title: [Biliary atresia and polysplenia syndrome].
abstract: Polysplenia syndrome is a rare malformation characterized by the association of multiple rates and other congenital anomalies dominated by cardiac, vascular, intestinal and bile malformations. We report the observation of a patient operated in the neonatal period (3 days) for an upper intestinal obstruction with situs inversus. Surgical exploration noted the presence of multiple rates, a preduodenal vein, a biliary atresia and a duodenal atresia. The surgical procedures performed were a latero-lateral duodeno-duodenostomy and hepatoportoenterostomy of KASAI with simple immediate and delayed outcomes. The follow up was of 23 years. We recall the epidemiological characteristics of this malformative association and we discuss the role played by the prognosis of polysplenia syndrome in the evolution of biliary atresia. The diagnosis and treatment of biliary atresia are always urgent to increase the chances of success of the Kasai, and the chances of prolonged survival with native liver. However, almost all long-term survivors (even anicteric) have biliary cirrhosis, which requires lifelong follow up.
authors.forename: Yosra
authors.initials: Y
authors.lastname: Kerkeni
authors.forename: Amine
authors.initials: A
authors.lastname: Ksia
authors.forename: Hayet
authors.initials: H
authors.lastname: Zitouni
authors.forename: Mohsen
authors.initials: M
authors.lastname: Belghith
authors.forename: Sahnoun
authors.initials: S
authors.lastname: Lassad
authors.forename: Imed
authors.initials: I
authors.lastname: Krichene
authors.forename: Mongi
authors.initials: M
authors.lastname: Mekki
authors.forename: Abdellatif
authors.initials: A
authors.lastname: Nouri
mesh_terms: D001656:Biliary Atresia / Q000150:complications* / Q000601:surgery; D059446:Heterotaxy Syndrome / Q000150:complications*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D035583:Rare Diseases