Los bebes con problemas para vaciar los intestinos a veces tienen un problema denominado enfermedad de Hirschsprung. El tratamiento para esta. Hirschsprung disease (HSCR), or congenital intestinal aganglionosis, is a birth defect characterized by complete absence of neuronal ganglion. In Hirschsprung disease, there are no ganglion cells in the wall of the affected intestine. % of children with Hirschsprung disease have the rectum and.

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Hirschsprubg is commonly characterised by a short segment of colonic aganglionosis affecting term neonates, especially boys. Hirschsprung disease affects approximately 1: In short segment disease, there is a significant predilection for males M: Interestingly, it is almost never seen in premature infants.

The condition typically presents in term neonates with failure to pass meconium in the first days after birth, although later presentation is also common. A very small number may ehfermedad in the adult population 1.

Enfermedad de Hirschsprung

Hirschsprung disease is characterised by aganglionosis absence of ganglion cells in the distal colon and rectum. It is thought to either occur from a failure of neuroblasts in neural crest cells to migrate into bowel segments or degeneration of already migrated neuroblasts.

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It affects cells both in the myenteric and submucosal plexuses 4.

Hence, functional obstruction develops as a result of a spasm in the denervated colon. It can be anatomically divided into four types according to the length of the aganglionic segment:. It is postulated that hypoganglionosis hirscsprung number of ganglion cells handles intestinal pseudo-obstruction 4.

Findings are primarily those of a bowel obstruction. The affected bowel is of smaller caliber and thus depending on the length of segment affected variable amounts of colonic distension are present.

In protracted cases, marked dilatation can develop, which may progress to enterocolitis and perforation. A carefully performed contrast enema is indispensable in both the diagnosis of Hirschsprung disease but enfermedax in assessing the length of involvement.

It should be noted however that the depicted transition hircshsprung on the contrast enema is not accurate at determining the transition between absent and present ganglion cells.

The affected segment is of small caliber with proximal dilatation. Surgical treatment is by removal of the affected portion of the colon. Where this is successful, the prognosis is good.


Hirschsprung Disease Overview – GeneReviews┬« – NCBI Bookshelf

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Log in Sign up. Articles Cases Enfermecad Quiz. About Blog Go ad-free. Epidemiology Clinical presentation Pathology Radiographic features Treatment and prognosis History and etymology Differential diagnosis References Images: Hirschsprung disease and hypoganglionosis in adults: Edit article Share article View revision history. Synonyms or Alternate Spellings: Support Radiopaedia and see fewer ads.

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