- Email: [email protected]
Recent experience with a modified Sawaguchi procedure for biliary atresia
Recent Experience With a Modified Sawaguchi Procedure for Biliary Atresia By T i m o t h y G. Canty, T h o m a s W. Self, David L. Collins, and Louis Bonaldi
San Diego, California 9 The treatment of biliary atresia by variations of the original Kasai hepatoportoenterostomy has shown early success with good bile flow and the elimination of jaundice in 5 0 % to 7 0 % of cases in many series. Long-term follow-up in many of these patients shows continued problems with ascending cholangitis and progressive liver disease leading to death. Our recent experience with a modified Sawaguchi hepatoportoenterostomy is encouraging. Twelve patients w e r e operated on before two months of age, All but one became jaundice free within 2 to 4 months and had biliary intestinal continuity reestablished within 3 to 6 months. These 11 patients have remained jaundice free with normal growth and development 1 to 8 years postoperatively. T w o patients had one and t w o episodes of cholangitis, respectively. All have continued mild elevations of hepatocellular enzymes but no patient has obvious signs of liver failure. Serial liver biopsies have shown clearing of bile stasis and continued periportal fibrosis. Size and number of ductules in the excised biliary remnant did not correlate with clinical outcome. One patient remained jaundiced after hepatoportoenterostomy and reoperation, and eventually expired. In contrast, two patients operated at 4 and 9 months of age never drained bile and eventually died of bleeding varices and hepatic failure, respectively. The atypical success and relative lack of cholangitis in this series is not readily explained, but may be related to specific technical modifications of the original Sawaguchi procedure. 9 1985 by Grune & Stratton, Inc. INDEX WORDS: Biliary atresia; Sawaguchi procedure; hepatopor toenterostomy.
H E T R E A T M E N T of biliary atresia by various modifications of the original Kasai hepatoportoenterostomy is now well established. 1 Typically there is early establishment of bile flow and the elimination of jaundice in 50% to 70% of cases undergoing operation before two months of age. A significant number of these early "successes" suffer progressive cirrhosis, portal hypertension, and hepatic failure. The incidence of nutritional and metabolic deficiencies and, most importantly, life-threatening ascending cholangitis, approaches 90% in some series. 2 10 Analysis of many variables has failed to reveal any consistent factor or group of factors, with the exception of age at the time of surgery, responsible for what appears to be true success in some cases and continued difficulties and/or eventual death in others. 4'11'12In spite of many modifications of the original operative procedure and perioperative management, the overall success rate over the past 5 to 10 years has remained at 30% to 40% of those cases treated before the age of two months. 2-~~
T
Journal of Pediatric Surgery, Vo120, No 3 (June), 1985: pp 2 t 1- 216
This, however, is a remarkable achievement in a group of youngsters in which mortality was virtually universal. 13 Success in infants operated upon after two months of age is rare. 2 9 M A T E R I A L S A N D METHODS From 1974 through 1982, 14 infants with mixed jaundice (direct and indirect hyperbilirubinemia) and operatively proven biliary atresia have undergone the first stage of the Sawaguchi procedure. This series includes 3 females and 11 males. Twelve children were operated upon before the age of two months. All had the onset of mixed jaundice between 2 to 4 weeks of age and were evaluated with serial bilirubin determinations, hepatic enzyme analysis, Torch titers, hepatic ultrasonograpy, and either I~3~ Rose Bengal or -IDA hepatobiliary radionuclide imaging, none of which excluded the diagnosis of biliary atresia. Exploratory laparotomy, operative cholangiography, and the first stage of the Sawaguchi procedure was performed in all 12 cases by 6 to 8 weeks of age. Bile output and liver function tests were closely monitored in the hospital and after discharge 7 to 10 days postoperatively. Infants were maintained on Portagen formula, supplemental vitamin D, calcium, and multivitamins, and weekly parenteral vitamin K. When bile output exceeded 50cc/d, salt replacement was calculated and added to the diet. The second stage surgery, take down of "biliostomy," and reestablishment of biliary intestinal continuity, was performed in 11 of 12 infants 4 to 6 months after stage 1 when bilirubin levels remained normal, enzyme levels remained stabilized, and bile output remained at a satisfactory level (greater that 80 to 100cc/d) for at least one month continuously. Liver function tests, growth, and nutritional status were monitored after discharge on a normal diet. Two infants underwent first stage Sawaguchi procedures after similar preoperative evaluation at 4 and 9 months of age. Second stage reconstruction was not performed in either case as bile drainage, liver function tests, and bilirubin levels never returned to normal.
Operative Technique
Stage 1: Exploratory Laparotomy, Operative Cholangiography, Sawaguchi Hepatoportoenterostomy, Liver Biopsy The area of the gallbladder and hepatoduodenal ligament are exposed through a small right subcostal incision. If a gallbladder or other biliary ductal structure is present, a catheter is placed and
From the University of California at San Diego School of Medicine, Childrens Hospital, San Diego, and Kaiser Permanente Medical Group, San Diego. Presented before the 16th Annual Meeting of the Pacific Association of Pediatric Surgeons, Fukuoka, Japan, May 15-19, 1983. Address reprint requests to Timothy G. Canty, MD, 8008 Frost Street, Suite 308, San Diego, CA 92123. 9 1985 by Grune & Stratton, Inc. 002~3468/85/2003~9002503.00/0 211
212
CANTY ET AL
operative cholangiograpy attempted. If no communication between the liver and GI tract can be demonstrated, or if there is no suitable structure for cholangiography, the incision is widened and the fibrous remnant of the biliary system is completely separated from the portal vein and hepatic artery to the liver parenchyma. The ductal remnant is ligated distally near the duodenum, cut off proximally at the liver surface beyond the branching of the portal vein, and submitted en bloc for serial histopathologic sectioning. Frozen sections are not performed intraoperatively. A 10 to 15cm segment of jejunum is isolated on its mesenteric pedicle such that the proximal end can reach the portahepatis through the transverse mesocolon (retrocolic position) and the distal end can reach the skin without tension, excess length, or kinking. Intestinal continuity is reestablished by two-layer interrupted silk anastamosis. The hepatoportoenterostomy is performed between the open proximal end of the isolated segment and the "adventitia" of the portal vascular structures posteriorly and the liver capsule anteriorly using 6-0 chromic sutures. The technique involves meticulous placement of sutures to avoid traumatizing in any way the cutoff end of the fibrous ductal structure. The distal end of the conduit is matured as a stoma below the right upper quadrant incision (Fig 1). A liver biopsy is performed and the abdomen closed. NG tube decompression is utilized postoperatively until normal intestinal activity resumes and oral feedings are begun, usually within 5 to 7 days. Broad spectrum prophylactic antibiotics are given preoperatively and continued postoperatively for 3 to 5 days. The infant is discharged 7 to 10 days postoperative.
Stage 2: Takedown of "Biliostomy'" and Anastomosis to the Jejunum The abdomen is opened through the previous right upper quadrant subcostal incision. The stoma is mobilized from the skin and discarded. The distal end of the conduit is carefully laid out alongside the jejunum just distal to the ligament of Treitz, and the open end further enlarged by incising the antimesenteric border 3 to 4cm proximally; a long two-layer silk "antimesenteric to antimesenteric border end-to-side" anastomosis is performed such that the final conduit, 5 to 7cm in length, lies in a straight path from porta to jejunum (Fig 1). A second liver biopsy is performed, followed by the
closureof the primaryincisionand the stoma opening.Postoperative nasogastric decompression is maintained until oral feedings are tolerated and perioperativeantibiotics are given for 3 to 5 days.
RESULTS Operation after Two Months of Age (Two Patients) The two children referred for surgery at 4 and 9 months of age have both died. Both had severe jaundice (total bilirubin greater that 16mg%, direct bilirubin greater than 10mg%) at the time of referral and the 9-month-old youngster was in early hepatic failure. At surgery both had severely cirrhotic livers. Postoperatively no bile drainage occurred, jaundice remained unchanged, and hepatic function tests continued to deteriorate. The 4-month-old died of exsanguinating hemorrhage from varices in the conduit at 7 months of age. Autopsy showed a shrunken, scarred liver and a patent conduit. The 9 month old died at 10 months of age from continued hepatic failure. Autopsy was not performed.
Operation Before Two Months of Age (12 Patients) One female had minimal and delayed drainage of bile from her stoma following stage 1 surgery. Ductules in the proximal end of the resected bile duct specimen were greater than 200 microns in diameter. Serum bilirubin and hepatic enzymes remained elevated. Four months postoperatively she underwent curettage of the porta from inside the conduit, which did not improve bile output. ~17 She expired at an outlying hospital at ten months of age from apparent sepsis. Autopsy was not performed. Eleven children are currently alive and well, jaundice free, with normal growth, development, and nutrition 2 to 8 years after reestablishment of biliary intestinal continuity. All are on a normal diet; supplemental vitamins were included during the first postoperative year. Long-term prophylactic antibiotics were not used. Bilirubin levels are less than 1 mg total in all children; hepatic enzymes, S G O T and SGPT, remain mildly elevated (150 to 200 IU); and alkaline phosphatase levels are moderately elevated (500 to 600 IU); prothrombin times are normal. Postoperative radionuclide scans have been performed in five patients and show normal excretion into the GI tract. Six patients have stable mild hepatosplenomegaly; no patient has signs of hepatic failure. Following stage 1, bilirubin levels returned to normal in all 11 children within four months as bile output increased. The average length of time for bile flow was 3 to 5 weeks; two children did not drain bile for six and eight weeks, respectively, and then rapidly increased over the next 2 to 3 weeks. Electrolyte content of the excreted bile was replaced by adding salt to the diet. Three children became sodium deficient when bile output suddenly increased to greater that 300cc/d and required brief hospitalization for intravenous fluid therapy. One other child developed intestinal obstruction from adhesions three weeks after stage 1, requiring operative lysis. Histopathologic study of liver biopsy and duetal specimens obtained at stage 1 in these 11 patients showed varying patterns. Bile stasis was seen in all, as well as varying amounts of hepatocellular giant cell transformation, periportal infiltration with lymphoid ceils, portal-portal fibrous bridging, and intraportal small bile duct proliferation. Bile duct specimens showed marked fibrosis, small lymphoid cell infiltration, and varying numbers of biliary ductules. The number of ductules was highest at the portal end of the specimen and in two cases became nonexistent in the area of the "common duct." In four cases, ductules were less than 200 microns in diameter. Following stage 2 surgery, seven of the eleven children had no further complications. One child, the
SAWAGUCHI PROCEDURE FOR BILIARY ATRESIA
213
B HEPATO-POR
CUTEND OF -." ~
~
RETROCOLIC
~
~"7/ ~)
STOMA
ANASTOMOSIS
IPI
HIGH, LONG, TAPERING END TO SIDE ANASTAMOSIS
Fig 1. (A) Stage 1. A f t e r portal dissection and excision of fibrotic biliary structures high in the porta, beyond the bifurcation of the portal vein, a short segment of jejunum (10 to 15cm) is selected for the h e p a t o p o r t o e n t e r o s t o m y conduit. (B) Stage 1. The proximal end of the conduit is brought to the porta in a retrocolic position; the distal end is brought to t h e skin as a stoma. Intestinal continuity is reestablished. (C) Stage 2. The stoma is taken d o w n from the skin and biliary intestinal continuity reestablished by a long tapering end-to-side anastomosis. Note: The conduit is v e r y short (5 to lOom) and lies in a straight direct path from porta to jejunum, just distal to the ligament of Treitz.
214
CANTY ET AL
oldest in the series (age 9), developed jaundice and fever at five years of age secondary to an adhesive band kinking the conduit just below the transverse mesocoIon. Temporary T-tube drainage of the conduit was maintained postoperatively as bilirubin levels fell to normal. This youngster remains jaundice free and otherwise well now 4 years later. A second child developed prolonged intermittent fever with elevated bilirubin and hepatic enzymes (cholangitis) two months following stage 2. Antibiotic therapy was ineffective. Reoperation with temporary tube decompression of the conduit resulted in a return to normal bilirubin levels. The tube has been out for 2 years with no further difficulties. A third male child had two episodes of cholangitis at four and seven months following stage 2 easily controlled with intravenous antibiotics. At 24 months since the last episode, he is anicteric, healthy, and off antibiotic therapy. A fourth child developed what was thought to be cholangitis one month following stage 2. Intense antibiotic treatment was ineffective. Hepatic sonography revealed a 3cm lucent area in the middle of the right lobe of the liver. Sonographically guided percutaneous drainage of this area proved it to be a "bile lake" contaminated with pseudomonas organisms.~8 Percutaneous drainage did not provide a permanent solution and at a third operation the lake was located on the undersurface of the liver to the right of the original hepatoporto enterostomy and internally drained into the Sawaguchi conduit by a second "hepatoenterostomy," temporarily stented with a T-tube (Fig 2). Temperature and bilirubin fell to normal within one week. The child is currently anicteric, on a normal diet, and off antibiotics 18 months postoperatively. Liver biopsies from stage 2 operations, and in three cases, third operations, have shown clearing of bile stasis, reversal of giant cell transformation, but moderate increases in periportal fibrosis and portal-portal bridging.
HEPATOPORTOENTEROSTOMY INTRA-HEPATICABSCESS
1 A NEO-HEPATOENTEROSTOMY
DISCUSSION
The hepatoportoenterostomy as originally pioneered by Kasai, and later modified by he and others, remains a milestone in the treatment of infants with otherwise fatal biliary atresia. The etiology, pathophysiology, and reason for success or failure following operation in this condition remain confusing. Long-term follow-up of initial successes shows continued active hepatic disease and deterioration in almost one half. 2-9 There is little to offer this group except liver transplantation, which has recently become more promising with the use of cyclosporin. 19 Portasystemic shunting and sclerotherapy for bleeding varices has been successful in
E Fig 2. (A) Position of the intrahepatic "bile l a k e " (abscess) in one patient discovered two months after Stage 2. (B) Internal drainage of "bile l a k e " by neohepatoenterostomy into original Sawaguchi conduit, stented temporarily with a T-tube.
SAWAGUCHI PROCEDURE FOR BILIARY ATRESlA
215
some children, while in others, bleeding spontaneously resolves without the need for specific treatment. 4'2~ The results in this small series parallel those of others in several respects. The lack of correlation between liver histology at initial and later biopsies and clinical course is notable. Except for extensive fibrosis and hepatocellular damage seen in the two late cases (4 and 9 months), the varying histologic patterns in the early (less than 2 months of age) cases were not of prognostic significance. 4'22 26 Similarly, ductal histology, initially thought by many to be of prognostic significance, did not correlate either with initial bile flow or late clinical c o u r s e . 4J2'23'27 Two other aspects of this study, however, are at marked variance with other series: the initial and long-term success to date (90%), and the relative lack of cholangitis. Cholangitis is by far the most disturbing postoperative complication in biliary atresia. It occurs more commonly following reestablishment of biliary intestinal continuity and in those patients with good bile flow. 4 Cholangitis does not necessarily signify end-stage disease and, therefore, methods for its control and/or prevention have importance. Continued or recurrent episodes can lead to eventual failure in a patient previously doing well. Cholangitis is often responsive to parenteral antibiotic treatment initially, but may become refractory and life threatening. There are varying opinions about what factors are important etiologically including liver histology, hepatic lymphatic flow, and the anatomic arrangement and timing of various one and two stage procedures. Lilly 7 and Altman 2 advocate the use of a long conduit exteriorized as a Mikulicz stoma for at least one year or longer, routed back into the intestine well down from the ligament of Treitz. Other Japanese surgeons have extensively modified the original Kasai procedure and are leaving an exteriorized stoma for longer and longer periods (2 to 3 years). 4 Kimura has returned to original
one-stage Kasai procedure with occasional percutaneous drainage and notes no difference in the incidence of cholangitis, which is still high at 30% to 50%. 28 Initial stenting of the conduit allowing antibiotic irrigation and a variety of other ingenious conduit configurations have not appreciably affected the incidence of this complication overall. Little attention has been given to the possibility of partial obstruction of the conduit; this is of interest in that the most common cause of ascending cholangitis in adults is distal biliary obstruction (choledocholithiasis, ampullary stenosis, etc). We have adopted a somewhat contrary approach. We use a very short initially externalized conduit, reconnected into the jejunum near the ligament of Treitz. This straight course from the porta to the bowel, and the long open anastamosis may reduce potential mechanical problems (partial obstruction) to a minimum. Stage 2 is performed early rather than late, usually within one month after the establishment of satisfactory bile flow and normalization of serum bilirubin. The low incidence of cholangitis and the overall initial and long-term "success" to date is not readily explainable. Perhaps these mechanical and technical factors (absence of partial obstruction in a long conduit loop) and timing are of importance. These initial results suggest that this modified Sawaguchi procedure is a satisfactory treatment for biliary atresia. Further long-term evaluation of these children, and additional trials of this procedure in the future will hopefully shed more light on this fascinating disease and its treatment. ADDENDUM
Two additional children have recently been reconnected and remain anicteric without episodes of cholangitis or hepatic decompensation 4 and 6 months postoperative.
REFERENCES
1. Kasai M, Kimura S, Asakura Y, et al: Surgical treatment of biliary atresia. J Pediatr Surg 3:665, 1968 2. Altman RP: The portoenterostomyprocedure for biliary atresia: A fiveyear experience. Ann Surg 188:351, 1978 3. Campbell DP, Smith EI, Bhatia M, et al: Hepatic portoenterostomy:An assessmentof its value in the treatment of biliary atresia. Ann Surg 181:591, 1975 4. Hays DM, Kimura K: Biliary Atresia--The Japanese Experience. Cambridge, Harvard Univ Press, 1980 5. Howard ER, McClement J, Driver M, et al: Results of surgery in 88 consecutivecases of extrahepatic biliary atresia. J Royal Soc Med 75:408, 1982 6. Lilly JR, Altman RP: Hepatic portoenterostomy(the Kasai operation) for biliary atresia. Surgery 78:76, 1975 7. Lilly JR, Airman RP, Schroter G, et al: Surgery of biliary atresia. Am J DiseasesChild 129:1429, 1975
8. Lilly JR: Surgical jaundice in infancy. Ann Surg 186:549, 1977 9. Smith El, Carson JA, Tunell WP, et al: Improvedresults with hepatic portoenterostomy:A reassessment of its value in the treatment of biliary atresia. Ann Surg 195:746, 1982 10. Barkin RM, Lilly JR: Biliaryatresia and the Kasai operation: Continuing care. J Pediatr 96:1015, 1980 11. Weber TR, Grosfeld JL, Fitzgerald JF: Prognostic determinants after hepatoportoenterostomyfor biliary atresia. Am J Surg 141:57, 1981 12. Kasai M: Hepatic portoenterostomyand its modifications, in Kasai M, Shirok K (eds): Cholestasis in Infancy. Tokyo, Univ of Tokyo Press, 1980, pp 337-344 13. Hays DM, Snyder WH, Jr: Life-span in untreated biliary atresia. Surgery 54:373, 1963 14. Altman RP, Anderson KD: Surgical managementofintracta-
216
ble cholangitis following successful Kasai procedure. J Pediatr Surg 17:894, 1982 15. Goltaday ES: Reoperation for failure of portoenterostomy. South Med J 75:927, 1982 16. Groeve AH, Volpicelli N, Kosloske AM: Endoscopic recanalization of a portoenterostomy. J Pediatr Surg 17:901, 1982 17. Suruga K, Miyano T, Kimura K, et al: Reoperation in the treatment of biliary atresia. J Pediatr Surg 17:1, 1982 18. Fonkalsrud EW, Arima E: Bile lakes in congenital biliary atresia. Surgery 77:384, 1975 19. Lilly JR, Starzl TE: Liver transplantation in children with biliary atresia and vascular anomalies. J Pediatr Surg 9:707, 1974 20. Altman RP: Portal decompression by interpostion mesocaval shunt in patients with biliary atresia. J Pediatr Surg 1:809, 1976 21. Miyata M, Satani M, Ueda T, et al: Long-term results of hepatic portoenterostomy for biliary atresia: Special reference to postoperative portal hypertension. Surgery 76:234, 1974 22. Gautier M, Moitier G, Odievre M: "Uncorrectable" extrahe-
CANTY ET AL
patic biliary atresia: Relationship between intrahepatic bile duct pattern and surgery. J Pediatr Surg 15:129, 1980 23. Haas JB, Bill AH: Hepatobiliary histopathology in biliary atresia, in Kasai M, Shiraki K (eds): Cholestasis in Infancy. Tokyo, Univ Tokyo Press, 1980, p 189 24. Kasai M, Oni R, Chiba T: lntrahepatic bile ducts in biliary atresia, in Kasai M, Shiraki K (eds): Cholestasis in Infancy. Toyko, Univ Tokyo Press, 1980, p 181 25. Shiraki K: Histopathology of the liver, in Kasai M, Shiraki K (eds): Cholestasis in Infancy. Tokyo, Univ Tokyo Press, 1980 26. Altman RP, Chaundra R, Lilly JR: Ongoing cirrhosis after successful portoenterostomy in infants with biliary atresia. J Pediatr Surg 10:685, 1975 27. Gautier M, Jehan P, Odievre M, et al: Histologic study of biliary fibrous remnants in 48 cases of extrahepatic biliary atresia: Correlation with postoperative bile flow restoration. J Pediatr Surg 89:704, 1976 28. Kimura K, Hashimoto S, Nishijima E, et al: Percutaneous transhepatic cholangiodrainage after hepatic portoenterostomy for biliary atresia. J Pediatr Surg 15:811, 1980
San Diego, California 9 The treatment of biliary atresia by variations of the original Kasai hepatoportoenterostomy has shown early success with good bile flow and the elimination of jaundice in 5 0 % to 7 0 % of cases in many series. Long-term follow-up in many of these patients shows continued problems with ascending cholangitis and progressive liver disease leading to death. Our recent experience with a modified Sawaguchi hepatoportoenterostomy is encouraging. Twelve patients w e r e operated on before two months of age, All but one became jaundice free within 2 to 4 months and had biliary intestinal continuity reestablished within 3 to 6 months. These 11 patients have remained jaundice free with normal growth and development 1 to 8 years postoperatively. T w o patients had one and t w o episodes of cholangitis, respectively. All have continued mild elevations of hepatocellular enzymes but no patient has obvious signs of liver failure. Serial liver biopsies have shown clearing of bile stasis and continued periportal fibrosis. Size and number of ductules in the excised biliary remnant did not correlate with clinical outcome. One patient remained jaundiced after hepatoportoenterostomy and reoperation, and eventually expired. In contrast, two patients operated at 4 and 9 months of age never drained bile and eventually died of bleeding varices and hepatic failure, respectively. The atypical success and relative lack of cholangitis in this series is not readily explained, but may be related to specific technical modifications of the original Sawaguchi procedure. 9 1985 by Grune & Stratton, Inc. INDEX WORDS: Biliary atresia; Sawaguchi procedure; hepatopor toenterostomy.
H E T R E A T M E N T of biliary atresia by various modifications of the original Kasai hepatoportoenterostomy is now well established. 1 Typically there is early establishment of bile flow and the elimination of jaundice in 50% to 70% of cases undergoing operation before two months of age. A significant number of these early "successes" suffer progressive cirrhosis, portal hypertension, and hepatic failure. The incidence of nutritional and metabolic deficiencies and, most importantly, life-threatening ascending cholangitis, approaches 90% in some series. 2 10 Analysis of many variables has failed to reveal any consistent factor or group of factors, with the exception of age at the time of surgery, responsible for what appears to be true success in some cases and continued difficulties and/or eventual death in others. 4'11'12In spite of many modifications of the original operative procedure and perioperative management, the overall success rate over the past 5 to 10 years has remained at 30% to 40% of those cases treated before the age of two months. 2-~~
T
Journal of Pediatric Surgery, Vo120, No 3 (June), 1985: pp 2 t 1- 216
This, however, is a remarkable achievement in a group of youngsters in which mortality was virtually universal. 13 Success in infants operated upon after two months of age is rare. 2 9 M A T E R I A L S A N D METHODS From 1974 through 1982, 14 infants with mixed jaundice (direct and indirect hyperbilirubinemia) and operatively proven biliary atresia have undergone the first stage of the Sawaguchi procedure. This series includes 3 females and 11 males. Twelve children were operated upon before the age of two months. All had the onset of mixed jaundice between 2 to 4 weeks of age and were evaluated with serial bilirubin determinations, hepatic enzyme analysis, Torch titers, hepatic ultrasonograpy, and either I~3~ Rose Bengal or -IDA hepatobiliary radionuclide imaging, none of which excluded the diagnosis of biliary atresia. Exploratory laparotomy, operative cholangiography, and the first stage of the Sawaguchi procedure was performed in all 12 cases by 6 to 8 weeks of age. Bile output and liver function tests were closely monitored in the hospital and after discharge 7 to 10 days postoperatively. Infants were maintained on Portagen formula, supplemental vitamin D, calcium, and multivitamins, and weekly parenteral vitamin K. When bile output exceeded 50cc/d, salt replacement was calculated and added to the diet. The second stage surgery, take down of "biliostomy," and reestablishment of biliary intestinal continuity, was performed in 11 of 12 infants 4 to 6 months after stage 1 when bilirubin levels remained normal, enzyme levels remained stabilized, and bile output remained at a satisfactory level (greater that 80 to 100cc/d) for at least one month continuously. Liver function tests, growth, and nutritional status were monitored after discharge on a normal diet. Two infants underwent first stage Sawaguchi procedures after similar preoperative evaluation at 4 and 9 months of age. Second stage reconstruction was not performed in either case as bile drainage, liver function tests, and bilirubin levels never returned to normal.
Operative Technique
Stage 1: Exploratory Laparotomy, Operative Cholangiography, Sawaguchi Hepatoportoenterostomy, Liver Biopsy The area of the gallbladder and hepatoduodenal ligament are exposed through a small right subcostal incision. If a gallbladder or other biliary ductal structure is present, a catheter is placed and
From the University of California at San Diego School of Medicine, Childrens Hospital, San Diego, and Kaiser Permanente Medical Group, San Diego. Presented before the 16th Annual Meeting of the Pacific Association of Pediatric Surgeons, Fukuoka, Japan, May 15-19, 1983. Address reprint requests to Timothy G. Canty, MD, 8008 Frost Street, Suite 308, San Diego, CA 92123. 9 1985 by Grune & Stratton, Inc. 002~3468/85/2003~9002503.00/0 211
212
CANTY ET AL
operative cholangiograpy attempted. If no communication between the liver and GI tract can be demonstrated, or if there is no suitable structure for cholangiography, the incision is widened and the fibrous remnant of the biliary system is completely separated from the portal vein and hepatic artery to the liver parenchyma. The ductal remnant is ligated distally near the duodenum, cut off proximally at the liver surface beyond the branching of the portal vein, and submitted en bloc for serial histopathologic sectioning. Frozen sections are not performed intraoperatively. A 10 to 15cm segment of jejunum is isolated on its mesenteric pedicle such that the proximal end can reach the portahepatis through the transverse mesocolon (retrocolic position) and the distal end can reach the skin without tension, excess length, or kinking. Intestinal continuity is reestablished by two-layer interrupted silk anastamosis. The hepatoportoenterostomy is performed between the open proximal end of the isolated segment and the "adventitia" of the portal vascular structures posteriorly and the liver capsule anteriorly using 6-0 chromic sutures. The technique involves meticulous placement of sutures to avoid traumatizing in any way the cutoff end of the fibrous ductal structure. The distal end of the conduit is matured as a stoma below the right upper quadrant incision (Fig 1). A liver biopsy is performed and the abdomen closed. NG tube decompression is utilized postoperatively until normal intestinal activity resumes and oral feedings are begun, usually within 5 to 7 days. Broad spectrum prophylactic antibiotics are given preoperatively and continued postoperatively for 3 to 5 days. The infant is discharged 7 to 10 days postoperative.
Stage 2: Takedown of "Biliostomy'" and Anastomosis to the Jejunum The abdomen is opened through the previous right upper quadrant subcostal incision. The stoma is mobilized from the skin and discarded. The distal end of the conduit is carefully laid out alongside the jejunum just distal to the ligament of Treitz, and the open end further enlarged by incising the antimesenteric border 3 to 4cm proximally; a long two-layer silk "antimesenteric to antimesenteric border end-to-side" anastomosis is performed such that the final conduit, 5 to 7cm in length, lies in a straight path from porta to jejunum (Fig 1). A second liver biopsy is performed, followed by the
closureof the primaryincisionand the stoma opening.Postoperative nasogastric decompression is maintained until oral feedings are tolerated and perioperativeantibiotics are given for 3 to 5 days.
RESULTS Operation after Two Months of Age (Two Patients) The two children referred for surgery at 4 and 9 months of age have both died. Both had severe jaundice (total bilirubin greater that 16mg%, direct bilirubin greater than 10mg%) at the time of referral and the 9-month-old youngster was in early hepatic failure. At surgery both had severely cirrhotic livers. Postoperatively no bile drainage occurred, jaundice remained unchanged, and hepatic function tests continued to deteriorate. The 4-month-old died of exsanguinating hemorrhage from varices in the conduit at 7 months of age. Autopsy showed a shrunken, scarred liver and a patent conduit. The 9 month old died at 10 months of age from continued hepatic failure. Autopsy was not performed.
Operation Before Two Months of Age (12 Patients) One female had minimal and delayed drainage of bile from her stoma following stage 1 surgery. Ductules in the proximal end of the resected bile duct specimen were greater than 200 microns in diameter. Serum bilirubin and hepatic enzymes remained elevated. Four months postoperatively she underwent curettage of the porta from inside the conduit, which did not improve bile output. ~17 She expired at an outlying hospital at ten months of age from apparent sepsis. Autopsy was not performed. Eleven children are currently alive and well, jaundice free, with normal growth, development, and nutrition 2 to 8 years after reestablishment of biliary intestinal continuity. All are on a normal diet; supplemental vitamins were included during the first postoperative year. Long-term prophylactic antibiotics were not used. Bilirubin levels are less than 1 mg total in all children; hepatic enzymes, S G O T and SGPT, remain mildly elevated (150 to 200 IU); and alkaline phosphatase levels are moderately elevated (500 to 600 IU); prothrombin times are normal. Postoperative radionuclide scans have been performed in five patients and show normal excretion into the GI tract. Six patients have stable mild hepatosplenomegaly; no patient has signs of hepatic failure. Following stage 1, bilirubin levels returned to normal in all 11 children within four months as bile output increased. The average length of time for bile flow was 3 to 5 weeks; two children did not drain bile for six and eight weeks, respectively, and then rapidly increased over the next 2 to 3 weeks. Electrolyte content of the excreted bile was replaced by adding salt to the diet. Three children became sodium deficient when bile output suddenly increased to greater that 300cc/d and required brief hospitalization for intravenous fluid therapy. One other child developed intestinal obstruction from adhesions three weeks after stage 1, requiring operative lysis. Histopathologic study of liver biopsy and duetal specimens obtained at stage 1 in these 11 patients showed varying patterns. Bile stasis was seen in all, as well as varying amounts of hepatocellular giant cell transformation, periportal infiltration with lymphoid ceils, portal-portal fibrous bridging, and intraportal small bile duct proliferation. Bile duct specimens showed marked fibrosis, small lymphoid cell infiltration, and varying numbers of biliary ductules. The number of ductules was highest at the portal end of the specimen and in two cases became nonexistent in the area of the "common duct." In four cases, ductules were less than 200 microns in diameter. Following stage 2 surgery, seven of the eleven children had no further complications. One child, the
SAWAGUCHI PROCEDURE FOR BILIARY ATRESIA
213
B HEPATO-POR
CUTEND OF -." ~
~
RETROCOLIC
~
~"7/ ~)
STOMA
ANASTOMOSIS
IPI
HIGH, LONG, TAPERING END TO SIDE ANASTAMOSIS
Fig 1. (A) Stage 1. A f t e r portal dissection and excision of fibrotic biliary structures high in the porta, beyond the bifurcation of the portal vein, a short segment of jejunum (10 to 15cm) is selected for the h e p a t o p o r t o e n t e r o s t o m y conduit. (B) Stage 1. The proximal end of the conduit is brought to the porta in a retrocolic position; the distal end is brought to t h e skin as a stoma. Intestinal continuity is reestablished. (C) Stage 2. The stoma is taken d o w n from the skin and biliary intestinal continuity reestablished by a long tapering end-to-side anastomosis. Note: The conduit is v e r y short (5 to lOom) and lies in a straight direct path from porta to jejunum, just distal to the ligament of Treitz.
214
CANTY ET AL
oldest in the series (age 9), developed jaundice and fever at five years of age secondary to an adhesive band kinking the conduit just below the transverse mesocoIon. Temporary T-tube drainage of the conduit was maintained postoperatively as bilirubin levels fell to normal. This youngster remains jaundice free and otherwise well now 4 years later. A second child developed prolonged intermittent fever with elevated bilirubin and hepatic enzymes (cholangitis) two months following stage 2. Antibiotic therapy was ineffective. Reoperation with temporary tube decompression of the conduit resulted in a return to normal bilirubin levels. The tube has been out for 2 years with no further difficulties. A third male child had two episodes of cholangitis at four and seven months following stage 2 easily controlled with intravenous antibiotics. At 24 months since the last episode, he is anicteric, healthy, and off antibiotic therapy. A fourth child developed what was thought to be cholangitis one month following stage 2. Intense antibiotic treatment was ineffective. Hepatic sonography revealed a 3cm lucent area in the middle of the right lobe of the liver. Sonographically guided percutaneous drainage of this area proved it to be a "bile lake" contaminated with pseudomonas organisms.~8 Percutaneous drainage did not provide a permanent solution and at a third operation the lake was located on the undersurface of the liver to the right of the original hepatoporto enterostomy and internally drained into the Sawaguchi conduit by a second "hepatoenterostomy," temporarily stented with a T-tube (Fig 2). Temperature and bilirubin fell to normal within one week. The child is currently anicteric, on a normal diet, and off antibiotics 18 months postoperatively. Liver biopsies from stage 2 operations, and in three cases, third operations, have shown clearing of bile stasis, reversal of giant cell transformation, but moderate increases in periportal fibrosis and portal-portal bridging.
HEPATOPORTOENTEROSTOMY INTRA-HEPATICABSCESS
1 A NEO-HEPATOENTEROSTOMY
DISCUSSION
The hepatoportoenterostomy as originally pioneered by Kasai, and later modified by he and others, remains a milestone in the treatment of infants with otherwise fatal biliary atresia. The etiology, pathophysiology, and reason for success or failure following operation in this condition remain confusing. Long-term follow-up of initial successes shows continued active hepatic disease and deterioration in almost one half. 2-9 There is little to offer this group except liver transplantation, which has recently become more promising with the use of cyclosporin. 19 Portasystemic shunting and sclerotherapy for bleeding varices has been successful in
E Fig 2. (A) Position of the intrahepatic "bile l a k e " (abscess) in one patient discovered two months after Stage 2. (B) Internal drainage of "bile l a k e " by neohepatoenterostomy into original Sawaguchi conduit, stented temporarily with a T-tube.
SAWAGUCHI PROCEDURE FOR BILIARY ATRESlA
215
some children, while in others, bleeding spontaneously resolves without the need for specific treatment. 4'2~ The results in this small series parallel those of others in several respects. The lack of correlation between liver histology at initial and later biopsies and clinical course is notable. Except for extensive fibrosis and hepatocellular damage seen in the two late cases (4 and 9 months), the varying histologic patterns in the early (less than 2 months of age) cases were not of prognostic significance. 4'22 26 Similarly, ductal histology, initially thought by many to be of prognostic significance, did not correlate either with initial bile flow or late clinical c o u r s e . 4J2'23'27 Two other aspects of this study, however, are at marked variance with other series: the initial and long-term success to date (90%), and the relative lack of cholangitis. Cholangitis is by far the most disturbing postoperative complication in biliary atresia. It occurs more commonly following reestablishment of biliary intestinal continuity and in those patients with good bile flow. 4 Cholangitis does not necessarily signify end-stage disease and, therefore, methods for its control and/or prevention have importance. Continued or recurrent episodes can lead to eventual failure in a patient previously doing well. Cholangitis is often responsive to parenteral antibiotic treatment initially, but may become refractory and life threatening. There are varying opinions about what factors are important etiologically including liver histology, hepatic lymphatic flow, and the anatomic arrangement and timing of various one and two stage procedures. Lilly 7 and Altman 2 advocate the use of a long conduit exteriorized as a Mikulicz stoma for at least one year or longer, routed back into the intestine well down from the ligament of Treitz. Other Japanese surgeons have extensively modified the original Kasai procedure and are leaving an exteriorized stoma for longer and longer periods (2 to 3 years). 4 Kimura has returned to original
one-stage Kasai procedure with occasional percutaneous drainage and notes no difference in the incidence of cholangitis, which is still high at 30% to 50%. 28 Initial stenting of the conduit allowing antibiotic irrigation and a variety of other ingenious conduit configurations have not appreciably affected the incidence of this complication overall. Little attention has been given to the possibility of partial obstruction of the conduit; this is of interest in that the most common cause of ascending cholangitis in adults is distal biliary obstruction (choledocholithiasis, ampullary stenosis, etc). We have adopted a somewhat contrary approach. We use a very short initially externalized conduit, reconnected into the jejunum near the ligament of Treitz. This straight course from the porta to the bowel, and the long open anastamosis may reduce potential mechanical problems (partial obstruction) to a minimum. Stage 2 is performed early rather than late, usually within one month after the establishment of satisfactory bile flow and normalization of serum bilirubin. The low incidence of cholangitis and the overall initial and long-term "success" to date is not readily explainable. Perhaps these mechanical and technical factors (absence of partial obstruction in a long conduit loop) and timing are of importance. These initial results suggest that this modified Sawaguchi procedure is a satisfactory treatment for biliary atresia. Further long-term evaluation of these children, and additional trials of this procedure in the future will hopefully shed more light on this fascinating disease and its treatment. ADDENDUM
Two additional children have recently been reconnected and remain anicteric without episodes of cholangitis or hepatic decompensation 4 and 6 months postoperative.
REFERENCES
1. Kasai M, Kimura S, Asakura Y, et al: Surgical treatment of biliary atresia. J Pediatr Surg 3:665, 1968 2. Altman RP: The portoenterostomyprocedure for biliary atresia: A fiveyear experience. Ann Surg 188:351, 1978 3. Campbell DP, Smith EI, Bhatia M, et al: Hepatic portoenterostomy:An assessmentof its value in the treatment of biliary atresia. Ann Surg 181:591, 1975 4. Hays DM, Kimura K: Biliary Atresia--The Japanese Experience. Cambridge, Harvard Univ Press, 1980 5. Howard ER, McClement J, Driver M, et al: Results of surgery in 88 consecutivecases of extrahepatic biliary atresia. J Royal Soc Med 75:408, 1982 6. Lilly JR, Altman RP: Hepatic portoenterostomy(the Kasai operation) for biliary atresia. Surgery 78:76, 1975 7. Lilly JR, Airman RP, Schroter G, et al: Surgery of biliary atresia. Am J DiseasesChild 129:1429, 1975
8. Lilly JR: Surgical jaundice in infancy. Ann Surg 186:549, 1977 9. Smith El, Carson JA, Tunell WP, et al: Improvedresults with hepatic portoenterostomy:A reassessment of its value in the treatment of biliary atresia. Ann Surg 195:746, 1982 10. Barkin RM, Lilly JR: Biliaryatresia and the Kasai operation: Continuing care. J Pediatr 96:1015, 1980 11. Weber TR, Grosfeld JL, Fitzgerald JF: Prognostic determinants after hepatoportoenterostomyfor biliary atresia. Am J Surg 141:57, 1981 12. Kasai M: Hepatic portoenterostomyand its modifications, in Kasai M, Shirok K (eds): Cholestasis in Infancy. Tokyo, Univ of Tokyo Press, 1980, pp 337-344 13. Hays DM, Snyder WH, Jr: Life-span in untreated biliary atresia. Surgery 54:373, 1963 14. Altman RP, Anderson KD: Surgical managementofintracta-
216
ble cholangitis following successful Kasai procedure. J Pediatr Surg 17:894, 1982 15. Goltaday ES: Reoperation for failure of portoenterostomy. South Med J 75:927, 1982 16. Groeve AH, Volpicelli N, Kosloske AM: Endoscopic recanalization of a portoenterostomy. J Pediatr Surg 17:901, 1982 17. Suruga K, Miyano T, Kimura K, et al: Reoperation in the treatment of biliary atresia. J Pediatr Surg 17:1, 1982 18. Fonkalsrud EW, Arima E: Bile lakes in congenital biliary atresia. Surgery 77:384, 1975 19. Lilly JR, Starzl TE: Liver transplantation in children with biliary atresia and vascular anomalies. J Pediatr Surg 9:707, 1974 20. Altman RP: Portal decompression by interpostion mesocaval shunt in patients with biliary atresia. J Pediatr Surg 1:809, 1976 21. Miyata M, Satani M, Ueda T, et al: Long-term results of hepatic portoenterostomy for biliary atresia: Special reference to postoperative portal hypertension. Surgery 76:234, 1974 22. Gautier M, Moitier G, Odievre M: "Uncorrectable" extrahe-
CANTY ET AL
patic biliary atresia: Relationship between intrahepatic bile duct pattern and surgery. J Pediatr Surg 15:129, 1980 23. Haas JB, Bill AH: Hepatobiliary histopathology in biliary atresia, in Kasai M, Shiraki K (eds): Cholestasis in Infancy. Tokyo, Univ Tokyo Press, 1980, p 189 24. Kasai M, Oni R, Chiba T: lntrahepatic bile ducts in biliary atresia, in Kasai M, Shiraki K (eds): Cholestasis in Infancy. Toyko, Univ Tokyo Press, 1980, p 181 25. Shiraki K: Histopathology of the liver, in Kasai M, Shiraki K (eds): Cholestasis in Infancy. Tokyo, Univ Tokyo Press, 1980 26. Altman RP, Chaundra R, Lilly JR: Ongoing cirrhosis after successful portoenterostomy in infants with biliary atresia. J Pediatr Surg 10:685, 1975 27. Gautier M, Jehan P, Odievre M, et al: Histologic study of biliary fibrous remnants in 48 cases of extrahepatic biliary atresia: Correlation with postoperative bile flow restoration. J Pediatr Surg 89:704, 1976 28. Kimura K, Hashimoto S, Nishijima E, et al: Percutaneous transhepatic cholangiodrainage after hepatic portoenterostomy for biliary atresia. J Pediatr Surg 15:811, 1980