Cholecystoappendicostomy in a child with alagille syndrome

Cholecystoappendicostomy in a Child With Alagille Syndrome By Michael W.L. Gauderer and John T. Boyle Cleveland, Ohio and Greenville, South Carolina l The authors performed a partial biliary diversion on a lo-month-old child with Alagille syndrome using the appendix vermiformis as a conduit between the gallbladder and the abdominal wall skin. Three years later the patient is well, and his previously severe pruritus is controlled. The appendix has a lumen closer to that of the biliary tree, does not accumulate significant amounts of bile, and provides a smaller stoma. The small bowel is undisturbed, and there is less interference with a possible future liver transplant. Copyright o 1997 by W.B. Saunders Company INDEX WORDS: At-teriohepatic dysplasia, Alagille intrahepatic cholestasis, pruritus, partial biliary appendiceal conduit. syndrome, diversion, A RTERIOHEPATIC DYSPLASIA or Alagille syndrome is a familial disorder characterized by chronic intrahepatic cholestasis beginning in infancy, congenital heart disease,distinctive facies, variable bone and ophthalmologic abnormalities, and other typical features.‘,* Intense pruritus is one of the most severe and difficult to treat complications of the disorder, presenting a seriousproblem in the treatment of our patient. Because the effect of partial biliary diversion for controlling pruritus in children with intrahepatic cholestasis was controversial,3 we chose the appendix vermiformis as a conduit between the gallbladder and the skin. blood supply. It was then opened and flushed with salme. The distal (blind) end was anastomosed side to side to a nearly normal-sized gallbladder; we used a running single layer 6-O synthetic absorbable suture. The cecal end was brought out as the stoma and secured with a few stitches of 6-O synthetic absorbable suture (Rg 1). The child’s hepatomegaly brought the gallbladder close to the level of the umbilicus, facihtating positioning of the appendiceal conduit. A gastrostomy button was Inserted in the stomach. The liver biopsy results showed multiple characteristic stgns of advancing cholangiopathy. Postoperatively his pruritus decreased markedly; his appetite improved, as did his sleep pattern, and the progression of developmental milestones. A limited, skin-level stoma revision was required 1 month after the procedure. The conduit has worked well without complications. The no-longer-needed gastrostomy was removed within 1 year. A sample of relevant btochemical data at 9 months preoperatively, and 12 and 36 months postoperatively include the followmg values: total bdirubin, 16.2, 1.8, and 2.2, respectively; direct bilirubin. 11.2,0.6, and 0.9, respectively; alanine amino transferase, 175, 86, and 105, respectively; alkaline phosphatase, 275, 365. and 556, respecttvely: gammaglutamyltransferase. 1748, 623. and 856, respectively; cholesterol: 352, 222, and 184, respecttvely. The child is now 46 months old, active. and happy. Stoma1 output averages 15 mL/d. Mild pruritus is controlled with ursodeoxycholic acid therapy. Weight and height remam below the fifth percentile; however. his weight to height ratio is m the 25th percentile and his current weight IS 80% of that expected for age. DISCUSSION From the Department of Pediatric Surge?, Children’s Hospital, Greenville Hospital System. Greenville, South Carolina. and the Division of Pedzatric Gastroenterology, Department of Pediatrics, Rambow Babies & Children k Hospital. Cleveland, OH. Presented at the 27th Annual Meeting of the American Pediatric Surgical Association, San Diego, California. May 20-23, 1996. Address reprint requests to Michael WL. Gauderer MD, ChreJ Department of Pediatric Surgery, Children k Hospital, Greenville Hospital System, 890 W Faris Rd. MMOB. Suite 440. Greenville, SC 29605-4253. Copyright 0 1997 by WB. Saunders Company 0022-3468/97/3202-0004$03.00/O Arteriohepatic dysplasia or Alagille syndrome is the principal syndromic form of intrahepatic cholestasis.2 Jaundice and hepatomegaly are present in the neonatal period. Liver histology is characterized by a reduction, but not absence, of interlobular bile ducts, and by diminutive portal tracts.‘,2The mostprominent physiological consequenceis intracellular and canalicular cholestasis, and there is a variable, slow, and gradual progression toward irreversible liver damage.’Medical therapy alone has limited value. However, there is now increasing evidence that biliary diversion can arrest and even reverse progression of hepatic fibrosis in children with Byler’s syndrome, another form of congenital cholestasis.4 However, this therapy is still largely untested in Alagille syndrome, and the exact mechanism of this phenomenon is not known. Pruritus is a prominent clinical feature in children with thesehepatic abnormalities. In 1988, Whitington suggested partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. He and his coworkers useda segmentof jejunum interposedbetween the gallbladder and the skin. We selectedthe appendix vermiformis as a conduit becauseof its anatomic advantages:the lumen is closer to that of the biliary tree; it does not accumulate and thus resorb significant amounts of bile; it provides a smallerstoma (Fig 2); the small bowel 166 Journal CASE REPORT The child presented here had a classic Alagille’s phenotype. By 9 months of age, he exhibited severe pruritus and was unresponsive to treatment with cholestyramine. phenobarbital, and ursodeoxycholic acid. Pruritus was associated with severe skin excoriation, excessive irritability, sleep disturbance. anorexia. and marked failure to throve. At 9 months of age his weight and height were less than the fifth percentile. His weight to height ratto was below the fifth percentile and his weight for age was 60% of expected. At 10 months of age he underwent a celiotomy with a triple purpose: a cholecystoappendicostomy for partial biliary diversion, a gastrostomy for the administration of nonpalatable medication, and an open liver biopsy to assess the histological status of the liver, For creation of the biliary conduit, the appendix was detached from the cecal wall and mobilized with its intact mesoappendix and ofPediafnc Surgery, Vol32, No 2 (February), 1997: pp 166-167 CHOLECYSTOAPPENDICOSTOMY Fig 2. The abdominal after cholecystoappendicostomy. needed. Fig 1. Diagram illustrating tomy, and liver biopsy. the cholecystoappendicostomy, gastros- is left undisturbed, and an additional anastomosis is avoided; and there is less interference with a possible future liver transplant. The use of the appendix as a catheterizable stoma dates back to the turn of the century5 and this short, thin tube has found its contemporary applications in accessto the cecum6 and urinary rese- wall with the The appendiceal gastrostomy stoma 3 years is no longer voirs7 The feasiblity of using the appendix as an internal conduit for bile or urine was demonstratedexperimentally by Grosfeld et al in 1971.* The appendix has since been used clinically for reconstruction of the biliary tree”.‘” and the urinary tract.1”t2 Cholecystoappendicostomy is a short, simple technique for biliary diversion in patients with cholestatic syndromes and intractable pruritus that does not “burn any bridges” if transplant is ultimately required. It is somewhat ironic that this child’s relief is basedon the “in-line” connection of the two most commonly removed intraabdominal organsin young patients. REFERENCES 1. Alagille D, Estrada A, Hadchovel M: Syndromic paucity of interlobular bile ducts (Alagille Syndrome or anteriohepatic dysplasia): Review of 80 cases. .I Pediatr 110: 195-200, 1987 2. Dehner LP: Liver, gallbladder and extrahepatic biliary tract, m Pediatric Surgical Pathology (ed 21: Baltimore, MD. Williams & Wilkins, 1987. pp 434-436 3 Whitington PF. Whmngton GL: Partial external diversion of bile for the treatment of intractable pruritus associated with mtrahepatic cholestasis. Gastroenterology 95: 130-136. 1988 4. Edmont JC. Whitington PF: Selective surgical management of progressive familial mtrahepatic cholestases (Byler’s Disease). J Pediatr Surg 30:1635-1641, I995 5. Cataldo PA: History of stomas. in MacKetgan JM, Cataldo PA (edsl: Intestinal stomas. St. Louis, MO, Quality Medical Pubhshing, 1993, pp 3-37 6. Squire R, Kiely EM. Carr 8, et al: The clmical apphcation of the Malone antegrade colonic enema. J Pediatr Surg 28: 10 12-10 1.5. 1993 7. Mitrofanoff P: Cystostomie contmente trans-appendiculaire dans Ie traitement des vessies neurologiques. Chir Pediatr 21:297-305, 1980 8 Grosfeld JL, Wemberger M. Clatworthy HW Jr: Vascularized appendiceal transplants m biliary and urinary tract replacement. J Pediatr Surg 6:630-638, I97 1 9. Greenholz SK, Lilly JR. Shiker RH, et al: Bihary atresia m the newborn J Pediatr Surg 21:1147-1148, 1986 10. Crombleholme TM. Harrison MR, Langer JC, et al: Bihary appendico-duodenostomy: A nonrefluxmg conduit for biliary reconstruction. J Pediatr Surg 24.665-667. 1989 I 1. Wemberg RW: Appendix ureteroplasty. Br J Urol48:234, 1976 12. Martin LW: Use of the appendix to replace a ureter. Case report. J Pediatr Surg 16:799-800, I98 1