Biliary Atresia Splenic Malformation Syndrome: A Single Center Experience

Abstract

Abstract:

Introduction: The cause of biliary atresia (BA) is not understood exactly as well as biliary atresia splenic malformation (BASM) syndrome. BA is destructive biliary fibrosis; the etiology may be multifactorial. Association of cytomegalovirus (CMV) and BA have been shown in many reports but CMV and BASM have not been mentioned in the literature. So we aimed to report BASM experiences, an association of CMV infection and need of duodenoduodenostomy if preduodenal portal vein exists.

Materials and Methods:  The data were collected retrospectively from Cukurova University which is one of the largest tertiary hospitals in Turkey between 2005-2017. The patients of sex, age, blood chemistry counts, TORCH infections blood parameters, BA types, operational findings and mortality were noted.

Results: In total, 59 BA patients were diagnosed between 2005- 2017. Seven of them were classified as BASM.  The median age of them was 60 days (45-90 days). Three of them were girl and 4 of them were male in gender. The main complaint of whole patients was jaundice. The jaundice of 6 patients began since birth. One of them began at 20 days-age. 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 immunoglobulin M. All had positive anti-CMV Ig G and anti-toxoplasmosis Ig G.

Evaluation of the types of BA revealed that one patient had type 2, while all others had type 3. Four of BASM patients had polysplenia and one had asplenia. Five of them had a preduodenal portal vein. All of them had midgut malrotation. One had inferior vena cava interruption. One had hepatic artery anomaly which was originated from SMA.

The median time of follow-up was 4 years (1-5 years). All of them are alive and 1 required liver transplantation.

Conclusion: BASM should be kept in mind by the surgeon for the requirement of additional surgical procedures such as Ladd procedure, duodenoduodenostomy with Kasai Porto-enterostomy.

Duodenoduodenostomy may be performed when the existence of preduodenal portal vein.

Further research is recommended for CMV infection and BASM.