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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The hirschsprunt of HD in adults is much harder than in children, due to their rarity as they are often short or ultrashort aganglionic segments, which produce mild symptoms in early stages of the disease. The objective of this study is to report a case of late diagnosis of the disease at age 13, with symptoms of fecal incontinence in its evolution.

In this case, the aganglionic segment is not removed, but a rear dissection is performed. Only cases of HD have been described in adults, with male predominance in a 4: It consists in a rear section of muscular wall of rectum starting from dentate line by transanal waywhich removes some of the top segment of internal anal sphincter, getting relief stenosis.

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

The lancet, Feb. The colon is a tube made of muscles. As observed in this patient, the association of HD and MWS can lead to the need of prolonged parenteral nutrition and frequently present diverting stoma leading to a greater number of postoperative complications in this population. This is the result of a lack of migration of neuroblasts from the neural crest to the large intestine during embryonic period. Rectum had a normal size. Emergency surgery was indicated, performing an anastomosis resection and end ileostomy.


The infrequency of this diagnosis in this age and the clinical course of this patient underscore the uniqueness of this case. 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.

Enfermedad de Hirschsprung | Grand Strand Heart & Vascular Care

This congenital disease is usually detected in neonatal period. A later surgery will be needed to reconnect the intestines. In addition, the upcoming issues associated with nutritional support and therapeutic strategies in the multidisciplinary context were evaluated. The diagnosis is made by barium enema, anorectal manometry and rectal biopsy. Enfermedad de Hirschsprung del adulto: Given these findings, emergency surgery was decided. HD presents itself by symptoms of constipation, such as a greater than h delay in elimination of meconium, abdominal distention and vomiting.

With this technique an extensive dissection of the anterior wall of the rectum is avoided, thus avoiding the risk of injuring adjacent structures. 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.

Our patient had a history of long-standing bowel constipation, progressing to fecal incontinence. Here are the surgery types: Hirschsprung’s disease HD or aganglionicmegacolon is a congenital disorder characterized by the absence of ganglion intramural cells of the submucosal myenteric plexus namely Auerbach and Meissner,respectively in distal sections of the intestine.


Accllracy of the hariulll enema examination.

No nausea or vomiting. Waste can pass through this opening to a bag. In older children, the doctor will ask about your child’s symptoms and health history. Hirschsprung disease and hypoganglionosis in adults: Pull-through—The hirschsprunv layer of the affected colon area is removed.

A hypothesis of HD was proposed; thus an anorectal manometry was asked, but its result was inconclusive due to patient’s lack of cooperation. Abdominal X-rays showed a dilated colon occupying abdominal cavity. The key finding in barium enema is the existence of a transition zone “funnel” between distal aganglionic bowel normal or narrow and proximal dilated.

Hirschsprung’s disease affects about 1 in 5, live hirsdhsprung and usually presents in neonatal period. Observing the Meissner and Auerbach plexus with immunohistochemical techniques and enolase S, loss of neurons ganglion cells and lower density of such plexuses were observed with respect to a normal colon Figs.

Enfermedad de Hirschsprung

Call Primary Health Group – Chippenham at Medicina de Caldas, vol. Suele diagnosticarse en el periodo neonatal, siendo muy poco frecuente que se descubra en el adulto. The primary defect is absence of ganglion cells in Meiss-ner plexus submucosal and Auerbach plexus myenteric in the affected segment of large bowel. A CT scan was performed, showing a massive dilatation of sigmoid colon, which compresses liver to the enfermeead upper enfermedas, stomach into the left upper quadrant and bowel loops into the right flank Figs.