J Korean Surg Soc.  2009 Dec;77(6):438-440. 10.4174/jkss.2009.77.6.438.

Hirschsprung's Disease in Two Siblings

• Jung E
• Choi SO
• Park WH

• KMID: 1464826
• DOI: http://doi.org/10.4174/jkss.2009.77.6.438

Abstract

Hirschsprung's disease (HD) is one of the major pediatric gastrointestinal disease entities which is associated with an absence or lack of intrinsic ganglion cells in the myenteric and submucosal plexus in the gastrointestinal tract. It is commonly assumed to be a sex-modified multifactorial trait. The development of diagnostic and therapeutic modalities has been ongoing. Herein, we experienced two siblings who were confirmed as having HD histologically and were treated. We think further family evaluation regarding HD is needed. Also we could see a changing modality of diagnosis and treatment.

Keyword

Hirschsprung's disease; Etiology; Siblings

MeSH Terms

Ganglion Cysts
Gastrointestinal Diseases
Gastrointestinal Tract
Hirschsprung Disease
Humans
Siblings
Submucous Plexus

Figure

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  Fig. 1 Family trees of four affected children in two siblings.

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