NUTRITIONAL SUPPORT IN THE SERIOUSLY ILL PATIENT i

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JOSEF E. FISCHER, M.D.

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TABLE

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DIAGNOSTIC TOOLS FOR THE STUDY OF ESOPHAGEAL DISEASE .

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Roentgenographic Examination . . . . . . . Esophagoscopy . . . . . . . . . . . . Motility Studies . . . . . . . . . . . E l e c t r i c a l Ac*Avity . . . . . . . . . . . BENIGN LESIONS

ASSOCIATED

WITH M O T O R DISORDERS

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D i s o r d e r s of P h a r y n g o e s o p h a g e a l S p h i n c t e r , I n c l u d i n g Pharyngoesophageal Diverticulum . . . . Traction Diverticul~ . . . . . . . . . . . P u l s i o n o r E p i p h r e n i c D i v e r t i c u l a of t h e L o w e r T h i r d of t h e E s o p h a g u s . . . . . . . . A c h a l a s i a of t h e E s o p h a g u s . . . . . . . . T H E SURGHCAL DISEASES OF THE ESOPHAGUS OF CO~GENITAL

ORIGIN

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Embryology . . . . . . . . . . . . . Tracheoesophageal Fistula and Esophageal Atresia . Esophageal Atresia without Tracheoesophageal Fistula Tracheoesophageal Fistula without Esophageal Atresia . . . . . . . . . Congenital Esophageal Stenosis . . . . . . . D u p l i c a t i o n s of t h e E s o p h a g u s . . . . . . . Chalasia . . . . . . . . . . . . . . BENIGN TUMORS OF THE ESOPHAGUS

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Epithelial Tumors . . . . . Nonepithelial Tumors . . . . Other Mesenchyma! Tumors . . . Heterotopic Tumors . . . . . . S u r g i c a l T r e a t m e n t of B e n i a ~ T u m o r s PERFORATION OF T H E ESOPHAGUS

CHEMICAL BURNS O F MALLORY-WEISS INFLAMMATORY

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SYNDROME DISEASES

ESOPHAGUS . OF,THE

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T u b e r c u l o s i s of t h e E s o p h a g u s . . . . . . M y c o t i c I n f e c t i o n of t h e E s o p h a g u s . . . . . . Primary Esophageal Moniiiasis . :. . . . . A c t i n o m y c o s i s of t h e E s o p h a g u s . . . . . Regional Esophagitis . . . . . .

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QUESTIONS OF CLINICAL USEFULNESS ANSWERED IN THIS ISSUE

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Enumerate the various abnormalities of the esophagus that may be delineated on barium swallow, p. 8 List the advantages and disadvantages of the fiberoptic esophagoscope, p. 9 List the basic equipment necessary for the performance of esophageal motility studies, p. 9 List the information that may be obtained from esophageal motility studies, p. 10 List the diseases in which esophageal motility studies are t h e most useful, p. 10 What are the factors that limit the usefulness of electromyographic studies of the esophagus? p. 11 Describe the cricopharyngeus muscle, p. 12 Classify dysfunctions of the cricopharyngeus muscle, p. 12 Describe the principal manometric abnormality that is found in Zenker's diverticulum, p. 13. Describe the procedure of cricopharyngomyotomy, p. 13 List and describe the usual locations of esophageal diverticulae. pp. 15-17 List the various types of surgical procedures that have been used in epiphrenic diverticula, p. 17 List the principal abnormalities in achalasia, of the esophagus. p. 17 Describe the most recent concept of the autonomic inervation of the esopha~s, p:: 18 L i s t t h e diagnostic procedures that should be carried out in patients suspected of having achalasia !of the ~esophagus. p. 20

16. Describe the operative procedure of esophagocardiomyotomy and list the advantages and: disadvantages of a ' thoraCic versus an abdominal approach: p p . 22-26 17. What is the prognosis following esophagocardiomyotomy? pp. 26 and27 3

18. What are the causes of unsatisfactory results? pp. 27 and 28 19. What is the relationship of the origin of the trachea and the esophagus embryologically? p. 28 20. What is the most common congenital anomaly of the esophagus? p. 29 21. What prenatal condition frequently is associated with congenital obstruction of the gastrointestinal tract? p. 30 22. What is the usual symptomatology of a patient with esophageal atresia? p. 30 23. How soon after birth can esophageal atresia be diagnosed and by what means? p. 30 24. What is the most significant factor in the development of pneumonia with esophageal atresia and tracheoesophageal fistula? p. 30 25. What steps should be taken in treating esophageal atresia with tracheoesophageal fistula? p. 32 26. What are the advantages and disadvantages of the transpleural and retropleural approach to the treatment of tracheoesophageal fistula with atresia? p. 32 27. What standard methods of esophageal anastomosis generally are employed? p° 32 28. What is the survival rate of patients with esophageal atresia and tracheoesophageal fistula and what are the principal causes of postoperative death? p. 33 29. Do all demonstrable anastomotic strictures need to be treated? p. 33 30. Under what circumstances is a staged procedure considered for treatment of esophageal atresia with tracheoesophageal fistula? p. 33 31. How can esophageal atresia without a tracheoesophageal fistula be simply differentiated from one with a tracheoesophagealfistula? p. 34 82. Why should an infant with gastrostomy feedings be fed orally as well? p. 34 33. If a primary anastomosis cannot be performed, how is the continuity of the esophagus best:achieved? pp. 3 4 and 35 34. What is the usual treatment of the " H " type congenital tracheoesophageal fistula? p. 38 35. What is the usual treatment necessary for congenital esophageal stenosis? p. 38 36. What are the complications of duplications of the esophagus? p. 39 4

37, What is chalasia and how is it treated? pp. 39-40 38. Classify benign tumors of the esophagus, p. 40 39, Describe the usual method of presentation of the benign esophageal tumor, pp, 40-41 40, Describe the histology of the adenomatous polyp, p. 41 41, Whm~, is the cause of acquired cyst of the esophagus? p. 41 42. What are possible etiologic factors in leiomyoma of the esophagus? p, 42 43. What are the risks of biopsy of benign tumors of the esophagus and when are biopsies absolutely contraindicated? p. 44 44, What are the indications for esophageal resection in ]eiomyoma of the esophagus? p. 45 45, Who described the first case of rupture of the esophagus? p, 45

46, What is the current mortality rate for esophageal perforation? p. 45 47. Give a method of classification for esophageal perforation. pp. 46 and 47' 48. Describe the inherent anatomic weakness in a normal esophagus, p, 48 49, What is the physiopathology of the mediastinitis associated with perforated esophagus? p. 49 50, What is the natural history of spontaneous rupture of the esophagus with and without treatment? p. 50 51. What is the value of thoracent~sis in diagnosis of perforation of the esophagus and what is the most important diagnostic clue? pp. 50-51 52, Describe the radiologic signs of esophageal perforation, p. 51 53. Outline the management program for perforation of the esophagus according to the anatomic site. pp. 51-52 54, How do you detect minute esophageal~perforation intraoperatively? p. 52 55. What are the operative methods and technics of repair of large perforation of the esophagus?p. 52 56. What concentration of solution of sodium hydroxide is sufficient to produce experimental corrosive esophagitis? p. 54 57, What is the physiopathology of co~Tosive burns of the esophagus? p. 54 58, What are the indications for esophagoscopy in chemical burns of the esophagus? DeScribe the hazards :and limitations, p. 54 5

59. Describe the early and late complications of chemical burns of the esophagus, p. 54 60. What are BAPN and penicillamine and what is their role in prevention of esophageal stricture? p. 57 61. Is the risk of carcinoma developing on an old esophageal burn a real one? p. 58 62. Who were Mallory and Weiss, what is their syndrome and how does it differ from Boerhaave syndrome? p. 60 63. Does the Mallory-Weiss syndrome occur only in an alcoholic patient? p, 60 64. Do you diagnose the Mallory-Weiss syndrome by esophagogram, esophagoscopy or by other methods? p. 60 65. What i s the minimal bleeding rate that may b ~ e c o g n i z e d in arteriography? p. 61 66. Outline the methods of treatment for the Mallory-Weiss syndrome and give the indications for surgical intervention. pp. 61 a n d 62

67. What is the pathogenesis of esophageal tuberculosis and what histologic types of esophageal tuberculosis are indications for surgical intervention? pp. 63 and 64 68. Is monfliasis of the esophagus a common entity and what are the associated conditions that promote the development of esophageal moniliasis? pp, 63 and 64 69. What is the most common symptom of moniliasis? Describe the typical esophagogram for esophageal candidiasis, p. 64

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is Professor "of Surgery in the Faculty of Medicine at the University of Pennsy]vania and Surgeon-in-Chief at the Graduate School of the same institution. Doctor N e m i r received his M.D. degree from the University of Texas in 1944, :followed by postgraduate training at the Hospital of the University of Pennsylvania. His research interests include the enzymic digestion of.blood, pulmonalT function and pulmonary embolism, and vascular prosthetics.

is Associate Professor of Surgery at the University of Pennsylvania School of Medicine. Doctor Wallace received his M.D. degree from the Tufts University School of Medicine. H e took b-oth~an internship and a residency in general surgery at the Tufts New England Medical Center, followed by thoracic and cardiovascular training at the M o u n t Sinai Hospital in New York. Doctor Wallace's clinical and research interests are in the field of thoracic a n d cardiovascular disease.

is Assistant Professor of Surgery at the University of Pennsylvania. After completion of his basic medical education in Tehran, he took a surgical i:esidency a t the University of Pennsylvania. Doctor Fallahnejad has served as Senior Registrar at the Hawkmbre Chest Hospital-in Devon, England. His primary clinical and research interests are in thoracic and cardiovascular surgery.

DIAGNOSTIC TOOLS FOR THE STUDY OF ESOPHAGEAL DISEASE T H E ]ESOPHAGUS is a muscular, tube-like structure generally flattened anteroposteriorly, measuring approximately :25 cm in length. It extends from the lower border of the cricopharyngeusmuscle, at about the~level of the 6th cervical vertebra, downward and t h r o u g h - t h e d i a p h r a g m to join the cardiac portion of t h e stomach. I t s principal function is to transport food and liquid from the pharynx to t h e stomach d u r i n g t h e third stage of deglutition. Participation in t h e act: of swallowing is t h e single most important kinetic function of this organ, and dysphagia is the major manifestation of esophageal disease. However, despite its r a t h e r straightforward role, its singularity of function in the swallowing act and its rea-

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sonable accessibility to diagnostic study by methods long used, the esophagus continues to p r e s e n t problems in diagnosis and m a n a g e m e n . It is not our purpose: to describe current diagnostic technics i n great detail, but rather to emphasize those aspects that w i l l improve clinical diagnosis or influence management.

ROENTGENOGRAPI~IC EXAMINATION A rapid and thorough assessment of the lumen of t h e esophagus is possible by means of a roentgenologic contrast study. T h e experienced roentgenologist can quickly evaluate the distensibility ~of the organ and the presence of outpouchings or filling defects of the lumen, a s well as displacement o f the organ, mucosal pattern, motor activity and competency of the esophagogastric junction. Our radiologists stress the importance of clearly delineating the distensibility of the organ and of varying the ingestion mixtures. For example, a thick barium m i x t u r e is essential for coating the esophageal mucosa w h e n moniliasis or esophageal varices is suspected. T h e difficulties of examining the hugely dilated organ in the patient with far-advanced acha-

Fig. 1. Severe achalasia of esophagus. The hugely dilated organ has a sigmoidal configuration. The difficulties on roentgen study are apparent. Careful lavage and removal of food debris is essential prior to study.

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lasia have been stressed. 77 A large growth may be overlooked on esophagography in an esophagus that is dilated and partly filled with retained food (Fig. 1). These patients should be thoroughly prepared for several days prior to study, with esophageal lavage through a large-bore tube and a liquid diet. Cytologic study of the secretions may be invaluable in these unusual cases. T h e radiologists at the Graduate Hospital of the University of Pennsylvania have for m a n y years placed great reliance on the response to ingested Seidlitz powders in studying narrowed areas in the lower esophagus and esophagogastric junction. On administration of Seidlitz powder, which is mixed in w a t e r and swallowed immediately, 5 0 ~ or more of the retained contrast material.will immediately e m p t y into the stomach. T h e sudden release of carbon dioxide will distend the functionally narrowed segment. T h i s does not occur with organic stricture. This reaction may be dramatically illustrated in cine studies and is pathognomonic of achalasia.

ESOPHAGOSCOPY T h e fiberoptic endoscopic instruments have allowed for a more thorough visualization of the esophagus with less risk of perforation. S y m p t o m s of dysphagia, regurgitation of food, hematemesis, retrosternal burning pain or a history of swallowing of a foreign body are indications for esophagoscopy. Marked deformity of the cervical spine due to arthritis or m a r k e d kyphosis may m a k e esophagoscopy impossible or limit the extent o f the examination to the upper part of the organ. Patients with thoracic aneurysms should not be endoscoped. Although the fiberoptic i n s t r u m e n t is used almost routinely in diagnosis and in biopsy, the rigid instr~lment still is used in those instances in which an open lumen is needed, as in dilatations or the passage of filiforms or strings. Occasionally the rigid instrum e n t has been necessary in order to obtain an adequate biopsy specimen. MOTmITY STUDIF_,S Although esophageal motility studies were first carried out in 1883 by Kronecker and Meltzer, the real forward t h r u s t in this method of investigation h a s occurred over the p a s t 20-25 years. TM While there is some difference of opinion with respect to the value of routine manometric study of the esophagus i n diseased states, sl there is increasing evidence that valuable information m a y ~be obtained that is not forthcoming from the m o s t t h o r o u g h and s k i l l e d roentgen a n d cinefluorographic study :or from endoscopy: Motility studies, o r i g i n a l l y of p r i m a r y physiologic interest, were instrumental in t h e study of the pathophysiology of c e r t a i n disease states. T h e y now have become useful in clinical medicine, n° not: onIy i n diagnosis b u t in t h e :selection and actual perf0rmance of certain surgical procedures. T h e equipment is not prohibitively expensive nor are the studies unduly difficult to perform. Moreover, p r e s s u r e transducers and muItichannel recorders a r e available i n m a n y hospitals for evaluation o f cardiopulmonary problems •and can be u s e d for t h e selected patient i n Whom m0tilitystUdies are desired. T h e system now widely used is to sense t h e peripheral pressure through water-filled s y s t e m s with open-ended:polyethylene: tubes. ~The orifice may be covered with a balloon, or the:liquid surface a t : t h e orifice

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m a y be continuously perfused. T u b i n g is connected to a sensitive p r e s s u r e t r a n s d u c e r external to the patient. W e routinely use S t a t h a m strain-gauge m a n o m e t e r s . T h e various methodologies, e q u i p m e n t a n d technics are a m ply documentedA 41. 16s Silber169 has believed t h a t balloons should not be used, since t h e y a c t as a f o r e i g n body in contact with the mucosa and, therefore, as a s t i m u l a n t to smooth muscle contraction. H e also believed t h a t t h e size, s h a p e a n d volume of the balloon m i g h t cause inaccuracies in p r e s s u r e and motility recordings. F o r this reason, he has favored the openended catheter, since absolute pressures m a y be m e a s u r e d in this m a n n e r . I n o u r early studies, ls2 we used a triple balloon m e t h o d a n d have believed t h a t this more closely simulated n a t u r a l events occurring during deglutition. It m u s t be pointed out t h a t it is necessary to record respiratory movem e n t s by m e a n s of a p n e u m o g r a p h s i m u l t a n e o u s l y with the i n t r a l u m i n a l pressures, so t h a t the true motor p h e n o m e n o n of t h e esophagus c a n be s e p a r a t e d from m o v e m e n t s resulting from inspiration a n d expiration a n d for a c c u r a t e delineation of the point of respiratory reversal. A m y o g r a p h or o t h e r recording device for the neck muscles is useful to signal the volunt a r y initiation of the act of deglutition, b u t this is not absolutely necessary for m o s t clinical studies. M a n o m e t r y provides the clinician with s t u d y of the r h y t h m i c i t y , a m p l i t u d e and d u r a t i o n of contractions at various levels of the esophagus. T h e e x t e n t of involvement of the esophagus in a m o t o r a b n o r m a l i t y m a y be obtained. This i n f o r m a t i o n is vital in differentiating between advanced scleroderrnic disease of the esophagus a n d achalasia, p a r t i c u l a r l y if t h e r e is no sclerodermic involvement of the skin. In scleroderma, the contractions of the u p p e r few centimeters of t h e esophagus are peristaltic a n d are not involved in t h e sclerodermic process 143 a n d there is no reaction to M e c h o l y l J 69 On the o t h e r hand, pressure waves t h r o u g h o u t the esophagus a r e involved in achalasia. An estimate of the a m p l i t u d e of esophageal contractions m a y help to p i n p o i n t the site of origin of pain in the chest. Excessive p r e s s u r e s in the s p h i n c t e r can be p r e s e n t a n d produce a functional obstruction a n d p a i n f u l dysphagia. Inability of the s p h i n c t e r to relax a n d p r e m a t u r e or excessive contractility after swallowing m a y also be seen. T r e a t m e n t m a y be guided m o r e intelligently w h e n i n f o r m a t i o n of this type is available. A n increase in the d u r a t i o n of contractions is helpful in reaching a diagnosis of diffuse spasm, since in this condition some of the contractions are prolonged. T h e m a n n e r in which disordered contractions m a y produce a t e m p o r a r y o b s t r u c t i o n have been elucidated p r i m a r i l y by study of p r e s s u r e records. 58 In some cases, motility studies will be the only m e t h o d t h a t will d e m o n s t r a t e the findings of diffuse spasm. This t y p e of spasm does not respond t o Mecholyl b u t relaxes with t h e a d m i n i s t r a t i o n of a n antispasmodic drug. 169 M o t i l i t y studies a r e essential in t h e d i a g n o s i s of achalasia of the esophagus a n d in d i f f e r e n t i a t i n g this condition from organic stricture. A l t h o u g h some a u t h o r s have b e l i e v e d that: the studies have been helpful i n - b e n i g n a n d m a l i g n a n t tumors, locating diverticuli a n d in d e l i n e a t i n g webs a n d rings of various types, s6 this has not b e e n a general view. T h e r e is increasing evidence t h a t esophageal m o t i l i t y studies are import a n t in l t h e assessment of p a t i e n t s w i t h h i a t u s h e r n i a preoperatively, 145 d u r i n g o p e r a t i o n 9~ a n d postoperatively. 4s M e a s u r e m e n t s of p H changes P',-°s. so are being used with greater frequency in p a t i e n t s w i t h reflux esophagitis and, although of limited useful. 10

ness in the diseases under discussion, should be an integral part of a thorough diagnostic work-up. ELECTRICAL ACTIVITY Recently there has been considerable interest in the study of the electrical activity of the musculature of the esophagus and its sphincters and it m a y be possible in this manner to have a more precise means of measuring the motor action and thus enhancing our understanding of disease states. Hellemans and his group sz have carried out elegant electromyographic and motility studies on the ~canine esophagus in the normal unanesthetized animals using two platinum needles embedded in glass-filled epoxy plates at a distance of 2 turn. The electromyographic tracings of the normal canine esophagus and gastroesophageal sphincter closely resembled the electromyographic recordings of the skeletal muscle contractions of progressively increasing and decreasing strength. They found the correlation between spike activity and pressure changes to be fairly good in the esophagus proper. O n the other hand, electrical activity and manometric data did not correlate well in a normal gastroesophageal sphincter. A specificelectrical activity could not be detected in the resting gastroesophageal sphincter with the technics used by them. There was no electromyographic evidence that atonic contraction of the circular muscle coat or the contraction of alternating units of this muscle layer is the cause of the high resting pressure in the sphincter. G o o d m a n and his group 70 have recorded the electrical potentials in the normal h u m a n esophagus and in patients with hiatus hbrnia. The slow electrical wave reported by them, which develops synchronously with deglutitive pressure complexes, was not observed by Hellemans in the cat. T h e difficulties with mucosal surface electrodes experienced by G o o d m a n and his group were apparent. W e have had similar problems. Although the electromyograrn can be recorded acutely with these implanted electrodes, it does not appear that they will be satisfactory with chronic preparations because of the tissue reaction and foreign body granulomata. W e have carried out electromyographic and pressure tracings in dogs with achalasia. Under anesthesia, we were unable to observe any ~lifference in tonic (resting) electrical and mechanical activity between normal and achalasic dogs. In addition, we were unable to record anything that resembled a regular peristaltic wave of electricalor mechanical activity. It was not possible to lighten the anesthesia sufficiently to obt~iin swallowing movements. In view of the work of Besancon, 26 it is likely that the most promising role for combined electromyographic and pressure studies will be in conjunction with drug studies, especially the adrenergic and cholinergic stimulating, and inhibiting agents. In subsequent studies by I-Iellemans and his group, ss comparisons were m a d e between the electrical activity in t h e lower half of the c a t and monkey esophagus (where there is a distribution of striated and smooth muscle similar to that found in the human) and the dog. They found the following: (1) As in the dog, a specific electrical activity could not be detected in the gastroesophageal s p h i n c t e r of cats and monkeys. (2) As in t h e dog, there was n o electromyograpHi'c evidence to show that atonic contraction of the circular muscle coat or the contraction of alternating functioning units of this mtrscle layer is the cause of the high resting sphincteric pressure normally obser~.ed. (3) In the smooth m u s c l e portion of the monkey esophagus, deglutition resulted in a burst of electrical activity that was 11

quite different from t h a t observed in striated esophageal muscle. T h e number of spikes in a burst of electrical activity was m a r k e d l y smaller, duration of the action potentials was m u c h longer and the amplitude was several times higher. I n t r a l u m i n a l pressure m e a s u r e m e n t s at the level of recording electrodes indicated that these bursts of electrical activity as well as the continuing bursts resulted in a monophasic pressure wave. T h e response of the lower third of the cat's esophagus closely resembled t h a t of the m o n k e y esophagus. (4) As in the dog, there is an absence of a specific resting electrical activity in the gastroesophageal sphincter of the m o n k e y and the cat. These studies indicate t h a t the motor and electrical responses of the esophageal tube, w h e t h e r covered largely with skeletal muscle (as in the dog) or skeletal and smooth muscle (as in the cat, monkey a n d hum a n ) , are highly similar.

BENIGN LESIONS ASSOCIATED WITH MOTOR DISORDERS

DISORDERS OF PHARYNGOF.SOPHAGEALSPHINCTER~ INCLUDING P~IARYNGOESOPHAGEALDIVERTICULUM Until recent years, little emphasis has been placed on the u p p e r esophageal or pharyngoesophageal sphincter as a source of difficulty in the swallowing function. T h e cricopharyngeus muscle now is recognized as a true sphincter and was anatomically described by Batson. 14 It arises from the lateral borders of the cricoid cartilage and runs posteriorly to the wall of the esophagus without having a m e d i a n r a p h e as do the constrictors of the pharynx. Superiorly, the cricopharyngeus muscle is bordered by the inferior constrictor and inferiorly it blends into the circular longitudinal muscle fibers of the u p p e r esophagus. Although there continues to be considerable controversy-with respect to the innervation of the cricopharyngeus muscle, animal experiments and clinical observations suggest t h a t both sympathetic and p a r a s y m p a t h e t i c systems a r e involved. It is probable t h a t the nerve of the sphincter is derived from pharyngeal branches of the vagus through the p h a r y n g e a l plexus. Henderson and his colleagues °° clearly demonstrated a relationship between dysphagia and diseases of the r e c u r r e n t laryngeal nerve and have shown anatomically t h a t the r e c u r r e n t laryngeal nerve has a direct branch to the proximal esophagus and to the cricopharyngeus. In 1919, Kelly first suggested t h a t there was a spasm at the entrance of the esophagus in patients with the disease, which later became known in G r e a t B r i t a i n as the Paterson, Brown, Kelly syndrome and in the United States as the P l u m m e r - V i n s o n syndrome; since t h a t time, a variety of disorders resulting in abnormalities of the pharyngoesophageal function have been published. Several classifications of " the dysfunction of the cricopharyngeus m u s c l e have been proposed, 1~. 5~; 56 which include most of t h e conditions likely to be encountered clinically: 1. Central n e r v o u s system disease, such as bulbar poliomyelitis or a cerebrovascular accident. 2. Diseases affecting muscle activity, such as m u s c u l a r dystrophy, dermatomyositis, m y a s t h e n i a gravis, thyr0toxic m y o p a t h y . 3 . PostsurgicaI dysphagia, including radical oropharyngeal surgery, tracheostomy, idiopathic incoordination. 4. Pharyngoesophageal diverticulum. H e n d e r s o n et al. 9o h a v e pointed out that pharyngoesophageal dysphagia 12

occurs frequently as a result of recurrent laryngeal nerve palsy. Associated with the dysphagia there may be significant aspiration symptoms. In patients who have had a pneumonectomy, the aspiration m a y be of sufficient severity to produce respiratory infection and potential damage to residual respiratory function. Eleven of 15 patients with bronchogenic carcinoma and recurrent laryngeal nerve palsy had pharyngoesophageal dysphagia that developed simultaneously with the voice change of vocal cord paralysis. Pharyngoesophageal dysphagia was also noted in patients with thyroid resections and in an instance of viral neuritis in which recurrent laryngeal nerve palsy was present. The diagnosis of cricopharyngeal dysfunction causing upper esophageal dysphagia is based mainly on the clinical history and cine studies. Although Mitchell and A r m a n i n P 23 have not found pressure studies of the cricopha, ryngeus muscle to be helpful, Ellis 55 and Belsey z~ have believed that manometric studies helped clarify the pathogenesis and contributed to the diagnosis. Ellis and his associates 5g have described a type of incoordination in patients with Zenker's diverticulum. In manometric studies on these patients there was shown a zone of elevated pressure at the pharyngoesophageal sphincter in all patients with pharyngoesophageal diverticula. The zone of elevated pressure was slightly longer and the maximal pressures within it slightly lower than those reported from normal individuals. With deglutition, all the patients studied had immediate sphincteric relaxation fallowed by contraction, although the magnitude of relaxation and its duration were slightly longer than normal and the amplitude of contraction •slightly less. These findings clearly excluded the possibility of cricopharyngeal achalasia being present in patients with this disease, but all of the patients were noted to have an abnormal temporal relationship between pharyngeal contraction and pharyngoesophageal sphincteric relaxation and contraction. These findings were n o t observed in the studies on normal individuals. Over the p,~t decade, m a n y authors have carried out cricopharyngomyotomy for the t r e a t m e n t of Zenker's diverticulum and there are increasing reports of its successful use in other disorders. Cricopharyngomyotomy may be indicated in p a t i e n ~ i n whom dysphagia is suspected to be on the basis of a motor d i s o r d e r of the cricopl/aryngeal sphincter. Recently, Bishop 27 reported a case of cricopharyngeal achalasia i n childhood. The roentgenograms revealed a spasm of the cricopharyngeus muscle. Extramucosal myotomy successfully eliminated the child's prolonged problems of swallowing. In those instances in which a s p i r a t i o n has been a major complicating problem, in patients with vagal nerve injuries, radical glossectomy, radical orophar~yngeal resections, supraglottic laryngectomies or p e r m a n e n t tracheostomies, cricopharyngomyotomy m~iy be helpful. OPERATION.--Access to the cricopharyngeus is o b ~ i n e d through a left oblique cervical incision anterior to t h e edge of the sternomastoid muscle, beginning at the suprasternal notch 'and c a r r i e d u p w a r d t o just below the base of the:ear. T h e s e n s o r y c u t a n e o u s bra~nches of the anterior Cutaneous n e r v e m a y be sacrificed. T h e descendens cervicalis a n d t h e branches of the hypoglossal, which innervate t h e strap muscles, should be preserved, The omohyoid inferiorly may be sacrificed, if necessary, and.the thyroid is retracted medially and the carotid s h e a t h laterally. For better exposure, the inferior thyroid artery and: the middle a n d t h e i n f e r i o r :thyroid vein

13

m a y be divided. Care is taken during this maneuver to avoid injury to the recurrent nerve. T h e cricopharyngeus muscle is exposed posteriorly by rolling the esophagus and the inferior p h a r y n x laterally and anteriorly. T h e muscle then is divided in the midline down to the mucosa, carrying the incision cephalad approximately 2 cm and caudad approximately 3 c m on the esophagus. After the myotomy, the esophageal and cricopharyngeal muscles are dissected from the underlying mucosa for about half the circumference of the mucosal tube to allow the mucosa to protrude freely through the incision. We have not had experience w i t h phar3mgoesophagea! myotomy alone for Zenker's diverticulum. Since almost invariably the diverticulum has been of considerable size, we have proceeded with resection (Fig. 2). It seems reasonable, however, that i f the diverticulum is small myotomy alone would appear to be satisfactory. Belsey 17 recently has presented 32 cases of pulsion diverticula treated with myotomy~ and diverticulopexy without resection of the diverticulum. Ellis e t al. 5s also have subscribed to the t r e a t m e n t of pulsion diverticulum with myotomy. If the procedure is being done for pharyngoesophageal diverticulum, the diverticulum is freed to its neck and the transverse fibers of the cricopharyngeus muscle borderFig. 2.--Large pharyngoesophageal diverticulum in a debilitated 70-yearold male treated by primary resection. Diverticulopexy and myotomy or a twostage procedure consisting initially of diverticulopexy and subsequent resection are alternative procedures and may be indicated in poor-risk patients.

14

ing the inferior m a r g i n of the neck of the diverticulum are identified and incised. T h e incision is carried down to the mucosa and extended caudally a short distance onto the esophagus. If myotomy alone is done, the wound is closed without drainage and, subsequently, oral feedings can be allowed over the next several days. A n advantage of the myotomy procedure alone is that the hospital stay m a y be sborte.ned appreciably. Some authors have recorded a reduction in the resting pressures of the pharyngoesophagea] sphincter; others have failed to note any changes either preoperatively or postoperatively.

TRACTION DIVERTICULA Although this lesion is n o t u s u a l l y identified with motor disorders or any characteristic p a t t e r n on motility studies, disordered peristaltic waves have been "noted occasionally during r o e n t g e n fluoroscopy. These diverticula occur in the region of the bifurcation of the trachea (Fig. 3). I n this Fig. 3.--Traction diverticula in region of tracheal bifurcation. The dependent portion of the sac is rare and probably was responsible for dysphagia and an abnormal peristaltic pattern noted in this patient. Operation generally is not indicated unless complications occur,

15

vicinity, where lymph nodes and lymphatics are abundant, the diverticulum usually is the result of extra-esophageal inflmnmatory disease rather than internal pressure. Acute and chronic inflammatory changes in the adjacent lymph nodes, particularly tuberculosis with subsequent fibrosis and contraction, produce focal traction diverticula, T h e sac usually is on the anterior aspect of t h e esophagt~s, is small and funnel-shaped and because of the fixation of the tip of the diverticulum it generally does not occupy a dependent position. F o r tl~is reason, there is little tendency toward accumulation and retention of food, with their Subsequent evils, and the diverticulum rarely is symptomatic. If and when symptoms occur, they usually are related to the disease process that produced the diverticulum originally. A periesophageal sinus or esophagotracheal, bronchial or esophagopleural fistula m a y ensue. Surgical treatment o f these conditions is directed toward the complication. PULSION OR EPIPHRENIC DIVF~TICULA OF THE LOWER THmU OF THE ESOPHAGUS Diverticula of the lower third of the esophagus, like those of the pharyngoesophageal junctionl are due to internal pressure with herniation of the mucous membrane through a weak area in the muscular wall and are, therefore, termed pulsion diverticula. T h e y are situated in the terminal

Fig. 4.-~----Large epiphrenic diverticulum in lower portion of esophagus. Distortion and angulation of the esophagus may occur readily, enhancing regurgitation esophagitis. Primary excision is the procedure of choice.

16

portion of the esophagus just above the diaphragm and are comparatively rare. T h e pouch orifice usually is situated posterolaterally, more often to the right; than to t h e left. As the pouch enlarges, it tends "to assume a more dependent position and produces distortion and angu]ation of the esophagus. L~D Thus, during deglutition, food m a y be directed into the pouch rather than along the normal esophageal course. Although cardiospasm has been associated in a number of the cases, there is little evidence that it is of etiol6gic significance. T h e diverticula at this site m a y be the seat of inflammatory changes, ulceration, p e r f o r a t i o n and hemorrhage. Owing to the resulting esophagitis and the angulation of the esophagus, stricture of the esophagus distal to the orifice of the diverticulum is not an infrequent finding. Procedures t h a t have been employed in the t r e a t m e n t of these diverticula fall in the following categories: (1) anastomosis between the sac and stomach, (2) excision of the sac, (3):"diverticulopexy, (4) inversion of the sac and (5) resection of t h e lower part of the esophagus, including the diverticulum and cardia of the stomach, followed by esophagogastrostomy. On the basis of our experience, as well as an analysis of t h e various methods of surgical t r e a t m e n t , it appears t h a t the excision of the diverticulum followed by closure:~f the defect in the esophagus is the method of choice. Such a procedure permits removal of the lesion and restoration of normal anatomy and function, thus achieving the ideal objective of surgical therapy. We have not believed it to be necessary to add a long extramucosal esophagomyotomy as reported by the Mayo Clinic group.14s If marked stricture formation is present in the esophagus distal to the diverticulum, it then will be necessary to proceed with a resection of the segment of esophagus, including the diverticulum, followed by an interposition procedure with j e j u n u m or colon or gastric tube. ACHALASIA OF THE ESOPHAGUS

Megaesophagus, cardiospasm, was first described around 300 years ago. Steichen, Heller and Ravitch l~s have given a lucid review of the history and t r e a t m e n t of this condition over the years. T h e disease is the most frequent cause of dysphagia in the female and second only to carcinoma as the cause of dysphagia in the male. 12-~The principal abnormalities in achalasia of the esophagus are the disappearance of normal motility, the increase in esophageal size, the abnormal contractions characterized by decrease in frequency and amplitude and the total lack of rhythm, the functional obstruction in the region of the esophagogastric junction and the increased sensitivity of the body of t h e esophagus to cholinergic drugs. Much work h a s been done on the motor abnormalities o f achalasia of the esophagus. T h e major physiologic derangement responsible for the obstruction i s controversial. For m a n y years it was believed t h a t there was a failure of relaxation in the region o f the inferior esophageal sphincter, m More recently, however, there is increasing evidence that the:major functional obstruction is on the basis of a spasm of:the smooth muscle. The etiology of achalasia still is not known. H u r s t and Rake 99 postul a t e d t h a t the disease was d u e :to an absence of A u e r b a c h ' s plexus. Others have p o s t u l a t e d t h a t t h e r e is c degeneration Of t h e plexus, xTs and others have p o s t u l a t e d t h a t it:is a disorganization of the intrinsic neural mechardsm.m 17

It is our view 131. is°- that Auerbach's plexus is present in those cases in which studies have been made of the esophageal muscle removed during operation or at autopsy but there is a decrease in the n u m b e r of ganglion cells. Cassella and his associates s7 found that only 6 8 ~ of 28 specimens studied had degeneration or absence of Auerbach's cells, and in the more recent studies from the Mayo Clinic,91 a concept was advanced that an extra-esophageal vagal lesion, of either the peripheral vagus nerve or its dorsal motor nucleus, is the p r i m a r y site of involvement in esophageal achalasia and that the esophageal changes are secondary. H a r m a n , O'Hegarty and Byrnes 79 have studied by electron microscopy the esophageal smooth muscle in patients with achalasia. They f o u n d alterations in the cellular connections when normal muscle was compared with achalasia muscle. They believed that both the absence of normal peristaltic movements and the chronic contraction in the cardia might be explained on the basis of the altered bonding. Besancon °-6 has presented evidence that motility studies carried out u n d e r conditions more nearly simulating n a t u r a l events would lead to rejection of the theory of failure of relaxation and suggest instead an interpretation based on mechanical and chemical hyperexcitability. F u r t h e r studies utilizing simultaneous recordings of motility and of the deglutitive electrical activity Of the esophagus m a y be expected to give valuable information on both the normal and disturbed esophageal motility. T h e mechanisms that enter into the control and regulation of the movements of the esophagus have not been clearly delineated. A detailed review of the ~hysiology and a n a t o m y of the esophagus h a s been presented by Ingelfinger. 1°° A m o r e recent review of the pharmacology of the esophageal motor function has been published by Christensen 39 and particular emphasis is given to the effect of the gastrointestinal hormones. There is increasing evidence that the innervation of the body of the esophagus is largely, if not entirely, cholinergic. Recent studies indicate that the adrenergic innervation of the esophagus is primarily located within the myenteric plexuses. 102 This is in agreement w i t h observations made on the gastrointestinal tract of other species: TM In the sphincter region of the dog esolJhagus, striking catecholamine fluorescent terminals are seen in the myenteric plexus, and the close anatomic apposition of adrenergic varicosities to enteric ganglion cells suggests that sympathetic fibers terminate on the nerve cell body or its dendrites. T h e adrenergic innervation to the smooth muscle i s relatively sparse i n Comparison to the density of the fluorescent fibers contained in the plexuses. T h e picture of the autonomic innervation of the esophagus suggests that contraction is due to the release of acetylcholine from the parasympathetic (cholinergic) innervation and that the sympathetic (adrenergic) inhibitory m e c h a n i s m takes place primarily at the level of the my.enteric ganglia of Auerbach. ~Vhether innervat~on t o the sphincter muscle emanates from the ganglion cells contained within t h e stoumch is n o t known. We have suggested, however, ithat part of Cne choiinergic innervation appar a t u s to the sphincter muscle is derived from ganglion cells of the stomach. A neuroanatomic basis for t h i s consideration was reported in the guinea pig,: a n d its nerve fibers were traced f r o m the ganglia i n the stomach to the esophagus. Such an a r r a n g e m e n t 6f nerves from the stomach to the esophagus could serve to control and coordinate the passage of food past the sphincter into the stomach.

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This concept of the autonomic innervation is attractive in considering the neuromuscular abnormalities not only in achalasia of the esophagus but also in congenital pyloric stenosis and congenit.al aganglionic megacolon, l~eyes and Friesen :Ss believe that in pyloric stenosis there is an arrest of normal development of the ganglion cells, since in this condition there is a lack of activity of mitochondrial and other oxidative enzymes, which ordinarily characterizes the m a t u r e cell. In congenital aganglionic megacolon there is an absence of ganglion cells together with a prominence of nerve fibers i n t r a m u r a l l y in the rectum. T h e finding of numerous nerve fibers in the intermyenteric layer is as diagnostic as the absence of ganglion cells. Using histochemical technics, Kamijo, H i a t t and Koelle :°6 demonstrated high concentrations of specific cholinesterase in the numerous bundles of nonmyelinated nerve fibers in the intermyenteric prunes of the spastic segments. Presence of the enzymes indicates that the fibers are cholinergic whereas their lack of myelination places them in the classification of postganglionic. T h e i r observations-provide the basis for an alternative interpretation to the widely accepted current concept of the etiology of congenital megacolon: namely, the cholinergic ganglion cells are present but outside the colon in a location more central than normal and that their postganglionic fibers extend to the usual sites of termination from somewhere outside the colon. Studies by Ambache and F r e e m a n s strengthen the impression that the myenteric plexus of the rabbit ileum contains both excitatory cholinergic and inhibitory adrenergic neurons, the participation of both of which is necessary for the normal integration of peristaltic contractions. If the same situation pertains in the h u m a n colon, it seems possible that the physiologic defect in the spastic segment in congenital rnegacolon might consist chiefly of the absence or paucity of adrenergic neurons, which in the presence of a normal component of centrally situated ganglion cells could result in the net effect of unopposed tetanic contraction. Okamoto e t al. 1~5 selectively destroyed the myenteric ganglion plexus and thus produced a nonorganic stenosis at any segment of the gut regardless of whether sphincteric or not and the mechanism was believed by them to be atonic contraction of the smooth muscle fibers. In the normal innervation of the GI tract, the i n t r a m u r a l ganglion cells generally a r e considered to be postganglionic parasympathetic neurons at which the preganglionlc parasympathetic fibers from the vagus, etc. terminate with synapses whereas the s y m p a t h e t i c fibers, being~postganglionic, pass through t h e i n t r a m u r a l ganglia Without synaptic interruption, directly to their termination. Absence or loss of ganglia t h u s has b e e n equated physiologically w i t h a depletion of preganglionic parasympathetic fibers, both resulting in sympathetic overactivity. T h e more recent concept of the innervation of the gut suggests that the i n t r a m u r a l ganglion plexus is a n isolated nervous system at .which postganglionic sym~a*~hetm fibers terminate with synapses in a w,ay similar to the preganglionic parasympathetic fibers. Thus, the myente~ic ganglion plexus would appear to have m a n y intrinsic reflex, arcs in itself t h a t directly manage intestinal activity. T h e extrinsic sympathetic and parasympathetic fibers regulate intestinal activity only via t h e i n t r a m u r a l plexus from a higher level, conveying inhibitory or peristaltic imlJulses from the central nervous system. In thisl-new .concept , the~ smooth muscle fibers at the site where ganglia are absent, i m m a t u r e or destroyed are considered to

19

have lost all nervous control the sympathetic as well as the parasympathetic fibers no longer can regulate the smooth muscle fibers, leaving them in a state of denervation. T h i s seems to be the basic pathophysiology of the stenosis in Hirschsprung's disease and achalasia. Applying these newer concepts~ and in the light of some of o u r previous studies, the reason for the beneficial effects of the :surgical procedure of esophageal cardiomy.otomy would appear to be clarified. F u r t h e r reference will be made to this. D~GNOSIS.. The presenting or predominant s y m p t o m is dysphagia in 85-90~o of patients23-~5 T h i s complaint is more manifest on the ingestion of solid foods and the-patients often l e a r n to force food t h r o u g h b y ' t a k i n g large volumes of warm liquids or carbonated beverages. :Regurgitation occurs in approximately 60%. Weight loss is~present in between 50% and 6 0 ~ of patients and has rahged u p t o as much as 85 pounds in our series. Occasionally there m a y be hypertrophy of t h e s a l i v a r y glands. In general, the diagnosis of achal~sia of the esophagus is not difficult and m a y be made by roentgen examination alone..The esophagus m a y be greatly dilated in a fusiform fashion or it m a y be sigmoidal in configuration. Regardless of the configuration, however, the important features are the smooth conical narrowing at the esophagogastric junction and the m a r k e d delay in~ emptying. At times i t i s extremely difficult to differentiate an organic obstruction from a functional one. Moreover, a carciimma of the lower esophagus m a y be missed on roentgen examination because of the widely dilated capacious organ. For these reasons, esophagoscopy is mandatory. Cineroentgenography has been helpful also, not only in clarifying the pathophysiology but in establishing the diagnosis. The dilatation, the delay in emptying, the narrowed s e g m e n t a n d the distorted peristalsis m a y be clearly demonstrated and documented in t h i s m a n n e r . Administrat-ion of Mecholyl will accentuate the reversed peristalsis, a feature of the distorted motility in achalasia, whereas it will not have this effect o n the n o r m a l esophagus or on an obstruction due to organic narrowing. I n our series there have been several instances in which both careful fluoroscopic examination and esophagoscopy have been inconclusiwe in differentiating between functional and organic obstruction. In such instances, esophageal motility studies have been most useful. The motility pattern of the esophagus of the patient with achalasia is markedly different from that of a n o r m a l esophagus and from that of an esophagus with an organic obstruction. It must be emphasized again that the p a t t e r n of the entire esophagus is altered and not just the lower or narrowed portion. T h e r e is a total lack of r h y t h m and: a m a r k e d decrease in a m p l i t u d e and frequency of contraction. Studies have further indicated that t h e r e i s no relationship or proportionality between the duration of the condition a n d the degree of motor abnormality. 85 Motility studies have also indicated t h a t : a l t h o u g h the operation of esophageal c a r d i o m y o t o m y i s effective in relieving the functional obstruction and in some cases decreasing the size of the esophagus, i t has no effect on:the altered motility pattern (Figs. 5 and 6). In t h r e e instances i n which difficulty was e n c o u n t e r e d in differentiatingbetween a functional and a n organic obstruction, motility studies revealed a 'pattern in the u p p e r e s o p h a g u s consistent w i t h organic obstruction and a p a t t e r n i n t h e l o w e r esophagus consistent with achalasia. I n t h e s e cases, a carcinoma spreading in the wall of the esophagus and replacing the m y e n t e r i c plexus was found to be present. TRP_~T~NT.~It is our belief that all cases should have a trial of forceful 20

Fig. 5.--Roentgenograms of 72-year-old patient with achalasia of 5 years' duration. The conical narrowing of the distal esophagus in the preoperative film is characteristic. The roentgenograms at the right were taken 6 weeks after operation and demonstrate a marked decrease in caliber of the esophagus with rapid emptying. Occasionally the size of the esophagus does decrease as demonstrated here, but this is not usual. The clips were placed on the diaphragmatic hiatus. (Reprinted with I~ermission of Surgical Clinics of North

America.) dilatation prior to consideration for cardi~myotomy. One or more proper dilatat;ions will be effective in the majority-of instances in relieving symptoms, It is well to point out, however, t h a t with the success of the esophageal cardiomyotomy, we have accepted for operation a number of patients who did not wish to undergq repeated dilatations. Moreover, dilatations in the presence of a markedly dilated or sigmoidal esophagus m a y be n o t only extremely difficult but also dangerous. In these instances, t h e m o r b i d i t y is less with operation. If the diagnosis has been ,accurate and i f there have been_n0 existing complications, we m a y reasonably guarantee successwith the operationl Obvionsly, i f the diagnosis has bee~ incorrect, if t h e r e i~ coexisting carcinoma, a diverticulum or esophagqtls with stenotic narrowing, the operation i s n o t going to a c c o m p l i s h its purpose. Preoperative preparation of these patients must b e careful. Debilitation frequently is a prominent feature. General. supportive measures, including careful attention to n u t r i t i o n , fluid a n d e l e c t r o l y t e equilibrium, should b e carried out u n t i l the optimal: time.for operati0n h a s b e e n obtained. In almost 10~o of the cases of achalasia s o m e p u l m o n a r y complications: are p r e s e n t due to nocturnal regurgitation. In order t0i keep pulmonary complications to a 21

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~:~-:;.~,:~.~i:~.;i:~, : ~ : , ' ~':~ ~V: .....~ ~ ~',i 'L~ '~ :'~.';:~•",• ~ ~ "~•- ~ ~,:,r. ~ . ,

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F'i~. 6. Esophageal motility studies in the same patient whose roentgenograms are shown in Figure 5. These studies were done 6 months postoperatively following esophagocardiomyotomy and pyloroplasty. Although all symptoms have been relieved and the esophagus has decreased markedly in size, the motility pattern has not been altered. There is the same dysrythmicity and decrease in the frequency and amplitude of contractions. The response to urecholine a n d atropine were the same as observed preoperatively. (Reprinted with permission of Surgical Clinics of North America.)

minim~lm, frequent esophageal aspirations m a y be necessary during the preoperative preparation and it m a y be' necessary to limit oral intake. P u l m o n a r y complications m a y r a n g e f r o m a mild aspiration pneumonitis to bronchiectasis and pulmonary abscess (Fig. 7). T h e presence of pulmon a r y lesions i n patients w i t h achalasia is another indication for operation, since the pulmonary lesions frequently will heal on removal of the obstructive mechanism. ~ATI~ PROC~tVR~:.mThe modified Heller procedure or esophagocardiomyotomy now is generally accepted as the t r e a t m e n t of choice. Heller's original operation consisted of an,anterior: an d a posterior esophagocardiomyotomy. 89 T h e modified Heller procedure consists of a simple, single linear esophagocardiomyotomy placed on the anterior aspect of the distal esophagus and cardiac portion of the stomach.:31~.In t h e uncomplicated case of achalasia, t h i s operative procedure h a s been carried out by us to the exclusion of all others since 1947. There has been considerable difference

22

Fig. 7.- .....Roentgenogram of chest of patient with achalasia demonstrating a pulmonary abscess in the right lower lobe. The lung lesion initially was thought to be neoplastic. Following esophagocardiomyotomy, the pulmonary complications are alleviated or markedly decreased.

of opinion w i t h respect to the surgical approach. Ellis 5r a n d others h a v e used the thoracic a p p r o a c h , believing t h a t surgical d i s r u p t i o n of the h i a t a l a t t a c h m e n t s is m o r e likely to occur w h e n the a b d o m i n a l a p p r o a c h is used thus, leading to a likelihood of postoperative herniation. Others ~2 h a v e b e lieved t h a t the thoracic a p p r o a c h is necessary i n o r d e r to c a r r y the m y o t o m y incision to a n a p p r o p r i a t e level in a c e p h a l a d direction. W e h a v e preferred the a b d o m i n a l approach. It is simpler, a n d since p y l o r o p l a s t y is a routine addition to the operation, t h e a b d o m i n a l a p p r o a c h allows easy access to the pyloroduodenal area. T h e a b d o m i n a l a p p r o a c h also enables us to d e t e r m i n e t h e presence of o t h e r lesions in the abdomen, w h i c h have b e e n v a r i o u s l y r e p o r t e d in 10-20~o of instances. T h i s l a t t e r consideration has added i m p o r t a n c e w h e n one considers t h a t : p a t i e n t s w i t h concomitant achalasia a n d active d u o d e n a l u l c e r disease a p p e a r to have a h i g h incidence of esophagitis after the H e l l e r operation, ss T h e a b d o m e n is e n t e r e d t h r o u g h a n u p p e r m i d l i n e i n c i s i o n e x t e n d e d to the left of t h e xiphoid process of the s t e r n u m i n o r d e r to g e t a s m u c h e x p o s u r e a s p o s s i b l e in a c e p h a l a d d i r e c t i o n . T h e l e f t t r i a n g u l a r l i g a m e n t s u s p e n d i n g the l e f t l o b e of the liver to t h e u n d e r s u r f a c e of t h e left hemid i a p h r a g m is identified, p u t on a s t r e t c h a n d divided. I f care i s ~t a k e n to a v o i d the veins o n the u n d e r s u r f a c e of the d i a p h r a g m , no bleeding of con-

23

sequence will result. Following division of this ligament, the left lobe is folded on itself a n d r e t r a c t e d to the right sicle of the midline,, thus exposing the c a r d i a c end of t h e stomach. T h e peritoneal reflection is incised a n d t h e esophagus is liberated circumferentially. It usually is quite easy to deliver 8-10 cm of the t e r m i n a l esophagus below the hiatus. It is i m p o r t a n t , a t this stage, to carefully identify the vagus nerves a n d protect t h e m from injury. Division. or i n j u r y of the vagus nerves will decrease~ t h e "gastric e m p t y i n g , thus i n c r e a s i n g the incidence of regurgitation. T h e m y o t o m y t h e n is carried out s t a r t i n g well above the esophagogastric j u n c t i o n a n d the fibers a r e gently s p r e a d a p a r t w i t h a right-angled i n s t r u m e n t . W h e n the e x t r a m u c o s a l plane is reached, a ~blunt-nosed right-angled c l a m p m a y be i n s e r t e d deep to the m u s c u l a r coat b u t superficial to the m u c o s a and, as it is opened, the a s s i s t a n t carefully severs the fibers stretched over the clamp. T h i s m a n e u v e r is similar to t h a t of the R a m s t e d t procedure. T h e muscle fibers are s e p a r a t e d for a distance of 6-8 cm above the esophagogastric junction a n d for 1-2 cm distal to the esophagogastric junction. It is i m p o r t a n t t h a t a l l of the circular muscle fibers be severed, and great care m u s t be t a k e n in .this m a n e u v e r (Figs. 8 a n d 9). T h e r e is a good deal of v a r i a t i o n a m o n g surgeons as to the l e n g t h of the m y o t o m y on the esophagus a n d this h s s been one of the most p e r p l e x i n g aspects of this operation. R a t h e r significant variations i n technic a p p a r e n t l y give almost identical results. H e l l e r originally reported a n incision 8 cm long on b o t h the anterior a n d posterior aspects of the .esophagogastric a r e a a n d pointed out t h a t it w a s - n e c e s s a r y to lengthen the incision p a r t i c u l a r l y d o w n w a r d , although~it need be carried u p w a r d only as far as the beginning of the esophageal dilatation. Silber TM incises the esophageal side for only 3/~-1 inch a n d believes t h a t it is i m p o r t a n t t h a t the incision stop short about 1/~ inch from the visible esophagogastric junction. H e believes t h a t it is illogical to e x t e n d the incision into the dilated a b n o r m a l p a r t of the esophagus. Ellis incises for a distance of from 6 t o 1 2 cm almost entirely on the esophageal side, w i t h the incision to the gastric m u s c u l a t u r e a l w a y s less t h a n 1 cm a n d u s u a l l y only a few m m . F o n t a i n e 62 m a k e s a v e r y long incision e x t e n d i n g f r o m the fundus to the a r c h of the aorta. G a m m e l g a a r d 67-makes a 6--10-cm incision extending from some w a y down the a n t e r i o r surface of the stomach to w e l l over the n a r r o w e d portion of the esophagus. Grimes, Stevens a n d BelF 2 m a k e a n incision beginning a t t h e level of the inferior pulmon a r y vein a n d going from 12 to 15 cm well down on the gastric cardia. O u r m e t h o d has been to incise for a dista~ace of 8-10 cm with 6-8 cm above the esophagogastric j u n c t i o n a n d 1-2 cm distal to, the esophagogastric junction. W e r o u t i n e l y a d d a p y l o r o m y o t o m y or pyloroplasty. T h e zone of elevated pressure i n the region of the a n t e r i o r e s o p h a g e a l s p h i n c t e r s is m a r k e d l y r e d u c e d by e s o p h a g o m y o t o m y and, a c c o r d i n g to Ellis, is all b u t e l i m i n a t e d w h e n extensive m y o t o m y is carried out. T h e p r e f e r r e d incision' a p p e a r s t o b e one t h a t is long enough to a p p r o p r i a t e l y reduce t h e resting t o n e in the inferior esophageal s p h i n c t e r but, a t t h e s a m e t i m e , a v o i d s i m p a i r m e n t of g a s t r o e s o p h a g e a l competence. TM I t is likely t h a t the ~o p e r a t i o n is too imprecise to t r e a d this n a r r o w line, a n d s p e c u l a t i o n is invited as to o t h e r possible responsible mechanisms. As m e n : tioned previously, in studies on dogs w i t h achalasia t h e r e was a n absence of a d r e n e r g i c fibers i n the A u e r b a c h plexus or a substantial r e d u c t i o n of t h e s e fibers: I n 5 dogs, a n e s o p h a g o c a r d i o m y o t o m y was c a r r i e d out a n d the a r e a was e x a m i n e d "7-10 d a y s after o p e r a t i o n using m e t h o d s that' we re24

Fig. 8.--Esophagocardiomyotomy for achalasia. Following exposure of the mucosa through the longitudinal incision, a'right-angled instrument is placed between the mucosa and circular muscle fibers and as the instrument is gently spread, the assistant severs the fibers. This is first carried out in an upward direction, then in a downward direction.

ported previously. 1°2 T h e animals with esophagocardiomyotomy showed a degeneration of acetylcholinesterase staining nerves in the region adjacent to the lesion. About 10-20~o of the cross-sectional area was involved. 13° These studies suggest t h a t the m y o t o m y procedure causes a reduction in the p a r a s y m p a t h e t i c predomin&nce in the esophageal sphincter. Thus, the reduction in resting tone and the degree of esophagogastric competence m a y be r e l a t e d to the extent of ~cholinergic nerve degeneration following myotomy r a t h e r t h a n to the a m o u n t of muscle incised. T h e concept of a strong autonomic component in the action o f the lower esophageal sphincter is f u r t h e r s u p p o r t e d in recent reports of reduction in the esophageal sphincter tone in h u m a n subjects after~ administration of isoproterenol s3 and relaxation o f the esophageal sphincter in the cat by stimulation of beta receptors, s4 T h e response to n o r e p i n e p h r i n e and e p i n e p h r i n e h a s been studied by m a n y investigators: Both catecholamines cause contractions. TM In these studies, which were carried out in cats, and in the study by 25

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Fig. 9.--Esophagocardiomyotomy for achalasia. An adequate bulge of the mucosa is obtained following the severance of the circular fibers and the small vessels in the region of the esophagogastric junction. Either a pyloromyotomy or a pyloroplasty, as shown here, is added.

Schenk, the most noticeable effect was at the lowermost segment of the esophagus. The findings of Christensen and Daniel were, however, closer to the views of Ellis and his group, 54 since they believed t h a t the effect of epinephrine was variable, sometimes causing contraction and sometimes relaxation. The fact that excitation was opposed by tolazoline and that inhibition was opposed by propranolol suggested to Christensen and Daniel that excitation is m e d i a t e d through the adrenergic alpha receptors and inhibition through adrenergic beta receptors. 4(j041 RESULTS.---Good to excellent results following esophagocardiomyotomY can be expected in 70"85% of patients, T h i s procedure h a s been performed on 87 patients at our institution with 1 postoperativedeath due to a cereb r o v a s c u l a r a c c i d e n t . Convalescence f o l l o w i n g the :abdominal procedure generally i s smooth: and uncomplicated. Rarely, d u r i n g the myotomy, a small perforation m a y be:made inadvertently t h r o u g h t h e mucosa' Simple closure'of the mucosa will suffice, and in 3. cases in w h i c h this did occur there were no untoward complications: Indwelling nasogastric tubes are not u s e d after operation for fear that they will erode the thin mucosa. 26

Most patients are able to tolerate liquids the day after operation, and over a period of from 5 to 7 days, the oral intake is increased to a regular diet. Follow-up studies in this group of patients have ranged from 6 months to 26 years. Seventy-seven per cent have been classified as having good to excellent results, with complete relief from the symptoms of dysphagia. Twenty patients (23%) are classified as having fair to poor results. Seven of the latter had been operated on initially elsewhere. Analysis of those patients classified as having fair to poor results revealed certain factors common to a significant number to suggest prognostic importance: All had longstanding disease, 16 had multiple dilatations, 3 had esophageal diverticula i n addition to the achalasia and 9 had severe scarring a r o u n d the esophagogastric junction, specifically mentioned at the time of myotomy. Regurgitation esophagitis with its accompanying complications of pain, ulceration, bleeding and stenosis was the most common cause of the unsatisfactory results, occurring in 15 of the 20 patients. Obstruction recurred at the esophagogastric junction in 3 patients and there was obstruction or marked delay in emptying at the py]oroduodenal area in 2. In our experience there has been only one instance in which we believed t h a t an inadequate incision in the distal esophagus accounted for the poor results. T h e methods of m a n a g e m e n t of those patients having recurrence of sYmptoms range from nonoperative m a n a g e m e n t to extensive interposition procedures. 130 In those patients having a recurrence of obstruction at the esophagogastric junction without evidence of esophagitis, a second myotomy may be Carried out. This is best performed through a thoracic approach, taking special care in mobilizing the esophagus in order to prevent i n j u r y to the vagus nerves and to prevent disruption of the normal hiata] attachments. Obstruction at the pyloroduodenal area has been observed in 2 patients in whom there was no evidence of ulcer or other gross pathology. These patients were successfully treated by pyloroplasty and antrectomy respectively. As a result of this experience, we routinely added a pyloroplasty to the initial esophagocardiomyotomy. We believe that the addition of the pyloroplasty results in better gastric emptying and a decreased incidence of reflux esophagitis. In 7 of the patients with regurgitation esophagitis there was an associated hiatus hernia. A hiatus hernia repair, vagotomy and pyloroplasty were carried out in 4 of these patients. All had been previously operated on through the abdominal approach. None of these patients had a hiatus hernia prior to the first operation and it is likely t h a t the postoperative hiatus hernia that occurred was the r e s u l t of operative ~lterafion of the hiatus or disruption of the phrenoesophageal ligamer ~her possible factor is eventual retraction of the intra-abdominal p~ the esopha~ gus secondary to fibrosis a n d scarring. T h e importance c~ ~ correction of the hiatus hernia, whether coexistent or surgically produced, has been stressed previously. 65 If an irreversible sten0sis is present secondary to the regurgitation esophagitis, interposition p r o c e d u r e s w i t h colon have been carried out. After a secondary operative procedure, approximately 80% of the patients have experienced improvement, ~a l t h o u g h t h e r e s u l t s certainly have not been as satisfactory as those obtained with a successful initial proce27

dure. Since all the patients who have had unsatisfactory results have had longstanding disease and since the majority of them have had multiple previous dilatations, we believe that early operation is indicated, particularly in those patients having a widely dilated esophagus.

THE SURGICAL DISEASES OF THE ESOPHAGUS OF CONGENITAL ORIGIN EMBRYOLOGY The development of the esophagus is intimately associated with those events affecting the development of the foregut, especially the differentiation of the trachea, lungs and stomach. 6. 71 Between the fourth and fifth weeks of gestation, the esophagus can be recognized as a short but definite tube between the p h a r y n x and the stomach. At about this time, the trachea becomes demarcated from the esophagus, with the appearance of a median anterior diverticulum of the esophagus. Growth of the body cephalad is accompanied by the ascent of the p h a r y n x and elongation of the esophagus. This gives the impression that the elongation of the esophagus carries the stomach below the forming diaphragm. A rapid development of the h e a r t and lung occurs during this esophageal elongation. Separation of the trachea from the esophagus from below upward occurs also during this period of elongation. Ridges appear on the lateral walls, which fold inward until they fuse and divide the foregut into tracheal and esophageal channels, which are joined at the pharynx (Fig. 10). Proliferation of the esophageal epithelium occurs during the seventh and eighth weeks of gestation until the lumen is nearly filled with cells. In humans, complete occlusion o~ the l u m e n does not normally occur but r a t h e r t h e r e is a series of communicating channels formed by irregular spaces within the cellular mass. The rare occurrence of a mucosal web n

Fig. 10.--Development of esophagus. A, 4 weeks. B, 5 weeks. C, 6 weeks.

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across the l u m e n of the esophagus would suggest t h a t complete l u m i n a l occluaion m a y occur occasionally. T h e vacuoles regress so t h a t b y the t e n t h week a s i n g l e l u m e n is restored a n d ciliated cells begin to a p p e a r , chiefly" on ".'r,folded ridges. T h e n u m b e r of ciliated cells progressively increases u p to the fifth m o n t h . D u r i n g the fourth m o n t h , s t a r t i n g in the mid-esophageal area, i s l a n d s of stratified s q u a m o u s e p i t h e l i u m begin to replace the ciliated epithelium. Small areas of c o l u m n a r cells n e a r the two ends of the esophagus a n d bet w e e n p r i m a r y folds, which are n o t replaced by stratified s q u a m o u s epit h e l i u m , become the superficial a n d deep esophageal glands.

TRACHEOESOPHAGEAL FISTULA AND ESOPHAGEAL ATRESIA T h e most common congenital a n o m a l y of the esophagus is a t r e s i a w i t h concomitant tracheoesophageal fistula. M o r e t h a n 90% of the p a t i e n t s have the classic form of a blind h y p e r t r o p h i e d a n d dilated u p p e r esophageal pouch t h a t u s u a l l y extends into the m e d i a s t i n u m below the thoracic inlet b u t does not reach as low as the c a r i n a of the t r a c h e a (Fig. 11). T h e lower esophageal s e g m e n t is small in caliber because of disuse a n d c o m m u n i cates w i t h the t r a c h e a via a fistula s i t u a t e d on the posterior t r a c h e a l wall above the carina. T h e incidence of esophageal a t r e s i a w i t h a n d w i t h o u t a fistula varies with reported series and is estimated to be a b o u t 1 p e r 3000 b i r t h s J v5 Association with o t h e r congenital anomalies is e x t r e m e l y . c o m m o n a n d is a m a j o r factor in the over-all m o r t a l i t y along w i t h p r e m a t u r i t y a n d infection. R a r e l y (1.5,°-/o of tracheoesophageal anomalies) is this combination of defects found with a fistula to the t r a c h e a from the u p p e r esophageal pouch or from both the u p p e r a n d lower esophageal segments. 9~ T h e s e l a t t e r two forms will be disregarded in the r e m a r k s t h a t follow. P o l y h y d r a m n i o s should a l e r t the p h y s i c i a n to the possibility of congenital o b s t r u c t i o n of the gastrointestinal tract. T h e absence o f this i n t h e m o t h e r does not exclude the diagnosis b u t it is p r e s e n t in about half of ~he obstructed infants. T h e obstruction in tl~e infant's gastrointestinal s y s t e m

Fig. 11,--Variations in types of esophageal atresia and tracheoesophageal fistula.

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29

is thought to prevent the ingestion and reabsorption of amniotic fluid, leading to the increase in amniotic fluid. At birth, these infants usually are normal ih appearance. During the first few hours of life, m a n y of them drool excessively and require frequent nasopharyngeal suctioning. T h e amount of saliva produced probably is normal; rather, it is the inability to swallow their saliva that gives the appearance of excessive production. Nonetheless, significant drooling should warn the attending medical and nursing staff that an atresia is present. Difficulty in feeding, with accompanying choking, cyanosis and shortness of breath, should leave little doubt of the diagnosis. T h e spillover of saliva and feedings, as well as the regurgitation of gastric'juice, into the trachea lead to p u l m o n a r y complications, which account for more than half of the deaths in these patients. 94 Crying and straining apparently force air into the gastrointestinal tract through the fistula, where it collects in the stomach and causes moderate distention, which may also lead to some respiratory embarrassment. From time to time, the gaseous distention in the stomach is relieved back through the fistula, carrying with it gastric juice into the tracheobronchial tree. This mechanism is of significance in the etiology of respiratory complications. A chest x-ray performed early m a y show no anomalies, but within a short period of time there usually is evidence of aspiration with atelectasis or pneumonia. I n most newborn nurseries, these patients are spared the insult of feedings and the delay in diagnosis by the routine passage of a firm catheter through the nostril and down into the stomach at the tiflle of delivery. Suspicion should be aroused immediately by any resistance to the passage of the catheter. T h e passage of t h e catheter in one nostril and eventual appearance out the other is almost diagnostic of a blind esophageal pouch (Fig. 12). T h e presence of obstruction indicates the need for roentgenographic studies. T h e catheter should be taped i n place and AP a n d lateral x-rays of both the chest and abdomen obtained. In most instances, this is sufficient to make a diagnosis. Although some authors consider it contraindicated, 1~5 it probably is wise to inject a small amount of water-soluble radiopaque material to rule out the possibility of a fistula from the u p p e r segment to the trachea. T h e contrast m e d i u m can be immediately suctioned back t h r o u g h the tube and thereby pulmonary aspiration of this material is prevented. T h e absence of air in the gastrointestinal tract on the x-ray of the abdomen indicates that the infant has esophageal atresia without a tracheoesophageal fistula. T h e abdominal film, in addition, helps detect associated obstructive lesions of the intestine. Only w h e n a baby is seen with no atelectasis or pneumonia is immediate p r i m a r y repair undertaken. Much more frequently, the child has marked pneumonitis. Since it now is obvious that the pneumonia results primarily from reflux of gastric secretion up through the fistula, a gastrostomy is performed as an urgent procedure u n d e r either local or generalanesthesia. Should intestinal atresia exist, i t m i g h t be dealt with at this time and the gastrostomy becomes p a r t of the same operation. T h e simplest pa!liative procedure t h a t will alleviate the situation should be utilized. For the next 24-72 or more hours, the p r i m a r y concern is t0 combat the existing pulmonary complications and to prevent others. T h e following steps should be taken: (1) antibiotics to combat bacterial infection; ( 2 ) h i g h h u m i d i t y to aid in liquefying secretions; (3) a sump suction catheter in the u p p e r esophageal pouch to prevent aspiration of saliva; and (4) the above-mentioned

30

Fig. 12.mEsophageal atresia a n d tracheoesophageal fistula. The passage of a catheter into and out of the inostrils is diagnostic of a blind esophageal pouch. The presence of gas in the gastrointestinal tract i s indicative of a communication between the lower esophageal segment and the trachea.

gastrostomy, which always should be left open until the fisi~la has been* closed to prevent reflux of gastric contents in the trachea. At the appropriate t i m e u~der elective conditions, the definitive operation then can be performed. It is important that the anesthesiologist b e experienced in infant anesthesia. In performing the definitive procedure, either a transpleural or a retropleural a p p r o a c h may be used. Better exposure, shorter operative time and ease of operative approach are the m a j o r advantages of the transpleural approach. T h e retropleural a p p r o a c h has

31

as its principal advantage the localization of infection through the retropleural space if an anastomotic leak occurs, r a t h e r t h a n c o n t a m i n a t i o n of the fr~e pleural space, with a resulting generalized e m p y e m a . In the initial period of experience in the t r e a t m e n t of tracheoesophageaI fistula with atresia, the retropleural a p p r o a c h was p r i m a r i l y utilized. W i t h more and m o r e experience, the availability of antibiotics and the progress in pediatric anesthesia, a greater n u m b e r of individuals turned to the franspleural approach. I n the mid-1960s, a survey was conducted by the surgical section of the American A c a d e m y of Pediatrics in an a t t e m p t to evaluate which of these approaches is safest for the patient. 94 Since then, the pendulurn has swung, With the retropleural approach regaining its former position. Anastomotic leaks occurred in 17% of the patients a n d - w e r e m a j o r causes of 21~o of the deaths with a retr6pleural approach. A leak occurring with a t r a n s p l e u r a l approach had a m o r t a l i t y of 63%. W h e n a leak occurred w i t h a retropleural approach, the m o r t a l i t y was 40%. 94 Most surgeons approach the posterior m e d i a s t i n u m t h r o u g h t h e bed of the extensively resected right fourth rib. Subperiosteal removal of the rib is carried out from the costochondral junction anteriorly to the angle of the rib posteriorly. Disarticulation of the rib more posteriorly=is avoided,, since the p l e u r a is quite a d h e r e n t in this area. An incision is made in the deep periosteum of the rib bed and a plane is developed by blunt dissection between the parietal pleura and the endothoracic fascia. T h e azygos vein m a y be divided, which we prefer; or left inplace,-removing the pleura from it; or reflected with the p l e u r a by ligating one or two of the intercostal veins as they enter the azygos. E v e r y a t t e m p t is m a d e to avoid entering the pleura, which is extremly delicate in these tiny infants. Should the p l e u r a be inadvertently entered, the opening is p r o m p t l y sutured. T h e lower esophageal segment is identified adjacent to the vagus nerve and is freed superiorly to its fistulous connection with the trachea. A section of the fistula is divided and closed on the tracheal side a n d then a n o t h e r one, until the esophagus is completely free. To avoid compromising the tracheal lu~nen, care must be taken not to divide the fistula too close to the )rachea. ~onversely, closure too far from the trachea will create a diverticulum t h a t m a y collect secretions. T h e tracheal closure is ~hecked for air leaks. T h e lower esophagus has its own segmental blood s~pply and therefore should be free only enough to allow/approximation of the esophageal s e g m e n t s without tension. T h e upper" segment now is identified and, if necessary, is freed well into t h e neck. T h e thyroc~rvical t r u n k supplies the blood vessels to the u p p e r s e g m e n t and is not interr u p t e d by extensive dissection. I L t h e r e is any difficulty in identifying the u p p e r segment, a catheter can be passed down by t h e anesthesiologist. To avoid a n y u n d u e t r a u m a , the esophagus is never handled with forceps, only w i t h a needle, blunt hook or traction sutures. T h e three methods of esophageal anastomosis generally employed are: (1) a single-layer end-to-end anastomosis using a suture t h r o u g h both musculari~ and mucosa of both u p p e r and lower s e g m e n t s ; (2) doublelayer end-to-end anastomosis using two layers of sutures, one for the mucosa ~a n d one for the muscularis; and (3) Haight's two-layer anastomosis, in which the mucosa of the u p p e r s e g m e n t is s u t u r e d to the full thickness of the lower segment as the i n n e r layer and the imuscularis is pulled down over the inner layer and s u t u r e d to the muscularis of the lower s e g m e n t .76 Since the H a i g h t a n a s t o m o s i s has the lowest incidence of leaks and since

32

a leak may jeopardize the infant's chance of survival, we prefer this type of anastomosis. We prefer to utilize a thin rubber drain down to the anastomosis, a l t h o u g h a small chest tube could easily be utilized. Postoperatively, the order of the day is high humidity, antibiotics and frequent pharyngeal suctioning in order to combat'pos~.operative p u l m o n a r y difficulties. If necessary~ tracheal aspiration utilizing a direct laryngoscopy is performed also. Gastrostomy feedings are begu n on the first or second postoperative day and glucose and w a t e r is allowed by m o u t h on the seventh postoperative day. If the~oral feedings are taken well, formula is offered orally the following day.. If all goes well, the child usually is on full formul~ by m o u t h b y the tenth postoperative day.an d the gastrostomy and ~chest tube can be removed. If a leak occurs, oral feedings are withheld Until the leak has closed and there is no demonstrable pocket at the area of the anastomosis by x-ray examination. The recurrence of the tracheoesophageal fistula now is an exceedingly rare complication that requires reoperation.17s, 17s P r e m a t u r i t y , associated anomalies and p u l m o n a r y complications are the principal, causes of postoperative death. T h e survival of patients with esophageal atresia and tracheoesophageal fistula, is about 6 0 % . 94

T h e primary long-term complication is anastomotic stricture. One or two esophageal dilatations are required postoperatively by m a n y patients. T h e child's symptomatology and not roentgenographic appearance of the esophagus should be used as the criterion for treatment of stricture. I n certain instances, a staged procedure should be considered. ~ T h e y include the p r e m a t u r e infant, operative findings, such as a wide gap between the upper and lower esophageal segments or extremely friable tissue, critically ill infants with pnemnonia or respiratory distress syndrome and the child with serious associated anomalies. Division of the stomach into two pouches with aspiration of the u p p e r gastric pouch and feeding through the lower pouch has been suggested. In our opinion, this procedure is too much of an operation and too long of an anesthesia for these critically ill infants. In addition, f u r t h e r major operating to restore continuity of the alimentary tract is required. We prefer a gastrostomy performed under local anesthesia as the initial procedure. Some 10 days later, utilizing an extrapleural approach, the tracheoesot~hageal fistula is divided and closed. The upper esophageal pouch i s d e c o m p r e s s e d with a surnp tube. Gastrostomy feedings are started and esophageal repair is performed when the patient's improvement has reached~its optimal level. If a prolonged period a p p e a r s to be necessary before the primary anastomosis is performed, a left cervical esophagostomy m a y be carried out. In those instances in which the p r i m a r y operation was incomplete because of the considerable distance between the two portions~of the ~esophagus, the tracheoesophageal fistula is~ divided and closed. Postoperativ~ley, dilatation and elongation of the u p p e r blind pouch by bougienage m a y be attempted. Although this technic i s advocated by some, 97 its true efficacy is doubted by others. TM A l t h o u g h the actual distance between the two portions m a y remain the same, a period o f growth of t h e infant and of the esophageal remnants often will r e s u l t in an easier: anastomosis. I n long-term follow-up Studies following correction of the esophageal atresia with tracheoesophageal fistula, disordered rnotiliiiy h a s been almost uniformly noted. Some studies revealed the disordered m o t i l i t y to be a l m o s t entirely in the segment below the anastomosis. T h e work of others,

33

however, h a s shown the whole esophagus to be involved in the abnormal peristalsis. 113 T h e evidence to date seems to indicate that disordered motility is an inherent defect and not an iatrogenic one.

ESOPHAGEAL ATRESIA WlTHOUT TRACHEOESOPHAGEALFISTULA It is i m p o r t a n t to differentiate between isolated esophageal atresia and esophageal atresia with tracheoesophageal fistula, sinccJ the operative approach is different. T h e symptomatology of the two lesions is the same: excessive salivation, coughing and cyanosis with feeding and respiratory problems. However, because there is no tracheoesophageal communication to allow gastric reflux, p u l m o n a r y difficulties are less severe. Again, the diagnosis is made by encountering an obstruction while attempting to pass a catheter into the stomach and by chest and abdominal x-ray. T h e absence of gas in the gastrointestinal tract associated with esophageal atresia has been reported in patients v~th a tracheoesophageal fistula. However, the combination remains excellent presumptive e~idence that a fistula does not e x i s t . High humidity, antibiotics and a sump catheter in the u p p e r esophageal pouch are utilized to combat pneumonia and atelectasis. T h e gastrostomy is performed shortly a f t e r m a k i n g the diagnosis, but the same urgency does not exist as with a tracheoesophageal fistula (Fig. 13). At the time of gastrostomy, the length of the lower esophagus is estim a t e d by probing with a radiopaque catheter. T h e use of an additional radiopaque catheter a n d / o r contrast medium" in the uppe~ esophageal pouch allows an estimation of the approximate distance between the two portions of the esophagus (Fig. 14). This information can determine if the two portions of esophagus are long enough for a p r i m a r y esophageal anastomosis to be performed wJ'~'hout the need for a thoracic operation to obtain this information. Unfortunately, the lower, esophageal segment usually is not more t h a n 1 or 2 cm in length. W h e n it has been determined t h a t a p r i m a r y repair cannot be performed and the patient's condition allows, a left or right cervical esophagostomy is performed just above the clavicle to the left o f the midline. T h e tip of the b l i n d pouch ~must be adequately mobilized to p e r m i t it to be sutured to the skin without tension, T h e esophageal cutaneous anastomosis is made by excising an ellipse of skin and subcutaneous tissue in a m a n n e r similar to performing an ileostomy. T h e entire procedure almost always can be performed through a neck incision. T h e optimal age for performing an esophageal replacement procedure is a m a t t e r of personal preference. T h e usual age is between 6 and 30 months, w h i l e ' t h e patient gains weight and strength with gastrostomy feedings. Experience has shown t h a t babies with gastrostomies s h o u l d be f e d o r a l l y despite the p r e s e n c e of a cervical esophagostomy. Ideally, the oral feedings should b e given once or twice a day at the same time the gastrostomy feedings are instilled. I f oral feedings are withheld until gastrointestinal continuity is re-established, the infants do not learn to enjoy the act of feeding and they become difficult problems after esophageal reconstruction. A v a i l a b l e for esophagoplasty are jejunum, colon and gastric t u b e . We reserve t h e g a s t r i c tube for cases in which sufficient colon is not available o r not suitable, since there is the p r o b l e m of reflux up the gastric tube in small infants and children. Although some s u r g e o n s prefer the jejunum, we use t h e colon. E a r l y experience consisted of t h e use of a substernally placed segment of colon with the cologastric anastomosis, on

34

Fig. 13.

Esophageal atresia without tracheoesophageal fistula. The course of the catheter confirms the presence of a blind upper esophageal pouch. The absence ;of gas in the gastrointestinal tract indicates the probable absence of a tracheoesophageal fistula.

the anterior gastric wall, T h e main objection to this procedure is the failure to use the distal esophageal remnant with t h e normally present distal esophageal sphincter. At present, we basically follow the technic advocated by Waterston, lss utilizing the left transverse colon with the splenic flexure. At ~times, a separate abdominal incision rather than incising the periphery of "the diaphragm through the chest is utilized: T h e colonic segment and the left branch of the midcolic a r t e r y are isolated; colocolostomy is performed. T h e thorax is entered through the left seventh interspace. A separate defect is created in the diaphragm lateral to the esophageal hiatus. 35

Fig, 14,-:-Esophageal atresia without a tracheoesophagehl, fistula. A retrograde barium study through the gastrostomy tube and the position of a catheter in the upper pouch indicate the length of the atretic segment.

;',/?

.'

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. ~ t.

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Through it, the colon is brought up behind the tail of the pancreas in an isoperistaltic fashion. This defect should be large enough to accommodate the blood supply to the colon comfortably and is sutured sufficiently to prevent small bowel herniation after the segment of colon has been positioned in the posterior left chest. T h e colon is anastomosed to the lower esophagus and brought behind the root •of the lung to the apex of the thoracic cage. The cervical esophagostomy then is freed up and the colon segr~ent is brought up through the thoracic inlets:into the neck. Proximal cervical esophagocolostomy then is performed. We prefer to do both anastomoses in two layers. A s o f t catheter is placed in the colonic segment to prevent overdistention in.the early 36

postoperative period. T h e gastrostomy is also kept open. Gastrostomy feedings are re-established in about 48 hours. T h e tube in the colon is removed in about 3-4 days and oral feedings are begun. When full and adequate intake by m o u t h is attained, gastrostomy feedings are stopped and the gastrostomy is discontinued. TRACHEOESOPHAGEAL FISTULA WITHOUT ESOPHAGEAL ATRESIA

The " H " type congenital tracheoesophageal fistula without esophageal atresia is uncommon. This type of lesion often is overIooked for days or weeks and on occasion for years. 2 Almost invariably there is a hia,tory of coughing with feedings. These infants can handle their saliva more effecFig. 15. A, esophageal atresia with fistula. Initial treatment of gastrostomy and aspiration of upper pouch. B, esophageal atresia with fistula. Definitive therapy: division of fistula and end-to-end esophageal anastomosis. C, esophageal fistula without atresia. Division of fistula via a cervical approach. D, colonic interposition with retention of esophagogastric junction when end-to-end anastomosis is not possible.

%

37

tively t h a n those w i t h esophageal atresias b u t still t e n d to have r e s p i r a t o r y difficulties. Confirmation of the diagnosis m a y be quite difficult. E n d o scopic visualization often is impossible. T h e most effective m e t h o d is to utilize cinefluorography while the p a t i e n t is ingesting r a d i o p a q u e material. Often the lesion c a n n o t be detected on the fluoroscope b u t r a t h e r is noted in reviewing the cine films. M a n y of the isolated t r a c h e o e s o p h a g e a l fistulae occur at a level h i g h e r t h a n those associated w i t h esophageal atresia. T h i s isolated defect can be a p p r o a c h e d e i t h e r thoracically or cervically, d e p e n d i n g on itq position. W h e n it is in the chest below t h e second or t h i r d dorsal vertebra, ~t is most conveniently a p p r o a c h e d t h r o u g h a right transp l e u r a l approach. However, w h e n it is located above the level of the second thoracic vertebra, a cervical a p p r o a c h is feasible. T h u s , m o s t of the fistulae c a n 5e closed by a cervical approach. It is i m p o r t a n t to visualize both sides of the fistula so t h a t a d e q u a t e closure can be performed. A d j a c e n t tissue is interposed as b u t t r e s s over the tracheal end of the closure to help p r e v e n t r e c u r r e n c e (Fig. 15). CONGENITAL ESOPHAGEAL STENOSIS

T h e r e does not a p p e a r to b e a n y good embryologic e x p l a n a t i o n for the occurrence of the congenital intrinsic n a r r o w i n g of the esophagus a n d esophageal web. T h e s e lesions m a y occur a t a n y level in the esophagus, a b o u t half of t h e m occurring in the middle third. I n general, w i t h congenital st enosis, only a s h o r t s e g m e n t of the esophagus is involved. If the n a r r o w i n g is u n u s u a l l y tight, s y m p t o m s m a y be noted w i t h i n the first 2 d a y s of life w h e n the i n f a n t has trouble feeding. Vomiting after 1 or 2 ounces of formula m a y occur, with the possibility of aspiration. Since the early diet is liquid, m a n y infant~ seem to get along fairly well until solids a r e introduced into the diet. It is believed by some t h a t the onset of s y m p tomatology w i t h i n the first 2 or 3 weeks of b i r t h is not related to the c h a n g e in the consistency of the feedings but r a t h e r because there has been constant r e t e n t i o n of ingested m a t e r i a l p r o x i m a l to the obstruction, w i t h progressive reaction and e d e m a l e a d i n g to complete obstruction. 14s T h e diagnosis is confil~ned by esophagogram. T h e esophagus p r o x i m a l to the s t r i c t u r e u s u a l l y ~411 show some degree of dilatation. Because most lesions of this t y p e are short or web-like, one or m o r e careful esophageal dilatations usually will be all t h a t is necessary. At times, it m a y be necess a r y to p e r f o r m a gastrostomy if the lesion is severely obstructed in order to provide the i n f a n t with nutrition. It t h e n is possible to do r e t r o g r a d e dilatations. A silk t h r e a d swall0~ved t h r o u g h the stricture can be retrieved via the g a s t r o s t o m y a n d m a y be used for safer antero- a n d r e t r o g r a d e dilat a t i o n in long or very tight strictures. If dilatation is successful, r e c u r r e n c e is not common, as opposed to the strictures caused by i n f l a m m a t o r y disease. I n a few instances it m a y become necessary to resect t h e lesion. U s u a l l y this c a n be done a n d end-to-end anastomosis accomplished by mobilization of the esophagus. R a r e l y is it necessary to i n t e r p o s e a segm e n t of colon. DUPLICATIONS OF THE ESOPHAGUS

I n m a n y r e s p e c t s , t h e word d u p h c a h o n , is a m m n o m e r , s nce i n the m a j o r i t y o f the lesions a t r u e double esophagus i s a ~very u n u s u a l occur~C

38

"

°

~:P

°

°

i

rence. These remnants from the embryonal state m a y either be cystic or communicate with the lumen of the esophagus. Esophageal cysts probably are the second most common benign neoplasm of the esophageal wall. Since these cysts are mutually related embryologically to both the esophagus and trachea, it is not uncommon that they have a lining of respiratory e p i t h e l i u m - - c l e a r evidence of early communication with the lung bud. It has been reported that malignancy m a y occur in these cysts. The true duplication of the esophagus ("double esophagus,') could also be considered a foregut cyst and usually is lined by stratified squamous epithelium. Twenty per cent of the alimentary duplications occur in the thorax and 60~o of: these occur in t h e lower third of the esophagus. 1~9 The duplication has a commori muscular wall with the esophagus and frequently the lining m e m b r a n e of the cyst is partially composed of gastric mucosa. Thus, aspiration of an intrathoracic cyst and determination of the p H m a y be helpful in identification. T h e acid fluid also accounts for such complications as ulceration, hemorrhage a n d perforation of the duplication. T h e r e is a frequent association of foregut cysts and duplications with vertebral abnormalities and malformations of the spinal cord. 20, 60. 1~9 Such vertebral abnormalities consist of Klippel-Feil deformity, anterior vertebral body defects or spina bifida. Most commonly, the cysts are asymptomatic a n d discovered incidentally on x-ray examination. T h e y m a y enlarge in size and on a rare occasion cause dysphagia. More likely the mass will exert pressure on the bronchial system rather t h a n obstructing the lumen of the esophagus and be a cause of repeated pulmonary infection. The ectopic gastric mucosa may be a cause of bleeding in the duplication of the adjacent esophagus. At times, this hemorrhage m a y be massive. Because of the possibility of hemorrhage or perforation, it is advisable to remove these cysts once the diagnosis is established. In m o s t instances, the duplication can b e enucleatecl without opening the esophageal lumen. At other times, it is not possible to excise them without resection of at least a segment of the associated esophagus. Occasionally there is a diverticulum-like duplication t h a t needs to be excised and the esophagus closed in two layers.

CI-IALASIA Chalasia was first described by Berenberg and N e u h a u s e r 21 as a condition in newborn infants with a p p a r e n t relaxation of the cardioesophageal sphincter mechanism without a hiatus hernia. T h e r e is a free reflu~ of gastric c o n t e n t s b a c k into the esophagus, and in the view of some authors this relaxation of the cardia is considered the earliest form of hiatus hernia.51. 73 These children begin to vomit between the third and tenth days of life. The regurgitation appears to be effortless and occurs when the stomach is f u l l and usually when the child is p u t down after feeding. Immediately after vomiting, these children still are h u n g r y and will readily take a second feeding. This loss of.nutrition occasionally m a y be sufficient to interfere with normal growth and weight gain. T h e symptoms m a y be confused with those of pj, loric s t e n o s i s . T h e diagnosis is made by fluoroscopic and x-ray demonstration of a wide-open esophagogastric junction, with free reflux of barium back and forth: f r o m the stomach to the esophagus, particularly when the baby is recumbent. T h e combination of the relatively low gastric.acidity of infants and the regular infant diet, which 39

i n itself is a good acid neutralizer, probably accounts for the general absence of peptic esophagitis in this condition. T h e t r e a t m e n t consists of propping the infant in an erect position in a small, well-padded chair during a n d after feedings. F r e q u e n t l y this upright position m u s t be m a i n t a i n e d constantly. W i t h the passage of time, usually within a m a t t e r of weeks or occasionally several months,' the s y m p t o m s are entirely relieved. Most of these infants, if re-examined at 2 or 3 y e a r s of age, w i l l have a n o r m a l cardia. Some, however, develop reflux esophagitis a n d a small hiatus hernia. 176

BENIGN TUMORS OF THE ESOPHAGUS Benign tumors of the esophagus are rare. In a systematic study by Moersch and H a r r i n g t o n TM of 11,000 patients suffering from dysphagia, benign tumors were found in only 15 instances. P l a c h t a TM reported only. 90 cases in some 20,000 autopsies performed over a 50-year period, a n d in a n o t h e r autopsy series o f more t h a n 6000 cases, no benign tumors were found.159 Although there are a n u m b e r of ways in which benign tumors of the esophagus have been classified, s°. is0 we have utilized the following anatomic system:

A. Epithelial Tumors 1. 2. 3. 4.

Papilloma Polyps Adenoma Cysts

B. Nonepithelial Tumors 1. M y o m a a. Leiomyoma b. F i b r o m y o m a c. L i p o m y o m a d. Fibroma 2. Vascular tumors a. H e m a n g i o m a b. L y m p h a n g i o m a 3. M e s e n c h y m a l a n d others a. Reticuloendothelial tumor b. L i p o m a c. Myxofibroma d. Giant cell t u m o r e. Neurofibroma f. Osteochondroma

C. Heterotopic Tumors 1. 2. 3, 4. 5. 6.

Gastric mucosa Melanoblastic elements Sebaceous gland G r a n u l a r cell myoblastoma P a n c r e a t i c gland T h y r o i d nodule

Benign t u m o r s of the esophagus characteristically are single, symptomless a n d t h e i r d i s c o v e r y ' o f t e n s e r e n d i p i t o u s ' T h e i n c i d e n c e is slightly 40

higher in males than in females, 17° and the ages range from 22 to 92 years. 9 On rare occasions, the tumors may protrude into the lumen, causing partial mechanical obstruction. ]~PITHELIAL TLrMORS T h e papillomas occur most often i n elderly patients and a p p e a r as an isolated or conglomerate wart-like overgrowth, often the r e s u l t of chronic irritation. Distal esophageal obstruction m a y occur. Histologically, this tumor is characterized by acanthosis due to hyperplasia of the malpighian corpuscles. A normal granular layer usually dominates the picture, but there may b e premalignant changes suggesting adenocanthoma, is° T h e adenomatous polyp is histologically a hyperplastic process involving the epithelial cell, basement membrane and underlying vascular and connective tissues 193 (Figs, 16 and !7). Subgroups are classified according to the predominant histologic component and include: acinous a d e n o m a , intracanalicular vegetative adenoma, p a p i l l a r y adenoma and pseudoacinous adenoma. Various combinations of the above often will intermingle in a single portion of the tumor. T h e polyps a r e chiefly composed of adipose, fibrous or muscular tissue, with scanty s~roma infiltrated by lymphocytes and plasma cells. T h e surface usually is covered by pseudostratified or squamous epithelium. Congenital cysts are thought to be duplications of portions of the primitive gut and were discussed previously. Acquired or r e t e n t i o n cysts result from obstruction of the esophageal glands. Although u s u a l l y small, they m a y grow large and become infected. Fig. 16.--Barium swallow roentgen study demonstrating a polyp in the lower third of the esophagus.

41

Fig. 1 7 . , Survey view of microscopic section (XIO) of entire specimen of squamous papilloma of esophagus. Note the differentiation and papillary arrangement. This lesion was removed endoscopicaily. N O N E P r r I ~ r A L TUMOr,S Myomas are by far the most prevalent of benign esophageal tumors. Virchow published the first pathologic description of leimyo~na of the esophagus in 1867,186 and the first successful operative removal of this tumor was performed by Sauerbruch in 1932. TM A detailed historical background and comprehensive review of the subject can be found in an elegant text by Skandalakis and Gray, iv° who reviewed a total of 390 smooth muscle tumors of th~ esophagus that had been repbrted. More t h a n 85~o were leiomyomata. E i g h t h u n d r e d thirty-eight cases of leiom y o m a h a v e been reported more recently by Seremetis et al. TM from the world literature. ETIOI~Y. Hypothetical etiologic factors that are common to all smooth muscle tumors of t h e gastrointestinal tract include acid pH, embryonic rest, estrogen sensitivity and gastrointestinal mardfestation of von Recklinghausen's disease. T h e etiologic factors directly related to leiomyoma of the esophagus are hiatus hernia, esophageal diverticula 9s and heredity. 16~ Histologically, m y o m a t a consist of smooth muscle fibers arranged in whorls of long spindle cells with eosinophilic cytoplasm surrounded by hypovascularlconnective tissue. Developing in the submucosa, their size m a y progressively increase t o as m u c h as 10 cm. Although they are well 42

ehcapsulated, they m a y partially or completely encircle the organ. T h e y rarely project into the mediastinum. T h e surgical specimen is firm, smooth, gray-white in color, avascular and sometimes calcified. C[JNICAL .~mT~Ja~..---Less than half of patients with benign esophageal tumors are symptomatic. When present, however, symptoms include dysphagia and pain, weight loss, obstruction and passive regurgitation and vomiting. T h e r e may be excessive salivation. F a t a l complications, such as asphyxia and hemorrhage, may occur. Clinical symptoms rarely a r e p r e s ent in small tumors. Large tumors may cause mechanical compression of adjacent structures such as the trachea, dysphagia o r regurgitation and vomiting on the basis of obstruction. F a t a l respiratory complications manifested by sudden respiratory distress and asphyxia m a y be due to airway obstruction by a protruding tumor or compression by a large mass. Less severe respiratory problems, such as asthmatic attacks or stridor, have been noted by Storey and Adams I74 in 12~oof their cases. DIAGNOSIS. - T h e diagnosis of these tumors is based on endoscopy and roentgenography. T h e details of t h e radiologic features have been re. portedJS, 160 Plain roentgenogram of the chest sometimes may indicate the presence of an esophageal tumor. O n fluoroscopy, these lesions usually do not interfere with peristaltic activity, but are detected by barium displacement, obstruction of the flow of contrast material or proximal dilatation of the esophagus. T h e roentgenologic characteristics of benign tumors vary Fig. 18,--Hemangioma of the esophagus. This highly vascular, well-encapsulated tumor measured 2.5 cm in widest diameter and was removed by thoracotomy and esophagotomy.

43

Fig. 19.. :..Survey view of microscopic section (X5) of the hemangioma shown in Figure 18. Note the numerous cavernous vascular spaces separated by a fibrous stroma.

according to their site o f origin. Mucosal lesions are i r r e g u l a r i n outline a n d m a y be polypoid or ulcer~itive. Submucosal t u m o r s are smooth, r a r e l y u l c e r a t e a n d show a s h a r p l y outlined filling defect. E s o p h a g o s c o p y will confirm ~t h e presence of a t u m o r , rule out the presence of m a l i g n a n c y a n d s e p a r a t e mucosal from submucosal abnormalities. E s o p h a g e a l b i o p s y should be avoided w h e n e v e r a h e m a n g i o m a or a l e i o m y o m a is suspected because of the r i s k s of bleeding, u l c e r a t i o n a n d infection. T h e s e m a y e n o r m o u s l y complicate e v e n t u a l surgical intervention. R h a b d o m y o m a is a n exceptionally r a r e s t r i a t e d muscle t u m o r t h a t occurs in the u p p e r t h i r d of t h e esophagus. L i p o m y o m a t a also a r e very rare, u s u a l l y single a n d t e n d to become p e d u n c u l a t e d . V a s c u l a r Lumors, i n c l u d i n g ~h e m a n g i o m a , h e m a n g i o e n d o t h e l i o m a , h a m a r t o m a a n d l y m p h a n g i o m a , p r e s e n t as h y p e r t r o p h y of blood vessels (Figs. 18 a n d 19). S o m e t i m e s t h e y occur with m u l t i p l e h e m a n g i o m a t o s i s or 44

Rendu-Osler~Weber syndrome. Often encapsulated, t h ~ e tumors arise from the submucosal vascular layer. TM Because they m a y cause massive hemorrhage, endoscopy, if performed, m u s t be done with extreme caution. OTHER MESENCHYMAL TuMoRS

Mostly intramural, the clinical picture of these tumors is similar to that of leiomyoma. Malignant or benign, they m a y contain reticuloendothelial, areolar, connective, adipose or myxomatous tissue and sometimes cartilage and bone. HETEROTOPIC TUlVIORS An island of gastric mucosa in the u p p e r third of the esophagus frequently is encountered as a heterotopic tumor. A true heterotopic gastric mucosa is to be differentiated from a displaced gastric m u c o s a , which commonly is found in the lower portion of the esophagus, 2'-' Melanoblastic elements are diagnosed histologically and rarely are of clinical significance. Sebaceous gland tumors have been reported only in autopsy cases. Granular cell myoblastoma, 45 thought to be of fibroblastic origin, is very rare, generally benign and presents mostly as a histologic diagnostic problem (Fig. 20). SURGICAL TREATMENT OF BENIGN TUMORS Although pedunculated polypoid lesions of the esophagus m a y be removed by endoscopy, we generally recommend operative m a n a g e m e n t for the majority of these benign lesions. Endoscopic removal should never be attempted in a submucosal or vascular tumor. Surgical exposure of the esophagus depends on the anatomic site of the lesion. A lesion located in the lower third should be approached throug:: a left thoracotomy whereas lesions in the middle and u p p e r third are best approached through a right thoracotomy. Leiomyomata account for 99°-/0 of i n t r a m u r a l tumors of the esophagus and are best removed by enucleation. Generally, this m a y be accomplished without technical difficulty. The i m p o r t a n t points to keep in mind include preservation of the integrity of the vagus nerve and protection of the esophageal mucosa. Inadvertent damage to the fragile mucosa should be repaired p r o m p t l y by p r i m a r y closure of the esophagus with interrupted nonabsorbable monofilament suture material. Resection of the esophagus is indicated only w h e n an extensive tumor TM cannot be managed b y enucleation, if there has been a recurrence, if disease is diffuse 115 or when danger of malignancy is suspected by the gross appearance of the tumor (Fig. 21).

PERFORATION OF THE ESOPHAGUS GENERAL CONSIDERATIONS.-Perforation of the esophagus i s a serious, life-threatening condition, frequently r e q u i r i n g emergency surgical intervention (Fig. 22). Since the initial description of spontaneous perforation by Booerhave in 1724, 4~ much progress has been made i n t h e diagnosis and t r e a t m e n t of this condition, b u t t h e mortality still is in the range of 25-50%.24. 31, 158. 183 45

Fig, 20, Photomicrograph (X350) of granular cell myoblastoma. Note the uniform arrangement of cells with abundant granular cytoplasm. For the purposes of diagnosis and treatment, esophageal perforation may be classified as follows: I. E x t r i n s i c T r a u m a

A. External injury 1. Penetrating wounds 2. Foreign body 3. Chemical injury 4. Air blast injury 5. Crushing injury 46

B. Iatrogenic 1. Instrumental perforation---esophagoscopy, nasogastric tube, balloon tamponade tubes, dilators, bougies, tracheostomy 12s. ls2 2. Intraoperative accidents--.occurring at time of vagotomy, hiatus hernia repair, cardiomyotomy, pharyngomyotomy, aneurysmectomy, pneumonectomy 17, 146.178 II. Intrinsic Trauma A. Overt strain (post-emetic, parturition, etc.) B. Neurogenic C. Agnogenic--neonatal, smooth muscle hypertrophy, esophagomalacia Fig. 21.--Resected surgical specimen with extensive, multiple leiomyoma of the distal esophagus.

47

PATHOPHYSlOL0OY.--Regardless of the etiologic factor, two important points must be made regarding the pathophysiology and pathogenesis of esophageal •perforation: (1) Anatomic considerations. The esophagus is a very friable organ, devoid of serosa and surrounded by fatty areolar tissue. The posterior distal pharynx and upper esophagus are devoid o f longitudinal fibers of the inferior constx'ictor muscle, leaving the mucosal support primarily to circular muscle fibers. The majority of instrumental perforations occur in this region. A similar anatomic weakness is •present in the distal esophagus, where there is a segmental defect in the circular muscle o n the left posterolateral aspect and less support by mediastinal struc-

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Fig. 23.--The cervical and distal esophagus have an absence of muscular support, which makes them common sites of instrumental perforation.

tures. 49 This is the most common site for spontaneous rupture of the esophagus. Moreover, there is considerable evidence 43 t h a t this portion of the esophagus is subject to preferential adverse pressure effects 3s,112 (Fig. 23). An additional anatomic consideration relates to the cervical and dorsal vertebrae: Positional extremes, as in hyperextension of tile neck, or anatomic abnormalities of the structures may potentiate t h e inherent hazards of endoscopy. (2) Mediastinitis. This is the most severe complication of all types of esophageal trauma. Complete perforation of the esophagus leaves the mediastinal structures wide open to bacterial contamination (often anaerobic), food particles and gastrointestinal secretions. T h e esophagus, along with the other intrathoracic strUctures, is subject to inspiratory and expiratory pressure changes and circulatory pulsatio_ns. These mech~misms m a y enhance permeation of the entire: mediastinum b y bacteria- and enzyme-laderi esophageal secretions when perforation occurs. T h e result m a y b e a p u t r i d necrotizing mediastinitis w i t h associated severe inflamm a t o r y reaction. Moreover, the pleura m a y perforate, producing pleural effusions a s w e l l . Sequestration of fluid in t h e neck, mediastinum, pleural a n d / o r p e r i c a r d i a l c a v i t y m a y lead t o hypovoiemia and severe circulatorY embarrassment. If left untreated, added respiratory d i s t r e s s a n d sepsis usilally will r e s u l t in d e a t h in 2-4 days.: I n Blichert-Toft's review of 221 cases, ~s 9251o of the patients who d i d n o t receive any t r e a t m e n t died within 48 hours whereas 60~o of those who received "conservative" o r delayed trea'tment (over 24 hours) died Within a week. Mediastinitis is not always a diffuse process. On occasion, it m a y r e m a i n localized within a confined fascia/ p l a n e or it m a y . produce a chronic 49

abscess that can erode into adjacent structures or through the sldn 7s. 16s (Fig. 24). CIaNzc~ PICTURE. T h e symptoms of esophageal perforation depend largely on its anatomic location. Perforation of the cervical esophagus usually presents with pain and discomfort in that area. If unrecog.nized, cellulitis and subsequently an abscess m a y develop. Sometimes the downward extension of the infection will result in mediastinitis. Perforation of the thoracic esophagus m a y begin rather insidiously but usually follows instrumentation or vomiting. In 1946, B a r r e t t I2 described the clinical triad consisting of pain a n d guarding in the upper abdomen, severe dyspnea and subcutaneous emphysema, and the first successful surgical , r e a t m e n t of this entity. Pain, usually localized in the left upper abdominal quadrant, is a rather constant symptom, but it m a y also be present in the chest, t h e retrosternal, subdiaphragmatic or s c a p u l a r regions. T h e pain u s u a l l y i s accompanied by severe tightness of the chest, dysphagia and sialorrhea. On physical examination, the patient is acutely ill, toxic and febrile with tachycardia. Cyanosis m a y be n o t e d and will be more pronounced in patients with borderline pulmonary function. Subcutaneous emphysema usually is p a l p a t e d at the base of the neck and in the suprasternal area and may spread rapidly. The fever usually ranges b e t w e e n 102 and 104 degrees F. Some form of dysphonia may b e present. T h e condition of the patient usually deteriorates quickly if left untreated. Associated circulatory and r e s p i r a t o r y failure herald almost certain fatality. DIAGNOSIS.., Thoracentesis often yields a pleural effusion containing food debris and various foreign materials. Recent studies 52 have shown t h a t the p H of the pleural fluid normally is about 7.4. P l e u r a l fluid With a p H of less than 6 is highly suggestive of perforation at the lower end of the esophagus. T h e amylase content of the pleural ifluid may be elevated and this m a y b e incorrectly attributed to pancreatitis. Roentgen examination of the neck a n d Chest often reveals the presence of air i n the neck or

Fig. 24.--Pathogenesis of esophageal perforation. DIRECT iNJURY IATROGENIC SPONTANEOUS

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Fig. 25.- Roentgen demonstration of subcutaneous emphysema and pneumomediastinum. mediastinum and a pleural effusion (Fig. 25). T h e more subtle manifestations of mediastinitis include global enlargement of the mediastinal shadow, cloudiness in the retrosternal space and V or Z signs:~ Roentgenfluoroscopy with the judicious use of water-soluble contrast material should be performed immediately in all cases for a definitive diagnosis and to localize the site of perforation (Fig. 26). This study is particularly important, since the type of perforation may be unusual, s u c h a s intramural, ~09 or i f the clinical :picture mimics a n u m b e r of life-threatening conditions, such as myocardial i n f a r c t i o n , perforated ulcer, necrotizing pancreatitis, p u l m o n a r y embolism or dissecting aneurysm. TR~ATM~TT:mThe time lapse prior to treatment and t h e site of perforation are the most significant factors in the morbidity and mortality.associated with this condition. 24. 53 Initial t r e a t m e n t consists of the correction of s h o c k a n d the administration of l a r g e doses of antibiotics. Often, large amounts of crystalloid solutions are necessary to restore third space losses. W e prefer the broad-spectrum antibiotics, such as cephalosporin, in doses of 8 g i n / d a y given immediately w h e n there is even a suspicion: of perforation. T h e respiratory distress and acid-base balance r e q u i r e p r o m p t attention. Perforations involving t h e cervical esophagus no longer are routinely operated on. These m a y be safely managed by antibiotics; discontinuance 51

Fig. 26.--A perforation of the dist.! esophagus demonstrated by esophagogram. Note the extravasation of the contrast material.

of oral intake and close observation of the clinical course. Immediate drainage is carried out if signs of cellulitis or abscess develop. T h e access to this area is gained through a curvilinear incision medial to the sternocleidomastoid muscle. T h e choice of treatment for perforation of the thoracic esophagus is surgical. We agree with Ravitch ~49 and Loop and Groves 114 t h a t nonoperarive t r e a t m e n t of thoracic perforations is n o t "conservative" but is both radical and imprudent. Operative intemzention consists of early thoracotomy, adequate drainage of the thoracic cavity and protection of the mediastinum from further contamination. A left or r i g h t thoracotomy is performed, depending o n the location of the injury. T h e pleural cavity and m e d i a s t i n u m a r e opened widely, visible necrotic tissue is excised and the area is irrigated thoroughly. A culture is obtained f o r further bacteriologic identification. If the site o f i n j u r y is not apparent, we have found t h a t forcing air through a t u b e in the esophagus usually produces bubbles in a fluid:filled thoracic cavity a t the perforation site. Small and early perforations are best managed with a two-layer closure of t h e esophagus, drainage of ithe t h o r a x and gastrostomy. A large spontaneous perforation will require a n additional supportive patch. Gastric fundus as coverage for the defect,18!: a diaphragmatic pedicle graft147 a n d fundoplication have been employed successfully. Recently w e successfully used: Hi~ashima's gastric patch 93 complemented by fundoplication i n a 36-year-old patient who had sustained a perforation of the t e r m i n a l esophagus following a pneumatic dilatation for achalasia. 52

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Fig. 27. Methods of managing distal esophageal perforations: a) simple two-layer closure and drainage, b) and c) bipolar exclusion, cervical esophagostomy and gastrostomy with closure of the esophagogastric junction, d) resection of obstructed segment with esophagogastrostomy, e)patch of gastric fundus, f)T-tube drainage. The defect may also be patched with g) diaphragm, h) stomach or i)fundus of stomach. If there is a significant delay i n t h e diagnosis of esophageal injury, the likelihood of success by p r i m a r y repair is very small. In most cases, adequate drainage and diversion of esophageal and gastric secretions givesthe best results.: This may include the insertion of a T-tube 1 or bipolar" (cervi' cal esophagostomy and closure :of E - G j u n c t i o n ) , exclusion of the e s o p h a ' gus along with :thoracic drainage and gastrostomy.Z04' !TM Esophagogastrec' tomy 1°4, 149.has been advocated if t h e r e is an: associated severe underlying esophageal stenosis.:We are h e s i t a n t to perform s u c h an o p e r a t i o n in t h e face of major contamination (Fig: 27). Postoperative management i s tedious and complicated. P a r e n t e r a l hyperalimentation 50 has been a:most i m p o r t a n t recent a d v a n c e in the: management of these patients. CHEMICAL BURNS..OF THE ESOPHAGUS E a c h y e a r i n the U n i t e d States .approximately 5000 children under .the a g e of 5 accidentallyswallow •strong alkali preparations, ~usually o n e of the commonly, available drain cleaners: " Ingestion of s u c h material by adults usually is in connection w i t h suicide a t t e m p t s : Because a larger

53

quantity usually is involved u n d e r such circumstances, the injuries are likely to be quite severe and often fatal. Experimentally, a 2~o solution of sodium hydroxide was found sufficient ~o produce a m a r k e d corrosion of the esophageal mucosa. 19° The concentration of this alkali in commercially available drain cleaners ranges from 25,w/o to 60/°go. Clinitest tablets also contain significant amounts of anhydrous s o d i u m hydroxide and m a y be accidentally swallowed by young children, with disastrous effect. 6s Other chemical agents, including acids, rarely are involved. Strong alkalis solubilize protein, saponify fat, dehydrate tissue by binding water and liberate significant quantities of heat while going into solution. In contrast, the coagulation of protein by acids is somewhat selflimited and usually more superficial. However, s t r o n g acids tend to produce greater damage to the stomach because they are not neutralized by gastric juices as are the alkalis. T h e damage seen to the mucosa of the m o u t h and throat may be misleading in evaluating the total quantity ingested and the eventual damage to the esophagus. T h i r d degree esophageal burns have occurred in patients who had only hyperemia Or mild mucosal ulcerations in the mouth. Conversely, others, in particular children, have severe burns of the face or m o u t h and no evidence of esophageal damage on esophagoscopy. 46 The d e g r e e of tissue destruction is proportional to the a m o u n t of alkali ingested a n d is reflected in the clinical condition of the patient. Symptoms range from mild chest pain with dysphagia to severe shock, asphyxia and even coma. Shock m a y be the result of hemorrhage but usually is caused by more subtle factors. Vomiting m a y account for a substantial loss o f body fluid. The epiglottis normally is not exposed to the chemical agents in the initial ingestion; however, during regurgitation, the esophagus is not only re-exposed to the ingested caustic material but also the epiglottis now comes into contact With it. 46, is0 Edematous swelling of the epiglottis m a y p r o d u c e dyspnea, hyperpnea and asphyxia. This m a y be aggravated by copious salivation in response to the chemical stimulation. Frequently there is epigastric burning, with some hematemesis. Hiccups m a y signal perforation or diaphragmatic irritation. Gastric i n j u r y m a y be followed by melena or mucoid diarrhea. Renal complications, when they occur, are a grave prognostic sign. Fever UsUally i s present and m a y persist for days. All degrees of i n j u r y m a y be obser;ced. Minimal changes are hyperemia and swelling of the mucosa (first degree), which resolve in a few days. I n m o r e severe cases, Superficial layers of necrotic tissues become detached, formin,g a "pseudomembrane" not unlike that seen in cases of diphtheria. At times, t h e entire esophageal m.ucosa m a y slough off as a cast. Beneath, there m a y be ulcers corresponding in severity to second and t h i r d degree burns ~of the skin,: T h e second degree burns involve only t h e epithelial layer, with possible blister f o r m a t i o n , A ~fibrous exudate usually is present as Well. Such lesions heal _rapidly provided that the process is not impeded by mechanical ~irritation. Deep ulcers can affect all layers of the esophagus and even perforate the wall and possibly lead to mediastinitis or hemorrhage as well. Similar catastrophic events may occur to the stomach. Healing occurs by scar formation. If the patient survives the initial episode, he almost'surely will develop varying degrees of esophageal stricture. Months o f hospitalization m a y be required before the victim regains his swallowing function.

54

DIAGNosis.---The principal technics of evaluating the extent of esophageal injury a r e roentgen examination and esophagoscopy. A barium swal. low is an accepted method of evaluation. During the early phase of injury, edema of the mucosa and submucosa slows the passage of barium through the lumen, and areas of spasm m a y be easily identified. In addition, barium m a y outline sites of ulceration or indicate points of perforation. Esophagoscopy is a more hazardous procedure but m a y be required if there is any doubt regarding the severity of the lesion. An experienced endoscopist, using one of the newer fiberoptic esophagoscopes, usually can perform this examination without complications. Esophagoscopy in "the early phase is useful to delineate the extent of tile chemical burn and to grade its severity. 44 A number of authors advocate the use of early esophagoscopy a s a diagnostic method of choice. 42, 46, 11~ However, as endoscopy is .only diagnostic, no a t t e m p t s h o u l d be made to advance the esophagoscope beyond the first visible ulceration, edema or mucosal abnormality. 1°5 F r o m that point on, the extent of mucosal a b n o r m a l i t y should be evaluated by esophagogram. W h e n e v e r the patient's clinical picture permits, early esophagoscopy (within 24--48 hours) provides a positive diagnosis of the degree of injury and allows early therapy. Terrocol and Sweet is° believe that esophagoscopy should not be attempted until a t least 10-11 days after the injury. By this time, granulation tissue is forming and there is less danger of perforation; scarring has not yet begun and there still is time to start bougienage if indicated. Webb 189 and others 34 have demonstrated that there is a good correlation between the estimated degree of the initial burns and the formation of subsequent stricture. Some degree of stricture will develop in 80-100~o of the cases with third degree burns, usually within 3-4 weeks after the injury. We reserve the use of esophagoscopy for selected cases. In our opinion, the risk of injury in the use of indiscriminate early esophagoscopy outweighs the potential advantages. T R ~ T ~ N T . - - A l k a l i granules tend to adhere to the mucous membrane of the mouth or pharynx and esophagus, causing vascular thrombosis and liquefactive necrosis. Copious amounts o f bland liquids such a s milk should be ingested to wash away the chemical. T h e alkali is diluted and is relatively harmless in the stomach. However, if the quantity swallowed is excessive, acute gastric perforation is a definite threat. Vomiting should not be induced because of the danger of possible aspiration as well as reexposing the esophageal mucosa to a second dose of chemical agents. E a r l y complications include massive hemorrhage and shock, which will require volume replacement and possible immediate surgical intervention. E n d o t r a c h e a l intubation m a y become necessary if the edematous p h a r y n x and epiglottis cause airway obstruction. Gago and hiS associates 66 recommend immediate celiotomy i n cases in which massive necrosis or perforation is suspected. This approach can provide important information concerning the viability of t h e esophagus, stomach a n d surrounding tissues and is a good s t a r t i n g point for subsequent radical procedures, such as esophagectomy and gastreCtomy. In our experience, this type of surgery rarely is indicated. T h e use of antibiotics has been advocated for routine treatment; however, the danger of overgrowth by resistant bacteria or fungi should be taken into consideration. NearLy all authors are convinced t h a t early u s e of massive doses of c0rtieosteroids has an i n h i b i t o r y effect on

55

Fig.: 2 8 , Extensive pneumonitis and multiple lung abscesses in 26-year.-old female with tracheoesophageal fistula following lye ingestion.

scar formation.- These: steroids, should be-administered very early (within the:!first 48!hours) in order to.have: their maximal effect. 19e Despite:satisfact0~::~ initial: .treatme~t,:~:some patients .will: develop-~late comp~cations:,such as:tracheoes0phagea]:fistula; lung abscessi;es0phageal stricture: and.malnutrition,: (Figs~ 28-31). Most:: aUth:0rs.egree that if early.:bougienage, is to be.. utilized,:.:it ishould be started aroUnd::iday: 10,12i :~at:.vchich;-:.time the esophageal:wall :.has::.re: gainedsome !of:its. strength: bUt still~:.~:ispliable:enough to :.permit bougien:. age: ,;Belin0ff~: !..andlothers..:believe::~:that~(:idilatation :j~ll:.i::alter::.the.:i:eiasti c. properties~:;of '~;:the!.::fibroblast,~:..but::;:::this;~has ~::n0t,:been :.-well: documented. B 0 U g i e n a g e : m u s t - : b e ~ 0 ~ e d :~th great:.Care:: sinceany:inew:inju~ will be .:followed by:increased scarring and further:contractions, ReC~nt experimental studies havebeen directed:: toward the pharma56

cologic prevention of esophageal stricture. Beta amino proprionitrile (BAPN) was administered to dogs w h o developed severe corrosive esophagitis following instillation o f sodium hydroxide/~4, 46, 11~,i~s B A P N is a powerful lathyrogen that inl~ibits t h e action of ]ysyl oxidase, thus: preventing collagen cross, linking. The collagen thus fol~ned a p p e a r s histologically normal but yields quite easily to mechamcal stretching, w i t h some decrease in tensile strength. Agents such as B A P N or penicillamine or a combination of both may prove useful in the prevention of esophageal stenosis, Recent experimental work b y Reys TM suggests that treatment

Fig. 29.--Esophagogram of patient shown in Figure 28 demonstrating tracheoesophageal fistula. Note leftbronchogram.

57

Fig. 30.--Barium swallow 6 months after corrosive esophagitis. Note the long segment of stricture. with an i n t r a l u m i n a l esophageal silicone splint for 3 weeks after fullthickness esophageal b u r n is an: effective method of preventing stricture formation. T h e r e are several isolated reports of esophageal achalasia following lye burns. 125 Carcinoma of t h e esophagus originating in t h e s c a r has been repOrted:iS, 69 The interval between lye ingestion and development of carcinoma usually is a t least 20 years. T h e clinical symptoms are attributed t~ the lye burn, and m a l i g n a n c y i s n o t suspected until very late. Removal of the::strictured segment of the esophagus as a prophylactic procedure has been considered° 58

Surgical excision and replacement of esophageal stricture m u s t be resorted to whenever all a t t e m p t s at dilatation fail. ~4 Stomach, reverse gastric tube, small bowel and l a r g e bowel have been utilized as an esophageal substitute. In our experience, the most satisfactory organ for replacement is the colon. Previous b a r i u m enema and mesenteric a r t e r i o g r a p h y to evaluate the a n a t o m y of t h e colon are helpful in deciding w h e t h e r t h e left or right ~side should be used. We favor the left colon when possib]e, since it is easily mobilized and its blood supply generally is adequate for cervical anastomosis w i t h o u t fear of necrosis. T h e colon is brought u p through the chest either in front of or behind the sternum. For benign diseases of the esophagus, we generally prefer the retrosternal position. T h e techniFig. 31.--Retrograde barium study of a 36-year-old female 1 year after lye burn of the esophagus showing proximal stricture and a fistula.

59

cal aspects of this procedure have been well outlined ss, 142, 18~; however, meticulous attention to detail, m a i n t e n a n c e of adequate blood s u p p l y (particularly an intact venous system), absence of tension and a well-sealed anastomosis are very important.

MALLORY-WEISS SYNDROME I!a 1929, Mallory and Weiss 121, 191 first described a syndrome consisting of (1) massive h e m a ~ m e s i s , (2) forceful, incoordinated vomiting and (3) a longitudinal laceration of the gastroesophageal mucosa. Although the true incidence of u p p e r gastrointestinal bleeding directly •attributable to this syndrome is not known, some investigators believe it to be as high as 3--5% of all patients presenting with h e m a t e m e s i s J 38 Forty-six per cent of the patients in a recent collected series required operationJ 97 Vomiting, p a r t i c u l a r l y if repetitive, almost always i s a s s o c i a t e d with the occurrence of this syndrome. Initially, ":,Jute alcoholism was thought to b e the only precipitating factor. In recent years, several other disease states, including peptic ulcer, pancreatitis, gastric tumor, pernicious vomiting of pregnancy, 139 Uremia, gastroenteritis and migraine headaches have been implicated: Rarely, massive bleeding m a y occur without a previous vomiting episode. Generally, in these r a r e instances, there is a history of paroxysmal coughing, ~9 singultus, status asthmaticus, epileptic seizures or closed chest massage. 116 Mallory and Weiss concluded t h a t the mucosal tear was produced by a unique set of c i r c u m s t a n c e s - - v o m i t i n g , regurgitation of gastric contents into a spastic esophagus and contraction of the d i a p h r a g m all combining to create an abnormally high pressure in the lower esophagus. Recent investigations b y Atkinson and Betrill 7 tend to ~support this hypothesis. In a study on cadavers, they found t h a t a constant intragastric pressure of 150 m m Hg, with the esophagus occluded, was necessary to produce lacerations consistently at the esophagogastric junction. T h e y have also found t h a t in normal healthy adults the intragastric pressure• m a y be as high as 200 m m H g during retching. Some • authors s6' 51 have related these mucos a l tears to the•presence of p r e v i o u s l y undiagnosed hiatus hernia. T h e y have postulated t h a t the relatively decreased intrathoracic Pressure i n creases the pressure within the herniated p o r t i o n o f the stomach, leading to more dilatation and eventually to mucosal disruption. Gross exarmnation of the gastroesophageal junction u s u a l l y r e v e a l s a single 1Anear t e a r at t h e most d i s t a l p o r t i o n of the esophagus o r straddling the e s o p h a g o g a s t r i c junction. Multiple a n d irregular tears, varying i n d i a m e t e r between a f e w millimeters a n d several centimeters: m a y occur (Fig. 32). DIAGNOSIS. Diagnosis should be suspected in those instances in which patients have experienced a severe episode of v o m i t i n g a n d retching followed by hematemesis, particularly during a b o u t of acute alcoholism. B a r i u m swallow r a r e l y is helpful 35 a n d m a y waste precious time. ~¢Ve discourage this method :of investigation i n the face o f acute hematemesis because residual b a r i u m m a y obscure future arteriographic findings. Esophagosc0py m a y be very helpful in visualizing the sites of bleeding and in evaluating associated esophageal abnormalities, m However, this study m a y b e l i m i t e d because of severe bleeding, and t h e r e is also the h a z a r d Of extending the mucosal tear. In 1963, N u s b a u m and B a u m 13s, 1~4 60

Fig. 32,--Gross specimen of Mallory-Weiss syndrome. established t h a t conventional selective arteriography of the celiac and mesenteric arteries can demonstrate sites of gastrointestinal bleeding with extravasation rates of blood as low as 0.5 ml/min. Besides establishing the primary diagnosis, this study often will provide valuable information regarding the status of portal hypertension in an alcoholic patient. W e have found this technic extremely helpful in the management of esophagogastric lacerations. TRF_~TI~ENT.--The choice of treatment depends on the: severity of bleeding, t h e n a t u r e o f associated diseases a n d the g e n e r a l condition of the patient. T h e initial clinical evaluation : o f the p a t i e n t should b e followed promptly by volume replacement, protection of the Upper airway from aspiration TM a n d adequate h e m o d y n a m i c monitoring: Coagulation deficiencies should be corrected as soon a s possible. Esophageal balloon tamponade is ineffective 95 and dangerous, and should be avoided. T h e stomach should be emptied by vigorous lavage with iced saline after carefully

61

Fig. 33.--Arteriogram of patient with massive hemorrhage from ,mucosal tear at esophagogastric junction, Note extravasation of contrast material. (Courtesy of Moreye Nusbaum, M.D.) inserting a moderate-sized nasogastric tube. T h e selective infusion of the left gastric artery with vasopressin has controlled bleeding in a significant number o f patients. T h e details of this technic were outlined by Nusbaum. 133 We recommend arteriographic examination for the diagnosis of the site of bleeding, with a therapeutic trial of vasopressin, in a suitable candidate with gastrointestinal hemorrhage (Figs. 33 and 34); i.e., all patients whose general condition and rate of bleeding allow• adequate time to use a therapeutic trial of vasopressin. Operation is indicated i n three circumstances: (1) Life-threatening hemorrhage (losses in excess of 500 m l / 8 h r after initial replacement). (2) R e p e a t e d bleeding episodes, despite adequate therapy. (3) Possible esophageal perforation. : T h e usual operation for gastric bleeding :begins w i t h a high gastrotomy, packing of the pylorus, fundus a n d esophagogastric junction and t h e n careful inspection of e a c h of these areas: A preoperative: arteriogram aids greatly in the rapid identification of the •bleedi n g site, Small, inapparent tears m a y be •easily overlooked and occasionally have resulted in postoperative fatalit~y.6~ C l o s u r e o f the mucosal rent is best accomplished using a continuous running lock suture, with generous deep bites, starting a t t h e m o s t distal point of the tear. Some authors a t t e m p t to control possible eti0logic f a c t o r s b y performing a vagotomy and pyloroplasty, but this has not been our general rule. 62

Fig. 34.--Arteriogram of same patient as in Figure 33 after selective left gastric artery perfusion of vasopressin wi.th cessation of bleeding. (Courtesy of Moreye Nusbaum, M.D.) INFLAMMATORY DISEASES OF THE ESOPHAGUS

Involvement of the esophagus by inflammatory agents is extremely rare and generally does not present a major surgical problem. However, recog, nition of these various entities is important in: the differential diagnosis and management of esophageal diseasesi W e shall briefly discuss the most important aspects of these diseases. TUBERCULOSIS OF THE ESOPHAGUS

The first diagnosis of this disease is credited :to Denovilliersin 1837:lsz Generally. :it is seen i n patients with far-ad~mnced pu_]monary :tuberculoSis and its pathogenesis ican ibe:attributed: to' the following factors: (1)i/SwalloWing/ a large number of tuberculous bacilli, a: few of which m a y penetrate through the esophageal mucobal barrier:: (2) Retrograde: lymphatic spreatt from :involved bronchoesophageal lymph :nodeS::(paraesophageal tubercu. 10sis) .:! (3):: Direct:: continuity: from spinalabscesses (Port's disease): `as reported by: Maillet.i2°: (4): Finally, esophageal tuberculosis result from blood,borne metastasis. 63

Grossly, the lesions m a y be hypertrophic, granular or ulcerative. 59 T h e clinical picture depends on the severity of the disease. Pain and dysphagia are the most common symptoms and simulate carcinoma of t h e esophagus. Roentgen a n d / o r endoscopic evaluation usually foretell the correct diagnosis, which is confirmed by bacteriologic and histologic examinations, On esophagography, 4 spasm, alterations in the mucosal pattern or stricture formation m a y not be differentiated from similar findings in other esophageal diseases. Complications of tuberculous esophagitis are obstruction and tracheoesophageal fistulae. T r e a t m e n t consists of antituberculous medications. However, in hypertrophic esophageal tuberculosis with obstructive dysphagia or complicated forms of esophageal stricture or fistula, surgical treatment is indicated. 10, 13 MYCOTIC INACTION OF THE ~SOPHAGUS Esophageal mycosis is a significant clinical problem t h a t appears with greater frequency t h a n realized previously. 182 T h e most commonly isolated pathogens are Candida and Actinomyces. PRIMARY ESOPHAGEAL MONm

IS

T h e incidence of this disease is much higher than usually suspected, 165 particularly i n individuals with diabetes mellitus, tuberculosis, blood dyscrasias a n d neoplasms. It is also found in patients receiving therapeutic agents such as steroids, broad-spectrum antibiotics and immunosuppressive drugs. T h e most common presenting complaint usually is dysphagia, and 5 0 ~ of the patients in Jensen's study l°s also had odynophagia. Other associated symptoms such as epigastric and substernal pain have also been reported. T h e diagnosis often is established by b a r i u m swallow and esophagoscopy. Roentgen findings include the presence of a shaggy, ulcerated mucosa,, commonly in t h e lower o r middle third of the esophagus. N o d u l a r filling defects produced by edema, ulceration or actual colonies of Candida on the surface of the esophageal mucosa m a y be noted. ~4 These findings m a y be associated with o t h e r abnormalities of spasm, diminished peristaltic activity and segmental narrowing s2.96 (Fig. 35). On endoscopic examination, a yellowish exudate covering an area of d i f f u s e esophagitis with hyperemia and u l c e r a t i o n m a y be s e e n . T h e mucosa i s v e r y friable and m u l t i p l e nodularities often a r e visible. Pathologic examination reveals pseudohyphae w i t h i n the pseudomembrane and underlying tissue. TM Mycostatinlis the t r e a t m e n t of choice. A m p h o t e r i c i n B is reserved for systemic forms of t h e disease. Operation rarely is indicated. ACTINOMYCOSIS OF THE ESOPHAGUS

Actinomycosis of the esophagus m a y develop as a primary infection following t h e inadvertent ingestion of contaminated material or as a seco n d a r y invasion caused e i t h e r :by d i r e c t extension Of the disease:from a nearby s t r u c t u r e or b l o o d b o r n e from a distant s i t e of infection. T h e i n i t i a l phase of esophageal actinomycosis usually is insidious, with mild dysphagia. T h e diagnosis usually is confirmed b y t h e multiple abscesses a n d ulcerations o f the mucosa in a very irregular pattern seen d u r i n g esOphagosco:py. Obstruction and bleeding seldom occur: T h e biopsy specimen histologically reveals the characteristic sulfur granules, inflam64

Fig. 35.wEsophageal ' moniliasis. Note the shaggy, ulcerated mucosa with nodular filling defects and segmental narrowing.

matory cells and granulomas. This infection can spread widely and may involve: vertebrae, pleural cavity and pericardium with fistulae formation,i40, 194 The usual treatment:ismedical; however, complications such as abscesses and fistulae m a y require surgicalintervention. REGIONAL ESOPHAGIT]S

Regional, esophagitis is rare. rl~rina e t al,lS51 report only1 case in which t h e involvement0f t h e esophagus Was~ia part of g e n e r a ] i z ~ regional enteritis o f t h e entire a l i m e ~ r y tract. Recently, Madden and P~vid 11s 65

reported a series of 3 cases. The clinical picture usually is one of esophageal stenosis and presumably is due to extensive submucosal fibrosis. These lesions probably start at the level of the esophagogastric junction, progress intramurally in the cephalad plane and finally penetrate the mucosal layer. Ischemic ulceration of the mucosa has occurred occasionally. Our only experience with this entity was a patient with diffuse involvement of his alimentary tract. He developed a clinical pictpre of esophageal stenosis that subsequently was'complicated b y tracheoesophageal fistula and lung abscess. Sections from his esophagus showed a nonspecific granulomatous inflammation. At the moment, information regarding this entity is very limited and awaits further clarification.

ACKNOWLEDGMENTS The authors wish to express their gratitude to the following individuals, who provided some of the material used to illustrate this manuscript: A. V. Valdes-Dapena, M.D. and Patricia Gureghian, M.D. of the Graduate Hospital of the University of Pennsylvania and Thomas J. Spackman, M.D. of the Children's Hospital of Philadelphia. Figures 16 through 20 are reproduced with the permission of W. B. Saunders Company, Philadelphia.

1. 2. 3. 4. 5. % 7. 8.

9. 10. 11o 12. 66

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114. 115. 116. 117. 118. 119. 120.

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Diagnosis and surgical management of benign diseases of the esophagus.

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