Impacts of Early Kasai Portoenterostomy on Short-Term and Long-Term Outcomes of Biliary Atresia.

There are discrepancies regarding the clinical impact of age at Kasai portoenterostomy (KP) on surgical outcomes. Hence, we re-assessed the clinical significance of age at KP. We analyzed 224 patients with type III (atresia of bile duct at the porta hepatis) biliary atresia at Tohoku University Hospital. We classified patients into two groups: KP at ≤60 days of age (group TE) and >60 days of age (group TL). Group TE was subdivided into three groups (TE1, TE2, and TE3) according to age at time of surgery. Subsequently, 2,643 patients in the Japanese Biliary Atresia Registry were classified similarly. Background and surgical outcomes were compared. Of the 2,643 cases, 323 patients who underwent revision KP were analyzed separately. The jaundice clearance rates (JCRs) were 81.4%, 100%, 64.7%, 83.0%, and 65.2% of patients in the TE, TE1, TE2, TE3, and TL groups, respectively. The 15-year native liver survival rates of patients in the TE, TE1, TE2, TE3, and TL groups were 62.2%, 88.9%, 33.9%, 64.4%, and 42.9%, respectively. The 30-year native liver survival rates of patients in the TE, TE1, TE2, TE3, and TL groups were 38.6%, 74.1%, 25.4%, 35.8%, and 31.7%, respectively. The JCRs were 66.2%, 69.4%, 64.1%, 66.7%, and 59.7% for patients in groups JE, JE1, JE2, JE3, and JL, respectively. The 15-year native liver survival rates were 48.1%, 56.7%, 43.9%, 48.9%, and 37.2% for patients in groups JE, JE1, JE2, JE3, and JL, respectively. The JCRs following revision KP were higher in the JE1 group than in the other groups. Early KP was associated with favorable outcomes except in patients aged 31-45 days.