MEGACOLON CONGÉNITO O ENFERMEDAD DE HIRSCHSPRUNG Esta enfermedad es un trastorno multigénico hereditario que se transmite de manera . Report. Megacolon Aganglionar Congenito (Enfermedad de Hirschsprung). LR. Luis Rivera. Updated 24 April Transcript. Megacolon Aganglionar. Hirschsprung disease (HD) is a motor disorder of the gut, which is caused by the failure of neural crest cells (precursors of enteric ganglion.

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Megacolon Aganglionar Congenito (Enfermedad de Hirschsprung) by Luis Rivera on Prezi

With diagnostic methods already established in the literature, the sole treatment is surgery. The objective of this study is to report a case of late diagnosis hirschaprung the disease at age 13, with symptoms of fecal incontinence in its evolution. 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.

The absence of ganglion cells results in permanent contraction of the affected segment, preventing the passage of fecal content through that region. HD presents itself by symptoms of constipation, such as a greater than h delay in elimination of meconium, abdominal distention and vomiting. The infrequency of this diagnosis in this age and the clinical course of this patient underscore the uniqueness of this case. Mmegacolon 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.

The girl refers onset of fecal incontinence at the age of A hypothesis of HD was proposed; thus an anorectal manometry was asked, but its result was inconclusive due to patient’s lack of cooperation. C Surgical margin with ganglion cells.

Serology for Chagas’ disease was carried out in order to exclude a diagnosis of Chagas’ Disease megacolon, with negative result. The girl was discharged on the 5th day after surgery. 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.

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In this latter case, HD may affect the entire colon and even the small intestine.

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HD occurs in approximately 1 in live births. Some cases involve gene mutations for endothelin-B receptor. In addition, it is established that factors related to gender play a role, since men are preferentially affected, at a frequency of 4: This disorder is uncommon in adolescence and in adulthood and when present in this age group, it shows up as an ultrashort-segment disease.

Some patients reach adulthood without a diagnosis for this disease. Typically, patients go to the doctor with a long-standing history of constipation requiring frequent laxative use. Our patient had a history of long-standing bowel constipation, progressing to fecal incontinence. Therefore, the most liquid stools upstream pass around the fecal impaction and produce the reported symptom, known as fecal incontinence soiling. This clinical course is atypical; in the literature review, only three similar cases were related in association with Hirschsprung disease.

Complaints of fecal incontinence are often reported in the literature in cases of functional constipation and of idiopathic megacolon.

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

This factor contributed to the delay in her diagnosis, driving the pediatrician out of a HD hypothesis. Therefore, fecal incontinence can cause emotional disturbances to patients, with subsequent relationship problems at school and with their own families.

The diagnosis of HD is supported by barium enema studies, anorectal manometry and rectal biopsy.

The technique considered as the golden standard for the diagnosis of HD is the absence of ganglion cells in a rectal biopsy specimen. Imaging studies such as computed tomography CT and barium enemas are usually accepted for evaluation of chronic constipation, which is a common disorder in adults.

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ENFERMEDAD DE HIRSCHSPRUNG, REVISTA DE MEDICINA, CONCLUSIONES

Several procedures are used to manage this disease after childhood; currently the option of choice is the surgical procedure of Duhamel. Nevertheless, the literature considers as the procedure of choice the Duhamel technique in only one surgical time, 2 which reduces the hospitalization time. This surgery is considered curative. However, post-operative bowel functioning is not always satisfactory. Enterocolitis, constipation and fecal incontinence represent the main megacoolon complications in children.

Enfermedad de Hirschsprung en el adulto. Informe de um caso. Niger J Clin Pract. Enfermedad de Hirschsprung del adulto: Rev Argent Resid Cir. cognnito

Enfermedad de Hirschsprung, Conclusiones

Gordon PH, Nivatvongs S. Principles and practice of surgery for the colon, rectum and anus.

Enfermedad de Hirschsprung en adultos. J Pediatr Rio de J. Hirschsprung disease and hypoganglionosis in adults: This is an enfdrmedad article distributed under the terms of the Creative Commons Attribution License. Case Reports Late diagnosis of Hirschsprung’s disease. Hirschsprung’s disease; Congenital megacolon; Fecal incontinence. Introduction Hirschsprung’s Disease HDalso known as congenital aganglionic megacolon, is an anomaly characterized by enfefmedad absence of ganglion cells in the myenteric and submucosal plexuses in a variable bowel segment.

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.

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.

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