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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 healthy colon is descended from behind of the aganglionic segment, performing the anastomosis between the two segments Fig. In this case, the aganglionic segment is not removed, but a rear dissection is performed. Ineluding sixty-seven consecutive Endorectal Pullthrough procedures.

Accllracy of the hariulll enema examination. Pathogenesis of Hirschsprung’s disease. Our patient had a history of long-standing bowel constipation, progressing to fecal incontinence. Some patients reach adulthood without a diagnosis for this disease.

In addition,it is also targeted to evaluate the clinical results from this patient. The infrequency of this diagnosis in this age and the clinical course of this patient underscore the uniqueness of this case.

Laboratory studies revealed leukocytosis with left enfermedas and elevated C-reactive protein. Como citar este artigo. A new surgical approach to extensive aganglionosis. Here are the surgery types:.

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

These patients tend to have milder forms of the disease, in which hypertrophy of innervated proximal colon compensates the functional obstruction by lack of innervation of the distal area 2. Clinical case Female patient, 13 years old, coming from Campo Grande – MS, reports that since birth had intestinal constipation, with mean bowel movements at every days with hardened feces, being followed-up by a pediatrician and in treatment for functional constipation. A healthy part of the small intestine or colon will be cut just above the damaged area.


Total colonic aganglionosis initially diagnosed in an adolescenl.

Enfermedad de Hirschsprung en el adulto. Pull-through—The inner layer hirscshprung the affected colon area is removed. Definition Hirschsprung disease HD is an area of the colon large intestine that has stopped working the right way. Severe constipation Diarrhea Growth delay. Congenital nirschsprung megacolon in Nigerian adults: This congenital disease is usually detected in neonatal period.

Other proposed mechanisms include defects in the neuroblast differentiation and accelerated destruction of ganglion cells in the intestine 4. It is rarely diagnosed in adult age. Peripheral aerial imagery was found in the dilated segment, gas-like wall, which is thickened in this segment. 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.

If you have one child with the disease, you could have more children with the disease. CT scan was performed, showing signs of peritonitis caused by dehiscence of ileum-rectal suture. HD is often diagnosed when an infant fails to have a bowel movement within 24 hours of birth.

Enfermedad de Hirschsprung by Yatziri Hdz on Prezi

Techniques chirurgicales appareil digestif. In our case, we are in a situation of irreversible dilatation of entire colon, so we opted for a total colectomy, restoring intestinal transit by ileo-rectal anastomosis.

The colon is a tube made of muscles. Organic constipalion in adults. Symptoms found in newborns include: A CT scan is a useful tool, not only to show the transition zone, but to exclude other causes of chronic constipation and megacolon in adults, such as colorectal cancer, volvulus, stricture, slowing of colonic motility, Chagas disease, anatomical or functional obstruction to defecation or idiopathic megacolon 2.

It usually presents as severe constipation with colonic dilatation proximal to the aganglionic segment.

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

Other things that may raise your child’s chance of HD are: This factor contributed to the delay in her diagnosis, driving the pediatrician out of a HD hypothesis. Fourtecn-ycars expcricnce in diagnosis and lrealment. An ostomy gives the remaining colon time to rest and heal. In this hirschspruny, it is probably due to a problem with genes.


The goal of surgery is to remove the part of the colon that does not work the right way. The healthy intestine will be connected to an opening in the abdomen.

Reviewing the medical history of the patient,the evolution of the anthropometric data weight and height as well as the analytical parameters for further studies were carried revvision. No nausea or vomiting. The key finding in barium enema is the existence of a transition zone “funnel” between distal aganglionic bowel normal or narrow and proximal dilated.

Plain films typically show a massive expansion of rwvision colon, with a small distal segment. The technique considered as the golden standard for the diagnosis of HD is the absence of ganglion cells in a rectal biopsy specimen.

Only a small number of cases remain undiagnosed after 5 years of age 2. This information is neither intended nor implied to be a substitute for professional medical advice.

Hirschsprung Disease

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. A later hirshsprung will be needed to reconnect the intestines. Megaeystis Illicrocolonintestinal hypoperistalsis synorollle: A resection of distal rectal mucosa of the aganglionic segment is performed, maintaining muscular wall of this segment.