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 language | English |
|---|---|
| Journal | Journal of Pediatric Surgery |
| Volume | 44 |
| Issue number | 8 |
| DOIs | |
| Publication status | Published - Aug 2009 |
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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
Cite this
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.
In: Journal of Pediatric Surgery, Vol. 44, No. 8, 08.2009.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Multiorgan developmental anomalies presenting as a variation of the serpentine-like syndrome
T2 - cervical fusion and brachioesophagus with intrathoracic stomach and malposition of duodenopancreas and spleen
AU - Deprez, Fabrice C.
AU - Debauche, Christian
AU - Clapuyt, Philippe
AU - de Ville de Goyet, Jean
PY - 2009/8
Y1 - 2009/8
N2 - 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.
AB - 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.
KW - Cervical vertebra fusion
KW - Congenital short esophagus
KW - Embryology
KW - Intrathoracic stomach
KW - Klippel-Feil syndrome
KW - Prenatal diagnosis
UR - http://www.scopus.com/inward/record.url?scp=67650935179&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=67650935179&partnerID=8YFLogxK
U2 - 10.1016/j.jpedsurg.2009.05.024
DO - 10.1016/j.jpedsurg.2009.05.024
M3 - Article
C2 - 19635288
AN - SCOPUS:67650935179
VL - 44
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 8
ER -