Hirschprung`s disease, a practical approach.
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Abstract
Initially, Hirschsprung's disease was
Hirschsprung's disease was described by the Danish pediatrician Harald Hirschsprung in 1888. It is the most common congenital enteric neuropathy, with a lack of relaxation of the enteric muscle, with subsequent intestinal obstruction. Its etiopathogenesis is given by aganglionosis in the digestive tract, mainly affecting the rectosigmoid region. Thus, keeping the internal anal sphincter contracted. It is classified into four types: short segment, the most common; long segment, ultrashort segment, and aganglionosis coli. The clinical presentation frequently begins in the neonatal period. The classic triad consists of abdominal distention, absence of meconium in the first 24 to 48 hours of life, and bilious vomiting with intolerance to the oral route. The diagnostic approach is performed by abdominal X-rays, contrast enema, or rectal manometry. The gold standard test is the rectal biopsy, which is considered positive if there are no ganglion cells in the myenteric and submucosal plexus of the colon. The initial management of the disease is based on intravenous fluid resuscitation, broad-spectrum antibiotics to prevent enterocolitis and bacterial translocation, decompression with a nasogastric tube, and colonic lavage. The definitive treatment is colonic resection of the affected segment. The main postoperative complications are perianal excoriation, constipation, dirt, diarrhea, fecal incontinence, and enterocolitis.
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