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.

Deprez, Fabrice C;Debauche, Christian;Clapuyt, Philippe;de Ville de Goyet, Jean
(2009) Journal of Pediatric Surgery — Vol. 44, n° 8, p. E25-28 (2009)

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Authors
  • Deprez, Fabrice CUCLouvain
    Author
  • Debauche, ChristianUCLouvain
    Author
  • Clapuyt, PhilippeUCLouvain
    Author
  • de Ville de Goyet, Jeanorcid-logoUCLouvain
    Author
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.
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Deprez, F. C., Debauche, C., Clapuyt, P., & de Ville de Goyet, J. (2009). 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. Journal of Pediatric Surgery, 44(8), E25-28. https://doi.org/10.1016/j.jpedsurg.2009.05.024 (Original work published 2009)