Obstrucción intestinal de debut tardío

  1. E. Cid París
  2. G. Galicia Poblet
  3. Gema Arriola Pereda
  4. M.E. Rubio Jiménez
  5. M. Pangua Gómez
  6. Ana Serrano González
  7. J. M. Jiménez Bustos
Revista:
Revista española de pediatría: clínica e investigación

ISSN: 0034-947X

Ano de publicación: 2010

Volume: 66

Número: 3

Páxinas: 193-196

Tipo: Artigo

Outras publicacións en: Revista española de pediatría: clínica e investigación

Resumo

Hirschsprung's disease is a congenital disorder of the colon in which certain nerve cells, known as ganglion cells, are absent, causing chronic constipation. A barium enema is the mainstay of diagnosis of Hirschsprung's, though a rectal biopsy showing the lack of ganglion cells is the only certain method of diagnosis. The usual treatment is excision of the aganglionic colon and "pull-through" surgery of ganglionic colon with a coloanal anastomosis. We report a case of Hirschsprung's disease with bowel obstruction in the infant period.

Referencias bibliográficas

  • De Manueles J. Enfermedad de Hirschsprung. Asociación Española de Pediatría 2008. 6: 56-61
  • Facer P, Knowles CH, Thomas PK, et al. Decreased tyrosine kinase C expression may reflect developmental abnormalities in Hirschsprung's disease and idiopathic slow-transit constipation. Br J Surg 2001; 88: 545-52.
  • Liu CP, Li XG, Lou JT, et al. Association analysis of the PHOX2B gene with Hirschsprung disease in the Han Chinese population of Southeastern China. J Pediatr Surg 2009; 44: 1805-11.
  • De Pontual L, Zaghloul NA, Thomas S, et al. Epistasis between RET and BBS mutations modulates enteric inervation and causes syndromic Hirschsprung disease. Proc Natl Acad Sci U S A 2009. 106: 13921-6
  • Karaca I, Turk E, Ortac R, el al. Waardenburg síndrome with extended aganglionosis: report of 3 new cases. J Pediatr Surg 2009; 44: E9-13.
  • Lorijn F, Kremer LC, Reitsma JB, et al. Diagnostic tests in Hirschsprung disease: a systematic review. J Pediatr Gastroenterol Nutr 2006; 42:496-505.
  • Guinard V, Bonnard A, De Lagausie P. Calretinin inmunohistochemistry: a Simple and efficient tool to diagnose Hirschsprung disease. Mod Pathol 2009; 22: 1379-84.
  • Kuyk EM, Brugman-Boezeman AT, Wissink-Essink M, et al. Defecation problems in children with Hirschsprung's disease: a prospective controlled study of a multidisciplinary behavioural treatment. Acta Paediatr 2001; 90:1153-9.