Surgical treatment of Hirschsprung’s disease at the National Children’s .. Enfermedad de Hirschprung. Fantobal-Rojas A. Constipación crónica en el niño. La enfermedad de Hirschsprung es un trastorno congénito infrecuente (afecta aproximadamente a 1 de cada recién nacidos en EE. UU.) que provoca. El estreñimiento se debe diferenciar de la enfermedad de Hirschsprung y de alteraciones anatómicas y metabólicas. La evaluación clínica incluye una historia.

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It 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 present in the adult population 1.

Enfermedad de Hirschsprung | Valley Regional Medical Center

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


Hence, functional obstruction develops as a result of hirschspruung 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 reduced 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 also in assessing the length of involvement. It should be noted however that the depicted transition zone 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. You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Unable to process the form. Check for errors and try again. Thank you for updating your details. Log in Sign up.

[Hirschsprung’s disease: the immunohistochemistry as ancillary method for the diagnosis.]

Articles Cases Courses 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. Case 2 Case 2. Case 3 Case 3.

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