ESOPHAGO ESOPHAGEAL FISTULA IN A PATIENT WITH ACHALASIA

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1 GASTROENTEROLOGY Copyright 1970 by The Williams & Wilkins Co. Vol. 58. No.2 Printed in U.S.A. ESOPHAGO ESOPHAGEAL FSTULA N A PATENT WTH ACHALASA C. MCHAEL KNAUER, M.D., WLLAM T. MCLAUGHLN, M.D.,.\ND JAMES B. D. MARK, M.D. Departments of Medicine, Radiology, and Surgery, Santa Clara Valley Medical Center, San Jose, California, and the Department of Surgery, Stanford University School of Medicine, Stanford, California A 62-year-old female presented with a previously unreported complication of achalasia, an esophago-esophageal fistula. The detailed evaluation of this patient's esophagus is described and the value of esophageal cine-roentgenography in delineating the fistula and the aperistalsis is noted. The surgical management of this lesion is reported. Achalasia of the esophagus is a neuromuscular disorder of the swallowing mechanism which leads to dysphagia and nutritional problems. - 3 More serious complications may occur; these include aspiration pneumonia, esophagitis, bleeding, a higher incidence of esophageal carcinoma, and esophageal perforation. 4-7 n this communication we describe a previously unreported complication of achalasia, an esophago-esophageaj fistula. Case Report E. K., a 62-year-old female, was first seen on June 10, 1968, for evaluation of persistent epigastric discomfort and a sensation of substernal fullness and dysphagia of 5-year duration. The dysphagia was particularly noticeable on ingestion of meat and became prominent in the 3 to 4 months prior to admission. The patient denied melena, emesis, and substernal chest pain, and had never had pneumonia. She had been treated for rheumatoid arthritis of the hands, hips, and knees for at least 10 years and had taken various medications, including corticosteroids and salicylates. n the several weeks prior to our first evaluation, prednisone had been Received April 7, Accepted September Address requests for reprints to: Dr. C. Michael Knauer, Department of Medicine, Santa Clara Valley Medical Center, San Jose, California The authors are grateful to Dr. Richard Osborne for performing the esophageal motility and manometric studies on this patient. gradually discontinued and acetylsalicylic acid had been substituted at a dose level of 2.4 g per day. The review of systems was noncontributory. The patient had had no cardiorespiratory or genitourinary symptoms and denied specifically the symptoms and signs associated with Raynaud's phenomenon. Physical examination revealed a well developed, elderly white female who was a poor historian. Vital signs were normal. Physical findings were essentially within the limits of normal except for thin, pale skin, a right epitrochlear lymph node, and decreased range of motion of the elbows, shoulders, hips, knees, and ankles. Laboratory studies including lupus erythematosis preparations, antinuclear antibody titer, serum creatine phosphokinase, electrolytes, and creatinine were normal except for a hematocrit of 36%, sedimentation rate of 27 mm per hr, and 20 to 35 white cells per high powered field on microscopic examination of the urinary sediment. A chest X-ray taken shortly after admission showed an infiltrate in the upper lobe of the right lung, dilation of the thoracic esophagus, and changes in the shoulder joints typical of rheumatoid arthritis. An upper gastrointestinal series confirmed the presence of a dilated esophagus which had a sigmoid appearance at the distal end, a possible esophago-esophageal fistula between the two adjacent segments of the esophagus, a "beaking" of the distal esophagus, and a duodenal ulcer (figs. 1 and 2). On introduction of a Hehfuss tube into the stomach for gastric secretory studies, under fluoroscopic control, the tube was observed to descend straight through the esophagus rather than around the sigmoid curve. Cine-roentgen studies of the esophagus 223

absence of primary peristalsis of the esophagus. A Mecholyl test, FG. 3.

Upper gastrointestinal roentgenogram demonstrates the dilated redundant

2 224 CASE REPORTS Vol. 58, No.2 confirmed the presence of an esophago-esophageal fistula as well as the absence of primary peristalsis of the esophagus. A Mecholyl test, FG. 3. Gross surgical specimen with the cephalad margin just proximal to the area of fistulization. The arrow points to the fistula, the other probe indicating the normal esophageal lumen. FG. l. Upper gastrointestinal roentgenogram demonstrates the dilated redundant esophagus. The arrow points to the esophago-esophageal fistula. FG. 2. Spot films from the upper gastrointestinal series showing the characteristic beaking of the distal esophagus. FG. 4. The surgical specimen opened with probes located as in figure 3. The arrow is directed at the mucosal demarcation, indicative of the area of fistulization.

Swallows are indicated by the horizontal bars at the bottom of the figure.

3 February 1970 CASE REPORTS 225 r; + + T + ~ ~ ~ r r r r' t r r T + + FG. 5. Motility study of the esophagus demonstrating aperistalsis. The simultaneous vertical deviations of the motility tracing represent artifacts secondary to moving the catheter assembly 1 cm orad. Swallows are indicated by the horizontal bars at the bottom of the figure. performed during the cine-roentgen study, resulted in considerable substernal chest pain but only a minimal increase in the motor activity of the esophagus. An attempt at esophagoscopy was unsuccessful because of the marked immobility of the patient's temporomandibular joints. Cultures of an aspirate from the distal esophagus revealed a heavy growth of Klebsiella. On June 18, 1968, an abdominal laparotomy was performed under general anesthesia. A scarred duodenal bulb was present. The distal esophagus was markedly narrowed, and the esophagus proximal to it was dilated to 4 cm in transverse diameter. Digital dilation of the esophagogastric junction was readily accomplished through a high gastrotomy incision. The distal esophageal mucosa was normal in appearance. Approximately 4 to 5 cm above the narrowed area, the normal opening of the esophagus was seen to deviate to the patient's left. On the right side of the esophageal lumen a second opening could be seen, confirming the presence of an esophago-esophageal fistula (figs. 3 and 4). A distal esophagectomy with an esophagogastric anastomosis, vagotomy, and pyloroplasty were performed. The patient tolerated the surgical procedures without difficulty and' she had an uneventful postoperative course. She was restarted on acetylsalicylic acid in a dose of 2.4 g per day for her rheumatoid arthritis. One year postoperatively the patient was reevaluated. On X-ray examination, moderate narrowing of the distal esophagus was evident, which on esophagoscopy, with a fiberoptic esophagoscope, was found to be due to exudative esophagitis. Motility and manometric studies of the esophagus, utilizing the constant infusion technique with the catheter openings 5 cm apart; were performed. The motility studies revealed aperistalsis of the esophagus (fig. 5) as there was no response to swallowing. The findings of the manometric studies included the absence of an esophagogastric sphincter, as would be expected from her surgery, and a positive Mecholyl test (fig. 6) which included severe chest pain necessitating the use of atropine to interrupt the response. The rheumatoid arthritis was controlled with 1.8 g of acetylsalicylic acid per day.

226 CASE REPORTS Vol. 58, No.2 -- 1..., -i, --, --.-- ~ N [ l.., -. rs: ft ~ P ~ : 1'1, ~ ~ y- ~ ~ t rn : ~ f' t fl 't - f!f rrl H ~ '. ' i!. A ~... 1l1lCA1D ""'"'" -il;..,.. FG. 6.

A pressure scale is superimposed on the tracing of the distal-most catheter. Each new level represents lo-cm water pressure.

4 226 CASE REPORTS Vol. 58, No , -i, --, ~ N [ l.., -. rs: ft ~ P ~ : 1'1, ~ ~ y- ~ ~ t rn : ~ f' t fl 't - f!f rrl H ~ '. ' i!. A ~... 1l1lCA1D ""'"'" -il;..,.. FG. 6. ~ a n o m estudy t r of i c the esophagus illustrating the positive Mecholyl response and its termination with atropine; the tracing rate is 1 mm per sec. A pressure scale is superimposed on the tracing of the distal-most catheter. Each new level represents lo-cm water pressure. The Mecholyl was administered 75 sec prior to the first major deviation from the base line. No voluntary swallows occurred during this test. ' ;. Discussion The diagnosis of achalasia of the esophagus is established by the history of dysphagia and by barium contrast studies showing the beaking of the distal esophagus, proximal esophageal dilation, and the absence of primary peristalsis. A capacious esophagus with an esophagogastric junction that is readily traversed on esophagoscopy provides diagnostic support. Esophageal manometry and motility studies are confirmatory, as is the Mecholyl test.s-lo Because of the inability to visualize the esophagus directly at the initial evaluation of this patient, we were dependent upon cineroentgenography to define the loss of primary peristalsis, to visualize the beaking of the esophagus, and to clearly show the presence of the esophago-esophageal fistula. The pathogenesis of the esophago-esophageal fistula in this patient cannot be definitely determined. With the prominence of the sigmoid portion of the esophagus, stasis and esophagitis may have led to fistula formation between the adjacent loops. Because of the relationship of these esophageal loops to each other, it is also possible that aspirin particles or tablets lodged in the dependent portion of the proximal loop could have been responsible for ulceration and eventual fistulization into the distal loop. The corticosteroids could have contributed to the fistula formation because of their antiinflammatory properties and influence on the composition of mucus.l1 A sharp foreign body could have initiated fistula formation. t is possible that this patient did not have achalasia, but instead aperistalsis of the esophagus in association with rheumatoid arthritis; this could lead to peptic esopha'-

5 February 1970 CASE REPORTS 227 gitis, stricture, and the ensuing complications. However, this is unlikely because of the absence of Raynaud's phenomenon.12 Furthermore, while there was no operative evidence of esophagitis or stricture, there were findings typical of achalasia. No evidence of vasculitis was noted in the surgical specimen on histological examination. The most likely antecedent factors for the fistula were a combination of achalasia with the sigmoid esophagus, stasis, and the salicylate medication. This patient required relief from the functional obstruction of the distal esophagus because of her symptoms and poor nutrition, as well as her complications of esophageal stasis, pneumonia, and esophago-esophageal fistula. Her continued need for pharmacological agents to maintain control of the rheumatoid arthritis was evident. Pneumatic dilation of the esophagus as therapy was considered and has been reportedly successful in patients with achalasia who had a sigmoid esophagus.13, 14 n this patient we felt that the presence of the esophago-esophageal fistula was a contraindication to pneumatic dilation because the procedure might disrupt the fistula and result in esophageal perforation. The surgical treatment of achalasia is usually reserved for failures of pneumatic dilation.15-l7 The current procedure of choice is modified Heller myotomy.17-2l This surgical procedure interrupts the functional obstruction at the esophagogastric junction by severing the circular muscle fibers in this area. The presence of the fistula in this patient necessitated resection of the distal esophagus; the redundancy of the remaining esophagus permitted an intraabdominal esophagogastrostomy. This surgical procedure is used infrequently for the treatment of achalasia, partly because of the prominent reflux which ensues. t should be noted that the incidence of reflux following the modified Heller procedure is also high and varies from 15 to 36%.22 n the 9 months following the operation the patient has been free of dysphagia. Her rheumatoid arthritis is adequately controlled with acetylsalicylic acid in a dosage of 1.8 g per day. She has been maintained on a program which includes elevation of the head of the bed on 6-inch blocks, nocturnal ingestion of antacids, and small frequent feedings, in order to minimize the deleterious effects of gastroesophageal reflux. REFERENCES 1. Kramer, P., and F. J. ngelfinger Cardiospasm, a generalized disorder of esophageal motility. Amer. J. Med. 7: Casella, R. R., A. L. Brown, Jr., G. P. Sayre, and F. H. Ellis, Jr Achalasia of the esophagus: pathologic and etiologic considerations. Ann. Surg. 160: Ellis, F. G The natural history of achalasia of the cardia (abridged). Proc. Roy. Soc. Med. 53: 6 ~ Seaman, W. B., J. Wells, and C. A. Flood Diagnostic problems of esophageal cancer-relationship to achalasia and hiatus hernia. Amer. J. Roentgen. 90: Hessler, C., Carcinoma of the esophagus and achalasia. J. Einstein Med. Cent. 11: Katz, R. 1., and R. R. Hughes Carcinoma of the esophagus in association with achalasia. Dis. Chest 46: Benedict, E. B., and H. C. Grillo Spontaneous rupture of megaesophagus in achalasia. J. Thorac. Cardiovasc. Surg. 44: ngelfinger, F. J The physiologic background of heartburn, esophagitis and cardiospasm. Arch. ntern. Med. (Chicago) 105: Code, C. F., B. Creamer, J. F. Schlegel, A. M. Olsen, F. E. Donoghue, and H. A. Andersen An atlas of esophageal disease, pp Charles C Thomas, Publisher, Springfield, ll. 10. Kramer, P., and F. J. ngelfinger Esophageal sensitivity to Mecholyl in cardiospasm. Gastroenterology 19: Menguy, R., and L. Desbaillets The gastric mucous barrier: influence of proteinbound carbohydrate in mucus on the rate of proteolysis of gastric mucus. Ann. Surg. 168: Stevens, M. B., P. Hookman, C. 1. Siegel, J. R. Esterly, L. E. Shulman, and T. R. Hendrix Aperistalsis of the esophagus in patients with connective tissue disorders and Raynaud's phenomenon. New Eng. J. Med. 270: Kurlander, D. J., H. F. Raskin, J. B. Kirsner, and W. L. Palmer Therapeutic value of the pneumatic dilator in achalasia of

6 228 CASE REPORTS Vol. 58, No.2 the esophagus. Gastroenterology 45: Rosenweig, N. R., and A. E. Cocco Treatment of a sigmoid esophagus in achalasia by pneumatic dilatation. Gastrointest. Endosc. 13: Nanson, E. M Treatment of cardiospasm by the expanding bag technique. Ganad. Med. Assn. J. 86: Benedict, E. B Bougienage, forceful dilatation, and surgery in treatment of achalasia. J. A. M. A.188: Olsen, A. M., S. W. Harrington, H. J. Moersch, and H. A. Anderson Treatment of cardiospasm: analysis of a twelveyear experience. J. Thorac. Gardiovasc. Surg. 22: Ellis, Jr., F. H., J. C. Kiser, J. F. Schlegel. R. J. Earlam, J. L. McVey, and A. M. Olsen Esophagomyotomy for esophageal achalasia: experimental, clinical and manometric aspects. Ann. Surg. 166: Drake, E. H Surgical treatment of cardiospasm. New Eng. J. Med. 266: Steichen, F. M., E. Heller, and M. M. Ravitch Achalasia of the esophagus. Surgery 47: Sawyers, J. L., and J. H. Foster Surgical considerations in management of achalasia of the esophagus. Ann. Surg. 165: Helsingen, N., Jr Gastroesophageal reflux as a complication of the Heller operation. Act. Clin. Surg. Scand., supp!

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