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

Fabrice C. Deprez, Christian Debauche, Philippe Clapuyt, Jean de Ville de Goyet

Research output: Contribution to journalArticle

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.

Original languageEnglish
JournalJournal of Pediatric Surgery
Volume44
Issue number8
DOIs
Publication statusPublished - Aug 2009

Keywords

  • Cervical vertebra fusion
  • Congenital short esophagus
  • Embryology
  • Intrathoracic stomach
  • Klippel-Feil syndrome
  • Prenatal diagnosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

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