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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Suele diagnosticarse en el periodo neonatal, siendo muy poco frecuente que se descubra en el adulto. Fifty per cent of the patients are younger than 30 years of age 3. Family members with the disease Presence of Down Syndrome Presence of other birth defects. Enfermedad de Hirschsprung en el adulto. After 24 hours, the patient presented hemodynamic instability, with severe abdominal pain.

A detailed description of the composition of parenteral nutrition administered within the period of 28 days of hospital admission. The work of the colon is controlled by a number of nerves. The technique considered as the golden standard for the diagnosis of HD is the absence of ganglion cells in a rectal biopsy specimen.

Enfermedad de Hirschsprung | Dallas Medical Specialists

Symptoms found in newborns include: It often happens in the lower part of the colon but can sometimes include the whole colon. The diagnosis is made by barium enema, anorectal manometry and rectal biopsy.


No nausea or vomiting. CT scan was performed, showing enfermedas of peritonitis caused by dehiscence of ileum-rectal suture. This content is reviewed regularly and is updated when new and relevant evidence is made available.

Genetic counseling can help you learn more about the risk. A new surgical approach to extensive aganglionosis.

Enfermedad de Hirschsprung

The treatment is surgical, in order to remove the aganglionic segment and restore digestive continuity. There was neuronal dysplasia with scarce presence of ganglion cells on both edges of resection.

The girl refers onset of fecal incontinence at the age of Myomectomy has the advantage of being technically easier and presenting a low morbidity, but it has worse functional outcomes and can only be used in ultra-HD 7,8.

It can then be joined to the rectum so that stool can pass through. With this technique an extensive dissection of the anterior wall of the rectum is avoided, thus avoiding the risk of injuring adjacent structures.

Hirschsprung disease associated with Mowat-Wilson syndrome: report of a case.

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. Serology for Chagas’ disease was carried out in order to exclude a diagnosis of Chagas’ Disease megacolon, with negative result.

Ahsense of hirschspprung neurons in the aganglionic colon. Waste movement can get slowed or stopped in the area. In this case, the aganglionic segment is not removed, but a rear dissection is performed. Accllracy of the hariulll enema examination. An x-ray using special dye to view the colon Biopsy of bowel tissue.


The disadvantage of this technique is that it requires a new intervention to restore the intestinal transit. Postoperative evolution was torpid, with difficulty feedback by adynamic ileus and infection of surgical wound.

Hirschsprung disease associated with Mowat-Wilson syndrome: report of a case.

A developmental model and approach. This information is neither intended nor implied to be a substitute for professional medical advice. Diagnostic Imaging in children with acute chest and abdominal disorders.

Hirschsprung’s disease HD or aganglionicmegacolon is a congenital disorder characterized by the absence of ganglion intramural enferkedad of the submucosal myenteric plexus namely Auerbach and Meissner,respectively in distal sections of the intestine.

HD that is less serious may not hirschspruung noticed until later in life.

Some cases involve gene mutations for endothelin-B receptor. Primary aganglionosis associated with imperforate anus review of the litcraturc pcrtinent to one observation.

Hirschsprung’s disease in a young adult: It is rarely diagnosed in adult age. Mechanisms of idiopathic constipation:

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