Biliary Atresia and the Polysplenia By F.M. Karrer,
Syndrome
Roberta J. Hall, and John R. Lilly
Denver, Colorado 0 There is a widely held but unsubstantiated belief that in infants with biliary atresia and coexisting polysplenia syndrome, the Kasai operation fails. An equally poor prognosis has been forecast for patients with this complex treated by liver transplantation. From 1975 to 1989. 16 of 131 infants with biliary atresia (12%) had one or more components of the polysplenia syndrome: polysplenia (8), intestinal malrotation (12), preduodenal portal vein (6). absent inferior vena cava (6). aberrant hepatic artery (4). or abdominal heterotaxia (4). Although technically more demanding, 12 of the 15 polysplenia patients achieved biliary drainage after operation. (One patient had exploration only, because of parental preference.) Four children are alive; two are anicteric and well at ages 5 and 8 following Kasai’s operation, and two by virtue of liver transplantation done at ages 4 and 7. Three of the five patients who had liver transplantation died. Actuarial survival was 44% at 5 years, not significantly different from that of the 115 nonpolysplenia patients (48%). When associated with the constellation of anomalies known as the polysplenia syndrome, biliary atresia is most likely caused by an early (at approximately the fifth week) embryonic insult. The anomalies do not preclude successful biliary reconstruction using the Kasai procedure or successful liver transplantation. Copyright o 1997 by W.B. Saunders Company INDEX WORDS: Biliary atresia; polysplenia portoenterostomy.
syndrome;
Kasai
A
LTHOUGH BILIARY atresia (BA) usually is considered to be an isolated lesion, associated congenital malformations occur in 10% to 25% of patients.‘.’ Most commonly, the coexisting anomalies are part of the “polysplenia syndrome,” which includes polysplenia, malrotation of the intestine, absent inferior vena cava with azygous continuation, symmetric bilobed liver, situs inversus, preduodenal portal vein, anomalous hepatic arterial supply, bilobed right lung, and cardiac malformations including anomalous pulmonary venous return and atria1 septal defeck6 The association between BA and polysplenia syn-
From the Department of Surgery, University of Colorado School of Medicine, Denver, CO. Presented at the 37th Annual International Congress of the British Association of Paediatric Surgeons, Glasgow, Scotland, July 25-27, 1990. Suppotied in part by a grant (RR-00069) from the General Clinical Research Centers Program of the Division of Research Resources, National Institutes of Health, and by the Pediattic Liver Center at The Children’s Hospital, Denver, CO. Address reprint requests to F.M. Karrer, MD, ChildrenS Health Center, 1950 Ogden St, 8323, Denver, CO 80218. Copyright o 1991 by W.B. Saunders Company 0022-3468/91/2605-0004$03.0010 524
drome has two important implications. First, the usual theory that BA is the result of a perinatal process that obliterates the normally developed biliary tract’ does not account for the concurrent congenital malformations. Second, the presence of coexisting malformations may influence the choice of treatment. Because it has been stated that portoenterostomy will fail in children with BA associated with polysplenia syndrome,‘,’ the Kasai operation may not be offered. Furthermore, initial attempts at hepatic transplantation failed because of the technical difficulties presented by the associated vascular malformations.” We believe that neither of these assumptions is true and that both Kasai portoenterostomy and hepatic transplantation may be successfully used in infants with BA and the polysplenia syndrome. MATERIALS
AND
METHODS
From 1975 through 1989, 16 of 131 consecutive infants with BA referred to the University of Colorado had one or more components of the polysplenia syndrome; namely polysplenia (8), intestinal malrotation (12), preduodenal portal vein (6), absent inferior vena cava (6), situs inversus (4), hepatic arterial anomalies (4), and dextrocardia or congenital cardiac defects (4) (Fig 1). There were 9 girls and 7 boys. Fifteen of the 16 patients underwent Kasai portoenterostomy or modifications thereof. One patient had an exploration only because of parental preference. In one case, polysplenia syndrome was suspected preoperatively on abdominal ultrasonography (Fig 2). In the remainder, the diagnosis was confirmed at operation. The average age at operation was 59.8 days (range, 25 to 82 days). Twelve infants had double-barrelled exteriorization of the Rouxen-Y loop, two had reconstruction using the appendix as a biliary conduit (facilitated by the associated intestinal malrotation), and one had a simple Roux-en-Y anastomosis. There were no examples of the “correctable” type or distal bile duct patency. Eleven of the remaining 115 infants had congenital defects not related to the polysplenia syndrome, including club foot (l), hydronephrosis (I), Meckel’s diverticulum (l), hernia (5), cataract (l), and small bowel atresia (2). Follow-up information was obtained for all patients. Survival curves were constructed by the method of Kaplan and Meier, and compared by Cox-Mantel test using Solo Statistical System (BMDP Statistical Software, Inc, Los Angeles, CA).
Technique The initial surgical approach to hepatic portoenterostomy in patients with the polysplenia syndrome is identical to that used in nonpolysplenia patients, ie, the fibrotic gallbladder is mobilized and dissected to the cystic duct-common bile duct junction. If a preduodenal portal vein is present, the atretic duct is often underneath the vein or is in a vascular complex of portal vein and hepatic artery branches. Under these circumstances, the customary proximal dissection of the duct to the porta hepatis is abandoned. Instead, the hepatic hilum is dissected, the bifurcation of the portal Journaloffediatric
Surgery, Vol26. No 5 (May), 1991:
pp 524-527
525
BILIARY ATRESIA AND THE POLYSPLENIA SYNDROME
Fig 1. Polysplenia malformations in BA patients in this series. (1) Multiple small spleens; (2a) aberrant left lateral segment hepatic artery (from left gastric artery); (2b) aberrant hepatic artery (from superior mesenteric artery); (3) preduodenal portal vein (note the hepatic artery lies posterior to the portal vein); (4) intestinal malrotation; (5) absent IVC (with azygous continuation); (6) bilobed liver. Not shown are the atretic bile ducts of BA.
vein is identified, and its branches are mobilized. The common hepatic duct is visualized between the left and right portal vein branches and is dissected free. Stay sutures are placed in the liver at both the lateral margins of the duct and the posterior duct margin, and the duct is transected flush with the liver. The Roux-en-Y portoenterostomy is done in standard fashion.
RESULTS
Twelve of the 15 infants (80%) had bile drainage postoperatively. Of the 12 patients, six achieved long-term ( > 5 years) survival. Many patients experienced complications related to their BA that did not
differ from infants without polysplenia, namely, recurrent cholangitis, portal hypertension with variceal bleeding, and jaundice. Five patients underwent liver transplantation at ages 10 months, and 21/, 4,5, and 7 years, three of whom died and two are alive at ages 5 and 10. Two other patients are still alive and free from jaundice, 5 and 8 years after Kasai’s operation. Thus, four of the 15 patients are still alive (27%). The polysplenia group was compared with the remaining 115 children with BA. The mean age at operation (59.8 v 66 days) was similar, as was the degree of fibrosis on liver biopsy. For those undergoing corrective operation the likelihood of achieving bile drainage was nearly identical (80% v 82%). Actuarial 5-year survival for the polysplenia group was 44%. This was not significantly different from the other patients with BA (48%) (Fig 3) by Cox-Mantel test (P > .5). DISCUSSION
The constellation of abnormalities comprising the polysplenia syndrome probably results from a teratogenie insult at about the fifth week of gestation, when the spleen and extrahepatic biliary ducts begin to develop.’ Ivemark found that the insult causing asplenia occurs between 30 and 40 days of gestation, whereas that causing polysplenia probably occurs slightly later.” The polysplenia sequence is thought to be a defect in lateralization with duplication of left-sided structures (levoisomerism) and absence of right-sided structures.” Situs inversus and abdominal heterotaxia probably result from this dysmorphic process involving determination of laterality and the embryonic midline. Development of the bronchial tree, endocardial cushion, and atria1 septa begins at week 5. The inferior vena cava (IVC) is produced by fusion of the subcardinal veins with the hepatic veins
0 0
1
2
3
4
5
6
7
8
9
10
YEARS Fig 2. Preoperative ultrasound examination of a 7-week-old jaundiced infant showing multiple spleens in the right upper quadrant.
Fig 3. Kaplan-Meier survival curves for children with BA alone and with polysplenia syndrome. The two groups are not statistically different by Cox-Mantel test (P > .5).
526
KARRER, HALL, AND LILLY
between day 25 and 50 of fetal growth.13 The development of the portal vein begins at about the fourth week when the paired vitelline veins are invaded by the liver cells from the septum transversum; subsequent cross linkage and then selective obliteration in the fifth week results in the normal S-shaped portal vein.14 Definitive growth of the extrahepatic bile ducts occurs in the fifth week of fetal life from the primordial bud of the intestine. At this stage, cellular proliferation occludes the duct lumen. Failure of normal canalization in the sixth week was formerly held to be the cause of extrahepatic BA.” The occurrence of these relatively rare malformations together, and their frequent association with BA (> 10%) seems unlikely to be a random event. Yet the modern theory of the pathogenesis of BA, namely that it results from an acquired intrauterine or perinatal obliterative process,’ does not explain the association of these early embryonic abnormalities. Because there are no demonstrable differences in the age at presentation, the histopathologic findings, or the outcome following portoenterostomy, it seems unlikely that there is more than one mechanism causing BA, ie, one for BA associated with polysplenia and another for nonsyndromatic BA. Parenthetically, we have seen polysplenia only in association with BA and not with infants with other types of liver disease, nor, to our knowledge, has this coincidence been reported. Thus, if components of the polysplenia syndrome are identified in the evaluation of a jaundiced infant, for instance, demonstration by ultrasound of polysplenia, absent IVC, or preduodenal portal vein, the diagnosis of BA is almost certain. Grosfeld, in discussion of Lilly et al’s Denver
series, commented that he “had seen no successes with the Kasai procedure in children with polysplenia syndrome, situs inversus, or with an anterior portal vein.“’ The New York group reported a similar experience.’ While acknowledging that these patients present a challenging surgical dilemma,16 we have five patients who survived 5 years without transplantation, two of whom are long-term jaundice-free survivors. Liver transplantation has been equally troublesome. In 1974, Lilly and Starzl” reported three infants with BA and vascular abnormalities who had hepatic transplantation. All were unsuccessful, and they concluded that these patients “are highly questionable candidates for transplantation.” Hoffman et al” reported two children with BA and polysplenia syndrome who had liver transplantation, both of whom died (one of thrombosis of the graft and the second with intractable rejection). Nonetheless, they believed that with technical modifications (anastomosis of the hepatic venous common orifice to the donor suprahepatic IVC cuff with ligation of the lower IVC), successful transplantation would be possible.” The Pittsburgh group recently has reported successful hepatic transplantation in children with absent IVC.18 Patients with situs inversus have also been successfully transplanted by anastomosis of the donor hepatic venous confluence to the left-sided cava.19 Preduodenal portal vein does not present any special difficulty and the hepatic arterial anomalies can be dealt with using standard techniques.“’ The polysplenia syndrome anomalies associated with BA do not preclude successful biliary reconstruction using Kasai’s procedure nor do they preclude successful liver transplantation.
REFERENCES 1. Chandra RS: Biliary atresia and other structural anomalies in the congenital polysplenia syndrome. J Pediatr 85:649-65.5, 1974 2. Dimmick JE, Bovo KE, McAdams AJ: Extrahepatic biliary atresia and the polysplenia syndrome. J Pediatr 86:644-645, 1975 (letter) 3. Maksem JA: Polysplenia syndrome and splenic hypoplasia associated with extraliepatic biliary atresia. Arch Path01 Lab Med 104:212-214,198O 4. Paddock RJ, Arensman RM: Polysplenia syndrome: Spectrum of gastrointestinal congenital anomalies. J Pediatr Surg 17:563-566, 1982 5. Miyamoto M, Kajimoto T: Associated anomalies in biliary atresia patients, in Kasai M (ed): Biliary Atresia and its Related Disorders. Amsterdam, The-Netherlands, Excerpta Medica, 1983. pp 13-19 6. Moller JH, Nahib A, Anderson RC, et al: Congenital cardiac diseases associated with polysplenia: A developmental complex of bilateral “left-sidedness.” Circulation 36:789-799,1967 7. Landing BH: Considerations of the pathogenesis of neonatal
hepatitis, biliary atresia and choledochal cyst: The concept of infantile obstructive cholangiography. Prog Pediatr Surg 6:113-139, 1974 8. Grosfeld JL, in discussion of Lilly JR, Karrer FM, Hall RJ, et al: The surgery of biliary atresia. Ann Surg 210:289-296, 1989 9. Berdon WE, Altman RP: Personal communication, 1986 10. Lilly JR, Starzl TE: Liver transplantation in children with biliary atresia and vascular anomalies. J Pediatr Surg 9:707-714, 1974 11. Ivemark BI: Implications of agenesis of the spleen on the pathogenesis of conotruncus anomalies in childhood. Acta Paediatr 44:1-110,1955 12. Jones KL: Smith’s Recognizable Patterns of Human Malformation (ed 4). Philadelphia, PA, Saunders, 1988, pp 543-545 13. Huseby RA, Boyden EA: Absence of the hepatic portion of the inferior vena cava with bilateral retention of the subcardinal system. Anat Ret 81:537-544, 1941 14. Greatrex GH: A rare developmental abnormality of the portal vein. Br J Surg 52:477-478, 1965
BILIARY ATRESIA AND THE POLYSPLENIA
SYNDROME
15. Ylppo A: Zwei Falle von Kongenitalem gallengangs verschluss. Z Kinderchir 9:319,1913 16. Lilly JR, Chandra RS: Surgical hazards of co-existing anomalies in biliary atresia. Surg Gynecol Obstet 139:49-54, 1974 17. Hoffman MA, Celli S, Ninkov P, et al: Orthotopic transplanration of the liver in children with biliary atresia and polysplenia syndrome: Report of two cases. J Pediatr Surg 24:1020:1022, 1989 18. Lerut J, Tzakis AG, Bron K, et al: Complications of venous
527
reconstruction in human orthotopic Surg 205:404-414,1987
liver transplantation.
Ann
19. Raynor SC, Wood RP, Spanta AD, et al: Liver transplantation in a patient with abdominal situs inversus. Transplantation 45:661-663,1988 20. Todo S, Makowka L, Tzakis A, et al: Hepatic artery in liver transplantation. Transplant Proc 19:2406-2411, 1987
Syndrome
Roberta J. Hall, and John R. Lilly
Denver, Colorado 0 There is a widely held but unsubstantiated belief that in infants with biliary atresia and coexisting polysplenia syndrome, the Kasai operation fails. An equally poor prognosis has been forecast for patients with this complex treated by liver transplantation. From 1975 to 1989. 16 of 131 infants with biliary atresia (12%) had one or more components of the polysplenia syndrome: polysplenia (8), intestinal malrotation (12), preduodenal portal vein (6). absent inferior vena cava (6). aberrant hepatic artery (4). or abdominal heterotaxia (4). Although technically more demanding, 12 of the 15 polysplenia patients achieved biliary drainage after operation. (One patient had exploration only, because of parental preference.) Four children are alive; two are anicteric and well at ages 5 and 8 following Kasai’s operation, and two by virtue of liver transplantation done at ages 4 and 7. Three of the five patients who had liver transplantation died. Actuarial survival was 44% at 5 years, not significantly different from that of the 115 nonpolysplenia patients (48%). When associated with the constellation of anomalies known as the polysplenia syndrome, biliary atresia is most likely caused by an early (at approximately the fifth week) embryonic insult. The anomalies do not preclude successful biliary reconstruction using the Kasai procedure or successful liver transplantation. Copyright o 1997 by W.B. Saunders Company INDEX WORDS: Biliary atresia; polysplenia portoenterostomy.
syndrome;
Kasai
A
LTHOUGH BILIARY atresia (BA) usually is considered to be an isolated lesion, associated congenital malformations occur in 10% to 25% of patients.‘.’ Most commonly, the coexisting anomalies are part of the “polysplenia syndrome,” which includes polysplenia, malrotation of the intestine, absent inferior vena cava with azygous continuation, symmetric bilobed liver, situs inversus, preduodenal portal vein, anomalous hepatic arterial supply, bilobed right lung, and cardiac malformations including anomalous pulmonary venous return and atria1 septal defeck6 The association between BA and polysplenia syn-
From the Department of Surgery, University of Colorado School of Medicine, Denver, CO. Presented at the 37th Annual International Congress of the British Association of Paediatric Surgeons, Glasgow, Scotland, July 25-27, 1990. Suppotied in part by a grant (RR-00069) from the General Clinical Research Centers Program of the Division of Research Resources, National Institutes of Health, and by the Pediattic Liver Center at The Children’s Hospital, Denver, CO. Address reprint requests to F.M. Karrer, MD, ChildrenS Health Center, 1950 Ogden St, 8323, Denver, CO 80218. Copyright o 1991 by W.B. Saunders Company 0022-3468/91/2605-0004$03.0010 524
drome has two important implications. First, the usual theory that BA is the result of a perinatal process that obliterates the normally developed biliary tract’ does not account for the concurrent congenital malformations. Second, the presence of coexisting malformations may influence the choice of treatment. Because it has been stated that portoenterostomy will fail in children with BA associated with polysplenia syndrome,‘,’ the Kasai operation may not be offered. Furthermore, initial attempts at hepatic transplantation failed because of the technical difficulties presented by the associated vascular malformations.” We believe that neither of these assumptions is true and that both Kasai portoenterostomy and hepatic transplantation may be successfully used in infants with BA and the polysplenia syndrome. MATERIALS
AND
METHODS
From 1975 through 1989, 16 of 131 consecutive infants with BA referred to the University of Colorado had one or more components of the polysplenia syndrome; namely polysplenia (8), intestinal malrotation (12), preduodenal portal vein (6), absent inferior vena cava (6), situs inversus (4), hepatic arterial anomalies (4), and dextrocardia or congenital cardiac defects (4) (Fig 1). There were 9 girls and 7 boys. Fifteen of the 16 patients underwent Kasai portoenterostomy or modifications thereof. One patient had an exploration only because of parental preference. In one case, polysplenia syndrome was suspected preoperatively on abdominal ultrasonography (Fig 2). In the remainder, the diagnosis was confirmed at operation. The average age at operation was 59.8 days (range, 25 to 82 days). Twelve infants had double-barrelled exteriorization of the Rouxen-Y loop, two had reconstruction using the appendix as a biliary conduit (facilitated by the associated intestinal malrotation), and one had a simple Roux-en-Y anastomosis. There were no examples of the “correctable” type or distal bile duct patency. Eleven of the remaining 115 infants had congenital defects not related to the polysplenia syndrome, including club foot (l), hydronephrosis (I), Meckel’s diverticulum (l), hernia (5), cataract (l), and small bowel atresia (2). Follow-up information was obtained for all patients. Survival curves were constructed by the method of Kaplan and Meier, and compared by Cox-Mantel test using Solo Statistical System (BMDP Statistical Software, Inc, Los Angeles, CA).
Technique The initial surgical approach to hepatic portoenterostomy in patients with the polysplenia syndrome is identical to that used in nonpolysplenia patients, ie, the fibrotic gallbladder is mobilized and dissected to the cystic duct-common bile duct junction. If a preduodenal portal vein is present, the atretic duct is often underneath the vein or is in a vascular complex of portal vein and hepatic artery branches. Under these circumstances, the customary proximal dissection of the duct to the porta hepatis is abandoned. Instead, the hepatic hilum is dissected, the bifurcation of the portal Journaloffediatric
Surgery, Vol26. No 5 (May), 1991:
pp 524-527
525
BILIARY ATRESIA AND THE POLYSPLENIA SYNDROME
Fig 1. Polysplenia malformations in BA patients in this series. (1) Multiple small spleens; (2a) aberrant left lateral segment hepatic artery (from left gastric artery); (2b) aberrant hepatic artery (from superior mesenteric artery); (3) preduodenal portal vein (note the hepatic artery lies posterior to the portal vein); (4) intestinal malrotation; (5) absent IVC (with azygous continuation); (6) bilobed liver. Not shown are the atretic bile ducts of BA.
vein is identified, and its branches are mobilized. The common hepatic duct is visualized between the left and right portal vein branches and is dissected free. Stay sutures are placed in the liver at both the lateral margins of the duct and the posterior duct margin, and the duct is transected flush with the liver. The Roux-en-Y portoenterostomy is done in standard fashion.
RESULTS
Twelve of the 15 infants (80%) had bile drainage postoperatively. Of the 12 patients, six achieved long-term ( > 5 years) survival. Many patients experienced complications related to their BA that did not
differ from infants without polysplenia, namely, recurrent cholangitis, portal hypertension with variceal bleeding, and jaundice. Five patients underwent liver transplantation at ages 10 months, and 21/, 4,5, and 7 years, three of whom died and two are alive at ages 5 and 10. Two other patients are still alive and free from jaundice, 5 and 8 years after Kasai’s operation. Thus, four of the 15 patients are still alive (27%). The polysplenia group was compared with the remaining 115 children with BA. The mean age at operation (59.8 v 66 days) was similar, as was the degree of fibrosis on liver biopsy. For those undergoing corrective operation the likelihood of achieving bile drainage was nearly identical (80% v 82%). Actuarial 5-year survival for the polysplenia group was 44%. This was not significantly different from the other patients with BA (48%) (Fig 3) by Cox-Mantel test (P > .5). DISCUSSION
The constellation of abnormalities comprising the polysplenia syndrome probably results from a teratogenie insult at about the fifth week of gestation, when the spleen and extrahepatic biliary ducts begin to develop.’ Ivemark found that the insult causing asplenia occurs between 30 and 40 days of gestation, whereas that causing polysplenia probably occurs slightly later.” The polysplenia sequence is thought to be a defect in lateralization with duplication of left-sided structures (levoisomerism) and absence of right-sided structures.” Situs inversus and abdominal heterotaxia probably result from this dysmorphic process involving determination of laterality and the embryonic midline. Development of the bronchial tree, endocardial cushion, and atria1 septa begins at week 5. The inferior vena cava (IVC) is produced by fusion of the subcardinal veins with the hepatic veins
0 0
1
2
3
4
5
6
7
8
9
10
YEARS Fig 2. Preoperative ultrasound examination of a 7-week-old jaundiced infant showing multiple spleens in the right upper quadrant.
Fig 3. Kaplan-Meier survival curves for children with BA alone and with polysplenia syndrome. The two groups are not statistically different by Cox-Mantel test (P > .5).
526
KARRER, HALL, AND LILLY
between day 25 and 50 of fetal growth.13 The development of the portal vein begins at about the fourth week when the paired vitelline veins are invaded by the liver cells from the septum transversum; subsequent cross linkage and then selective obliteration in the fifth week results in the normal S-shaped portal vein.14 Definitive growth of the extrahepatic bile ducts occurs in the fifth week of fetal life from the primordial bud of the intestine. At this stage, cellular proliferation occludes the duct lumen. Failure of normal canalization in the sixth week was formerly held to be the cause of extrahepatic BA.” The occurrence of these relatively rare malformations together, and their frequent association with BA (> 10%) seems unlikely to be a random event. Yet the modern theory of the pathogenesis of BA, namely that it results from an acquired intrauterine or perinatal obliterative process,’ does not explain the association of these early embryonic abnormalities. Because there are no demonstrable differences in the age at presentation, the histopathologic findings, or the outcome following portoenterostomy, it seems unlikely that there is more than one mechanism causing BA, ie, one for BA associated with polysplenia and another for nonsyndromatic BA. Parenthetically, we have seen polysplenia only in association with BA and not with infants with other types of liver disease, nor, to our knowledge, has this coincidence been reported. Thus, if components of the polysplenia syndrome are identified in the evaluation of a jaundiced infant, for instance, demonstration by ultrasound of polysplenia, absent IVC, or preduodenal portal vein, the diagnosis of BA is almost certain. Grosfeld, in discussion of Lilly et al’s Denver
series, commented that he “had seen no successes with the Kasai procedure in children with polysplenia syndrome, situs inversus, or with an anterior portal vein.“’ The New York group reported a similar experience.’ While acknowledging that these patients present a challenging surgical dilemma,16 we have five patients who survived 5 years without transplantation, two of whom are long-term jaundice-free survivors. Liver transplantation has been equally troublesome. In 1974, Lilly and Starzl” reported three infants with BA and vascular abnormalities who had hepatic transplantation. All were unsuccessful, and they concluded that these patients “are highly questionable candidates for transplantation.” Hoffman et al” reported two children with BA and polysplenia syndrome who had liver transplantation, both of whom died (one of thrombosis of the graft and the second with intractable rejection). Nonetheless, they believed that with technical modifications (anastomosis of the hepatic venous common orifice to the donor suprahepatic IVC cuff with ligation of the lower IVC), successful transplantation would be possible.” The Pittsburgh group recently has reported successful hepatic transplantation in children with absent IVC.18 Patients with situs inversus have also been successfully transplanted by anastomosis of the donor hepatic venous confluence to the left-sided cava.19 Preduodenal portal vein does not present any special difficulty and the hepatic arterial anomalies can be dealt with using standard techniques.“’ The polysplenia syndrome anomalies associated with BA do not preclude successful biliary reconstruction using Kasai’s procedure nor do they preclude successful liver transplantation.
REFERENCES 1. Chandra RS: Biliary atresia and other structural anomalies in the congenital polysplenia syndrome. J Pediatr 85:649-65.5, 1974 2. Dimmick JE, Bovo KE, McAdams AJ: Extrahepatic biliary atresia and the polysplenia syndrome. J Pediatr 86:644-645, 1975 (letter) 3. Maksem JA: Polysplenia syndrome and splenic hypoplasia associated with extraliepatic biliary atresia. Arch Path01 Lab Med 104:212-214,198O 4. Paddock RJ, Arensman RM: Polysplenia syndrome: Spectrum of gastrointestinal congenital anomalies. J Pediatr Surg 17:563-566, 1982 5. Miyamoto M, Kajimoto T: Associated anomalies in biliary atresia patients, in Kasai M (ed): Biliary Atresia and its Related Disorders. Amsterdam, The-Netherlands, Excerpta Medica, 1983. pp 13-19 6. Moller JH, Nahib A, Anderson RC, et al: Congenital cardiac diseases associated with polysplenia: A developmental complex of bilateral “left-sidedness.” Circulation 36:789-799,1967 7. Landing BH: Considerations of the pathogenesis of neonatal
hepatitis, biliary atresia and choledochal cyst: The concept of infantile obstructive cholangiography. Prog Pediatr Surg 6:113-139, 1974 8. Grosfeld JL, in discussion of Lilly JR, Karrer FM, Hall RJ, et al: The surgery of biliary atresia. Ann Surg 210:289-296, 1989 9. Berdon WE, Altman RP: Personal communication, 1986 10. Lilly JR, Starzl TE: Liver transplantation in children with biliary atresia and vascular anomalies. J Pediatr Surg 9:707-714, 1974 11. Ivemark BI: Implications of agenesis of the spleen on the pathogenesis of conotruncus anomalies in childhood. Acta Paediatr 44:1-110,1955 12. Jones KL: Smith’s Recognizable Patterns of Human Malformation (ed 4). Philadelphia, PA, Saunders, 1988, pp 543-545 13. Huseby RA, Boyden EA: Absence of the hepatic portion of the inferior vena cava with bilateral retention of the subcardinal system. Anat Ret 81:537-544, 1941 14. Greatrex GH: A rare developmental abnormality of the portal vein. Br J Surg 52:477-478, 1965
BILIARY ATRESIA AND THE POLYSPLENIA
SYNDROME
15. Ylppo A: Zwei Falle von Kongenitalem gallengangs verschluss. Z Kinderchir 9:319,1913 16. Lilly JR, Chandra RS: Surgical hazards of co-existing anomalies in biliary atresia. Surg Gynecol Obstet 139:49-54, 1974 17. Hoffman MA, Celli S, Ninkov P, et al: Orthotopic transplanration of the liver in children with biliary atresia and polysplenia syndrome: Report of two cases. J Pediatr Surg 24:1020:1022, 1989 18. Lerut J, Tzakis AG, Bron K, et al: Complications of venous
527
reconstruction in human orthotopic Surg 205:404-414,1987
liver transplantation.
Ann
19. Raynor SC, Wood RP, Spanta AD, et al: Liver transplantation in a patient with abdominal situs inversus. Transplantation 45:661-663,1988 20. Todo S, Makowka L, Tzakis A, et al: Hepatic artery in liver transplantation. Transplant Proc 19:2406-2411, 1987
