Biliary Atresia Splenic Malformation Syndrome: A Single Center Experience

Objective: Biliary atresia splenic malformation (BASM) syndrome which is a subgroup of BA is associated with situs inversus, intestinal malrotation, polysplenia, preduodenal portal vein, interrupted vena cava, congenital portocaval shunts and cardiac anomalies. We aimed to report our experiences in BASM management and association of CMV infection. Materials and Methods: The data were collected retrospectively from medical records of patients treated in Cukurova University between 2005-2017. Sex, age, liver function tests, serological test results, BA types, surgical findings, and mortality were noted. Results: Fifty-nine BA patients were diagnosed in the study period. Seven of them were classified as BASM. The median age was 60 days (45-90 days) with a female/male ratio of 3/4. The main complaint of all patients was jaundice. The jaundice of 6 patients began since birth and one began at 20 daysage. Median total/direct blood bilirubin levels were 9.6/5.4 mg/dL. Median values of liver function tests; ALT, AST, and GGT were 77 IU/L, 201 IU/L and 607 IU/L respectively. Five of the patients showed positive results for anti-CMV Ig M. All patients had positive anti-CMV Ig G. One patient had type 2 BA and all others had type 3 BA. Associated anomalies were polysplenia (n=4), asplenia (n=1), preduodenal portal vein (n=5), midgut malrotation (n=7), inferior vena cava interruption (n=1) and hepatic artery originating from superior mesenteric artery (SMA) (n=1). Patients had Ladd procedure (n=7), duodenoduodenostomy (n=5) along with Kasai portoenterostomy. The median follow-up time was 4 years (1-5 years). All patients are alive and one had liver transplantation. Conclusion: Patients with BASM represent a distinct subgroup of BA which may have additional gastrointestinal anomalies such as midgut malrotation and preduodenal portal vein. Thus additional procedures such as duodenoduodenostomy and Ladd procedure may be added to Kasai portoenterostomy. Further research is recommended for CMV infections role in BASM pathogenesis.