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1595 Postnatal Management for Prenatally Diagnosed Biliary Cystic Malformations (BCM)
  1. T Okada,
  2. S Honda,
  3. H Miyagi,
  4. A Taketomi
  1. Department of Gastroenterological Surgery I, Hokkaido University Graduate School of Medicine, Sapporo, Japan

Abstract

Background and Aims The aim of this study was to determine an appropriate postnatal management plan for prenatally diagnosed BCM including cystic biliary atresia (BA) and choledochal cyst (CC).

Methods From 2002 to 2011, a total of 27 consecutive children with CC were treated at our institute. Eight of our 27 patients with CC were diagnosed prenatally and examined clinically. Of these 8 patients, 2 (Group A) underwent delayed primary definitive surgery after percutaneous transhepatic cholangiodrainage (PTCD), 2 (Group B) underwent early definitive surgery in the neonatal period, and 4 (Group C) underwent delayed primary definitive surgery without PTCD in early infancy. Prenatally diagnosed cystic BA was consisted with 2 of patients with type 1 cystic BA (Group D).

Results The operation was difficult for adhesion in Group A. The diameter of the anastomosis in the hepaticojejunostomy was small and anastomotic leakage occurred in one of Group B. In all 2 patients, BA was recognized as final diagnosis at laparotomy and a hepaticojejunostomy was carried out because of correctable type. The pathological findings of liver biopsy revealed slight fibrosis of Glisson’s sheath in 6 of 8 CC patients. Severe liver fibrosis presented in one of two in cystic BA patients.

Conclusion In symptomatic CC patients, PTCD appears to be indicated only under certain circumstances, and delayed primary definitive surgery should be performed as early as possible thereafter. Clinicians need to be aware of cystic BA and how to distinguish it from CC to avoid inadequate primary surgical intervention.

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