Biliary atresia splenic malformation syndrome: an etiologic and prognostic subgroup.

摘要: BACKGROUND The polysplenia syndrome is the most common extrahepatic anomaly found in association with biliary atresia. This subgroup may have a different cause and worse prognosis than do infants atresia alone, this hypothesis has been tested by analyzing King's College Hospital series. METHODS case records of 308 treated between 1975 1991 for were examined anomalies. RESULTS Twenty-three (7.5%) had There also four other types splenic malformation: two double spleen asplenia. presence anomalies such as situs inversus portal vein all categories malformation suggests that they formed part larger which we now propose term (BASM) syndrome. was no difference age at presentation biochemical test results liver function before operation BASM those alone. Four (15%) born to mothers diabetes (three insulin dependent one gestational diet alone). cases maternal series whole. Actuarial "survival" (death or transplant) after initial corrective control group without anatomic (p < 0.05). CONCLUSIONS appears be distinct tends subjects. Whether caused (e.g., cardiovascular anomalies), are themselves detrimental, unclear.