title: Multiorgan developmental anomalies presenting as a variation of the serpentine-like syndrome: cervical fusion and brachioesophagus with intrathoracic stomach and malposition of duodenopancreas and spleen.
abstract: Congenital brachioesophagus with secondary intrathoracic stomach is an extremely rare condition. In association with rachischisis, the latter condition has been described recently as "serpentine-like syndrome." We report here a unique case of a male infant with normal karyotype from healthy nonconsanguinous parents, presenting with a complex malformative syndrome combining ultrashort brachioesophagus with intrathoracic stomach, duodenum, pancreas, and spleen associated with short neck because of posterior fusion and scoliosis. This case report details aspects of diagnostic and management of this unusual clinical presentation and includes a review of the literature.
authors.affiliation: Radiology Department, Cliniques St Luc-UCL, Brussels, Belgium.
authors.forename: Fabrice C
authors.initials: FC
authors.lastname: Deprez
authors.forename: Christian
authors.initials: C
authors.lastname: Debauche
authors.forename: Philippe
authors.initials: P
authors.lastname: Clapuyt
authors.forename: Jean
authors.initials: J
authors.lastname: de Ville de Goyet
mesh_terms: D002574:Cervical Vertebrae / Q000002:abnormalities; D000013:Congenital Abnormalities / Q000000981:diagnostic imaging*; D004386:Duodenum / Q000002:abnormalities; D004947:Esophagus / Q000002:abnormalities; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010179:Pancreas / Q000002:abnormalities; D011247:Pregnancy; D011256:Pregnancy Outcome; D013154:Spleen / Q000002:abnormalities; D013270:Stomach / Q000002:abnormalities; D013577:Syndrome; D016216:Ultrasonography, Prenatal