probably due to the low blood presslu-e and rapid cardiac rate. Anatomically, the aorta lay in the position of the esophagus beneath the azygos veinonthe right side, and the error illustrates the difficulty in identifying anatomical structures in a limited operative field in a premature infant. With the more complete ex- posure of the mediastinum employed in later cases, this error would probably not be made. It is of interest that the infant lived for a period of fifteen hours after operation. Dextraposition of the aorta was present in one of the cases reported by Lanman (11), in which the operation consisted in exploration of the mediasti- num by the transpleural route, but the infant died just as the mediastinum was entered. In this case a patent ductus arteriosus was also present.
Case 3. History (Adm. 519456) : J. S., a male infant, aged 6 days, was admitted to the Mount Sinai Hospital on April 26, 1944. The birth weight was 6 pounds. The infant had vomited all feedings since birth. During the first four days of life he required oxygen therapy. On the fifth day of life, in another hospital, barium was passed into the esoph- agus by catheter, visualizing a blind upper pouch; some barium passed into the lungs and the stomach; air was present in the stomach and intestines (fig. 3).
Examination: The temperature was 98°F., and the weight was 6 pounds. The infant was dehydrated and coughing large amounts of mucus. Cyanosis was marked and the respiratory rate was about 100 per minute. There was considerable difficulty with breath- ing and retraction of the thoracic wall on inspiration. Examination of the chest revealed numerous rales throughout both lungs. Other abnormal findings were an absence of the left thumb, and an Erb's palsy of the left upper extremity.
Laboratory Data: Urine: no abnormalities except for some white blood cells and casts on microscopic examination. Blood: hemoglobin, 116 per cent; leucocytes, 16,300, of which there 51 per cent segmented polymorphonuclear leucocytes and 14 per cent non-segmented, 26 per cent lymphocytes, 5 per cent monocytes, 1 per cent eosinophils, and 3 per cent myelocytes.
A roentgenogram of the chest revealed scattered areas of consolidation throughout both lungs.
18 ERNEST E. ARNHEIM
Fig. 3. Roentgenogram of the chest and abdomen (barium administered) 1 day before admission show ing a dilated upper pouch of esophagus, barium deposits in the lungs, and air in the intestines.
The infant was observed for a period of twenty-four hours during which time lie was kept in an oxygen cubicle, and 250 cc. of a mixture of 5 per cent dextrose in distilled water and physiologic solution of sodium chloride were administered through a continuous intravenous
COXGEKITAL ATRESIA OF ESOPHAGUS
19
drip; 60 cc. of plasma, and sulfadiazine, were also given intravenously. There was no im- provement in the condition of the infant during this period.
Operation: Operation was started under local anesthesia with the infant in the left lateral decubitus position. A curvilinear periscapular incision was made on the right side extend- ing from the base of the neck around the vertebral border of the scapula to the axilla. The scapula and its attached muscles were reflected upward from the thoracic wall. The third and fourth ribs were reseceted subperiosteally from their spinal attachments to the axilla. The intercostal vessels between the ribs were divided and ligated and the intercostal mus- culature removed. This allowed exposure of a segment of parietal pleura, measuring about 6 by 3 cm. in diameter.
The parietal pleura was stripped down by blunt dissection off the vertebral bodies until the posterior mediast ilium was exposed, and the dissection was continued until the esoph- agus and the trachea were exposed. The right vagus nerve was clearly visualized. The communicating branch of the azygos vein was clamped, divided and ligated. The lower segment of the esophagus was clearly visualized and found to be rjuite adherent to the trachea; it was cjuite narrow, measuring aliout 4 mm. in diameter. It entered the trachea through a small cniiinuinication which was clamjied, divided, and ligated with 4-0 silk. The upper segment of the esophagus was exposed and found to end blindly about 1.5 cm. proxi- mal to the distal end. Visualization of the upper segment was difficult because of its height, and assistance was rendered by pressure on the catheter which had been inserted prior to operation. The proximal segment of the esophagus was approximately 1 cm. in diameter. The blind end was opened. The catheter was i)assed through it into the open end of the distal segment of the esophagus and down into the stomach. Tension sutures of 5-0 silk had been passed into the four quadrants of the csoiihajieal wall prior to the opening of the proximal end of the e.soiihagus. An end-to-end anastomosis of the esophagus was carried out by interrupted opposing sutures of 5-0 silk through the open ends of the esoph- agus. Tliese were reenforced b\- a tew mattress sutures of the same material, and then by tying oi the stay sutures whicli liad l>een inserted first; the latter were tied under slight tension. About 0.5 gm. of sulfiinihnnide powder was scaitei-eil in the posterior mediasti- num. The thoracic wall was closei I wuh iiiten-u])ted sutures oi 1-0 silk. The operation was considerably prolonged by the fre(i\ieiit rapid c\\pansion and deflation of the lung.
Course: The infant withstood tlie operative procedure well. Oxygen therapy in a cubi- cle, pharyngeal suction, sulfadiazine, and intra\enous fluids were continued. In addition, an infusion of 60 cc. of citrated blood was administered during the first postoperative day. The postoperative course during the first twenty-four hours was verj' satisfactory. The respiratory rate remained rapid, but there was no cyanosis nor dyspnea. Examination of the lungs revealed ihonchi on both sides. I'eedings of 5 per cent (iextrose in distilled water through tlip catheter passing through the anastomosis were st arteil on the second postopera- tive day. A mixture of sulfadiazine in water was made up and administered through the catheter. On the fifth administration oi ihe sulfadiazine, it was noted that the catheter was obstructed and did not allow passage (jf Huid through it. The lack of available veins for intravenous therapy and the inadequacy of hypodermoclyses for sufficient nutrition made gastrostomy imperative.
Operation: Through a 6 cm. left upper rectus incision (under local anesthesia), the stom- ach was exposed and found to be of normal size. .\ Witzel gastrostomy, using a no. 12 cathe- ter, was performed. The infant's condition during operation was poor; he appeared pale and cyanotic and had irregular rapid respirations.
Course: The infant's condition remained poor, and he died one hour after the gastros- tomy, at the age of 9 days.
Necropsy Findings: The right pleural cavity contained a small amount of fluid. The operative incision was made through the chest wall outside of the pleura; the third and fourth ribs had been partially removed. The wound appeared clean, and the parietal pleura and the mediastinum were focally congested.
20 ERNEST E. ARNHEIM
The esophagus showed several black silk sutures around its entire circumference, about 1 cm. above the bifurcation of the trachea. The upper portion of the esophagus was widely patent, measuring about 0.7 cm. in diameter. There was an end-to-end anastomosis of the esophagus about 1 cm. above the bifurcation of the trachea (fig. 4). On filling the stomach with water, a small trickle was noted through the antastomosis. The entire esophagus could
Fig. 4. Photograph of the thoracic organs at necropsy showing the site of the end-to-end anastomosis of the esophagus (arrow).
be probed. The trachea showed on its posterior wall, 1 cm. above the bifurcation, a small funnel-shaiM'd orifice ciidiiin lilindly, about 2 mm. in length; one silk suture was found about 0.5 cm. l.cliiw ii ( tii; 'd. a narrow tube, stitched to the wall of the stomacli, led into the lumen of the sioniach nrar I lie greater curvature.
The other ahnurinal findings were bronchopneumonia and atelectasis of both lower lobes of the lungs, congenital absence of the left kidney and ureter; congenital absence of the left thumb; patent ductus arteriosus.
COXGEXITAL ATRESIA OF ESOPHAGUS
21
Comment: The infant's condition upon admission was very poor and there were evidences of a bilatei-al ]:)neiimonia. Therapeutic measures for a period of twenty -four hours, did nni pi (k luce any appreciable improvement, and it was questionalile whetlier the inlaiil cMuld survive operation. In spite of this, an
Fig. 5. Photograph of the opened trachea at necropsy shownif^ tin' siic of the closed tracheo-esophageal fistula on the posterior wall.
anastomosis of the esophagus was performed. The unfortunate plugging of the tube with sulfadiazine contributed to the death of the patient, in that feedings through the tube could have been continued for a longer period, and, if a gastros- tomy did become necessarj^, it cotild have been performed at a later date when the condition of the infant had improved. The other congenital anomalies were not incompatible with life.
22
ERXEST E. ARNHEIM
Case 4- History (Adm. 520728) : C. M., a male infant, aged 4 days, was admitted to The Mount Sinai Hospital on May 25, 1944. The infant was a second child, born at term. The birth weight was 5 pounds and 6 ounces. He had regurgitated all feedings since birth. Be- fore admission the infant had been given a barium mixture by mouth which met an obstruc- tion in the upper esophagus.
Examination: The temperature was 98.6°F., and the weight 5 pounds and 6 ounces. The infant was normally developed. The abnormal findings were retraction of the thoracic wall on inspiration, and rales at both bases of the lungs.
Laboratory Data: Urine: no abnormalities except for albumin. Blood: hemoglobin, 127 per cent; red cells, 6,400,000; leucocytes, 10,000, of which there were 13 per cent segmented polymorphonuclear leucocytes and 28 per cent non-segment, 53 per cent lymphocytes, 2 per cent monocytes, and 4 per cent eosinophiles.
A roentgenogram of the chest (fig. 6) revealed scattered infiltrations throughout the right upper lobe, both pulmonary bases, and the parahilar area; a catheter was passed into the esophagus and was seen to be obstructed in its upper portion; air was present in the gastro- intestinal tract.
The infant was observed for a period of twenty-four hours during which time he was kept in an oxygen cubicle, and 150 cc. of physiologic solution of sodium chloride were adminis- tered through a continuous intravenous drip; 50 cc. of plasma, and sulfadiazine were also given.
Operation: The operation was started under local anesthesia with the infant in the left lateral decubitus position. A curvilinear periscapular incision was made on the right side, extending from the base of the neck around the vertebral border of the scapula to the axilla. The scapula and its attached muscles were reflected upward from the thoracic wall. The third and fourth ribs, for an area of about 5 cm. were resected subperiosteally from their spinal attachments to the axilla. The intercostal muscles between the ribs were divided and ligated and the intercostal musculature removed. This allowed exposure of a segment of parietal pleura, measuring about 6 by 3 cm. in diameter.
The parietal pleura was stripped down by blunt dissection from the vertebral bodies until the posterior mediastinum was exposed. Dissection was continued until the esophagus and trachea were visualized. The communicating branch of the azj'gos vein was at a higher level than usual and was not divided. The lower segment of the esophagus was visualized and found to be quite adherent to the trachea; it measured about 4 mm. in diameter and entered the trachea through a small communication above the bifurcation. The fistula was clamped on its tracheal side and sutured with 4-0 silk. The upper segment of the esophagus exposed and found to end blindly about 2 cm. above the distal end. Visualization of. the proximal end was aided by pressure on the catheter which had been inserted into the esoph- agus prior to operation. The proximal end of the esophagus was about 1 cm. in diameter. Tension sutures of 5-0 silk were passed through the walls of the four quadrants of both seg- ments of the esophagus and left untied. The proximal end of the esophagus was then opened. The catheter was passed through it down into the distal end of the esophagus and into the stomach. An end-to-end anastomosis of the esoi)li:inus w is < ai ried out by inter- rupted opposing sutures of 5-0 silk through the open ends of the i si)|ihagus. These were reenforced by a few mattress sutures of the same material and by the stay sutures which had first been inserted. About 0.5 Gm. of sulfanilamide powder was scattered in the posterior mediastinum, and a small rubber dam was inserted. The thoracic wall was closed with interrupted 4-0 silk sutures uniting the musculature and skin.
Prior to operation the infant had respiratory difficulty as evidenced by retraction of the thoracic wall on inspiration. There was more difhculty than usual with the anesthesia, necessitating frequent interruptions in tlic ojx'iative procedure. At the termination of the operation, however, the infant's condition appealed fairly good. Intravenous fluids, sup- plemented by a blood transfusion, were administered.
Course: Oxygen therapy in a cubicle, sulfadiazine, and intravenous fluids were continued. The postoperative course during the first twelve hours after operation was satisfactory, but
COXGEXITAL ATRESIA OF ESOPHAGUS 23
Fig. 6. Roentgenoui mi ihrc lic-t ^iml w iihi.tit the use of contrast medium)
on admission showing air m the Ka.stniuitesliiial trart, a catheter in the esophagus ob- structed at the superior thoracic aperture, and scattered areas of consolidation in both lung fields.
soon thereafter the respirations became labored and marked cyanosis was noted. The tem- perature rose progressively to 105.8°F.. and he died eighteen hours after operation. ^
Necropsy Findings: The incision had been made through the thoracic wall outside the
24
ERXEST E. ARXHEIM
pleura. The second and third ribs had been partially removed. The wound was clean- The parietal pleura in the operative site was congested. There was no free fluid in the pleural cavities.
The esophagus, about 1 cm. above the bifurcation of the trachea, showed sutures around its entire circumference (fig. 7). A probe could be passed through the anastomosis into the
Fig. 7. Photograph of the thoracic organs at necropsy show m^i i In sn , .,i i he end-to-end anastomosis of the esophagus.
stomach. The suture line appeared intact and clean. The trachea, about 1 cm. above the bifurcation, showed on its posterior wall a funiiol-shaped orifice leading into a tube which could not be probed because of closure by a silk suture next to its opening.
The other abnormal findings were bilateral bronchopneumonia of all lobes; patent fora- men ovali; accessory pancreas.
CONGENITAL ATRESIA OF ESOPHAGUS
25
Comment: An anastomosis of the esophagus was performed and death was due to pneumonia. At the present time, operation in such a patient would be de- ferred until a course of penicillin had been administered. The other congenital anomalies were not incompatible with life.
SUMMARY
The clinical features, the surgical problems, and the results of operations for atresia of the esophagus with tracheoesophageal fistula are presented. The operation of choice is an extrapleural ligation of the tracheoesophageal fistula and restoration of the continuity of the esophagus by an anastomosis of the proximal and distal ends. This procedure was carried out in 2 patients; the infants died, two days and eighteen hours respectively, after operation, of bronchopneumonia.
BIBLIOGRAPHY
1. Ashley, J. D., Jr.: Congenital Atresia of the Esophagus with Tracheoesophageal
Fistula. Radiology, .36: 621, 1941.
2. Carter, B. X.: An Operation for the Cure of Congenital Atresia of the Esophagus.
Surg., Gynec. & Obst., 73: 485. 1941.
3. Gage, M., and Ochsner, A. ; The Surgical Treatment of Congenital Tracheoesophageal
Fistula in the Xew-born. Ann. Surg., 103: 725, 1936.
4. Haight, C: Congenital .\tresia of the Esophagus with Tracheoesophageal Fistula;
Reconstruction of Esophageal Continuity by Primary Anastomosis. Ann. Surg. 120: 623, 1944.
5. Idem: Personal communication. September 13, 1944.
6. Idem: Personal communication. October 24, 1944.
7. Haight, C, and Towsley, H. A.: Congenital Atresia of the Esophagus with Tracheo-
esophageal Fistula; Extrapleural Ligation of Fistula and End-to-End Anastomo- sis of Esophageal Segments. Surg., Gynec. & Obst., 76: 672, 1943.
8. HuMPHREYf^, G. H.: The Surgical Treatment of Congenital .\tresia of the Esophagus.
Surgery. 15: 801, 1944.
9. Ladd, W. E.: The Surgical Treatment of Esophageal Atresia and Tracheoesophageal
Fistulas. New England J. Med., 230 : 625, 1944. 10. Idem: Discussion of article by Haight (4).
IL Lanman, T. H.: Congenital Atresia of the Esophagus; A Study of Thirty-two Cases. Arch. Surg. 41 : 1060, 1940.
12. Leven, N. L.: Surgical Management of Congenital Atresia of the Esophagus with
Tracheoesophageal Fistula. J. Thoracic Surg., 6: 30, 1936.
13. Idem: Congenital Atresia of the Esophagus with Tracheoesophageal Fistula; Report of
Successful Extrapleural Ligation of Fistulous Coniniuni<;ation and Cervical Esophagostomy. .J. Thoracic Surg., 10: 648, 1941.
14. Idem: Discussion of article by Haight (4).
15. Mixter, C. G. : Discussion of article by Gage and Ochsner (3).
16. O'H.ARE, H. A.: Imperforate .\nus and Tracheoesophageal Fistula. Pennsylvania M.
J., 40: 914, 1937.
17. Plass, E. D.: Congenital .\trosia of the Eso|)hagus with Tracheoesophageal Fistula;
Associated with Fused Kidney; A Case l{eport and Survey of the Literature on Congenital Anomalies of the Esophagus. Johns Hopkins Hosp. Rep., 18: 259, 1919.
18. RiCHTER, H. M.: Congenital Atresia of the Esophagus; An Operation Designed for its
Cure. Surg., Gynec. & Obst., 17: 397, 1913.
26
ERNEST E. ARNHEIM
19. Rosenthal, A. H.: Congenital Atresia of the Esophagus with Tracheoesophageal
Fistula; Report of Eight Cases. Arch. Path., 12: 756, 1931.
20. Scott, W. J. M.: Quoted hy Heatly, C. A.: Some Problems in Esophageal Atresia.
Ann. Otol., Rhin. & Laryng., 45: 1122, 1936.
21. Shaw, R.: Surgical Correction of Congenital Atresia of the Esophagus with Tracheo-
esophageal Fistula. J. Thoracic Surg., 9: 213, 1939.
22. Idem: Personal communication. June 12, 1942.
23. VoGT, E. C: Congenital Esophageal Atresia. Am. J. Roentgenol., 22: 463, 1929.
HERNIA REPAIR
HUGH AUCHINCLOSS, M.D. FOREWORD
It is a pleasure to contribute a tribute to a Moschowitz. About forty years ago Eli cut tissue sections, stained, mounted, studied and discussed them in "Dr. Prudden's Laboratory," near the top floor of "the Old P and S" on 59th Street. Hodenpyl, Larkin, "Pa" Freeborn, Hiss, Wadsworth, Miller, Wood had work rooms. Tilney, Vogel, Zinsser, Ely, Matthews, Norris, Connell, Downs, Dayton, McWhorter, Sharp, Pool, Frank, Flint, Blake, Brewer, Darrach, Dowd, Van Beuren, Eli Moschowitz and Walton Martin were frequent visitors to "Bill Clarke's" desk where "surgical pathology" at P and S started. "Ed- ward" and "Jimmy" kept order. The pathological tissues from Roosevelt Hospital were brought there by its pathological internes. St. Mary's Hospital for Children in 34th Street and General Memorial Hospital on 103rd Street and Central Park, West, sent their specimens there.
What talk! What arguments! Eli's dissertations were priceless!
His brother, Alexis, did more than his share at the meetings of the New York Surgical Society, in those days. Not once, but many times, he told stories of hernia that needed telling. One of the things he stressed was the Transversalis Fascia. Let what follows be a tribute to the thoughts of a Moschowitz. The Moschowitz mind sought essentials.
Alexis Moschowitz contributed to the knowledge of abdominal wall herniae and their repair. He emphasized the importance of the transversalis fascia.
Though these lines use inguinal herniae as a text, their intent is to emphasize essential factors common to abdominal wall herniae of other sort.
The internal ring of the inguinal canal can be likened to the neck of a funnel into which the abdominal wall ingredients have been poured so that they come to line the canal. The result is that the structures of the abdominal wall have their representative parts lining the wall of this funnel into the inguinal canal and below. In the normal human being the peritoneal Hning of the abdomen crosses the internal ring without going down it. Should there be a bulge or a little prolongation we consider it abnormal and call it a tendency towards hernia or an early, or small, hernia. There is no normal representation of the perito- neal lining in the canal or below imtil the tunica vaginalis is reached in the scrotum.
Next to the peritoneal lining of the abdominal wall, are the "subserous," or "properitoneal," tissues. In the canal these tissues include the spermatic ar- tery, the vas deferens, the artery to the vas deferens, veins, lymphatics and sym- pathetic nerve fibres in a loose, areolar arrangement. Should a hernial sac exist, these properitoneal tissues are the tissues most intimate to it.
Outside the "properitoneal tissues" of the abdominal wall is the transversahs
27
28
HUGH AUCHINCLOSS
fascia. Its counterpart in the inguinal canal is called "the infimdibuliform fascia."
The next most external layer of the wall of the abdomen is the transversus, or transversalis muscle. Its counterpart in the inguinal canal is the fascia of the transversus, or transversalis muscle. This fascia and the infundibuUform fascia are scarcely recognizable. They are of little importance in repairing inguinal herniae.
One must avoid confusing the terms "Transversalis Fascia" and "Fascia of the Transversalis Muscle." Suture of the TransAeisalis Muscle or its fascia must not be confused with suturing of the "Trans\-ersalis Fascia." Suture of the transversalis fascia is the most important step of the Avhole operation for repair. Suture of tlic tians\'ersalis muscle, or its fascia, where it merges with the lower border of the internal oblique, often kills these muscles, weakens the wall, and may contribute toward the production of a new hernia.
The internal oblique muscle is next outside the trans\-ersus muscle. They fuse in their lower, inner portions to form a short tendon, to be inserted into the pubic bone, as the so-called "conjoined tendon." The term "conjoined tendon" is often used incorrectly to describe the lower edges of these two muscles before they become the "conjoined tendon," (see below). The counterpart of the internal oblique muscle in the inguinal canal, and below it, is the cremasteric fascia containing the cremasteric muscle fibres.
Outside of these muscles is the aponeurosis of the external oblique with its external spermatic fascia crossing the external inguinal ring.
Too many inguinal herniae recur after operation. The records of all our large hospitals show this. A vast literature on the subject bears witness to this fact.
A peritoneal sac, or intestine only partly lined by pei'itoneum, as in a "sliding" hernia, begins its "bulge" through the abdominal ^\•all where the wall's "first line of defense" is absent or weak. It is absent at tlie internal ring and at other places such as the femoral canal and obturator foramen. It may be weak in the region where direct herniae occur, or at the umliilicus, or above, in the epigastric region, or at the site of operative scars.
Intra-abdominal pressure, intensified by stresses and strains of many sorts produces a "bulge" wherever the "tirst line of defense" is absent or weak, and the story of "great" hernial "oaks from little" bulging "acorns" begins. Persis- tent pressure from within push slippeiy A'iscera through the "fii'st line of defense" into the "bulge" that, little by little, enlarges into a "hernia."
Peritoneum and propertitoneal tissues offer so little restraint to evisceration that they can practically be discounted. The "first line of defense" is trans- versalis fascia . 'Iliis is the important thing to bear in mind. Where it is absent, or weak, hciniation can occur. Once a "bulge" has begun, a "wedge" has been set and started that splits abdominal walls wide open with the "hammer blows" of intra-abdominal pressure. Coughing, sneezing, hiccough, weight-lifting, pregnancy and straining to defecate, may all be thought of as "hammer blows."
If absence or weakness of the "first line of defense" be the essential factor in hernia creation, it is evident that a new "first line of defense" must Ije made to prevent recurrence.
HEENIA REPAIR
29
Fig. a. Appearance of tlie ext(>iiial obliciue aponeurosis after the skin incision. The sac has not protruded through the e.xternal ring.
A. External ObH(|ue .\poneurosis.
B. External Inguinal Ring.
C. Deep P^ascia of the Thigh.
@. Site of tiif Inteinal Inguinal King. Sometimes the External Oblique Aponeurosis is relaxed and ma>- Iniigc slightly u Ikm'o a hoi-nia that has not protruded through the external ling cxisis. Tlii.s is usually, however, not discernible and the presence of a Sac may not be evident until the .Aponeurosis has been divided and the Cremasteric Muscle split.
Fig. B. Appearance of the FJxternal Oblique Aponeurosis where the Hernial Sac pro- trudes through the external ring and can readily be felt, or seen, on physical e.xamination. A. External Oblique Aponeurosis.
@. Hernial Sac protruding from the External Ring, showing the enlargement of the
External Ring. C. Deep Fascia of the Thigh. ®. Location of the Internal Inguinal Ring.
30
HUGH AUCHINCLOSS
Fig. C. Internal Oblitiue Muscle and Cremasteric Fascia after division of the Externa Oblique Aponeurosis.
A. External Oblique Aponeurosis.
©. Inner portion of Internal Oblicjue Muscle that at this point has begun to fuse with the Transversu.s Muscle to form the: —
©. Conjoined Tendon. The term "Conjoined Tendon" has been used incorrectly for many years in descriptions of inguinal hernia. Repeatedly in these descriptions ad- vice is given to sew the "conjoined tendon" to Poupart's ligament when it is meant to advise sewing the Internal Obli(iue, and the subjacent Transversus Muscle, to Pou- part's ligament. The term "Conjoined Tendon" should be used to describe a short, strong tendon made by the fusion of the Internal Oblique and Transversus Muscles, running to the Puhic Spine aiul Linea Alba in front of the Rectus Muscle. The me- dial portion of the Cremasteric nuiscle fil)res are inserted into its lower, inner margin.
D. Posterior surface of the External Oblique .\poneurosis commonly referred to as the "Shelving Margin of Poupart's Ligament."
®. Cremasteric Muscle fibres over the hernial sac and spermatic cord, spoken of as "Cremasteric Fascia." Sometimes these muscle fibres are conspicuous and evident, — at other times, scarcely seen.
F. Deep Fascia of the Thigh.
HERNIA REPAIR
31
Failure to create this "first line of defense" is the cliief cause for the persistence, or recurrence, of herniae following operation. This is not done because, in the first place, its importance is not appreciated and, in the second place, a reliable technique for recognizing the transversalis fascia, i.e. the "first line of defense," has not been emphasized nor taught.
Figures A, B, C, D, E show, diagramatically, steps in the technique of ex- posure of the indirect hernial sac and the search for latent weakness in the region of direct hernia. Subsequent figures F, G, H, I, J, K, L, M deal with the technique of repair.
Repair can be considered under three headings:
1. Opening and closure of the sac.
2. Creation of a "first line of defense."
3. Reenforcement of the "first line of defense."
1. Opening and closure of the sac. The sac must first be found. After di- viding the external aponeurof^is from the external inguinal ring, upwards and outwards along Poupart's ligament and freeing it from the internal oblique above, the "shelving margin" of Poupart's ligament is exposed by freeing it from the cremasteric fascia below by blunt dissection. The cremasteric fascia is then incised close to the deep edge of the "shelving margin." This incision is length- ened and widened by the introduction of the finger tips at either end and drawing them apart down the cord internally, and up to the origin of the internal oblique muscle externally. The "neck" of the sac is then sought. The "neck" is that portion of the sac in the canal just after its emergence from the internal ring. The "neck" is the least complicated portion of the sac. Once seen, the dissec-
G. Site of the Internal Inguinal Ring.
@. Internal Oblique muscle taking origin from the outer half of Poupart's Ligament.
Fig. D. The Cremasteric Fascia, with its muscle fibres, has been recognized and incised close to Poupart's Ligament. It is usually possible to identify this Fascia. Beneath this Fascia, covering the cord and the hernial sac, are two layers of fascia not so readily identi- fied. 1. The Fascia of the Transversus Muscle. 2. The Infundibuliform Fascia. This is the funnel shaped prolongation of the Transversalis Fascia down the cord. Immediately beneath this Fascia are the Subserous, or Properitoneal tissues in which run the vessels and nerves to the Cord, the Hernial Sac and the Peritoneum.
A. External Oblique Aponeurosis.
(b). Cremasteric Muscle, or Fascia.
©. Bulging of the Sac after the Cremasteric Fascia and the subjacent Fasciae have been divided and split apart by means of a pair of blunt scissors or the finger tips.
D. Internal Oblique Muscle.
E. Conjoined Tendon.
F. Spermatic Cord with the Vas Deferens readily identified by its little "curlicue" Artery from the Superior Vesical branch of the Internal Iliac or "Hypogastric" Ar- tery.
©. Shelving Margin of Poupart's Ligament.
H. Deep Fascia of the Thigh.
(J). Few fibres of the Cremasteric Muscle, or "Fascia" near Poupart's Ligament. In splitting the fibres of the Cremasteric Muscle, it is advisable to do this close to the Ligament. On the other hand, one should be cautioned against dividing these fibres too deeply, especially if the muscle and the subjacent structures have been drawn up by the operator's fingers so as to put them on the stretch. Should one divide too deeply, one might cut through the Transversalis Fascia. This might weaken it so as to create a direct hernia.
.J. Site of the Internal Inguinal Ring.
K. Origin of the Internal Oblique from the Outer Half of Poupart's Ligament.
32
HUGH AUCHINCLOSS
Fig. E. The Cremasteric Fascia has been so thoroughly split and disposed of that it has become almost indistinguishable from the Internal Oblique Muscle. The Sac, stripped of the fascia of the Transversus Muscle, the Infundibuliform Fascia and the Areolar, Sub- serous or Properitoneal Tissues, with their vessels and nerves, is outlined. The Cord and its Vas, well identified by its characteristic appearance, its almost wiry feel, and the so- called "curlicue" Artery of the Vas Deferens running on its surface, must be separated from the Sac. To do this, dissect close to the Sac wall, even inside its small blood vessels. This will save injury to the Cord. Aim at dissecting the sac at its "neck," — that is, just mesial to the internal ring. Once, having completely freed the neck of the Sac, its junction with the free Peritoneal Cavity can easily be determined, and the distal portion freed and either left in, or removed.
A. External Oblique Aponeurosis.
®. Hernial Sac, ready to open so that its further dissection can be done with its wall demonstrated by fingers introduced within.
C. Internal Oblique, where it fuses with the Transversus Muscle to form the,
D. Conjoined Tendon.
(E). Cord, with the Vas Deferens and its "curlicue" Artery.
F. Shelving Margin of Poupart's Ligament.
G. Deep Fascia of the Thigh.
®. Site of Internal Inguinal Ring.
HERNIA REPAIR
33
tion should be carried in the preperitoneal tissues around the "neck" of the sac, and as close to the sac as possible. This is the surest way to avoid injuring the vas and other cord structures. It may help, and often does, to open the sac during this part of the procedure. Whether to remove all of the sac, or leave its distal portion, requires discretion on the part of the operator. Where the sac is long and adherent, where the sac is "congenital," the lower portion may be left in. Once the sac has been dissected free to the level of the abdominal cavity, it must be emptied of its contents. If adherent, they must be freed.
At this point there is opportunity for studjang the immediate interior of the peritoneal cavity for possible pathological conditions or removal of an easily delivered appendix, and the region inside the epigastric vessels for a direct hernia. The sac should then be Hgated by suture or transfixion, its reduntant portion cut away, and the suture passed through the transversalis and internal oblique muscles above and outside the internal ring. Here it can be sutured to the fascia covering the internal oblique muscle. This draws the closed, sac opening away from the cord at the internal ring.
2. Creation of a "First Line of Defense." The transversalis fascia must be found. This is essential to effectively close the internal inguinal ring. "The internal arcuate fibi'es" forming the inner edge of the internal inguinal ring are fibres of the transversalis fascia. They are the outer margin of Hesselbach's ligament. The deep epigastric vessels run upwards and inwards close to the edges of these fibres and beneath them. Were the operator to dissect the tissues to show the deep epigastric vessels, he would be more than likely to cut these "internal arcuate fibres" of the transversalis fascia that are so important to preserve.
In some instances, these fibres can be seen. Usually, however, they are not
I. Edge of Poupart 's Ligament with a few fibres of Cremasteric Fascia.
J. Origin of Internal Oblique Muscle from outer half of Poupart's Ligament.
Fig. F. The Hernial Sac has been dissected to the Peritoneal Cavity and a purse string suture taken after freeing visceral attachments to its wall. At this time it is well worth while to feel in the Peritoneal cavity for pathological possibilities and inspect, using three retractors, so as to give the best possible view.
On the right side it is frequently easy to remove the Appendix. Some surgeons think this should not be attempted. When it can readily be done, it should be done, but done carefully and without contamination. A Direct Hernia may, or may not be obvious.
A. External Oblique Aponeurosis.
@. Purse string suture about the neck of the Sac. Whether this type of purse string be used, or a single or multiple transfixion suture, varies with the size and site of the Sac. In either case, after tying a pur.se string or transfixion stitch of fairly strong chromic gut, the ends should be made to encircle the neck at least once, or twice in some instances, to make closure quite secure.
©. Free, Peritoneal Cavity.
@. "Bulge" suggestive of a direct hernia. Many "recurrences" of Indirect Hernias have proven to be Direct Hernia.
E. Transversalis Fascia of the "Undefended Space" of the Direct Hernia Region.
F. Internal Oblique Muscle.
G. Conjoined Tendon.
H. "Shelving Margin" of Poupart's Ligament.
®. Cord, with Vas Deferens and its characteristically conspicuous, "curlicue" Artery. J. Deep Fascia (Fascia Lata) of the Thigh.
K. Origin of the Internal Oblique Muscle from the outer half of Poupart's Ligament.
34
HUGH AUCHIN'CLOSS
Fir; ( ; . Tho neck of the Siic has been opened. The purse string suture about the Neck of the S:ic IS not shown. A finger is being introduced to, not only palpate the interior of the Pcritiiiical Cavity, but to search for a True Hernia, a "Weakness" or the "Initial Bulge" of a Direct Hernia in this so-called "Undefended Space."
A "Bulge" is here demonstrated. Sometimes, however, the "Bulge" is wholly different and may occupy the whole of the "Undefended Space" and show no evidences of strengthen- ing, Transvorsalis Fa.scia .strands. Sonietiines the Bladder wall occupies part, or all, of this space, l- iiifici inl idduci idii of iliis soi t effectually reproduces on the operating table an "intra'abddininal tension " (■oinparaiilo to ordinary circumstances when the patient is up and about, lu this way tiic cailx inatiilestation of Direct Herniae can be demonstrated.
A. External Oblique Aponeurosis.
®. Margin of the Hernial Sac.
©. Evidence of the "Initial Bulge" of a coexisting hernia in the "Undefended Space"
of the Direct Hernia Region, inside the Deep Epigastric Vessels. @. Transversalis Fascia of the "Undefended Space" of the Direct Hernia Region.
E. Inner portion of the Internal Oblique.
F. "Conjoined Tendon."
G. Deep Fascia of the Thigh.
H. "Shelving Margin" of Poupart's Ligament.
I. Spermatic Cord.
HERNIA REPAIR
35
apparent. The way they can be recognized is by feeling for them with the finger. This technique should be cultivated in perfoiming inguinal hernia opera- tions. Their sharply defined margin can be felt in practically all instances if one learns how to feel for them. Figure I demonstrates the technique for finding these fibres by jialpation.
Silk sutures arc UNed to sew them to the "shelving margin" of Poupart's liga- ment, snugly around the cord and thus create a "first line of defense" across the internal inguinal ring.
If there be a direct hernial bulge, or true hernial sac, the edges of the defect in the transversalis fascia can likewise be felt from within the peritoneum before the closure of the indirect hernial sac. If there be no indirect hernia, after the direct hernial sac has been opened, the margins of the transversalis fascia fibres are frequently recognized by palpation. Accurate closure of such a direct hernia
J. Origin of the Internal Oblique Muscle from the outer half of Poupart's Ligament. ®. Finger being introduced through the neck of the Hernial Sac to explore for patho- logical possibilities within the peritoneal cavity and study the Direct Hernia Region.
Fig. H. The Neck of the Sac has been ligated with chromic gut and the ends of the liga" ture passed through the Transversus and Internal Oblique Muscles so as to draw the closure away from the Internal Ring preparatory to its repair.
The inner margin of the Internal Ring is shown where the "Internal Arcuate" Fibres of the Transversalis Fascia create a sharp, readily palpated, but not generally seen, edge just outside the epigastric vessels. This is the Ligament of Hesselbach. Repair of the Internal Ring weakness by recognizing this edge and suturing it to Poupart's Ligament should be one of the chief purposes of the Indirect Hernia operation. It was a feature frequently emphasized by Alexis Moschowitz over twenty years ago. It is a step in the successful re- pair of an Indirect Hernia rarely pjr.'ormed by surgeons. It is the most important step in the whole operation, toward permanently successful repair.
Suture of the edges of a cleft in the Transversalis Fascia protecting the "Undefended Space" of the Direct Hernia region is also demonstrated.
It is the Transversalis Fascia, not the Muscles, nor the External Aponeurosis that is of prime importance in Inguinal Hernia Repair (see text).
®. Purse string ligature of the Hernial Sac passed through the Transversus and In- ternal Oblique Muscles above and outside the region of the Internal Ring so as to bring the sac closure to the under surface of the abdominal wall well away from the region of the Internal Ring. (B). "Internal Arcuate" Fibres of the Transversalis Fascia, or "Ligament of Hesselbach". Sometimes this edge can be seen. More often it cannot be. But, it can always be felt. It is a short distance outside the course of the Deep Epigastric Vessels and it can be sutured with silk sutures to Poupart's Ligament without injuring the vessels. ©. Deep Epigastric Vessels behind Hesselbach's Ligament, which is a thickening of the
Transversalis Fascia at this point. ®. Silk sutures closing the cleft in the Transversalis Fascia of the "L^ndefended Space" in the Direct Hernia Region.
Direct Hernial weakness varies considerably from a vertical cleft, as depicted here, to a diffuse bulge, or real sac, with little or no recognizable Transversalis Fascia over the greater part of the Direct Hernia region.
Plication, or sutures taken from above to Poupart's Ligament, or transplantation of a fascial sheet behind the muscles, ma}' have to be resorted to to secure an ade- quate "first line of defense" against such herniae (see text).
E. Internal Oblique Muscle.
F. Conjoined Tendon.
G. Deep Fascia of the Thigh.
H. "Shelving Margin" of Poupart's Ligament.
I. Transversalis Fascia of the "Undefended Space" in the Direct Hernia region.
J. Spermatic Cord with Vas Deferens and its "curlicue" Artery from the Superior Ves- ical Artery.
®. Ligated neck of Hernial Sac ready for transplantation to posterior surface of Trans- versus Muscle.
36
HUGH AUCHINCLOSS
Fig. I. Feeling for the Internal Arcuate Fibres of the Transversalis Fascia, or Hessel- bach's Ligament.
Palpation is the surest way to accurately find these fibres before suturing their edge to Poupart's Ligament.
A. The Ligature about the neck of the Hernial Sac has been sutured to the fascia over the Internal Oblique Muscle after transplanting the closed sac orifice away from the Internal Inguinal Ring, to the posterior surface of the Transversus Muscle (see text).
B. External Oblique Ai)on(Hir()sis.
©. Edge of the Internal Arcuate Fibres of the Transversalis Fascia, or Hesselbach's Ligament, being jjalpated by the finger tip. This is, perhaps, the most essential feature in the techiiiciue of the operation.
D. Deep Epigastric \'essels behind Hesselbach's Ligament and the Transversalis Fascia.
E. Repair of the Direct Hernia by Closure of the Transversalis Fascia.
F. Transversalis Fascia of the Direct Hernia Region.
G. Internal Oblique Muscle, near its insertion.
H. Conjoined Tendon.
I. Deep Fascia of the Thigi\.
J. "Shelving Margin" of Poupart's Ligament.
HERNIA REPAIR
37
defect in the transversalis fascia, whether done from side to side, or from above downwards, should be done with silk sutures so as thus to create a "first line of defense" (figs. J, K).
Should there be an inadequate "first line of defense" in either the indirect or direct hernial regions, in other words, should the transversalis fascia be so weak or practically absent, from the standpoint of repair, the best technique for creat- ing a new "first line of defense" is the transplantation of an adequately broad sheet of fascia from the outer side of the thigh, or elsewhere, to form a new trans- versalis fascia. This is a better method than the use of fascial sutures.
3. Reenforcement of the "First Line of Defense." Once an adequate "first line of defense" has been created, reenforcement is helpful. Reenforcement without the creation of a "first line of defense" is probably the cause of most of our recurrences. Reenforcement, without a "first line of defense," done in such a way as to destroy or weaken the muscle fibres (see below), used to "reenforce" with, contributes, still further, towards recurrences.
The lower edge of the internal oblique and transversalis muscles over the inguinal canal have been frequently written and spoken about as the "conjoined tendon." Many times has it been advised to suture the "conjoined tendon," meaning these muscle fibres, to the "shelving margin" of Poupart's ligament.
All over the world this advice has been given and followed. This advice is wrong.
In the first place, the lower margins of these two muscles do not become a
K. Spermatic Cord, showing tiie "telltale", "curlicue" Artery to the Vas Deferens so
often helpful in identifying it (see text). ©. Finger, palpating the edge of the Internal Arcuate Fibres of the Transversalis Fascia
so as to locate it for suture to Poupart's Ligament and thus close the Internal Ring. This is the most important step in prevention of an indirect hernia recurrence.
Fig. J. Repair of the Internal Inguinal Ring by suturing the Edge of the Internal Arcu- ate Fibres of the Transversalis Fascia to Poupart's Ligament with interrupted silk sutures.
A. Knot in the fascia over the Internal Oblique of the Hernial Sac ligature. This chromic ligature has been passed through the Internal Oblique and Transversus muscles above and outside the Internal Ring.
B. External Oblique Aponeurosis.
C. Peritoneum and Subserous Tissues behind the Internal Inguinal Ring.
@. The Internal Arcuate Fibres of the Transversalis Fascia, or the Ligament of Hessel- bach. Suturing of this edge securely to Poupart's Ligament snugly about the emer- gence of the cord is the most important step in preventing recurrence of an Indirect Inguinal Hernia.
®. Silk sutures uniting the Internal Arcuate Fibres of the Transversalis Fascia to Pou- part's Ligament to close the Internal Inguinal Ring and prevent the initiation of a "bulge" of peritoneum that precedes the formation of a hernia.
d). Deep Epigastric Vessels.
G. Direct Hernia Repair. This is but a diagrammatic representation of the principle of Transversalis Fascia repair. Often the repair has to be done from above down- wards, or by plication, or by transplanting a fascial sheet. Fascial sutures are not as effective.
H. Transversalis Fascia of "Undefended Space" of the Direct Hernia Region.
I. Inner Portion of the Internal Oblique Muscle. J. Conjoined Tendon.
K. Deep Fascia of the Thigh.
L. "Shelving Margin" of Poupart's Ligament.
M. Spermatic Cord.
38
HUGH AUCHIXCLOSS
Fig. K. Repair of the Internal Ring by suturing the edge of the Internal Arcuate Fibres of the Transversalis Fascia, i.e., Hesselbach's Ligament, to Poupart's Ligament.
A method for fixing Poupart's Ligament to the posterior aspect of the Internal Oblique and Transversus Muscles so as to reenforce the Transversalis Fascia repair of the Direct Hernia region, without destroying Muscle Fibres.
A. Suture of the Hernial Sac ligature to the Fascia over the Internal Oblique Muscle (see Fig. H.)
B. Aponeurosis of the External Oblique Muscle.
©. Outer knot of a chromic gut "Staple" suture tied in the External Oblique. This suture is to bring the shelving margin of Poupart's Ligament to the posterior surfaces of the Transversus and Internal Oblique Muscles without destroying Muscle Fibres.
The outer portion of this "Staple" suture is taken first to run less risk of injuring the femoral vessels. After knotting it to the External Oblique Aponeurosis, it is passed directly through the Internal Oblique and Transversus Muscles,— then, just below the edge of the shelving margin of Poupart's Ligament to and through the deep fascia of the thigh below Poupart's Ligament and returned in the reverse direction (H") 2-3 cm. internally.
Tying a suture about a muscle is comparable to tying a suture about a bit of brain or spinal cord. Voluntary muscle fibres are one of the most highly specialized tissues of the body. The muscle cell is worthless without its nerve attachment. Suturing
HERNIA REPAIR
39
M
of these delicate structures in sucii a way as to strangle them, defeats the very pur pose desired.
Indeed, Bassini, in his original description of hernial repair advised suturing the fascia over the muscle and as little as possible of the muscle to Poupart's Ligament.
This "Staple" type of suture gives far better approximation with minimal trauma.
It is not an uncommon finding in operating on herniae that have been repaired by suturing the muscle to Poupart's Ligament to find black silk sutures in scar tissue and no muscle remaining. Of course, where very loose sutures have been taken, tension is lessened and such damage is less likely to occur. However, whenever stress and strain with muscle contraction occurs, tension mounts so that nerve and muscle damage does occur.
This "Staple" suture approximates Poupart's Li'gament to the posterior surface of the muscles practically without constricting muscle and nerve fibres. It improves whatever transversalis fascia repair has been effected behind it. D. Deep Epigastric Vessels.
(E). Repair of Transversalis Fascia in Direct Hernia region (see fig. H.).
F. Transversalic Fascia of Direct Hernia Region.
G. Inner Portion of Internal Oblique Muscle. (§)' Ending of the "Staple" Suture.
I. Conjoined Tendon. J. Deep Fascia of Thigh.
®" Inner Portion of "Staple" Suture returning from its fixed bearing on the deep fascia of the thigh. In passing the needle from the thigh upwards beneath or through Pou- part's Ligament at this point, there is no danger of harming the Femoral Vessels.
K. Spermatic Cord.
(E). Repair of the Internal Inguinal Ring with silk sutures through the edge of the In- ternal Arcuate Fibres to Poupart's Ligament close about the emergence of the Sper- matic Cord.
Fig. L. a "Staple" chromic gut suture reenforces the Direct Hernia Region. First row of an "Overlapping" interrupted, silk suture repair of the External Oblique Aponeurosis and its "Shelving Margin" of Poupart's Ligament.
®. LTpper edge of the incised Esternal Oblique Aponeurosis being sutured near the deep
edge of the Shelving Margin of Poupart's Ligament, with: — B. Interrupted, silk sutures. ©. Staple Suture (See Fig. K.) knotted ends.
D. Internal Oblique Muscle.
E. Deep Fascia of the Thigh.
F. "Shelving Margin" of Poupart's Ligament. ©. Staple Suture seated on Deep Fascia of Thigh.
H. Spermatic Cord, transplanted to in front of Internal Oblique and External Oblique. Fig. M. Repair, completed.
®. Lower Margin of External Oblique Aponeurosis incision overlapped over Spermatic
Cord with a continuous chromic suture. B. Spermatic Cord.
©. "Staple Sutures" (figs. K. L.) bearing on Deep Fascia of Thigh, D. Deep Fascia of Thigh.
40
HUGH AUCHIXCLOSS
tendon until it comes close to the os pubis. In the second place, suturing muscle fibres calls for a technique that Avill not destroy them. Sutures under tension about muscle fibres will kill them. A muscle fibre without nerve supply "dies." Sutures around delicate nerves and muscle fibres kill them. It is difficult to understand how circular sutures around muscles, even though not apparently tied tightly, can avoid tension, once the patient is up and about and subjects the muscles to stress and strain. At operations for recurrent hernia sutures that appear to have been taken about muscle fibres have often been found surround- ing, not muscle fibres, but white strands of thinned out scar tissue.
Figures K and L sho-w, diagrammatically, how a suture, tied on the external oblique aponeurosis, can be taken through the "conjoined muscles" and under the "shehang margin" of Poupart's ligament so as to draw Poupart's ligament up behind the "conjoined muscles." This provides a far more effective re- enforcement of the repaired transversalis fascia without killing the muscles.
Figures L and M indicate the overlapping of the external oblique in com- pleting the reenforcement to the "first line of defense." The cord has been "transplanted."
Recurrent hernias have not been included. Their scarred and compromised tissues present problems quite their own. Their repair calls for individualiza- tion and a variety of technique. Though not described, the underlying prin- ciples of "first line of defense" and subsequent "reenforcement" are the guides to success.
THE DIAGNOSTIC DIFFICULTIES IN UNCOMPLICATED SYPHILITIC AORTITIS WITH A NOTE ON ROENTGENKYMOGRAPHY OF THE AORTA*
SAjVIUEL H. AVERBUCK, M.D., F.A.C.P.
[Adjunct Physician, The Mount Sinai Hospital, New York]
INTRODUCTION
Possibly before but most certainly very soon after the initial lesion of syphilis makes its appearance the blood stream is invaded by large numbers of spiro- chaetes. In this way they are distributed throughout the body and deposited in all its structures and organs. The survival and further development of the Treponema pallidum in these disseminated foci depends on many factors con- cerned with the relationship between the virulence of the spirochaete and the resistance of the body. The part played by the number and concentration of spirochaetes present in any organ, and the role of the natural local tissue resis- tance and immunity are apparent. In this connection the phagocytic activity of special tissues like those composing the reticulo-endothelial system are of paramount importance. Undoubtedly many other forces, not thoroughly defined, are significant determinants of the extent and severity of any particular localization. It has been established that one site of invariable invoh'ement is the root or ascending portion of the aortic arch (1). Many theories have been projected to explain this phenomenon. McMeans (2) believes that the intima of the aorta is involved mainly by direct infection from the blood stream. Others maintain that this part of the aorta contains many small vessels which are primarily affected via the hematogenous route with the production of a syphilitic endarteritis. More widely accepted are the conclusions of Klotz (3) and of Martland (4) who stress the lymphatic and mediastinal factors in the consistent involvement of the aortic root in syphilis. They visualize a massive filtration of spirochaetes out of the blood stream by the lungs during the spirochaetemia which occurs early in the infection. The lungs rid them- selves of these spirochaetes by many routes, one of the most important of which is by way of their lymphatics. The pulmonary lymphatics drain into the peribronchial and mediastinal lymph nodes which quickly become infected. By further retrograde lymph flow there is a migration of spirochaetes into the peri-aortic structures. The perivascular spaces about the vasa vasorum in the adventitia become infiltrated with lymphocytes. The endothelial cells of the intima of these arteiioles proliferate and finally occlusion by thrombosis occurs. Secondary involvement of the aortic media now takes place with destruction and fragmentation of the elastic fibers and fibrous tissue replacement.
Aortitis is the significant lesion of cardiovascular syphilis for it constitutes the starting point from which all other types of syphilitic heart disease arise. Extension towards the heart results in involvement of the aortic cusps with
* From the Cardiac Clinic (Dr. I. R. Roth).
41
42
SAMUEL H. AVERBUCK
characteristic luetic aortic insufficiency. Tfie sinuses of Valsalva from which the coronary arteries take their origin in the great majority of individuals are in the first part of the aorta. Encroachment on the orifices of these important vessels causes reduced coronary blood flow with the anginal syndrome, and some- times, because of complete orificial obliteration, coronary artery occlusion. A distal extension of the aortitis may narrow the orifices of the great vessels arising from the aortic arch with similar occlusive vascular effects. Finally it can be readily understood how a diseased and weakened aortic wall may stretch and give rise to aneurism and similar distortions. It becomes clear then that the earliest and most important lesion of cardiovascular syphilis is the mesaortitis from which all other lesions spring and which, for an undetermined time, exists without any associated valvular or arterial complications. Obviously it is extremely important to determine the presence of this initial aortic involvement as early as possible (5). The diagnosis of uncomplicated luetic aortitis i.e. syphilitic aortitis without encroachment on the coronary orifices, insufficiency of the aortic valve, or aneurism of the aorta, has tried the ingenuity of clinicians since its importance was recognized.
The symptoms considered bj^ some to be diagnostic of uncomplicated luetic aortitis are: (1) Dyspnea, occurring upon exertion or appearing paroxysmally at rest. (2) Substernal burning pain most often situated above the third costal interspace and not invariably related to exertion. (3) Cardiac insufficiency or frank failure without any other obvious etiology. All of the foregoing symptoms undoubtedly arise, from an involvement of the orifices of the coronary arteries, resulting in reduced coronary blood flow and coronary insufficiency, with even- tual degeneration and scarring of the myocardium and cardiac weakness. Such symptoms are common in middle aged patients with arteriosclerotic narrowing of the coronary vessels even in the absence of syphihs. In fact Wilson (6) who correlated the symptomatology presented by 211 cases of luetic aortitis with the necropsy findings concluded that when any of the foregoing symptoms existed either an extension or complication of the luetic process or some other condition accounted for their presence. Keefer and Resnik (7) had come to a similar conclusion some years before.
Signs constantly referred to as diagnostic of luetic aortitis are (1) an accen- tuated tympanitic aortic second sound having a bell-like or tambour (drum- like) quality, (2) increased retromanubrial percussion dullness indicative of a widened aortic arch and (3) a systolic aortic murmur. Attention must be directed to the fact that similar signs are present in nonluetic subjects with widened or dilated aortas associated with arteriosclerosis or hypertension.
Maynard (8, 8A) and his a.ssociates who have been patient students of cardio- vascular lues for many years recommend the following six criteria:
1. The undoubted evidence that the patient has syphilis.
2. The absence of any other disease that might cause aortic dilatation, viz; arteriosclerosis, hypertension.
3. The patient must be younger than 40 years of age.
4. X-ray evidence of a dilated aorta.
DIAGNOSTIC DIFFICULTIES IN SYPHILITIC AORTITIS
43
5. The presence of a hollow accentuated aortic second sound.
6. The possible presence of a systolic murmur at the aortic area.
It is interesting to note that Maynard's criteria are signs and not symptoms, implying that luetic aortitis is an asymptomatic condition or that symptomatol- ogy is not specific enough to be reliable. The requirements set up by Maynard and his group are extremely rigid and allow of successful identification of specific aortitis in comparatively few cases. Furthermore serological syphilis is fre- quently discovered for the first time in patients over 40 and the ciuestion of aortic involvement demands attention in such individuals too. Realizing tlie thorough- ness with which the problem had been approached by previous workers our only justification for another effort to illuminate the diagnostic difficulty was the application of one new method of investigation i.e. roentgenkymography of the aorta. The rationale for utilizing this kind of study was based on specula- tion arising from the following known facts. The early syphilitic aortic lesion is a mesaortitis. As a result of an obliterating endarteritis of the vessels in the adventitia, the media of the aorta, which is composed mainly of elastic fibers and which possesses a minimum of muscle tissue, is very early the site of focal areas of degeneration and fibrous tissue replacement. Such changes in the anatomical structure of an artery could very well alter the elasticity of the vessel wall. Therefore it appeared to be a logical assumption that with an. alteration in the elasticity of the aorta there might be a change in the character of the pulsations of the aortic wall. If this could he detected by roentgenkymography one could establish the presence of aortitis in its incipiency before any of the more obvious complications appeared. This method had already proved its value in the detection of non-contractile portions of ventricular muscle resulting from previous myocardial infarctions. (Dack, Master, Sussman (9), Gubner and his associates (10).
MATERIAL
It is the established routine of the anti-luetic clinic to submit every patient for cardiac study before beginning therapy. In addition, patients are referred for periodic recheck of their cardiac status during the course of treatment. With rare exceptions patients in an out-patient department anti-luetic clinic have had syphilis for several years and usually have not had treatment early in the course of their infections. It was felt that the best opportunity for this study was afforded by these patients because aortitis or any syphilitic lesion localized in the cardiovascular system could be expected in greater frequency in such a group than in an earlier detected and treated group of cases. The cardio- vascular status of 47 patients from those referred to the cardiac clinic from the anti-luetic clinic of The Mount Sinai Out-Patient Department were investigated in an effort to determine the presence of uncomplicated luetic aortitis. Patients who presented unmistakable evidence of undoubted syphilitic heart disease i.e. patients in whom subsequent examination disclosed definite signs or clinical evidence of aneurism, aortic insufficiency or coronary ostial involvement were not included in this study.
44
SAMUEL H. AVERBUCK
All patients were interrogated for the presence of the usual manifestations of heart disease with special emphasis on the already reviewed symptoms of aortitis. Careful physical examination was then done. Fluoroscopic visualization of the heart and great vessels and electrocardiogram were part of every patients study. One new type of observation was added, i.e. roentgenkymographic study of the aortic arch in the postero-anterior and left oblique posticus. A discussion of the principles of this technique which records graphically the pulsations of the heart or blood vessels follows later.
TABLE I
Summarizing Analysis of 47 Luetic Patients Studied for Evidence
Aortic Involvement
Males
Females. . .
Age Range
Average Duration of Infection.
fMales. . . \ Females.
Average Duration of Adequate Treatment.
Positive Serology at some time during observation. Average Systolic and Diastolic Blood Pressure
Range of Blood Pressure.
Cases witli Associated Hypertension
Cases showing p]lectrocardiographic changes
Cases showing Fluoroscopic findings
Cases showing Clinical Evidence of Heart Disease.
32 (4 colored) 15 (6 colored) 24 to 67 years 23 to 63 years
16.1 years (Range 6 mos. to 50 yrs.). Known in 23 of 47 cases
3.4 years. (Range 6 mos. to 10
years.) 100% 133.5
mm. Mercury
mm. Mercury
Table I summarizes the essential data about the 47 cases studied. Some of the.se features will be discussed.
AGE, SEX, AND RACE
Interesting but not unusual were the patients of 63 and G7 who gave a history of having contracted the initial infection many years before, in one instance 50 years pre\iously. The.se two patients like the great majority of the 47 cases studies were included becau.se they manifested no definite clinical evidence of heart disease in spite of the long period of their infections. It was felt that an asymptomatic aortitis might be detected l)y lal)()ratory juethods. Cole and Usilton (11) ob.served two cases of uncompliratcil luetic aortitis which ap- peared after the age of G5. Roughly twenty percent of the patients were Negroes with a predominance of males. Although it is a clinical obserx ation that Negroes are more liable to suffer cardiox-ascular complications of lues than white persons, the relatively .small number of cases considered here precluded any verification of that point.
DIAGNOSTIC DIFFICULTIES IN SYPHILITIC AORTITIS
45
DURATION OF INFECTION
In 23 of the 47 cases there was an average duration of 16.1 years since the initial infection. In the other 24 cases this interval was not known. Maynard (12) showed that in syphilitic patients examined 10 to 19 years after the initial lesion, cardiovascular syphilis was discoverable in 56.8 percent of the cases. Examination after a shorter interval (4 to 9 years) revealed an incidence of 28.6 present, while after longer intervals (20 to 29 years) 77.8 percent of patients were affected. From this standpoint the patients in our series present a fair sampling of potential cardiacs.
WASSERMANN REACTION
All of the cases had a positive blood Wassermann reaction some time during the period of their olis(M\'ation and were undoubted luetics. Although patients Avith obvious syphilitic cardiovascular disease may have negative serologic tests in as high as 15 to 20 percent of the cases it would be extremely hazardous to attempt to identify as difficult a condition as uncomplicated specific aortitis in anyone who Avas not definitely a luetic. Maynard (8A) also lists positive evidence of syphilis as one of his essential criteria.
BLOOD PRESSURE
The average systolic pressure for the entire group was 133.5 mm. Hg. with a diastolic pressure of 80.3 mm. Hg. The range of systolic pressures was from 95 to 200 mm. Hg. Four (8.5 percent) cases had systolic values over 150 and diastolic levels over 85. The common association of luetic cardiovascular disease and hypertension has been noted (13, 14). It has been maintained that the aortic disease, in some manner, perhaps by depressing the inhibitor nerves causes the elevated blood pressure. In this study where clinical evidence for the presence of cardiovascular syphilis is lacking the presence of hypertension probably indicates that the elevated pressure is unrelated to the lues and that it is of the essential type. There are no grounds for believing that the hyper- tension could be considered a sign of aortic involvement. This agrees with Moore's (15) conception that essential hypertension occurs in patients with aortitis with about the same frequency as in non-syphilitic patients of analagous age. However, he stated that when hypertension was present the diagnosis of uncomplicated luetic aortitis was made more successfully because it directed attention to the cardiovascular system in luetics. From our experience we are of the opinion that the presence of hypertension in syphilitic patients makes the diagnosis less secure. In fact the two cases which we suspected to have aortic involvement had an associated hypertension and this circumstance tended to invalidate the certainty of the diagnosis, for the criteria, symptomatic and objective, can be manifested equally by both conditions (16). It is of interest to note that the prognosis in luetic aortitis with hypertension is much worse and the development of the graver foiTns of cardiovascular syphilis is more common.
46
SAMUEL H. AVERBUCK
ELECTROCARDIOGRAPHY
There were no charactoristic changes produced in the electrocardiogram. With severely compromised coronary orifices the electrocardiogram may reflect the extent of the myocardial damage resulting from reduced blood supply to the heart muscle. In the 3 cases showing significant variations from the normal the presence of hypertension or the age of the patient, or both, made it a proper assumption that the electrocardiographic changes might be due to associated arteriosclerotic coronary disease. These changes consisted of RT depressions, a Q wave in the first lead, and T wave inversions in the third lead. Slurring of the QRS complex, left axis deviation and in one patient a right bundle branch block completed the alterations, which could not be considered specific in any sense. INIaynard and his associates rarely found significant changes in early cases. With an associated arteriosclerosis or hypertension electrocardiographic changes were more common. Berk (17) has recently described his experiences with the use of the exercise test to bring out electrocardiographic changes in early luetics. The effect of exercise on the electrocardiogram of normal individ- uals casts doubt on the wisdom of drawing definite conclusions about the presence of relative coronary insufficiency and heart disease in cases where such changes are induced by exertion (18).
FLUOROSCOPIC OR X-RAY FINDINGS
Only 8 of the 47 cases (17 percent) showed fluoroscopic or X-ray evidence of significant aortic changes which were suggestive of luetic involvement. Par- ticular importance was attached to the presence of localized dilation of the ascending part of the aortic arch (21). Other alterations were definite and marked elongation and tortuosity of the thoracic aorta or diffuse aortic widening associated with a heart of normal size and contour. Of these 8 there were 3 who had definite diastolic as well as systolic hypertension. In these cases it was impossible to tell whether or not the changes in the aorta were associated with the elevated blood pressure. One of the 8 patients was a woman of G2 with a long history of syphilis. In spite of the long luetic history one could not be sure whether the degree of arteriosclerosis usual in the age group was not the cause of the findings. In the remaining 4 patients, 30, 38, 39, and 44 years of age, the variations in the aorta could have been considered evidence of luetic involvement. But the absence of confirmatory evidence made such an assumption hazardous.
Percussion is notably unreliable for slight degrees of dilatation so complete dependence has been placed on fluoroscopic and roentgenologic evidence of aortic widening. Many methods for determining the dimensions of the aortic root and of the ascending part of the aortic arch have been described (19, 20). Most of these employ the technique of direct measurement from standardized distance and position teleroentgenograms. Normal average dimensions have been re- corded ])y Hampton, Bland and Sprague. They set a limit of 5.3 centimeters for the normal or for the arteriosclerotic aorta without dilatation and they were able to conclude that the ascending aorta dilated from whatever cause
DIAGNOSTIC DIFFICULTIES IN SYPHILITIC AORTITIS
47
measures over 6 centimeters. Their measurements were made of the transverse diameter of the aorta at its root with the patient in the left anterior oblique position. Details of their technique can be found in their paper. With laudable caution they state that even in those cases with aortic widening a final diagnosis of luetic aortitis can be made only by taking into account all the other clinical and physical findings. Edeiken (22) has shown that in teleroentgenography of the aorta considerable magnification and distortion may occur. Ingraham and Maynard (24) studied 24 young individuals with a short history of lues and no other clinical condition. Teleroentgenograms showed slight dilatation of the aorta in 6. They considered these changes within the realm of normal. Other authors deny the accuracy and usefullness of any specific numerical values and suggest dependence on fluoroscopy of the aorta and the detection of such changes as 1) elongation or tortuosity 2) widening of any portion 3) pulsation to the right of the sternmn 4) increased density permitting visualization of the descend- ing aorta — especially in patients under 50 years of age (21). Such criteria are not always valid, for many factors, both normal and pathological, influence the appearance of the aorta. In sthenic individuals with high lying diaphragms the aortic arch is compressed from below and it assumes a broad silhouette with bulging sides that can easily be confused with true dilatation. Conversely asthenic subjects possess a long narrow aortic shadow and bona fide segmental dilatations may be hidden. In the middle years of life the thoracic aorta begins to elongate and distortions in its shape result. With increasing tortuosity the aortic knob becomes more prominent and variations in the right and left borders of the aorta appear. Unless such changes are extreme and unmistakably abnor- mal they are difficult to interpret. Very often hypertension — even of slight degree — complicates the picture still further, for dilatation, elongation and in- creased tortuosity and density of the thoracic aorta result from this condition too. Where unquestionable aortic changes, particularly dilatation of the ascending portion of the arch, are seen in j^oung syphilitic patients without hypertension the diagnosis of early luetic aortitis is justified, especially if one or more of the previously discussed signs or symptoms is present. Although the interval between an initial lesion and the appearance of aortic syphilis varies greatly aortic changes are rarely demonstrable in the younger luetics. In the middle aged group Avhere the question most frequently arises, the physi- ological variations of aortic anatomy make the problem of identifying early pathological changes a perplexing one.
CASES WITH CLINICAL EVIDENCE OF HEART DISEASE
Only two patients complained of symptoms suggesting cardiac involve- ment. A 38 year old negro who had had a primary lesion 10 years before and who had been treated inadequately with Bismuth for one year complained of a "pulling down" precordial pain, dyspnea upon exertion and occasional attacks of paroxysmal dyspnea. Physical examination revealed a blood pressure of 150 systolic and 110 diastolic with a moderately enlarged heart. The second aortic sound was accentuated and had a tambour quality. Fluoroscopy con-
48
SAMUEL H. AVERBUCK
firmed the cardiac enlargement and disclosed a slightly dilated aortic arch. The electrocardiogram revealed a left axis dex'iation ^^■ith RT segments depressed in leads 2 and 3 and the T wave inverted in lead 3. It was difficult not to conclude that both the symptoms and signs were associated with the hypertension. The second patient with clinical heart disease was a 55 year colored male with lues for an undetermined duration who had had a year of anti-luetic treatment. He complained of exertional dyspnea and palpitation. Examination revealed a h}^perten.sion of 200 systolic and 110 diastolic, and an enlarged heart with a "tambour" second aortic sound. Fluoro.scopy showed a dilated and tortuous aorta. The electrocardiogram revealed a left axis deviation with a low^ T wave in lead I. Here too tlie hypertension and its associated phenomena could very well explain the entire clinical picture. If it is assumed for purpo.ses of dis- cussion that the associated syphilis was of etiologic importance, the significant fact that comes to light is that in only two (4.25 percent) of a group of 47 cases could heart disease be susi)ectc(l on clinical grounds. In other words upon objective findings alone hietic aoi titis in the absence of aneurysm and aortic valve insufficiency could be cDiisidered a possiblity in only 2 cases. This is in contrast to the expectancy justified on pathological grounds. Warthin (23) found either macroscopic or microscopic evidence of luetic involvement of the aorta in 90 percent of 490 syi)hiHtic j):iticuts coming to autopsy. Langer (25) reported that in a series of 23, 105 ncci i)!)si('s 70 to 80 percent of all luetic patients had aortic invoh'ement. He further reportetl that whereas aortitis had been suspected during life in 39.3 percent of the 721 instances of luetic aortitis dis- covered at autopsy, it had remained undiagnosed in the remaining 437 (GO. 7 percent) cases. Moore, Danglade and Reisinger (20) reviewing 105 cases of pathologically prox'ed uncomplicated luetic aortitis fomid that in only 4 cases was the diagnosis made b(>fore death. In 13 it had been suspected while in 52 patients the diagnosis might have been established, had a correct interpretation of the elicited signs and symptoms followed. In the remaining 4G patients either other cardiovascular conditions had obscured the luetic signs or the possibility of luetic aortic disease had not been given serious or direct attention. In a later publication Moore and Metildi (27) reported the verification of the diagnosis of uncomplicated luetic aortitis in 19.1 per cent of 115 cases in which the diagnosis was estai)Iished by the application of 7 criteria they outlined. In an additional 35.0 pejcent of the group they noted presumptive evidence of the correctness of the original diagnosis. In contra distinction to our own experi- ences they conclutled that an a(!curate diagnosis of uncomplicated luetic aortitis could be established in a considerable proportion of patients with late syphilis.
Undoubtedly there were many more instances of aortic .syphilis in our 47 pa- tients than the 2 described above. Yet on purely clinical grounds, with the aid of carefully taken histories, complete physical examination, supplemented by fluoroscopic and electrocardiographic study the diagnosis was justified only in the limited munber. It is obvious from this alone that additional diagnostic aids are necessary.
DIAGNOSTIC DIFFICULTIES IN SYPHILITIC AORTITIS
49
ROENTGEXKYMOGRAPHY OF THE AORTA
Roentgenkymograph}' is a method of recording the movements of the actively beating heart and pulsating aorta on an X-ray film. This is done by interposing a lead grid between the X-ray film and the subject. The lead grid is fenestrated by horizontally placed slits 0.4 mm. wide, spaced 12 mm. apart, allowing X-rays to fall on the films through the open slits. In this way narrow transverse seg- ments of the beating heart and aorta are recorded on the X-ray film and pulsa- tions at respective levels on the right and left borders of the heart are visualized. The film is automatically moved down for distance equal to a little less than the slit separation distance (12 mm.) during an exposure period of from 1 to 2 sec- onds, thereby recording, at normal heart rates, two or three cardiac cycles. For complete technical details reference can be made to the writings of Stumpf (28) and of Hirsch (29). The films obtained by this method show, on the borders of the heart, a series of waves of varying depth, dependent upon the extent of systolic contraction and diastolic relaxation at any level, ^'entricular systole is recorded as a straight horizontal inward thrust while A entricular diastole is reflected as a curved outward excursion. The auricular contractions have a characteristic appearance as do pulmonary artery and aortic curves of the heart border. The diagnosis of myocardial infarctions, ventricular and aortic an- eurysms, dilated chambers in varied types of valvular disease, pericardial lesions, solid neoplasms of the mediastinum, etc. is aided by this method of X-ray visuali- zation (30, 31, 32). It was with the idea of detecting early variations in aortic pulsations that kymograms in the postero-anterior and left oblique positions were taken in these 47 patients with a luetic infection and no clinical evidence of aortitis. In brief it can be stated that kymography of the aortic arch for the early detection of luetic lesions is disappointing as a diagnostic aid. There are many reasons for this. The aorta, so closely attached to the heart, and subject to the influences of so actively contracting an organ, shares in the movement of each cardiac contraction. Xot only does the aorta pulsate expansively with each ventricular systole and diastole but it also moves en masse with each cardiac contraction. The degree of intrinsic pulsation and of en masse movement are variables determined by many factors among which are: 1. The blood pressure, 2. The age of the patient and the consequent degree of arteriosclerotic vessel changes, 3. The force of the myocardial thrust, the stroke volume, and the car- diac rate, 4. The size of the heart, 5. The position of the diaphragms, 6. The phase of respiration, 7. The interfering effect of adhesions from previous medi- astinal or hilar infections. Because of the number of varying factors it is ex- tremely difficult to establish standards of aortic pulsation sufficiently well defined and constant to allow the detection of the minimal variations which would have interested us. Furthermore early luetic changes are found most often in the intra- ventricular portion of the ascending part or root of the aortic arch. In many cases, this part of the aorta could be poorly visualized, even in the oblique position. Diagnostically helpful kymograms of the aorta have been described in outspoken luetic aortic insufficiency or aneurysm but in such cases
50
SAMUEL H. AVERBUCK
a clinical diagnosis is relatively easy and makes this type of X-ray study unneces-l difi sary. The difficulties encountered with kymography of the aorta in early luetic th( aortitis are analagous to the already established shortcomings of the fluoroscopic tic or simple roentgenographic study of the aorta for uncomplicated early aortitis, tb There too the normal variations of aortic size and contour diminish the possi- « bility of ascertaining the minimal deviations which would indicate an incipient j pathological state. As a result of this study it can be stated that roentgen- kymography of the aorta is of no help in the detection of simple aortitis in luetic i subjects without clinical signs or symptoms j
Perhaps from the recent perfection of the direct visualization in vivo of the cardiac chambers and great vessels with intravenous Diodrast (33) will come a method for measuring accurately the size of the aorta both in normal and abnormal states.
SUMMARY
Forty seven patients with positive serological evidence of syphilis were studied for evidence of cardiovascular involvement. These subjects were a mixed group including men and women of all ages. Some had acquired their infections comparatively recently while others in the group gave a history of having been infected many years before. All were under treatment and had been receiving anti-luetic therapy for varying periods of time. None presented symptoms or signs of outspoken or advanced cardiovascular syphilis, such as aneurysm, aortic insufficiency or coronary artery orifice narrowing. The exclusion of such cases was intentional for they present no unusual or exceptionally difficult diagnostic problem. Signs or symptoms that would lead to a justified diagnosis of uncom- plicated luetic aortitis were particularly sought for. The criteria of the Coopera- tive Clinical Group with modifications suggested by Maynard and his co-workers were used as guides. In addition to careful questioning and examination each patient was further studied by fluoroscopy of the chest, electrocardiography, and roe-ntgenkymography of the aorta. All the data were assembled and analyzed for a final evaluation of the validity of the diagnosis of uncomplicated luetic aortitis. In only 2 of the 47 patients was such a diagnosis warranted. It was recognized that in the light of the higher incidence of luetic aortitis (GO-80 percent) reported in necropsy studies of luetics, many cases of specific aortitis in this series were undetected. The additional method of study employed here— roentgenkymography of the aorta — gave no assistance. The difficulty of estab- lishing nornial standards of visualized aortic pulsation made interpretations impossible. A multitude of varying factors made the problem of determining a normal kymographic picture of the aorta at different age periods etc. an ex- tremely difficult one. Similar obstacles surround the question of establishing normal limits for the dimensions of the aorta when studied fluoroscopically or roentgenologically.
COMMENT
It is apparent that simple luetic aortitis is a clinically elusive condition. Symptomatology is not specific and objective data are made unreliable by the
DIAGNOSTIC DIFFICULTIES IN SYPHILITIC AORTITIS
51
difficulty of establishing normal standards for aortic size and pulsation. Perhaps the only set of circumstances which justifies a definite diagnosis is the visualiza- tion of an unmistakably dilated aorta — particularly if the widening involves the ascending portion of the arch — in the luetic patient without hypertension whose age and general condition would preclude the possibility of ascribing the aortic change to arteriosclerosis.
Realization that uncomplicated luetic aortitis is virtually impossible to detect in vivo and in the light of the knowledge that it exists frequently in syphilitic subjects, should encourage the attitude that it is justifiable, for the benefit of the patient, to hazard the diagnosis on less than perfect grounds. Furthermore because of the esoteric nature of the diagnostic criteria the condition cannot possibly be discovered in any but known luetics. Fortunately syphilis is de- tectible by a simple blood test which should be applied to all patients who seek medical attention. Only by adopting these viewpoints will it be possible to discover incipient cardiovascular lues and to deal with uncomplicated luetic aortitis, the initial lesion of all other foiTns of syphilitic heart disease, in a manner which its importance and seriousness deserves.
CONCLUSION
Early luetic aortitis, before the occurrence of coronary artery orifice involve- ment, aortic insufficiency or aortic aneurysm, is clinically undetectible. Excep- tionally it may be diagnosed when specific signs are found in a young luetic who has neither arteriosclerosis or hypertension. Uncomplicated specific aortitis is an asymptomatic condition. Symptoms imply an extension or complication of the syphilitic process in the aorta. Roentgenkymography of the aorta is of no aid in the early discovery of uncomplicated luetic aortitis. In view of the high incidence of uncomplicated luetic aortitis established by necropsy studies it must be recognized that though undiagnosable it is often present in kno\\Ti luetics.
BIBLIOGRAPHY
1. Saphir, O., and Scott, R. W.: Am. Heart. J., 6: 56, 1930.
2. McMeans, J. W.: Am. Heart J., 6: 42, 1930.
3. Klotz, O.: Tr. Am. Physicians, 1917.
4. Martland, H. S.: Am. Heart J., 6: 1, 1930.
5. Thompson, W. P., Comeau, W. J., and White, P. D.: Am. Heart J., 17: 286, 1939.
6. Wilson, R.: Am. J. Sc., 194: 178, 1937.
7. Keefer, C. S., and Resnik, W. A.: Arch. Int. Med., 37: 264, 1926.
8. Maynard, E. p. et al.: Brooklyn Hospital J., 2: 69, 1940.
8a. Maynard, E. P.: Modern Concepts of Cardiovascular Disease, 11: 12, 1942.
9. Dack, S,, Su.ssman, M. L., and Master, A. M.: Am. Heart J. 19: 453, 1940.
10. GuBNER, R., AND Crawford, J. H.: Am. Heart J., 18: 8, 1939.
11. Cole, H. N., and Usilton, L. J.: Arch. Int. Med., 57: 893, 1936.
12. Maynard, E. P.: Arch. Int. Med., 55: 873, 1935.
13. ScHERF, D., AND BoYD, L. J.: Cardiovascular Diseases, St. Louis, Mosby, 1939.
14. ScHLE.siNGER, H.: Handbuch der Haut und Geschlect Krankheiten (Jadassohn) bd.
16, pt. 2, p. 272.
15. Moore, J. E., and Metildi, P. F. : Arch. Int. Med., 52: 978, 1933.
52
SAMUEL H. AVERBITCK
16. PuRKS, W. K.: Am. Heart J., 9: 660, 1933.
17. Berk, L. H.: New York State Med. J., 41: 223, 1941.
18. RiSEMAN, J. F., Waller, J. V., and Bro\vn, M. G. : Am. Heart J., 19: 6S3, 1940.
19. Hampton, A. O., Bland, E. F., and Sprague, H. B.: Am. Heart J., 6: 77, 1930.
20. \^aquez, H., and Bordet, E.: The Heart and Aorta: New Haven, Yale Univ. Press,
1920.
21. Kurtz, C. M., and Eyster, J. A.: Am. Heart J., 13: 67, 1937.
22. Edeiken, J.: Am. Heart J., 20: 77, 1940.
23. Warthin, a. S.: South. M, J,, 24: 293, 1931.
24. Ingraham, R., and Maynard, E. P.: Am. Heart J., 6: S2, 1930.
25. Langer, E. Munchen: Med. Wchnschr., 73: 17S2, 1926.
26. Moore, J. E., Danglade, J. H., and Reisinger, J. C: Arch. Int. Med., 49: 753, 1932.
27. Moore, J. E., and Metildi, P. F.: Arch. Int. -Med. 52: 978, 1933.
28. Stumpf, F., Weber, H. H., and Weltz, G. A.: Roentgenkymographische Bewe-
gunzslhre innerer organe. Leipsig, 1936 g. Thieme.
29. HiRsCH, I. S.: Radiology, 22: 403, 1934. .30. Wood, F. G.: Lancet, 1: 249, 1939.
31. Gubner, R., Cr.\wford, J. H., Saiith, W. A., .\nd Ungerleideb, H. E.: Am. Heart.
J., 18: 729, 1939.
32. Back, S., Sussman, M. L., and Master, A. M.: Am. Heart J., 19: 464, 1940.
33. RoBB, G. P., and Steinberg, I.: Am. J. Roentgenol., 41; 1, 19.39.
CONTINUOUS IMMOBILIZATION OF BOTH LUNGS BY AIR PRES- SURE AND ITS APPLICATION TO THE TREATMENT OF PULMONARY TUBERCULOSIS
ALVAN L. BARACH, M.D.
[Frotn the Department of Medicine, College of Physicians and Surgeons, Columbia University and the Presbyterian Hospital, New York]
Inhalational therapy has modified in various waj's the breathing pattern of patients with disturbances in respiratory function. In clinical dyspnea due to anoxia the inhalation of oxygen-enriched atmospheres has been shown to result in a decrease in the volume of breathing. When increased percentages of car- bon-dioxide are inspired, the pulmonary ventilation is progressively increased. The inhalation of helium with oxygen is responsible for a decrease in the phys- ical effort of breathing in conditions characterized by obstruction in the respira- tory passageway. The volume of breathing may or may not be lowered, but the effort is diminished. During positive pressure respiration the physical eft'ort of breathing is also decreased in obstructive dyspnea when it is applied during inspiration or both cycles of respiration, and the total pulmonary ventilation is often diminished. The employment of positive pressure in expiration only is designed for the treatment of pulmonary edema and is less apt to result in note- worthy changes in the amount of air or oxygen breathed. Under the latter con- dition there is increased work on the part of the patient who breathes outward against an arbitrarily imposed obstruction.
Cessation of all lung movement may be temporarily produced by hyperventi- lation with pure oxygen, which reveals the importance of the chemical regulation of breathing. A normal individual may show a cessation of all chest motion for a period as long as 5 minutes without discomfort or signs of anoxia.
The purpose of this communication is to describe a method of producing continuous arrest of lung movement and at the same time provide a normal pul- monary ventilation with an adequate exchange of oxygen and carbon-dioxide between the pulmonary epithelium and the alveolar air. In 1926 Thunberg constructed a respirator, which he called the barospirator, in which an alter- nating pressure of one-sixth of an atmosphere Avas produced 25 times a minute. A person enclosed in this chamber obtained an adequate pulmonary ventilation with little change in the volume of the chest (1). The dcA-ice was employed to maintain artificial respiration in patients with poliomyelitis in whom respira- tory paralysis had taken place. The principle of this method depends on the physical law that the number of gas molecules in a container, with the volume and temperature constant, varies with the degree of pressure to Avhich the gas is expo.sed. Thus, if the lungs hold 3000 cc. of air at the end of a normal ex- piration increasing the pressure one-sixth of an atmosphere would result in an increase in the number of gas molecules present in them equivalent to 500 cc. Similarly, when the pressure change was decreased one-sixth of an atmosphere there would be a diminution in the number of molecules in the lung of approxi-
53
54
ALVAN L. BARACH
mately 500 cc. If one Aveie to assume a rigid container, as Thunberg did, no change in the size of the container would naturally take place. Although Thunberg obser\'ed little or no movement of the chest wall in normal subjects, our studies soon revealed a slight initial compression of the chest which took place when the positive pressure wave was applied to the thorax and, conversely, a slight expansion of the chest wall during the negati\'e pressure pliase. When patients with pulmonary disease were exposed to an alternating pressure of one- sixth of an atmosphere, expansion of the chest during the negative cycle was quite marked, as well as the subsecjuent compression of the chest during the height of the positive pressure wave. The degree of alternate enlargement and contraction of the thorax varied Avith the extent of resistance in the tracheo- bronchial passageway. It A\-as evident that the Thunberg barospirator could not be used for the treatment of patients with pulmonary tuberculosis since our object was complete arrest of lung movement.
Since the walls of the thoracic cage are somewhat flexible, the project of immobilizing them required that an equal pressure be exerted on the outer and inner surface of the chest wall (and on the upper and lower surface of the dia- phragm) at precisely the same moment. Since the air which enters the lung, \\hich ultimately builds up a pressure on the upper surface of the diaphragm and on the inner surface of the chest, must pass through the pharj^nx, larynx, trachea, and the large and smaller bronchi, a certain time interval is necessarily present during which a higher pressure is applied to the external surface of the thorax than to its inner surface. The problem resolved itself into a method of delaying the application of pressure to the outer chest wall until the time of the arri\-al of an equivalent pressure within the lung. As a result of experimental and clinical investigation, it became apparent also that in most subjects 5 cm. pressure was consumed by the tracheo-bronchial resistance in the passage of air from the pharynx to the lungs.
At first the body of the patient was surrounded by a box, with closure at the neck, and the patient and this box were then placed into a large room in which an alternating pressure of 55 mm. Hg above and below the atmosphere was ob- tained. By adjusting the number of holes in the inner box the pressure, as well as the time of arrix al of pressure on the outer chest wall, could be made equiva- lent to that which entered the lung through the nose. Later a transportable type of chamber (fig. 1) was developed in which this object was obtained by first passing the air into the head end of the chamber and then, through a slightly constricted orifice, into the body end of the chamber. In this way a slightly smaller total pressure^ A\as ajiplied to the chest wall at a slightly later moment in time than that wKwh was applied to the nose or mouth of the patient. This chamber, which we call the (M|ualizing ])i'essure chamber, accomplishes normal or increased pulmonary Aontilation without movement of the chest wall or diaphragm.
Complete arrest of spontaneous respiration is obtained in patients after a little preliminar\- training. At first, the impulse to breathe recurs in cases of pul- monary tul)erculosis who are treated l\v this methotl of i)ro\iding local lung
IMMOBILIZATION OF BOTH LUNGS BY AIR PRESSURE
55
rest. However, when the chemical requirements of the body are met by an adequate supply of oxygen and elimination of carbon-dioxide, the impulse for spontaneous respiration ceases. This arrest of voluntarj^ breathing can only be made continuous if the pressure on each side of the chest wall and the cUaphragm is made equal. When the pressure is allowed to arrive at the chest wall before adequate delay has been accomplished there appears to be a cumulative com- pression of the chest. Expressed differently, when alternating pressiu-e is used without the differential resistance which equalizes the pressure relationships, being maintained, a feeling of increased compression of the chest takes place which ultimately forces the individual to take an inspiration. Continuous immobilization of the lungs therefore can only be maintained when the pressure
Fig. 1. Equalizing pressure chamber open
on each side of the chest wall has been made equal and in addition an adequate exchange of gases in and out of the lungs be provided.
In animal experiments in which asphyxia was produced by excessive doses of nembutal, the use of the barospirator principle, namely, alternating pressure, was unable to maintain respiratory function. A gradual increase in carbon- dioxide and a progressive lowering of the oxygen saturation of arterial blood was found. When equalizing pressure was added under the same circum- stances, the animals showed maintenance of normal respiratory function, with- out significant lowering of the arterial oxygen saturation or retention of CO2 (2).
The equalizing pressure chamber was de^•eloped in order to provide complete lung rest in patients with pulmonary tuberculosis. Individuals who reside in this chamber also obtain an unexpected form of body rest. One of the re- markable effects of arrest of voluntary breathing is the type of bodily relaxation
56
ALVAN L. BARACH
which is observed when the effort of breathing is performed by the machine and not by the patient. An indi\ iclual who is in such a chamber without the ap- paratus in use reveals the same restlessness and movement of hands or legs seen in the patient who is at bed rest. However, as soon as the breathing is taken over by the application of eciualizing pressure, the impulse for spontaneous movement of the hands and legs is strikingly diminished. In some instances a patient will lie for 4 hours without turning or manifesting any change in body contour. The desire to smoke cigarettes which patients show at ordinary bed I'est is much less or almost absent when breathing is performed by the chamber. As a result of cessation of normal respiration, a degree of body rest is obtained which is not possible during ordinary bed rest. No patient has been encountered who could lie c^uietly in a bed for long periods when normal breath- ing was takmg place. The emplo^Tiient of continuous immobilization of both lungs, with provision of an addiuatc pulmonary ventilation, makes pos.sible l)oth a degree of local lung icst and body rest not hitherto available.
The application of this type of therapy has been undertaken in 10 patients ^\•ith advanced pulmonary tuberculosis. The patients generally reside in the chamber from 8:30 in the morning until 9:30 or 10 o'clock at night, and are only remo\-ed at meal time. A course of therapy of four months duration has arbi- trarily been selected. The preliminary resvdts of a series of 5 patients revealed marked improvement in 4 of them (3). Of the 10 patients who have been treated with one or more coiu'ses of immobilizing lung therapy, 3 of thefn have been clas.sified as being citiicr little imi)rov('d or transiently improved, without significant alteration in their course. A fourth patient showed a collapse of a large cavity in the left lung with a recurrence on slight activity; this patient is now being treated in a second course. Of the remaining G patients, 5 of them have been discharged as al)le to work and in 4 in whom a follow-up has been possible, all are at work without signs of active disease. In some there has been a complete clearing of tuberculous lesions and ca\ ities, and in others there has been complete arrest of the disease, with e\ idence of fibrosis on x-ray examina- tion. The sixth patient who showed marked improvement was ambulatory for a period of two years but is now in a municipal institution and reveals on x-ray examination what appears to be a small cavity at the site of his original large lesion. His sputum has been and is now consistently negative since the second course of treatment and he is in apparent good condition, manifesting no signs of clinical tuberculosis, except the area of apparent cavity on x-ray.
Two of the patients who have shown a favorable outcome as a result of this treatment had one course of treatment. Three patients had two courses of approxiinatoly 4 months each and one jiatient had three courses of treatment bef(.)rc all >igns of the disease cleared and l)efore the cavities in both lungs were completely al)sent. Tlie se\ enth patient will have had two courses of treatment.
The meclianisni by w hich clearing of advanced tuberculous disease is accom- plished in this chamber may be discussed provisionally. Local lung rest in- cA-itably decreases the diffusion of toxins from the tuberculous lesion. There is a striking diminution in the impulse to cough, which also makes less likely a
IMMOBILIZATION OF BOTH LUNGS BY AIR PUESSURE
57
spread of the disease. Since arrest of lung movement is begun at the conclu- sion of a normal expiration, the elastic tension of the lung is at a minimum. A specific factor in causing collapse of cavity is in all probability the removal of the ball-valve mechanism which is commonly responsible for enlargement and main- tenance of tuberculous cavities. Thus, in ordinary breathing, an inspiration is accompanied by an enlargement of the cavity and, when the respii-atory passageway between the cavity and the bronchi is small, by a decreased rate of emptying of the cavity, with a consequent pressure on its walls. Since there is no movement of the cavity as long as there is a passageway between it and the bronchi during immobilizing lung therapy, this alternate modification of the size of the cavity does not take place. When arrest of lung movement is pro- vided by equalizing pressure therapy, there is a passage of air into the cavity during the positive phase and an exit of air from the cavity during the negative phase. As long as some opening exists between the cavity and the bronchi this interchange of air results without compression of the walls of the cavity. It is possible that in cavities of very large size the diameter of the bronchus leading to the cavity may be so small in relation to the total size of the cavity as to result in some compression and expansion of the cavity during the phases of positive and negative pressure. In two of the cases in which no significant improvement took place the very large size of the cavity may have been re- sponsible for a situation in which complete absence of movement of their walls was not secured. In one of these patients an evening temperature of 104. 5°F. on admission to the hospital represented the terminal stage of a bilateral con- fluent type of tuberculosis.
In the presence of cavities of moderate size the cessation of lung movement ol)\-iously prevents the inspiratory enlargement of the cavity which necessarily takes place during ordinary breathing. That this type of local lung rest has a specific effect in producing collapse of cavity has been demonstrated in an un- questionable way. In a number of patients in this series, a cavity has disap- peared during the first course of treatment only to reappear after discharge from the hospital during convalescent care. A subsequent course of treatment resulted in complete closure of the cavity in the same length of time as was required during the first course. This type of evidence shows the specific ef- fect of local lung rest as provided by immobilizing lung therapy in achieving the closure of cavity as well as the clearing of infiltrative lesions (4).
During the course of this therapy gain in weight was sometimes striking, as much as 35 pounds in three months. The cough and expectoration are notably decreased in a period of 2 or 3 weeks. In fact, there is not infrequently on x-ray examination signs of scattered atelectasis as the result of the decreased cough and therefore diminished ventilation of certain areas of the lung. Although the patient is quite able to cough in the chamber and could be instmcted to ex- pectorate the secretions which accumulate during the absence of normal breath- ing, our procedure has been to suggest no postural drainage or no deliberate coughing in order to keep lung rest at a maximum. The possibility presents it- self, however, that emptying of the cavity by deliberate coughing may be de-
58
ALVAN L BARACH
sirable. There is one other characteristic of this tj'pe of therapy that may be mentioned, namely, the effects on the eardrums of the alternating pressure. In all the patients that have been treated this sensation, which may be uncom- fortable at first, is one to which they become oblivious, generally within 2 or 3 days. However, our more recent custom is to place sponge-rubber containers over each ear in order to make the sensation minimal.
Patients have not found this treatment uncomfortable or burdensome. In every instance in which the disease of the patient recurred after the first course of treatment, the patient urgently requested another course of therapy. In some instances thoracoplasty had been ad\dsed when one lung cleared under immobilization therapy, but the patient invariably expressed the desire for a second course in order to have the opportunity for complete arrest of the dis- ease without thoracoplasty.
SUMMARY
A method of Continuous immobilization of both lungs hy air pressure is de- scribed. The principle of alternating pressure, originally reported by Thun- berg, accomplishes an adequate ventilation of the lung but does not provide arrest of lung movement. An alternating pressure of one-sixth of an atmosphere results in compression and expansion of the chest during the positive and nega- tive pressure waves respectively in patients with pulmonary tuberculosis.
The development of a method of immobilizing the chest wall and diaphragm, with the production of complete arrest of lung movement, was made possible by decreasing the pressure applied to the chest wall through enclosing the body of the patient in a separate chamber from that of the head. At the same time the pressure applied to the thorax was delayed for a moment of time until an equivalent pressure passed through the tracheo-bronchial passageway to the inner surface of the chest.
The application of local lung rest in the equalizing pressure chamber was em- ployed in 10 patients with advanced pulmonary tuberculosis. In 6 patients a significant improvement took place. The seventh pat'ient is still under treat- ment. Five patients have been discharged as able to work and reveal either no evidence of tuberculous disease or complete arrest of disease ; these five pa- tients have been clinically well for periods of 1 to 4 years and in 4 in whom follow-up has been possible all are at work.
The procedure has been employed up to this time on patients in whom no other form of therapy was available. Pnevmiothorax had either been tried or was considered unsuitable and the bilateral nature of the disease precluded thoracoplasty except in one patient in whom a double thoracoplasty had been advised.
When voluntar,y respiration is dispensed with and the physical effort of breath- ing is no longer present in the chamber, a special type of relaxation takes place in which the impulse for movement of the voluntary musculature is either strik- ingly diminished or absent. Patients are able to lie for hours at complete body rest in the chamber although at ordinary bed rest this type of complete physical immobility is impossible to maintain.
IMMOBILIZATION OF BOTH LUNGS BY AIR PRESSURE
59
Immobilization of both lungs by this type of equalizing air pressure has been shown to have a specific effect not only on the clearing of infiltrative, tubercu- lous lesions, but also on the collapse of cavity.
BIBLIOGRAPHY
1. Thumbekg, T.: The barospirator: a new machine for producing artificial respiration.
Skandinav. Arch. f. Physiol., 48: 80, 1926.
2. Barach, a. L.: Immobilization of lungs through pressure. Am. Rev. Tuberc, 42:
586, 1940.
3. Barach, A. L.: Continuous arrest of lung movement. Treatment of pulmonary tuber-
culosis in an equalizing alternating pressure chamber. Am. Rev. Tuberc., 43: 56, 1941.
4. Barach, A. L.: Principles and Practices of Inhalational Therapy, Philadelphia, J. B.
Lippincott Co., 1944.
MULTIPLE PURULENT ARTHRITIS DUE TO MENINGOCOCCUS IN VERY EARLY INFANCY
Report of a Case in an Infant Suffering from Congenital Cataract and Cardiac Disease Whose Mother Had Rubella during First Month of Pregnancy
MURRAY H. BASS, M.D., and GERHARD XOTHMAX, M.D.
The neAvbom infant whose history folloAvs is of interest for the following reasons: During tlu' first month of pregnancy the infant's mother suffered from an attack of Cierman measles. The infant was born with two congenital deformities, a cataract in the right eye and a cardiac lesion. The relationship of th(>s( dcf()imiti(-s to the mother's previous illness will be only briefly discussed as the ocular Hntlings in this infant have already been published by Dr. Algernon Reese who oj^erated on and reported this (■as<> together with two other similar cases under the title "Congenital Cataract and Other Anomalies Following German Measles in the Mother" (1). At the age of six weeks the infant developed an acute fel)rile illness characterized by purulent arthritis in several joints aspiration of w hich yielded a pure culture of meningococcus. The infant made an excellent recovery.
CASE report
Hislory: R. S. (Adm. ^515748), aged 7 weeks, was admitted to The Mount Sitiai Hospital on January 23, 1944 because of dyspnoea and lack of apj)etite. Ho was the first child of healthy parents. During the first month of pregnane}' the mother had surferoil from an attack of German measles. Delivery was by forceps at full term after an 18 hour labor. The birth weight was 6 pounds. At the age of four days cyanosis was first noted and a diagnosis of congenital heart disease was made. E.xamination also revealed a cataract of the right eye and a right palpebral aperture slightly smaller than the left. At the re- quest of his pediatrician. Dr. Erich Siegel, he was seen by one of us two weeks later and it was decided that on account of his small size and general debility he should be kept in the infants nursery of the hospital for several weeks more. He was visited by his father and hy an uncle, both of whom were soldiers stationed at an army camp. At the age of six weeks the infant developed purulent conjunctivitis of the right eye which lasted about two days and was followed hy pliai> n<iitis, ccrx ical adenitis, low grade fever, mild dj'spnoea and anore.xia. The last two symptoms pci-sistctl until admission to The Mount Sinai Hospital when the infant was 7 weeks old.
Examination: On admission the temperature was 99.2°P\ The weight was 7 pounds 8 ounces. The infant appeared ill. He was somewhat dyspnocic and his color was dusky. Ears, nose and throat were ncjrmal. In the right vyv Www was a dense white cataract which occupied the entire pupillary area. liic pupil reacted slightly to light. The cornea and iris were normal. The right eye api)eared slightly smaller than the left. Examination of the left eye revealed the cornea, iris and lens to be normal and the pupil reacted promptly to light. The left fundus examination showed a grayish-white disc with clear margins. The peripapillary region was depigmented. In the lower temporal cjuadrant and less so in the nasal quadrant the pigment was irregularly distributed. The heart was enlarged and a loud rough systolic and a short blowing diastolic murmur were heard loudest at the base and along the sternum. The lungs were normal. The liver was felt two fingers
MULTIPLE PURULENT ARTHRITIS DUE TO MENINGOCOCCUS
61
breadth below the costal margin. The spleen was just palpable. The right kidney was palpable and seemed enlarged. Neurological examination was negative. The fontanelle was flat.
Laboratory data: On admission: hemoglobin, 74 per cent; red blood cells, 3,700,000; white blood cells, 37,600 with segmented polynuclear neutrophiles, 41 per cent; non-seg- mented, 30 per cent; lymphocytes, 23 per cent; monocytes, 6 per cent. The cells showed moderate toxic granulation. Urine normal. The Wassermann, Patch and Mantoux tests were negative. A throat culture disclosed Streptococcus viridans A, Staphylococcus albus B and Staphylococcus aureus A. A nose culture showed Staphylococcus albus B. X-ray examination of the chest revealed enlargement of the heart, both at the base and in its transverse diameter. Electrocardiograpliy showed right axis deviation and a polyphasic QRS in the standard leads. PhonucardiDgram revealed a crescendo systolic murmur of very high amplitude closely followed by a deerescendo diastolic murmur of moderate amplitude. The murmurs were loudest below the pulmonary area.
Course: On the second day after admission the child's temperature rose to 103 °F. and ranged from 100°F. to 104°F. for the following eight days. On the fourth day after ad- mission fluctuant areas were noted over both ankle joints. Aspiration of the swelling below the left external malleolus revealeil pus. Attempts to do femoral punctures to obtain blood for culture yielded pus from both sides which was apparently aspirated from the hip joints. Culture of this pus and also a blood culture revealed meningococcus type 1. Spinal tap at the same time produced normal spinal fluid. Roentgenograms of the lower extremities failed to shov osteomyelitis. A small dose of Antimeningococcus serum was injected intramuscularly without untoward effect and a mixture of the serum diluted with an equal part of normal saline solution was injected slowly intravenously. However, the patient developed dyspnoea and the injection had to be discontinued. Sulfadiazine, 0.125 gram every 6 hours by mouth was given for a period of six days. The swelling of the ankles disappeared gradually. The temperature declined by lysis and was normal for the last two weeks of the child's hospital stay. Blood culture after chemotherapy was sterile. The white cell count gradually came down to 9,300 (Feb. 8). Because the hemo- globin had dropped to 40 per cent (Jan. 30) the patient was transfused twice, with subse- quent rise in hemoglobin to 74 per cent. The patient was discharged four weeks after ad- mission in good general condition. The temperature was normal. The weight was 7 pounds 8 ounces. There was mild dyspnoea but no cyanosis.
The infant was later successfully operated on for his cataract by Dr. Reese and is now at the age of ten months in good general condition.
DISCUSSION
a. The Congenital defects. In recent articles piil)li.sh('(l in Australian medical journals Gregg (2), Swan and his a.ssociates (3, 4) have pointed out that an attack of German measles in the mother during the early ni(;nt]is of pregnancy may be followed by congenital defects in the sul:sequ('iitly born infant. The abnormal- ities encountered include cataract, heart tlisease, deaf nuitism, microcephaly, ol)literation of the bile ducts, eciuinovarus deformity, anomalies of the kidneys and mental deficiency. This observation was first made by Gregg, when in the early part of 1941 an unusual number of congenital cataracts ai)jM'ared in Sydney following a severe epidemic of German measles in the summer of 1940. A subsequent investigation conducted by the above mentioned authors re\ealed that cases also occurred in many other parts of Australia during the year 1939 and 1943. The total nimiber of cases recorded in Australia is 109. In most cases Rubella was contracted diu'ing the first three months of pregnancy. The number of cataracts following Rubella in the mother so far recorded in Australia
62 MURRAY H. BASS AND GERHARD NOTHMAN^
is 93, of which 20 cases were unilateral and 73 bilateral. In this country, of the three cases reported by Reese, two were bilateral and one unilateral. The reader is referred to Dr. Reese's paper for the details of the ophthalmological findings and for a more detailed summary of the Australian literature.
Congenital cardiac defects were present in 65 cases reported by the Australians. In this country the three cases published by Reese all had cardiac anomalies. Most of the cases seem to belong to the acyanotic group of congenital cardiac disease. A systolic or less frequently a systolic-diastolic murmur with the maximum intensity over the pulmonary area was heard in most cases. In some a thrill was present. In many, x-raj^s showed an enlarged and globular shaped heart. In the seven cases that came to autopsy a widely patent ductus arteriosus was found beside other minor lesions.
The latest report of this condition appeared in the October 1944 number of the Journal of Pediatrics by Carl A. Erickson who reported 11 infants with con- genital cataracts whose mothers had suffered from Rubella within the first 3 months of pregnancy.
According to Gregg most of the infants are small sized, poorly nourished, difficult to feed and mentally retarded. Swan gives the average birth weight as 5 pounds 7 ounces. 15 of the 75 cases reported b\'' Gregg died, several of bronchopneumonia. Our patient was of small size, a poor feeder and his gain in weight during the first few weeks of life was most inadequate. In this con- nection it is interesting to note how well the infant got over his severe meningo- coccus infection in spite of all of his handicaps.
b. The Meningococcus infection. In childhood, meningococcal infection in the great majority of cases takes the form of meningitis. However it is well known that there are conditions in Avhich the meningococcus causes disease by attacking other organs than the meninges thus producing very dissimilar clinical pictures. In especially susceptible indi-\-iduals or perhaps when the meningococci are particularly virulent, blood stream infection results in such o^-erwhelming sepsis that death occurs before the meninges have a chance to become invaded. Such cases may take the form of the Waterhouse-Friderichsen syndrome. In other instances the disease is not so acute nor so virulent and a picture of a subacute febrile illness results which may drag on undiagnosed for a long time. In such cases the joints may become the foci of infection and mono-articular or polyarticular inflammation may result. The case described in this communi- cation is remarkable for the following reasons : the disease occurred in very early infancy (7 weeks of age) ; it began as a fever of unknown origin, the joint involve- ment only appearing in the second week of illness ; in spite of the fact that the patient was suffering from two severe congenital malformations, he made an excellent reco\ ery Avithovit any apparent residual arthritic damage.
In the neonatal period multiple joint infections are Avell known, appearing as septic foci in so called sepsis of the newborn, ^^arious organisms may be the etiologic agent, most common being the streptococcus, the staphylococcus, the gonococcus and the colon bacillus. We have had on our pediatric service a
MULTIPLE PURULENT ARTHRITIS DUE TO MENINGOCOCCUS
63
child a few weeks of age with purulent arthritis of both shoulders, elbows, wrists, both hips, knees and ankles. This baby survived, only the two hip joints being permanently damaged, the other joints returning completely to normal.
Meningococcus arthritis is of particular interest because it may be part of meningeal disease or it may occur as an independent focus. Schein (5) in 1938 reported on 23 cases and thoroughly reviewed the whole subject. He divided the meningococcal arthritis as follows:
Type I. Meningococcal arthritis may be associated with epidemic menin- gitis.
(a) It may occur from a few days to as long as 2 months before the
meningitis, in polyarticular fashion, resembling rheumatic fever, gonorrhoeal arthritis, etc.
(b) It may occur during the course of the meningitis.
(c) It may occur after the meningeal symptoms have disappeared,
usually from the 4th to the 7th day. Type II. Arthritis may occur with sepsis apart from meningitis. Type III. It may occur as an isolated, localized infection with no other foci in the body.
Our case should be classified under Type II as there was no evidence of meningeal in^•olvement and the blood culture was positive.
The frequency of arthritic complications in meningococcus disease is difficult to determine. Most of the statistics deal with groups of cases composed of both children and adults, and in almost all the cases the meninges are involved. Fox and Gilbert (6) reported 10 cases of arthritis among 26G meningococcus infections seen during the part 12 years. There were 4 cases in 215 patients before the era of chemotherapy (1.9 per cent) and G cases in the 51 patients treated in 2 years with chemotherapy (11.8 per cent). This increased percentage they ascribe to the survival of more severe cases. They quote the percentage mcidence as found by other authors as follows: RoUeston 4.8 per cent; Councilman 5.4 per cent; Herrick 6.5 per cent; Bellevue Hospital series 7.7 per cent.
Of interest in this connection is a group of cases dealing with children only, recently reported from Chili by H. W. Jaeger (7). Within the space of 10 months 1,000 children suffering from meningococcus infections were treated in his hos- pital. In these arthritis was diagnosed clinically in 37 cases (3.7 per cent). However the author comments on the fact that in 52 cases which were examined at postmortem, 13 cases of purulent synovitis were discovered which had not been diagnosed clinically. Incidentally he also describes bursitis and tenosynovitis due to meningococci. His youngest case was aged 11 months. It had menin- gitis with puinlent arthritis of one knee joint and recovered.
As we mentioned before, all these series deal with epidemic meningitis. Here and there in the literature an occasional case of meningococcic arthritis is re- ported in a very young infant not suffering from meningitis, but such cases are extremely rare.
Campbell and Greenfield (8) describe a previously healthy 15 months old in-
64
.MURRAY H. BASS AND GERHARD NOTHMAN
fant who de^•eIoped fever and a swollen knee joint. There was no history of previous infection. Pus aspirated from the joint revealed meningococci. The outcome is not known.
Kobayashi (9) reports a 4 months old infant previously well who developed high fever and a morbilliform eruption. On the third day of illness there was invoh'ement of the ankles, elbows, knees and wrist. Blood culture was sterile but fluid from the joints re^'ealed meningococci. At no time were there menin- gitic symptoms. After an illness of 3 months, the baby recovered.
In 1921 French authors (10) recorded a fatal case in a 17 day old infant with arthritis of the knee and a cer\ ical abscess secondary to an inflammation of the sterno-clavicular joint without meningitis.
The interesting question arises as to how these very young infants acquire theii' infection. In our case the infant Avas in an excellent obstetrical ward where infection with the meningococcus was most imlikely. He was then taken home where he was \-isited by the father and an uncle, hoih of whom were in the armed services and may well have been cairiers due to sojourn in camps. The father's throat culture was negatiA C for meningococci. The uncle's could not not be obtained.
SUMMARY ■
An infant with congenital cataract and cardiac disease whose mother had Ru- bella during the first month of pregnancy is described. The relationship of the mother's illness to the child's defects is emphasized and the recent literature on this subject is brieflj' discussed. At 6 weeks of age the same infant developed meningococcus blood stream infection that localized in several large joints with- out meningitis. The infant was treated with sulfadiazine and anti-meningo- coccus serum and made an excellent recovery without any remnants of joint disturbance. The literature of meningococcus arthritis is very briefly sum- marized.
BIBLIOGRAPHY
(1) Reese, Algernon B.: Congenital cataract and other anomalies following german
measles in the mother. Am. J. Ophth., 27 : 483, 1944.
(2) Gregg, N. M.: Congenital cataract following german measles in mother. Tr.
Ophth. Soc. Australia, 3: 35, 1942.
(3) Swan, C, Tostevin, A. L., Mayo, H., and Black, G. H. Barham: Congenital defects
in infants following infectious diseases during pregnancy, with special reference to the relationship between german measles and cataract, deaf-mutism, heart disease and microcephaly, and to the period of pregnancy in which the occur- rence of rubella is followed by congenital abnormalities. M. J. Australia, 2: 201, 1943.
(4) Swan, C, Tostevin, A. L., Mayo, H., and Black, G. H. Barham: Further observa-
tions on congenital defects in infants following infectious diseases during preg- nancy, with special reference to rubella. M. J. Australia, 2: 409, 1944.
(5) ScHEiN, Albert J.: Articular manifestations of meningococci infections. Arch.
Int. Med., 62: 963, 1938.
(6) Fox, M. J., AND Gilbert, Jos. : Meningococcus infections with articular oi:)mplications.
Am. J. M. Sc., 208 : 63, 1944.
MITLTIPLE PX-RIiLENT ARTHRITIS DUE TO MEXIXGOCOCCUS
65
(7) Jaeger, H. W.: Meningocosis del aparto locomotor. Eev. chilena de pediat., 14:
414, 1942.
(8) Ca.mpbell, W., and Greenfield, .E. C: Meningococcal suppurative arthritis of
cryptogenic origin. South African M. J., 10: 545, 1936.
(9) KoBAYASHi, MiTSUGo: Ein Fall von Meningokokken Polyarthritis des Saiiglings.
Orient. J. Dis. Infants, 16: 22, 1934.
(10) Weill, DuFOVRT andBocca: M^ningococc^mie sans ^I6ningite mais avec Arthrites
purulentis multiples, chez un enfant de 17 jours, contagionn6 par sa mere. Lyon m^d. 130: 504, 1921.
(11) Erickson, Carl A.: Rubella early in pregnancy causing congenital malformations
of eyes and heart. J. Pediat., 25: 281, 1944.
HYPERINSULINISMi
SOLON S. BERNSTEIN, M.D.
The term hyperinsulinism was first employed by Seale Harris (1) in 1924 to describe a spontaneous syndrome indistinguishable from the hypoglycemic shock induced by an overdose of insulin. He hypothesized that the hypoglycemia Avith its attendant manifestations represented islet cell stimulation due to ex- cessive food intake A\hich might lead, in some instances, to insular exhaustion and diabetes. Nicholls (2) reported the first case of adenoma of the islands of Langcrhans in 1902 and Warren (3) collected 20 cases of islet cell adenoma in 1926. In no instance w as there clinical evidence of an associated hypoglycemic syndrome. Most of the reports, however, antedated the era of routine blood sugar determinations. In the following year Wilder and his associates (4) de- scribed the now classical example of carcinoma of the island of Langerhans with hepatic and lymphatic metastases associated with hyperinsulinism. Alcoholic extraction of a metastatic nodule in the liver, which bore a striking histologic resemblance to the cells of normal islet tissue, disclosed a high insulin assay and possessed the jjroiM'rty of markedly lowering blood sugar concentrations when injected into labbits. In 1929 the first reported instance of the postmortem finding of an islet cell adenoma in a patient with clinical hyperinsulinism appeared (5) and soon thereafter Howland and his associates (6) reported com- plete cure in a case of hyperinsulinism with coma and convulsions following the resection of an insular adenoma. Abundant reports of surgical cures in this condition soon followed.
Despite the growing recognition of this disease as a clinical entity, the term hyperinsulinism was often loosely applied to any form of hypoglycemia whether of hormonal, hepatogenic, central nervous or functional origin. In the absence of dependable criteria for differentiating insular from non-insular causes of hy- poglycemia, exploration was usually confined to severe or fulmmating cases or those which did not respond adequately to diet. As late as 1936 Harris (7) pleaded for the establLshment of such criteria. This period of confusion was finally terminated with "Wliipple's (8, 9) now classical triad defining the bases for the accurate diagnosis of islet cell adenoma. These criteria are: A. An un- usually low postabsorpti\-e (fasting blood sugar level, 50 mg. per cent, or less).
B. Symptomatic attacks of extreme hypoglycemia with signs of central nervous disorder, vasomotor or psychic, Avith, in some instances, coma or convulsions.
C. Dramatic and immediate recovery following the administration of glucose orally or by xein.
With adherence to these criteria Whipple (10) found tumors of the islands of Langerhans in 19 or 22 cases in which operation was performed. Similar accu- racy in diagnosis has l)een reported by many other observers. In a review of 105 cases of islet adenoma surgically removed, Whipple found only one patient with a fasting blood sugar level above 50 mg. per cent and this was 53 mg. It
1 From the services of Drs. George Baehr and J'ohn H. Garlock.
66
HYPERINSULINISM
67
is significant that in another clinic where these rigid criteria were not observed, tumors were found in only IG of 46 cases.
At the Mayo Clinic (11) the surgical selection of cases differs in minor partic- ulars from the Whipple standards. They are: a. normal health and evidence of stability of the autonomic nervous system prior to the first episode of hy- poglycemia, b. postabsorptive blood sugar levels of less than 60 mg. per cent, c. intolerance to fasting. Although it cannot be gainsaid that most instances of endocrine and hepatic hypoglycemia are excluded by the first criterion, the stigmata, in varying degree, of emotional and autonomic instability are so general that the exclusion of such cases from operation is hazardous. The case to be re- ported presents an emphatic refutation of this somewhat arbitrary criterion . The choice of 60 mg. per cent as the uppermost level, although probably dependable in the great majority of cases, has not been found necessary in Whipple's much larger series. The "intolerance to fasting" which paraphases Whipple's "postab- sorptive state" is properly emphasized. In hyperinsulinism, hypoglycemic at- tacks often appear in from 12 to 20 hours following the ingestion of food and the "fasting test" in which food is withheld for as long as 30 hours will almost in- variably precipitate an attack. A delayed breakfast, a skipped meal, anorexia, unusual physical exertion, menstruation or diarrhea may each provoke an epi- sode. In the so-called functional, emotional and "nervous" hypoglycemias symptoms usually make their appearance in from 2 to 4 hours following meals and are frequently accentuated by a high or concentrated carbohydrate diet. During attacks the blood sugar level rarely falls below 70 mg. per cent. Pro- longed fasts are, as a rule, well tolerated and postabsorptive blood sugar levels are not depressed.
Fraser and his associates (12) in an extensi\'e metabolic study of a case of hyperinsulinism, found a continuous excess of an insulin-like substance as shown by the abnormal tendency of starvation or insulin to produce an hypoglycemic attack. They recommend an insulin tolerance test after 12 hours fasting thus obviating the need for prolonged starvation. Campbell and his co-workers (13) studied the insulin concentration in several islet cell tumors and found that it ranged from 4 to 40 times that of a normal pancreas per gram but did not exceed the insulin content of the entire organ. They conclude that the uncontrolled and erratic, rather than continuous, production of insulin in an islet adenoma is the probable cause of the hypoglycemic symptoms, especially in the early stages of the disease.
In hyperinsulinism the electrocnfc]ihaloorani, as recently " cmpliasized by Wechsler and Garlock (14) presents a iaiiiy characteristic pattcin: sliort bui'sts of delta activity which become constant on hyper\('ntilation. Following the admmisti'ation of glucose the delta activity promptly disappears and the en- cephalogram becomes normal. These observers believe that the presence of an hypoglycemic pattern is a valuable diagnostic aid and that its persistence follow- ing resection of an islet cell adenoma indicates the presence of one or more residual functionuig tumors. The encephalographic jjattcrn of hyperinsulinism although indistinguishable from that of epilepsy, reverts promptly to normal following the ingestion of gluco.se.
68
SOLON S. BERNSTEIN
The presence of wide and bizarre variations in sugar tolerance curves in hyperinsulinism has led to the belief that they are not of diagnostic importance. "Wliipple (15) and others (14, IG) howcn-oi-, doscril)'' a paradoxical or diabetic pattern in many cases. On the other hand, ( 'urin (17) and later Duncan (18) in- sist that the assimilative curve can be of diagnostic value if performed under standard conditions. With the experience that a diet which has been either higli low in carbohydrate content preceding the test materially affects the gluco.vr- T(!!rranco, these authors recommend 1. the avoidance of undernutrition and 2. the oral ingestion of 250 gms. of carbohydi-ate daily for 3 days preceding the test. Under these ccMiditiuns the postabsorptive level in hyperinsulinism was found to be almost uniformly 50 mg. per cvnt or lower, the highest level thereafter rarely exceeding 120 mg. pei- cent, with a return to a subnormal level in 2 hours, wh^re it is maintained for as much as (i hours. This type of curve differs ivtdically from those found in functional ner\'ous disorders and infectious hepatitis. As an additional diagnostic feature Duncan (19) cites the ex- traordinarily rapid rate with which glucose was removed from the blood in the cases he studied.
PSYCHOSOMATIC ASPECTS
The nervous and emotional derangements frequently identified with hyper- insulinism have led to many erroneous diagnoses. In the period preceding the more geneial recognition of the disease, many cases were referred to neuro- psychiatrists, and Kepler and Moersch (20) collected 9 cases in which the early diagnoses included unexplained unconsciousness with or without convulsive featiu'es, acute confusional states, coma, alcoholism, brain tumor, hysteria, epilepsy and encephalitis.
Per.sonality and behavior alterations of variable severity are frequently obser\ ed in the presence of an islet adenoma, and may run the whole gamut of psycholosiic symptomatology. Such i)henomena have, to be sure, been noted in most hypoglycemic states, whatever the cause. Transient or prolonged pe- riods of apathy, loss of zest, amnesia and fatigue nia>' Mltcinate with more acute attacks of trcmulousness, weariness, vertigo and sweating. Wauchope (21) in 1933 described the pi-otean manifestations of hypoglycemia in detail. He linked the symptomatology to disturbances at various cerebi'al le^•els and men- tions such psychic disturbances as anxiety, negativism, irritability, querulousness, compulsions, etc. Romano and Coon (22) cite the case of a man with an islet adenoma who had recurrent attacks of confusion and bizarre behavior over a five year period which were interpreted as hysterical fugue states. During the hypoglycemic j^hase he showed uninhibited emotional behavior, incoherence, verbigeration. negati\ ism and disconnected movements. Previously inhibited aggressi^•e and sadistic tlreams were released periodically due to exhaustion of higher cerebral activity and a weakening of the ego structure. To quote Evans and McDonough (23) "hyperinsulinism has taken another syndrome from the waste basket of the neuroses." Alexander and Port is (24) in a psychosomatic study call attention to the profound inertia and relati\'e]y flat sugar tolerance
HYPERINSULINISM
69
curves found in hypoglycemic fatigue. Himwich and his co-workers (25, 20) divide the psychological characteristics of hypoglycemia into 5 progressive phases and ascribe this sequence to variable metabolic rates throughout the brain. The cortical phase is indicated by sweating, muscular relaxation, salivation, tremors and gradual clouding of consciousness. In the second or subcortico-diencephalic phase, motor restlessness and primitive movements appeal'. The mesencephalic or third phase is characterized by tonic spasm and a positive Babinski freciuently appears. A premyencephalic phase follows with tonic episodes, chiefly exten.sor, simulating that of Sherrington's decerebrate dog. In the fifth or myeleuceplialic phase there is deep coma. These stages of hypoglycemic response have been best obser\-ed during the insulin treatment of schizophrenia.
PATHOLOGY
Islet cell tumors may be divided into two groups from a functional and clinical standpoint, those that produce the characteristic syndrome of hyperinsulinism and those that do not. At least one half of the recorded instances of islet adenoma have been chance findings at autopsy and clinically inert (27). They cannot, howeA^er, be distinguished histologically from functionally active tumors. Duff (27) finds the incidence of islet adenomas with hypoglycemia somewhat higher among males than among females and predominating between the ages of 20 and 40. Xon-functioning insular adenomas are found chiefly in the later age periods. Although generally single, insular adenomas have been found to be multiple in 14 per cent of the reported cases. They are usually from 1 to 3 cm. in size but wide variations from microscopic tumors (at times indistinguish- able from huge islet cells) to adenomas 15 cm. in diameter have been described. The severity of the hypoglycemia and attendant symptoms does not bear any proportional relationship to the size of the tumor; profound coma and convul- sions have been reported in cases with a single minute adenoma. The tumors are, as a mle, discrete, sharply circumscribed and highly vascular. They pre- sent a purplish or reddish-gray color in situ and have a slightly firmer consistency than the suri ounding pale yellow acinar tissue. Fibrosis, hyalinization and even calcification can occur. They are more commonly found in the tail of the organ. Tumors hidden deep in the sub.stance o^ the head of the pancreas are not un- common and pre.sent one of the more frequent causes of operative failure. Se\ eral instances of multiple adenoma requiring 2 or more explorations before a complete cure could be achieved have been reported (9).
The histologic arrangement of the islet cell adenoma consists of undulating and anastomosing ribbon-like strands of cells and closely resembles the morphol- ogy of normal or hyperplastic islands of Langerhans (fig.l). Some minor devia- tions have been recorded (27). The adenoma cells contain in varying proportion granules which stain like those of normal beta cells. Although beta cells have been held responsible for the elaboration of in.sulin (28) they ha\'e also been demonstrated in apparently inert adenomas (29).
Frantz (30) (table 1) in a series of comprehensive re^•iews of all the published
70
SOLON S. BERNSTEIN
cases of hyperinsulinism to date, divides the tumors histologically into a. benign adenomata with well defined capsules, b. tumors with ill-defined capsules and questionable infiltration into the acinar tissue, c. malignant tumors with or without metastases, d. multiple microscopic and gross adenomatosis with, in some instances, infiltrative characteristics and e. a group of 11, somewhat ill- defined cases where in the pathologist's opinion there is merely hypertrophy and hyperplasia of the islets without neoplasia. Earlier observers were inclined to interpret the so-called hyperplastic group as analagous to the hypertrophic histologic picture seen in Graves' disease.
In another recent clinicopathologic study Frantz (31) describes several ex- amples of islet cell adenomatosis associated with hyperinsulinism. The tumors
Fig. 1. Islet cell adenoma of pancreas
are small, usually multiple, at times numerous and are diffusely scattered throughout the pancreas. They arc often characterized merely by hyperplasia and hypertrophy of islet ti.s.sue. It is probable that the 11 reported instances of "hyperinsulinism with functional hyi)eri)lasia of insular tissue" belong to this group. The extreme insular hyp(M])lasia r;u( l>- ()l)scr\( (l in infants of diabetic mothers is presunial)ly due to increased maternal demands. In most instances following a variable period of hypoglycemia the carl:)ohydrate mechanism tends to re^'ert to normal. I'ollowing partial pancreatectomy similar insular hyper- plasia has been obser\-ed in the remaining pancreas.
Histologic differentiation l:)etween benign and malignant islet tumors is often difficult, sometimes impossible (31, 32). In Frantz' group (28), however, none of the 15 cases of so-called malignancy have shown any tendency to recurrence for periods of one to twelve years following resection. Other recent obsei-vers
HYPERINSULINISM
71
share the impression that the morphologic picture of neoplasia or invasiveness is not clinically ominous and that the only true evidence of malignancy is the presence of metastasis. Frantz further states that in the group of questionable tumors "ihe suspicion of the pathologist, not the surgeon, has yet to be confinned in a single case by follow-up data." She also believes that in the multicentric cases, radical resection of the pancreas is indicated both because of the possibility of malignant disease and the danger of islet cell hyperplasia and neoplasia in the remaining pancreas with a return of hypoglycemia.
TABLE 1
Summary of slatislics (from Frantz)
|
SINGLE |
MULTIPIE |
TOTAL |
MULTIPLE |
|
|
Tumors removed at operation and considered benign . Tumors found at autopsy and considered benign |
65 26 |
11 4 |
76 30 |
|
|
Total benign tumors |
91 |
15 |
106 |
14.2 |
|
Tumors removed at operation and suspected malig- |
22 |
4 |
26 |
|
|
Tumors removed at autopsy and suspected malig- nant |
2 |
0 |
2 |
|
|
Total suspicious tumors |
24 |
4 |
28 |
14.3 |
|
Total cases of tumor without proven malignancy Carcinoma with metastases, -proven malignancy |
115 12 |
19 3 |
134 15 |
14.2 20.0 |
|
Total Cases of True Neoplasm |
127 |
22 |
149 |
14.8 |
|
Hj'pertrophy and hyperplasia without neoplasia |
11 |
Extrapancreatic islet cell adenomas have been described but they rarely pro- duce symptoms. Bell and his associates (33) report a cure of hyperinsulinism following the removal of an islet tumor which was almost completely extrapan- creatic, lying adjacent to the inferior border of the tail of the pancreas.
Cerebral changes in hyperinsulinism: The need for early recognition and prompt resection in cases of suspected in.sular adenoma is of especial importance in in- dividuals where if operation is ^vithheld, irreversible cerebral changes may occur. Kerwin (34) cites a case of abrupt onset with violent convulsions; coma ensued after 8 days despite the intravenous administration of glucose, with death 16 days after the onset of symptoms. Multiple pancreatic adenomas were found at necropsy with cerebral congestion, edema, hemorrhages, neural degeneration and gliosis. Malamud and Grosh (16) describe a case of islet adenoma A\dth convulsions and psychotic manifestations which was correlated with advancing destruction of the cerebral cortex and basal ganglia. The diffuse degenera-
72
SOLOX S. BERNSTEIN
tive process in the brain was ascribed to the direct toxic effect of insulin on the parenchyma. Moersch and Kernohan (35) describe the pathologic changes in 2 cases; although some petechial hemorihages in the pons were noted in one case, the most striking change in Ijoth instances was degeneration of the n('r\(> cells. They stress the narrow margin between reparable and irreparable damage in both insulin induced shock and spontaneous hypoglj-cemia.
SURGICAL ASPECTS
In the exploration of islet cell tumors careful inspection and palpation of the ■adequatel}^ exposed tail, body and head of the pancreas is essential. Mobiliza- tion of the duodenum to the left will expose the posterior surface of the head. \'\Tiipple (9) found 3 tumors in this area in patients who had previouslj' been unsuccessfully explored. The possibility of multiple tumors must always be borne in mind. If no adenoma is found, subtotal or total resection of the pan- creas must be considered. David (36) collected 17 cases in the literature in which subtotal procediu-es were followed by fa^•orable results and recommends removal of almost the entire pancreas (leaving only a small collar of the gland within the second portion of the duodenum) to insure a satisfactory result. This, it is hoped, would succeed in removing sufficient hypersecreting islet tissue as well as small adenomas otherwise overlooked.
Priestley and his co-workers (37) have recently described the first case of total pancreactectomy for an islet cell adenorna which was located only after serial section of the excised gland, deep in the substance of the head of the pancreas. The tumor measured 8x5x5 mm., one of the smallest on record. The surgical procedure was ciuite extensive and, because of the inadequate blood supply to the duodenum, a partial gastrecton\y with removal of the first and second por- tions of the duodenum was required; a cholecystgastrostomy was done to establish internal biliary drainage. The patient is living and well 10 months after operation. The diabetes that supervened was surprisingly mild, requiring the daily use of only 16 units of insulin. No disturbance of carbohydrate diges- tion was detected but there was distinct diminution in the digestion of protein and fats. The unexpectedly small insulin requirement following total pan- createctomy has been further substantiated in 2 cases by Goldner and Clark (38). Blood sugar levels between 90 and 200 mg. per cent were maintained postoperatively with but minimal supplementary insulin. Both patients showed considerable insulin sensitivity, going promptly into hypoglycemia if the insulin taken was not adecjuately "covered" with carboh.ydrate. From the evidence of these 3 cases there is a strong likelihood that the insulin requirement of the depan- creatized man is less than in many diabetics. This arouses speculation as to the existence of an insulin inhibiting mechanism, as well as a lack of insulin, in the causation of diabetes (39).
We have recently observed a case, reported by Umansky (40), with persistent hypoglycemia following subtotal resection of the pancreas. As the resected gland was found to be histologically normal, the existence of an islet cell adenoma in the small unresected portion of the head of the pancreas is presump- tive. A total pancreatectomy will probably be required to effect permanent cure.
HYPEHINSULINISM
73
Diet in hypcrinsulinism: Dietary control of hypcrinsulinism is, at best, pal- liative and the symptomatic relief thus secured should not permit the postpone- ment of operative cure. A high fat-high protein-low carbohydrate regimen with frequent feedings will in most instances prevent hypoglycemic attacks in the interval between diagnosis and operation. Prolonged dependence on the varied dietary regimes with which the literature abounds may induce a false sense of security and thus seriously compromise the outcome. There appears to be little virtue in other non-operative measures ^^•hich have from time to time been rec- ommended. These included the use of small doses of insulin, anterior pituitary, adrenal cortical hormone, thyroxin, Kendall's carbohydrate-stimulating factor E, epinephrin, ephedrin, bromides and the barbiturates.
Alloxan, the ureide of mesoxalic acid, has recently been employed in a case of carcinoma of the islet cells with multiple hepatic metastases (41). Based on the knowledge of its known necrotizing eifect on the islands of Langerhans in the rabbit and dog, it was hoped that carcinomatous islet tissue in the human would be similarly destroyed. Temporary, and not cleai-ly explained, relief folloAved its use in one case, but autopsy failed to disclose any necrosis in the malignant islet cells.
CASE UKPOKT
M. B., (Adm. *517713), aged 29 years, white married pianist had been in good health until July 29, 1943 when she retired at 11 p.m. and continued to sleep until 4:30 the follow- ing afternoon despite all efforts to awaken her. When finally aroused, she was bewildered and disoriented, perspiring profusely; her speech was thick and she complained of vertigo and diplopia. After eating breakfast, recovery was prompt. For the next three weeks, however, there was persistent drowsiness and she could rarely be awakened before 1 p.m. There were no convulsive seizures. Although conscious of the overpowering nature of the lethargy she found it impossible to arouse herself. Similar episodes would occur when a meal was inadvertently skipped. She soon learned the efficacy of sweetened drinks in preventing and controlling her symptoms and if awakened during the night to drink orange juice fortified with sugar, the morning attacks would be averted.
Her own description of her symptoms is of interest: "For several successive days I would be in a semiconscious state before arising. During these periods, which would last for 10 minutes to as much as 5 hours, I was told that I answered (luestions and would even eat breakfast without the slightest knowledge of having done so. At other times I was told that every possible attempt to awaken me would be futile, particularly if I clenched my teeth and refused food; the attacks would then last much longer. Shortly after eating I would become vaguely aware of my surroundings and on fully awakening would demand to know the date and hour. Early in my illness I would frequently awaken at night in a profound daze and find myself incapable of consecutive thought. I also found it im- possible to organize my daily routine. 1 w <juld f.ill :isl( ( p while reading letters, my fingers would fail to find the piano keys and I w as uiiablc to concentrate on reading. During the latter part of the day, although I never lost con.sciousncss, I had frequent sensations of vagueness, listlessness, unreality and total e.xhaustif)ii , sometimes associated with drench- ing perspiration which were relieved as soon as I took .sweet or fatty foods; relief would then last for an hour or two when the "haze" would return. I was fully aware of my grow- ing apathy, dullness and stupidity. Even when I was jjlaccd on a high fat diet with fre- quent meals these sensations, though milder and more transient, did n<it entirely disappear."
The clinical picture was at first interpreted as a ijrofound i)sycliasthenia which had been precipitated by separation from her parents, hard work and the prolonged, intense heat of the city of Washington, where she resided. She had been emotionally unstable,
74
SOLON S. BERNSTEIX
introspcctive and easily frightened since early childhood; trivial annoyances would loom large and fresh situations precipitated intense anxiety. She had developed an obsessional fear of cats and would fly into a panic at the mere sight of one. A moderate hypochondri- acal tendency had appeared in the last year.
She was admitted to the George Washington University Hospital in Washington, D. C. on August 23, 1943. Physical examination there was negative in every detail. On the regular hospital diet and bed rest she had no discomfort until August 27th, at 9 a.m. when she was found to be semicomatose and perspiring profusely. She was aroused with dif- ficulty and resisted examination; her responses were sluggish and inaccurate; speech was slurred, her expression stupid and she was unable to focus adequately; her gait was stag- gering. The blood sugar level during the attack fell to 37 mg. per cent. On the following morning she had a similar episode with inability to speak, expressionless facies and involun- tary movements of the right angle of the mouth. A "fasting test" was performed on August 30th and when breakfast was withheld until 9 a.m. she suddenly became dazed, was drenched in perspiration and unable to speak; within two minutes after the intravenous administration of 40 cc. of 50 per cent glucose she was fully alert, inquired the cause of the commotion and fell to eating her breakfast voraciously. On September 3rd she was found deeply comatose at 9 a.m.; there was marked twitching of the left corner of the mouth; the blood sugar level was found to be 10 mg. per cent. Response to intravenous dextrose was almost immediate. On the next day she automatically drank orange juice while in a semi-stupor with prompt response and no memory of the episode.
Laboratory investigations: Fasting bh)od sugar levels were successively 37, 28, 43, 36 and 45 mg. per cent. A sugar tolerance test with a fasting figure of 28 mg. showed, after the ingestion of 100 gm. of glucose, hourly figures of 36, 47, 52 and 59 mg. per cent thereafter. The hemoglobin was 95 per cent, the leukocytes numbered 10,150 and the differential count was normal. The spinal fluid was clear, under normal pressure, with adequate rise on jugular compression; total protein, 0.22 cm. per liter, globulin was absent. A skull roent- genogram was negative and the sella turcica normal. The urine was normal; specific gravity 1.019.
A high fat and protein diet with frequent feedings both day and night was instituted and the hypoglycemic episodes disappeared. At follow-up on November 30, 1943 the blood sugar level was 86 mg. per cent.
Although the major hypoglycemic episodes had been adequately controlled by the high fat-frequent feeding regimen, she complained of extreme fatigue, absent initiative, aim- lessness, tremulousness, inability to concentrate and an oppressive sensation which she described as a "veil" over her head. The liigh fat diet had induced a degree of obesity which she felt was both cumbersome ami unsightly. She was admitted for further study to The M<iunt Sinai Hospitnl on March 14, 1944.
Exaiiii initmii : Tlic p.iticiil was listless and her responses were sluggish. The ocular fundi wen negative. There was slight areolar hypertrichosis and the left breast was distinctly larger than the right. The systolic pressure was 134 mm. of mercury and the diastolic, 72. The muscular and neurologic status were negative. Despite her languor she was intelligent and cooperative.
Course: She was given the regular hospital diet and on the day following admission she was lethargic before breakfast but was perfectly well 20 minutes after eating; the fasting blood sugar level was 50 mg. per cent. On March 17th breakfast was delayed because of a gastrointestinal roentgen series and she had a seizure characterized by weakness, hunger and sweating. On March 21 food was purposely withheld for 18 hours and at 12:30 p.m. she became apprehensive, irritable and complained of vertigo and diplopia; articulation was clear and orientation good. The blood pressure was 150 mm. of mercury systolic and 100 diastolic. After drinking orange juice with sugar her symptoms vanished. On March 23 she experienced a very severe hypoglycemic attack at 5:30 a.m. with profound lethargy during which the blood sugar reached the level of 10 mg. per cent. The episode was fol- lowed by retrograde amnesia.
HYPEUINSULINISM
75
Laboratory Data: Fasting venous blood sugar figures were successively 50, 42, 50, 30, 10 and 34 mg. per cent. A sugar tolerance test with a fasting figure of 50 mg. per cent showed, following the oral ingestion of 100 gms. of glucose, 100, 200, 240, 240 mg. per cent at hourly intervals respectively ; at the expiration of 4 hours there was 3.3 per cent sugar in the urine. Other tests disclosed: total blood protein, 7.9 mg. per cent; chlorides 1060 and sodium, 146.0 milliequivalents per liter; cephalin fiocculation, 2 plus; icteric index, 6; lactose toler- ance test, normal; hemoglobin 93 per cent, leukocytes 5,700; basal metabolism, minus 17 per cent. Gastrointestinal roentgenograms disclosed no abnormality; the gall bladder, however, did not visualize adequately; an electroencephalogram taken when the patient was fasting but not in hypoglycemic shock gave a normal record with bursts of 3 per cent activity following hyperventilation.
Fig. 2. Islet cell adenoma with hyaline changes
It was felt that the recurrent episodes of hypoglyceinic sliock as.sociated with blood sugar levels of 10 to 50 mg. per cent appearing with some regularity from 12 to 18 hours following the ingestion of food indicated the presence of an islet cell adenoma with hyper- insulinism. Laboratorj^ and clinical studies had e.xcluded such causes of hypoglycemia as hepatic disease, organic disease of the nervous system, Addison's disease, myxedema and Simmond's disease. The possibility of a spontaneous hypoglycemia as a manifestation of her emotional instability was deemed hardly tenable. Exploration was therefore advised.
Operation: On March 29, 1944 a laparotomy (Dr. John H. Garlock) was performed under cyclopropane anesthesia after pre-operative preparation with 5 per cent intravenous glucose. Through an upper abdominal transverse incision the lesser sac was entered by cutting through the gastrocolic ligament. A careful exploration was made of the body and tail of the pancreas but no adenoma was found. This ixi|uired mobilization of the organ from below upward. Most of the head of the i);i?iri(