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Pediatric Surgery International

Erschienen in:

01.01.2012 | Case Report

Uncommon co-occurrence of ileal atresia and total colonic aganglionosis in two unrelated newborns

verfasst von: Paul Daher, Lara Raffoul, Edward Riachy, Rana Mitri

Erschienen in: Pediatric Surgery International | Ausgabe 1/2012

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Abstract

We report two recent cases of distal ileal atresia associated with total colonic aganglionosis (TCA). It is well known that ileal atresia and Hirschsprung’s disease (HD) are individually frequent causes of intestinal obstruction. However, the association of both these diseases is an extremely rare event. To our knowledge, only 19 cases of ileal atresia associated with HD have been described so far. When a child is diagnosed having ileal atresia, the possibility of associated TCA should be considered. Therefore, intra-operative staged biopsies should be sent for histological examination in order to rule out or confirm this very rare co-occurrence.

Gupta M, Beeram MR, Pohl JF et al (2005) Ileal atresia associated with Hirschsprung’s disease (total colonic aganglionosis). J Pediatr Surg 40:5–7CrossRef

Moore SW (2009) Total colonic aganglionosis and Hirshsprung’s disease: shades of the same or different? Pediatric Surg Int 25:659–666CrossRef

Horigome F, Seki T, Kobayashi H et al (2007) Developmental anomalies of the enteric nervous system in normoganglionicsegments of bowel from rats with total colonic aganglionosis. Pediatr Surg Int 23:991–995PubMedCrossRef

Janik JP, Wayne ER, Janik JS et al (1997) Ileal atresia with total colonicaganglionosis. J Pediatr Surg 32:1502–1503PubMedCrossRef

Escobar MA, Grosfeld JL, West KW et al (2005) Long-term outcomes in totalcolonic aganglionosis: a 32-year experience. J Pediatr Surg 40:955–961PubMedCrossRef

Marquez TT, Acton RD, Hess DJ et al (2009) Comprehensive review of procedures for total colonic aganglionosis. J Pediatr Surg 44:257–265PubMedCrossRef

Titel: Uncommon co-occurrence of ileal atresia and total colonic aganglionosis in two unrelated newborns
verfasst von: Paul Daher
Lara Raffoul
Edward Riachy
Rana Mitri
Publikationsdatum: 01.01.2012
Verlag: Springer-Verlag
Erschienen in: Pediatric Surgery International / Ausgabe 1/2012
Print ISSN: 0179-0358
Elektronische ISSN: 1437-9813
DOI: https://doi.org/10.1007/s00383-011-2967-4

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Uncommon co-occurrence of ileal atresia and total colonic aganglionosis in two unrelated newborns

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