Tohoku J. Exp. Med., 1997, 181 (1)

Biliary, Anorectal and Esophageal Atresia: A New Entity ?

ANTONIO DESSANTI1, GIOVANNINO MASSARELLI2, MARIA T. PIGA3, ALBERTO PORCU1 and GIUSEPPE DETTORI1

1Unit of Pediatric Surgery -Department of Surgical Clinic, 2Department of Pathology; 3Institute of Neonatology; School of Medicine, University of Sassari, Sassari, 07100, Italy

  • A unique case of newborn biliary atresia associated with esophageal atresia and tracheoesophageal fistula, anorectal atresia, Reovirus type 3 infection and an early switch of fetal into adult hemoglobin is reported. At birth, the infant, who had only one umbilical artery, was operated on by primary anastomosis of the esophagous, and descending colostomy. At six weeks of age the baby underwent a "Kasai hepatic portoenterostomy-Type I" for a EHBA Type III, Subtype C2, Subgroup O ( "aplasia" of all extrahepatic biliary ducts, including the gallbladder). The absence of an artery branch for the left lobe of the liver was observed. Histologically, the liver showed a hyperplasia of the intrahepatic bile ducts due to persistence of an excess of embryologic bile ducts in "ductal plate malformation" (DPM). Specific Reovirus type 3 antibodies were found in both the mother's and baby's sera. In the postoperative period the infant developed rapid and severe liver failure and underwent a successful liver transplantation. Although in most cases EHBA appears to be a perinatal event due to a necroinflammatory process of unknown etiology, cases associated with complex extrahepatic anomalies, may be due to different pathogenetic mechanisms supported by different causative agents operating very early in the fetal period. Viral infection seems to be the most reliable etiology.
    Key word(s)--- biliary atresia; congenital malformations; anorectal atresia; esophageal atresia; Reovirus type 3 infection

    Tohoku J. Exp. Med., 1997, 181, 49-55
    Address for reprints: Antonio Dessanti, M.D., Unit of Pediatric Surgery-Department of Surgical Clinic, University of Sassari School of Medicine, Viale S. Pietro 43 Sassari 07100, Italy.

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