Original communication| Volume 113, ISSUE 6, P662-668, June 1993

Biliary atresia splenic malformation syndrome: An etiologic and prognostic subgroup

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      Background. The polysplenia syndrome is the most common extrahepatic anomaly found in association with extrahepatic biliary atresia. This subgroup may have a different cause and a worse prognosis than do infants with biliary atresia alone, and this hypothesis has been tested by analyzing the King's College Hospital series.
      Methods. The case records of 308 infants treated between 1975 and 1991 for biliary atresia were examined for extrahepatic anomalies.
      Results. Twenty-three (7.5%) infants had polysplenia and biliary atresia. There were also four infants with other types of splenic malformation: two with double spleen and two with asplenia. The presence of other anomalies such as situs inversus and portal vein anomalies in all the categories of splenic malformation suggests that they formed part of a larger association for which we now propose the term biliary atresia splenic malformation (BASM) syndrome. There was no difference in age at presentation and in biochemical test results of liver function before operation between infants with BASM and those with biliary atresia alone. Four (15%) infants with BASM were born to mothers with diabetes (three insulin dependent and one with gestational diabetes treated by diet alone). There were no other cases of maternal diabetes in the series as a whole. Actuarial “survival” (death or transplant) of infants with BASM after initial corrective operation was worse than that in a control group without anatomic anomalies (p < 0.05).
      Conclusions. BASM syndrome appears to be a distinct subgroup in infants with biliary atresia. This subgroup may have a different cause and tends to have a worse prognosis than do control subjects. Whether this is caused by the presence of the other anomalies (e.g., cardiovascular anomalies), which are in themselves detrimental, is unclear.
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        • Landing BH
        Considerations of the pathogenesis of neonatal hepatitis, biliary atresia, choledochal cyst: the concept of infantile obstructive cholangiography.
        Prog Pediatr Surg. 1984; 6: 113-119
        • Silvera TR
        • Salazano FM
        • Howard ER
        • Mowat AP
        Congenital structural abnormalities in biliary atresia: evidence for aetiopathogenic heterogeneity and therapeutic implications.
        Acta Paediatr Scand. 1991; 80: 1192-1199
        • Choulet JJ
        • Gautier M
        • Eliot N
        Les malformations associees a l'atresie de voies extrahepatiques.
        Arch Fr Pediatr. 1979; 36: 19-24
        • Miyamoto M
        • Kajimoto T
        Associated anomalies in biliary atresia patients.
        in: Kasai M Biliary atresia and its related disorders. Excerpta Medica, Amsterdam1983: 13-19
        • Karrer FM
        • Hall RJ
        • Lilly JR
        Biliary atresia and the polysplenia syndrome.
        J Pediatr Surg. 1991; 26: 524-527
        • Hall RJ
        • Greenholz SK
        • Vasquez-Estevez JM
        • Lilly JR
        Biliary atresia and the polysplenia syndrome.
        Pediatr Res. 1987; 21 ([Abstract]): 269A
        • Howard ER
        Biliary atresia: aetiology, management and complications.
        in: Howard ER Surgery of liver disease in children. Butterworth-Heinemann, London1991: 40-59
        • Helwig FC
        Multiple spleen combined with other congenital abnormalities.
        Arch Pathol. 1929; 8: 761-767
        • Rumler W
        Ueber die kongenitale Gallenwegssatresia.
        in: Zum familiaeren Vorkommen und zur Genese dieser Fehlbildung. 2nd ed. Arch Kinderheikd. 164. 1961: 238-248
        • Strauss L
        • Valderam E
        • Alpert L
        Biliary tract anomalies: the relationship of biliary atresia to neonatal hepatitis.
        Birth Defects. 1972; 8: 135-148
        • Chandra RS
        Biliary atresia and other structural anomalies in the congenital polysplenia syndrome.
        J Pediatr. 1974; 85: 649-655
        • Abramson SJ
        • Berdon WE
        • Altman RP
        • et al.
        Biliary atresia and noncardiac polysplenic syndrome: US and surgical considerations.
        Radiology. 1987; 163: 377-379
        • Lilly JR
        • Chandra RS
        Surgical hazards of co-existing anomalies in biliary atresia.
        Surg Gynecol Obstet. 1974; 139: 49-54
        • Maksem JA
        Polysplenia syndrome and splenic hypoplasia associated with extrahepatic biliary atresia.
        Arch Pathol Lab Med. 1980; 104: 212-214
        • Gershoni-Baruch R
        • Gottfried E
        • Pery M
        • Sahin A
        • Etzioni A
        Immotile cilia syndrome including polysplenia, situs inversus and extrahepatic biliary atresia.
        Am J Med Genet. 1989; 33: 390-393
        • Teichberg S
        • Markowitz J
        • Silverberg M
        • et al.
        Abnormal cilia in a child with the polysplenia syndrome and biliary atresia.
        J Pediatr. 1982; 100: 399-401
        • McClean JM
        Embryology of the pancreas.
        in: Howat HT Sarles H The exocrine pancreas. WB Saunders, Philadelphia1979: 3-15
        • Mowat AP
        • Psacharopoulos HT
        • Williams R
        Extrahepatic biliary atresia versus neonatal hepatitis.
        Arch Dis Child. 1976; 51: 763-770
        • Rose V
        • Izukawa T
        • Moes CAF
        Syndromes of asplenia and polysplenia.
        Br Heart J. 1975; 37: 840-852
        • Lowy C
        • Beard RW
        • Goldschmidt J
        Congenital malformations in babies of diabetic mothers.
        Diabetic Med. 1986; 3: 458-462
        • Fullana A
        • Garcia-Frias E
        • Martinez-Frias ML
        • Razquin S
        • Quero J
        Caudal deficiency and asplenia anomalies in sibs.
        Am J Med Genet. 1986; 2 (suppl): 23-29
        • Crace CJ
        Mechanisms of teratogenesis in diabetes mellitus.
        in: 2nd ed. Textbook of diabetes. Blackwell Scientific, London1991: 851-855
        • Ruben GD
        • Templeton JM
        • Zeigler MM
        Situs inversus: the complex inducing neonatal intestinal obstruction.
        J Pediatr Surg. 1983; 18: 751-756
        • Niikawa N
        • Kohasaka S
        • Mizumoto M
        • Hamada I
        • Kajii T
        Familial clustering of situs inversus totalis, and asplenia and polysplenia.
        Am J Med Genet. 1983; 16: 43-47
        • Opitz JM
        Editorial comment on the papers by de la Monte and Hutchins on familial polyasplenia.
        Am J Med Genet. 1985; 21: 175-176
        • Hutchins GM
        • Moore GW
        • Lipford EH
        • Haupt HM
        • Walker MC
        Asplenia and polysplenia malformation complexes explained by abnormal embryonic body curvature.
        Pathol Res Pract. 1983; 177: 60-76
        • Putschar WG
        • Manion WC
        Congenital absence of the spleen and associated abnormalities.
        Am J Clin Pathol. 1956; 26: 429-470
        • Marks C
        Surgical implications of portal venous system malformation.
        Ann R Coll Surg Engl. 1974; 55: 299-306
        • Dickson AD
        The development of the ductus venosus in man and the goat.
        J Anat. 1957; 91: 358-368
        • Campbell M
        • Denchar DC
        Absent inferior vena cava, symmetrical liver, splenic agenesis and situs inversus and their embryology.
        Br Heart J. 1967; 29: 268-275
        • Brassett C
        • Ellis H
        Transposition of the viscera.
        Clin Anat. 1991; 4: 139-147
        • Peoples WM
        • Moller JH
        • Edwards JE
        Polysplenia: a review of 146 cases.
        Pediatr Cardiol. 1983; 4: 129-137