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[Associated complication of biliary atresia: cystic dilatation of intrahepatic biliary system]
H Kawarasaki1, M Itoh, K Mizuta
1Department of Pediatric Surgery, University of Tokyo, Japan.
Insights
Cystic dilatation of the intrahepatic biliary system (CDIB) in biliary atresia patients presents differently. Early diagnosis and treatment of solitary cysts (Groups A & B) yield good outcomes, unlike multi-cystic forms (Group C).
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Medical Imaging
Background:
- Biliary atresia (BA) is a severe neonatal liver disease.
- Postoperative complications can arise, including biliary system abnormalities.
- Cystic dilatation of the intrahepatic biliary system (CDIB) is a rare sequela.
Observation:
- Ten patients with biliary atresia developed CDIB postoperatively.
- Symptoms included fever, jaundice, and acholic stools.
- Diagnostic tools: ultrasonography, CT, and cholangiography.
- CDIB classified into three groups based on morphology: solitary non-communicating (A), solitary communicating (B), and multi-cystic (C).
Findings:
- Group A (n=3) and Group B (n=1) showed favorable outcomes after percutaneous transhepatic bile drainage (PTBD) or re-do Kasai operation.
- Group C (n=6) had a poor prognosis, with two deaths, three requiring liver transplantation, and one ongoing PTBD.
- Outcomes correlate with the morphological classification of CDIB.
Implications:
- Early differentiation of CDIB subtypes is crucial for patient management.
- Solitary cystic forms (A & B) are amenable to surgical or interventional treatment.
- Multi-cystic dilatation (C) indicates a poorer prognosis and may necessitate advanced interventions like liver transplantation.
Abstract:
Cystic dilatation of intrahepatic biliary system (CDIB) was observed on 10 postoperative patients with biliary atresia (BA). Fever, jaundice and alcoholic stool were found when CDIB was diagnosed with ultrasonography and computed tomography assertained by cholangiography. CDIB was divided into three groups by the shape of intrahepatic biliary system. They were group A (n = 3); solitary non-communicating cyst, group B (n = 1); solitary communicating cyst and group C (n = 6); multi-cystic dilatation. Treatments of CDIB were percutaneous transhepatic bile drainage (PTBD) and reanastomosis of hepatic portoenterostomy. All of our patients of group A and B were doing well after PTBD in two and re-do Kasai operation in two. However, out of 6 patients of group C, two died without any treatments, three were transplanted liver from their parents and the rest one is still in the hospital and PTBD is continued. Outcome of CDIB is good in group A and B, and poor in group C.