title: Long-term outcomes of biliary atresia with splenic malformation.
abstract: BACKGROUND
  We assessed the long-term outcomes of patients with biliary atresia with splenic malformation (BASM).
  
  
  METHODS
  We retrospectively assessed outcomes of 255 patients who underwent the Kasai procedure (KP) at our hospital between 1972 and 2014. Clinical outcomes of 11 patients with BASM (group A: nine with polysplenia, two with asplenia) and 244 patients with isolated BA (group B) were compared.
  
  
  RESULTS
  The incidence of early cholangitis and hepatopulmonary syndrome (HPS) was significantly higher in group A than in group B. Of the 11 group A patients, three died of severe cardiac defects during early infancy. Seven became jaundice free following KP, with three patients subsequently requiring liver transplantation (LTx). Four survived with their native livers for 2, 5, 22, and 23years, respectively. Overall 20-year survival rates were 63.6% and 66.5% and 20-year native liver survival rates were 29.0% and 47.3% in groups A and B, respectively. No significant difference in cumulative survival rates was observed between both groups.
  
  
  CONCLUSIONS
  Long-term outcomes in BASM patients without lethal cardiac defects were comparable to patients with isolated BA. Careful follow-up may be required in patients with BASM because of a potentially higher risk of secondary complications such as HPS.
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan. Electronic address: mnio@ped-surg.med.tohoku.ac.jp.
authors.forename: Masaki
authors.initials: M
authors.lastname: Nio
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Motoshi
authors.initials: M
authors.lastname: Wada
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Sasaki
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Hiromu
authors.initials: H
authors.lastname: Tanaka
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Tomohiko
authors.initials: T
authors.lastname: Watanabe
mesh_terms: D001656:Biliary Atresia / Q000150:complications* / Q000601:surgery*; D002761:Cholangitis / Q000209:etiology; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000209:etiology; D006801:Humans; D007223:Infant; D007565:Jaundice / Q000209:etiology; D016031:Liver Transplantation; D008297:Male; D011183:Postoperative Complications; D012189:Retrospective Studies; D013154:Spleen / Q000002:abnormalities* / Q000601:surgery; D015996:Survival Rate; D016896:Treatment Outcome
keywords: Asplenia; Biliary atresia; Long-term outcome; Polysplenia; Splenic malformation