Hirschsprung disease is a developmental disorder characterized by absence of If a rectal biopsy does not show ganglion cells, revision of the. Publisher: Introducción: La enfermedad de Hirschsprung (EH) o Métodos: A través de la revisión de la historia clínica, se estudió la evolución. Palavras-chave: Doença de Hirschsprung; Megacolo congênito; Incontinência fecal. Introduction . Revisión: Enfermedad de Hirschsprung. Rev Ped Elec.

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A physical exam will be done. This means that part of the colon can fe relax and fully open. The goal of surgery is to remove the part of the colon that does not work the right way. Some patients reach adulthood without a diagnosis for this disease.

It is rarely diagnosed in adult age.

Hirschsprung’s Disease HDalso known as congenital aganglionic megacolon, is an anomaly characterized by an absence of ganglion cells in the myenteric and submucosal plexuses in a variable bowel segment. Rectal lI1yectoll1Y for aganglionic megacoIon. CT scan was performed, showing signs of peritonitis caused by dehiscence of ileum-rectal suture.

Hirschsprung Disease | Primary Health Group – Chippenham

Department of General and Gastrointestinal Surgery. Some families may have a strong history of HD.

It can then be joined to the rectum so that stool can pass through. Family members with the disease Presence of Down Syndrome Presence of other birth defects.

Other things that may raise your child’s chance of HD are: EnBretano, informa casos similares. Hirschsprung’s disease HD is a malformation of the large intestine characterized by the absence of ganglion cells in submucosal and myenteric plexus, which produces a functional obstruction and dilatation proximal to the affected segment 1.


Rectum had normal caliber. Comparison with c1inical amI radiological criteria. Primary aganglionosis associated with imperforate anus review of the litcraturc pcrtinent to one observation.

Enfermedad de Hirschsprung

A transition zone was appreciated in distal sigmoid colon. Another option in these cases is the realization of an end ileostomy following colectomy, to avoid the risk of a possible wound dehiscence, as it happened in our case. Diagnosis HD is often diagnosed when an infant fails to have a bowel movement within 24 hours of birth.

In addition, it is established that factors related to gender play a role, since men are preferentially affected, at a frequency of 4: In addition, the reported symptoms caused the girl’s parents to begin to justify such a fact as a result of some psychological, rather than organic, disorder.

Changes in colorectal function in severe idiopathic chronic constipalion. Definition Hirschsprung disease HD is an enfermeedad of the colon large intestine that has stopped working the right way.

An experimental study on aganglionosis produced by a new method in the rat. Previous history of meningioma operated inintestinal resection in childhood unknown etiologychronic constipation, smoker of 20 cigarettes per day and moderate drinker.

Hirschsprung Disease

A midline laparotomy was performed, finding a massive dilatation of sigmoid colon, attaining a diameter of 44 cm at its more dilated portion.

The endermedad is surgical, in order to remove the aganglionic segment and restore digestive continuity. This information is neither intended nor implied to be a substitute for professional medical advice. The colon is a tube made of muscles. Discussion HD is a congenital anomaly that occurs due to a discontinuation of the cranial-caudal migration of neural crest cells, which are responsible for innervation of the colon, or when the ganglion cells undergo premature death between 5th and 12th weeks of pregnancy.


Serology for Chagas’ disease was carried out in order to exclude a diagnosis of Chagas’ Disease megacolon, with negative result. Peripheral aerial imagery was found in the dilated segment, gas-like wall, which is thickened in this segment.

For our patient, fecal incontinence is a more limiting symptom than the constipation itself, because the girl was living in constant fear of occurrence enfemedad such fact in environments like her school, which would cause great embarrassment before other people.

Introduction Hirschsprung’s Disease HDenfermeadd known as congenital aganglionic megacolon, is an anomaly characterized by an absence of ganglion cells in the myenteric and submucosal plexuses in a variable bowel segment. Hirschsprung’s disease; Congenital megacolon; Fecal incontinence. A CT scan was performed, showing a massive dilatation of sigmoid colon, which compresses liver to the right upper quadrant, stomach into the left upper quadrant and bowel loops into the right flank Figs.