Biliary atresia-splenic malformation (BASM) syndrome: A case report

Highlights

• The exact cause of biliary atresia is unknown.

• The prenatal diagnosis of BASM remains challenging.

• The operation time for biliary atresia splenic malformation syndrome (BASM) is still the major prognostic factor.

• The pathology in BASM can continue even after a postnatal period.

• Although it is controversial, the patient can benefit from steroid administration.

Abstract

Introduction and importance

Biliary atresia (BA) is characterized by the presence of persistent cholestatic jaundice during the neonatal period. This group of patients is at higher risk for liver disease and/or portal hypertension compared with other chronic liver diseases.

Case presentation

We present a newborn patient who had biliary stools early postnatally, but her gallbladder was not seen on radiological examination and was referred to our clinic. On examination, polysplenia was seen, and the inferior vena cava was not seen. The patient's stool color changed to a creamy stool without bile three weeks postnatally. The neonate was diagnosed with biliary atresia and splenic malformation (BASM) syndrome. The patient underwent a Kasai portoenterostomy in the 3rd week of age and was preoperatively diagnosed with malrotation and treated with a Ladd procedure at the same time. The patient had a postoperative stool with bile and decreased bilirubinemia with a normal defecation period.

Clinical discussion

BASM syndrome, clinically has different causes and a worse prognosis than isolated BA. The syndrome can lead to end-stage cirrhosis and liver failure if left untreated. Although timely Kasai surgery is the standard treatment for BA, the age of the patient at the time of the surgery is different depending on BA groups.

Conclusion

The most important prognostic factors for BASM syndrome are early diagnosis and treatment. Despite this, the patients need careful follow-up. Postoperative adjuvant steroid therapy may have a good impact on the outcomes.