Rowe, MI

Smith, S

Starzl, TE

Wiener, ES

English

D-Scholarship@Pitt

Stoma-Related Variceal Bleeding: An Under-Recognized Complication of Biliary Atresia By Sam Smith, Eugene S. Wiener, Thomas E. Starzl, and Marc I. Rowe Pittsburgh. Pennsylvania • The medical records of 52 children with biliary atresia treated by portoenterostomy and evaluated for liver trans-plantation were reviewed to determine the frequency of stoma variceal bleeding and the optimal strategies for prevention and treatment. Eighteen patients had had prior stoma closure. four by pre peritoneal closure without take-down from the abdominal wall. Three of the four developed occult variceal bleeding from the stoma closure site. Twen-ty-two patients had a stoma present at evaluation. All 22 patients with stomas (100%) had at least one bleeding episode requiring transfusion. Treatment included transfu-sion and local pressure (9). suture ligation of the bleeding site (5). and stoma closure and / or takedown (11). local treatment led to recurrences in eight of 14 (57%) of the cases. To reduce the high mortality in patients with biliary atresia awaiting liver transplantation, multiple variceal bleeding episodes should be prevented. To eliminate one source, stoma variceal bleeding, the stoma. whether func-tioning or nonfunctioning should be taken down and closed. Preperitoneal closure alone does not prevent stoma bleed-ing. © 1988 by Grune & Stratton. Inc. INDEX WORDS: Biliary atresia; portal hypertension; stoma varices; liver transplantation; portoenterostomy. P A TIENTS with failed portoenterostomy develop progressive liver disease and die unless liver transplantation can be provided. Seventy-three percent to 90% of children with successful portoenterostomy develop significant biliary cirrhosis with portal hyper-tension. 1•4 Ultimately, many of these patients also become liver transplant candidates. Unfortunately, 27% of pediatric liver transplant candidates die while awaiting transplantation.5 Repeated episodes of vari-ceal bleeding contribute to this high mortality. Preven-tion of bleeding from portal hypertension may decrease the high pretransplant death rate. Although esopha-geal varices are the most common source of blood loss, bleeding from portosystemic collaterals between the portoenterostomy stoma and the abdominal wall is also frequent, serious, and preventable. The purpose of this study is to evaluate a group of children with advanced liver disease following portoenterostomy to determine the incidence of stoma variceal hemorrhage, the opti-mal management of this complication, and the out-come of treatment. MATERIALS AND METHODS The medical records of 52 children with biliary atresia treated by portoenterostomy and evaluated for liver transplantation from March 1981 to July 1985 had adequate information for this review. Twelve patients without stomas were eliminated from the study. The Journal of Pediatiic Surgery, Vol 23, No 3 (March). 1988; pp 243-245 records of the remaining 40 children were analyzed for the following: status of the stoma (open or closed), type of stoma closure, number of bleeding episodes, methods of diagnosis, type of treatment, and outcome of treatment. A significant bleeding episode was defined as blood loss requiring transfusion of at least 10 mL/kg of packed red blood cells. The average age at portoenterostomy was 2.7 months. RESULTS Open Stomas Twenty-two patients had a biliary stoma present at the time of transplant evaluation. All had at least one severe bleeding episode. The stoma was nonfunction-ing in 15 of 22 (68%) cases. The average time from portoenterostomy to onset of bleeding was 18.7 months (range 1 month to 9 years). Treatment (Table 1) consisted of transfusion and local pressure to the bleeding site in nine (41 %) patients, suture ligation of a bleeding varix in five (23%) patients, and stoma take-down and closure in eight (36%) patients. Local treat-ment failed to control bleeding in eight of 14 (57%) patients. Bleeding continued until the patient either underwent transplantation or died. Stoma closure and takedown successfully controlled the stoma hemor-rhage in all cases. There was one death directly related to a bleeding stoma. The patient had four episodes of stoma hemorrhage treated by local pressure and trans-fusion during the first two bleeds and suture ligation of the bleeding varix during the third. The final bleeding episode led to consumptive coagulopathy and death at age 2 years, 8 months. Closed Stomas Eighteen patients underwent stoma closure prior to transplant evaluation. Four had a preperitoneal closure without takedown from the abdominal wall. Occult intestinal variceal bleeding from the stoma closure site occurred in three of these four patients. The bleeding site was identified by arteriogram in two cases (Fig 1) and by radionuclide bleeding scan in one case. One From the Department of Surgery. Children's Hospital of Pitts-burgh. Presented at the 34th Annual Congress of the British Association of Paediatric Surgeons. Dublin. July 22-24. 1987. Address reprint requests to Marc I. Rowe, MD. Department of Surgery, Children's Hospital of Pittsburgh, 3705 Fifth Ave of De Sofo St, Pittsburgh. P A 152 J3. © 1988 by Grune & Strallon. Inc. 0022-3468/88/2303-()OI0$03.00/0 243 -.--- .. -, .. -K<>-K~-........ """'"--------------244 Table 1. Treatment of Bleeding Open Stomas Type of Treatment Transfusion and local pressure Suture ligation of varix Stoma closure and takedown Total 9 (41).! 5 (23) 8 (36) 22 Recurrence of Bleeding 8 (62) o child had intermittent bleeding episodes over a 7-year period attributed to esophageal variceal hemorrhage. A mesenteric angiogram ultimately demonstrated the preperitoneal stoma closure site as the source of the occult bleeding. Bleeding was successfully controlled in all three patients by detaching the closed stoma from the abdominal wall, dividing the portosystemic connec-tions, and placing the intestinal segment intraperitone-ally. DISCUSSION It has become increasingly evident that portal hypertension is a major late complication in both functioning and nonfunctioning portoenterostomy patients. Lilly initialIy reported that variceal hemor-rhage was unusual following successful operation for biliary atresia.6 More recently, he revised this opinion and reported a 23% incidence of variceal hemorrhage STOMA-RELATED VARICEAL BLEEDING in patients with sustained bile drainage.7 Gautier et al8 reported the 5-year evaluation of 20 cases of extrahe-patic biliary atresia with functioning portoenterosto-mies. Definite biliary cirrhosis was noted by serial liver biopsy in 18 of 20 patients. Portal hypertension was confirmed by the presence of esophageal varices in 16 of 20 patients. Four of the 20 children (20%) bled profusely from esophageal varices. Stoma variceal hemorrhage is a problem unique to patients who have had portoenterostomy for biliary atresia. Andrews et al reported significant stoma bleeding that necessitated stoma closure before 1 year of age in 12 of 40 (30%) patients.9 In this report, all patients with open stomas developed significant bleed-ing and one patient died. Of the 22 stomas present at evaluation, 15 were nonfunctional. Since bleeding from this site is almost inevitable and the nonfunctional stoma serves no phys-iologic function, closure and takedown is recom-mended. The management of a functioning stoma in a patient with advanced liver disease deserves reevaluation. Burnweit and Coln lo reported no difference in inci-dence of cholangitis between 12 children with diverting stomas and portoenterostomies and 19 children with nondiverting portoenterostomies. Four of the 12 chil-Fig 1. Angiogram demonstrat-ing varices at preperitoneal stoma closure site. SMITH ET AL dren with stomas developed stoma hemorrhage and two required stoma closure to control the bleeding. In our series seven of 22 stomas were functional and all bled. We recommend takedown and stoma closure in patients with advanced liver disease following portoen-terostomy for biliary atresia whether the stoma is functioning or not. An aggressive approach to the prevention and treatment of stoma varices is warranted as more patients with advanced liver disease following 245 portoenterostomy proceed to successful liver trans-plantation. Preperitoneal closure as recommended by Lilly and Altman i i should not be used in these patients. The persistent connection of the closed intestine to the abdominal wall allows for continued portosystemic connection. A high risk of subsequent variceal hemor-rhage at the stoma site results. This source of bleeding is particularly difficult to diagnose because of its preperitoneallocation. REFERENCES I. Hays OM, Kimura K: Biliary atresia: New concepts of man-agement. Curr Prob Surg 18:1593·1594, 1981 2. Kasai M: Advances in treatment of biliary atresia. Jpn J Surg 13:265-276, 1983 3. Altman RP, Chandra R, Lilly JF: Ongoing cirrhosis after successful portoenterostomy in infants with biliary atresia. J Pediatr Surg 10:685·691, 1975 4. Alagaille 0, Valayer J, Odievve M, et al: Long term followup in children operated on by corrective surgery for extrahepatic biliary atresia, in Kasai M (ed): Biliary Atresia and Its Related Disorders. Amsterdam, Excerpta Medica, 1983, pp 233-238 5. Pettitt BJ, Zitelli BJ, Rowe MI: Analysis of patients with biliary atresia coming to liver transplantation. J Pediatr Surg 19:779-785, 1984 6. Lilly JR, Ohi R: Complications of surgery for biliary atresia and choledochal cysts. in deVries PA, Shapiro SR (eds): Complica-tions of Pediatric Surgery. New York, Wiley, 1982, pp 155-165 7. Lilly RJ, Stellin G: Variceal hemorrhage in biliary atresia. J Pediatr Surg 19:476-479, 1984 8_ Gautier M, VaJayer J, Odievre M, et al: Histological liver evaluation 5 years after surgery for extrahepatic biliary atresia: A study of 20 cases. J Pediatr Surg 19:263-268, 1984 9. Andrews HG, Zwiren GT, Caplan DB, et al: Biliary atresia: An evolving perspective. South Med J 79:581-584,1986 10. Burnweit CA, Coin 0: Influence of diversion on the develop-ment of cholangitis after hepatoportoenterostomy for biliar.y atresia. J Pediatr Surg 21: I 143-1146, 1986 II. Lilly JR, Altman RP: Hepatic portoenterostomy (the Kasai operation) for biliary atresia. Surgery 78:76-86, 1975 

Stama-related variceal bleeding: An under-recognized complication of biliary atresia

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Stama-related variceal bleeding: An under-recognized complication of biliary atresia

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