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Pediatric Surgery International
Published in: Pediatric Surgery International 1/2012

01-01-2012 | Case Report

Congenital brachioesophagus with secondary intrathoracic stomach associated with rachischisis described as “serpentine-like syndrome”: a case report and literature review

Authors: Hiroki Nakamura, Tadaharu Okazaki, Hiroyuki Koga, Geoffrey J. Lane, Atsuyuki Yamataka

Published in: Pediatric Surgery International | Issue 1/2012

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Abstract

We report a male infant with severe brachioesophagus with intrathoracic stomach, duodenum, pancreas, and spleen associated with short neck because of rachischisis and scoliosis, an extremely rare condition described recently as “serpentine-like syndrome”. Our case died and we review four similar cases reported in the English literature.
Literature
1.
Woodward M, Crabbe DC, Kiely EM, Spitz L (1996) Congenital intrathoracic stomach. Pediatr Surg Int 11:116–118CrossRef
2.
Skandalakis JE, Gray SW, Ricketts R (1994) Embryology for surgeons. The embryological basis for the treatment of congenital abnormalities, 2nd edn. Williams and Wilkins, Baltimore, pp 98–100
3.
Katz MS, Hess DJ, Caty MG, Khan AR, Glick PL (2008) Of snakes and babies: intrathoracic stomach and vertebral rachischisis. A sepentine-like syndrome? J Ped Surg 43:1385–1389CrossRef
4.
Deprez FC, Debauche C, Clapuyt P, Goyet JV (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. J Ped Surg 44:E25–E28CrossRef
5.
Leung AWK, Lam HS, Chu WCW, Lee KH, Tam YH, Ng PC (2008) Congenital intrathoracic stomach: short esophagus or hiatal hernia? Neonatology 93:178–181PubMedCrossRef
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Pierre R, Eduardo DR, David AP (2004) Pathology of pediatric gastrointestinal and liver disease. Springer, New York, pp 3–18
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O’Rahilly R, Muller F (1996) Human embryology and teratology, 2nd edn. Wiley, New York, pp 329–344
Metadata
Title
Congenital brachioesophagus with secondary intrathoracic stomach associated with rachischisis described as “serpentine-like syndrome”: a case report and literature review
Authors
Hiroki Nakamura
Tadaharu Okazaki
Hiroyuki Koga
Geoffrey J. Lane
Atsuyuki Yamataka
Publication date
01-01-2012
Publisher
Springer-Verlag
Published in
Pediatric Surgery International / Issue 1/2012
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI
https://doi.org/10.1007/s00383-011-3000-7

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