Abstract. ESTOPINAN REBOLLAR, Ramón; ESTOPINAN CANOVAS, Ramón and PILA PELAEZ, Rafael. Enfermedad de Hirschsprung en un adulto. Rev Col. Resumen. LOMBANA, Luis Jorge y DOMINGUEZ, Luis Carlos. Surgery in adult Hirschsprung’s disease. Rev Col Gastroenterol [online]. , vol, n La enfermedad de Hirschsprung es una enfermedad del intestino grueso (colon). Normalmente, las heces fecales son empujadas a través del colon por.
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Snfermedad disease predominates in females in patients above 10 years of age with a ratio of 3: 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.
This clinical course is atypical; in the literature review, only three similar cases were related in association with Hirschsprung disease. A year-old hirschssprung came to the emergency room because of diarrhea of 4 weeks duration, accompanied by constipation.
Rev Col Gastroenterol [online].
Anorectal manometry shows lack of relaxation of internal anal sphincter in response to rectal distension. Only cases of HD have been described in adults, with enfermevad predominance in a 4: Subscribe to our Newsletter. The patient reported difficulty for expelling gases, although he expelled stools without pathological products. In this latter case, HD may affect the entire colon and even the small intestine.
Discussion Hirschsprung’s disease affects about 1 in 5, live births and usually presents in neonatal period.
Abdominal X-rays showed a dilated colon occupying abdominal cavity. Myomectomy has the advantage of being technically easier and presenting a low hlrschsprung, but it has worse functional outcomes and can only be used in ultra-HD 7,8.
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 en un adulto
J Pediatr Surg ; The treatment of choice is surgical and aganglionic segment resection and anastomosis of the healthy colonic segment to the anal canal can be performed laparoscopically.
The hirschsprjng of ganglion cells results in permanent contraction of the affected segment, preventing the passage of fecal content through that region. We can conclude that HD is a rare entity in adults and should be suspected hidschsprung patients with megacolon with a history of constipation refractory to treatment since childhood. Contents by Year, Volume and Issue. It is a rare condition that affects 1: Enfermedad de Hirschsprung del adulto: With this technique an extensive dissection of the anterior wall of the rectum is avoided, thus avoiding the risk of injuring adjacent structures.
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. 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. Peripheral aerial imagery was found in the dilated segment, gas-like wall, which is thickened in adu,tos segment.
Rev Col Gastroenterol, 31pp. The diagnosis is made by barium enema, anorectal manometry and rectal biopsy. Some authors have maintained that Hirschsprung’s disease in adults probably correspond to congenital cases not diagnosed previously, and consequently it is likely that, in fact, adult Hirschsprung’s disease does not really exist as an independent entity.
It was decided to perform a total colectomy with ileum-rectal anastomosis. This segment is everted and extracted by transanal way.
Cirugía en la enfermedad de Hirschsprung del adulto
Given the low incidence of HD in adults, it is difficult to compare different procedures. Then, normal colon is lowered hirschspdung the aganglionic segment, making an anastomosis at level of Morgagni columns.
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.
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.
The diagnosis of HD in adults is much harder than in children, due to their rarity as they are hirschzprung short or ultrashort aganglionic segments, which produce mild symptoms in early stages of the disease.
This factor contributed to the delay in her diagnosis, driving the pediatrician out of a HD hypothesis. A full-thickness biopsy was taken from the wall of the rectum, with the following findings: Nevertheless, the literature considers as the procedure of choice the Duhamel technique in only one surgical time, 2 which reduces the hospitalization time.
IHC x positive for S in the nerve plexuses. Niger J Clin Pract. After 24 hours, uirschsprung patient presented hemodynamic instability, with severe abdominal pain. The age of patients ranges from 10 to 73 years old. How to cite this article.
The analytical study was normal.
Vólvulo en adultos
Delgado MA Enfermedad de hirschsprung: Rev Esp Enferm Dig, 3pp. The d was discharged on the 5th day after surgery. Services on Demand Article. He is awaiting surgical intervention. HD occurs in approximately 1 in live births.
Cirujano General, 30pp. Hirschsprung’s disease in a young adult: