Hirschsprung disease: Description, Causes and Risk Factors:Congenital dilation and hypertrophy of the colon due to absence (aganglionosis) or marked reduction (hypoganglionosis) in the number of ganglion cells of the myenteric plexus of the rectum and a varying but continuous length of gut above the rectum.Hirschsprung disease is a congenital illness in which there is a lack of intrinsic nerves (ganglion cells) in the distal segments of the intestinal tract. These abnormal segments produce mechanical obstruction because of failure to relax during peristalsis. Hirschsprung's disease always starts in the anal verge, but the length of the segment without ganglion cells (aganglionic) varies: it is limited to the rectum and sigmoid in 75% of patients; involves the whole colon in 8%; and rarely involves the small bowel.The incidence of Hirschsprung's disease varies from 1 in 5,000 to 1 in 10,000 live births. There appears to be a male preponderance with a ratio of 3:1 to 5:1, particularly in those with short segments. However, the incidence in both sexes seems to be the same in those with long segment disease.The average age at the time of diagnosis has been decreasing over the years. The diagnosis is established in 15% within the first month of life, in 40-50% in the first 3 months, in 60% at the end of the first year of age, and in 85% by 4 years. Occasionally, the diagnosis of Hirschsprung's disease is not made until adulthood.In a study of 29 patients in which the diagnosis was made after 10 years of age, the mean age at the time of diagnosis was 26 years (range 11-73 years). Most of these adults reported symptoms from an early age, but others reported the onset during adulthood. Adults report constipation as the predominant symptom, and most evacuate the bowel only with enemas. The presentation in the adult may also be atypical, and there are at least two reports of patients presenting with chronic colitis and pseudopolyps.If the diagnosis is not established in the newborn, the infant may present with mild constipation that may be followed with acute obstruction, frequent episodes of fecal impaction or with the development of acute life-threatening enterocolitis. The latter develops in from 15% to 50% of cases, and may be the presenting feature of Hirschsprung's disease in up to 12% of patients. It remains the main cause of death, and the mortality rate can reach 20 to 50%. From infancy until adulthood, mild-to-severe constipation may be the only symptom, so Hirschsprung's disease must be differentiated from functional constipation. Because clinical features do not allow a complete differentiation between these problems, the diagnosis of Hirschsprung's disease must always be considered in any child, adolescent or adult with severe intractable constipation.Symptoms:Symptoms of Hirschsprung's disease usually show up invery young children but can sometimes show up in olderchildren or teenagers.Most babies with Hirschsprung's disease do not pass stoolon the first or second day of life. After that, almost allbabies with Hirschsprung's disease are constipated andhave difficulty passing stool. The children may vomit andtheir tummy swells up because they cannot pass stooleasily. Some babies have diarrhea instead of constipation.Children and teenagers with Hirschsprung's disease usuallyexperience constipation their entire life. Normal growth anddevelopment may occur more slowly in some children.Diagnosis:A physician will examine your child and obtain a medical history. Other tests may be done to evaluate whether your child has Hirschsprung disease. These tests may include:Abdominal x-ray - a diagnostic test which may show a lack of stool in the large intestine or near the anus and dilated segments of the large and small intestine.
Barium enema - a procedure performed to examine the large intestine for abnormalities. A fluid called barium (a metallic, chemical, chalky, liquid used to coat the inside of organs so that they will show up on an x-ray) is given into the rectum as an enema. An x-ray of the abdomen shows strictures (narrowed areas), obstructions (blockages), and dilated intestine above the obstruction.
Anorectal manometry - a test that measures nerve reflexes which are missing in Hirschsprung disease.
Biopsy of the rectum or large intestine - a test that takes a sample of the cells in the rectum or large intestine and then looks for nerve cells under a microscope.
Treatment:The treatment of Hirschsprung disease is surgical. Initial medical management is important, however, in stabilizing the patient before surgical therapy is undertaken. This includes the correction of fluid and electrolyte imbalances, antibiotic therapy if enterocolitis is present and rectal decompression with the use of rectal irrigations and rectal tubes until the time of surgery.The basic principle for the definitive surgical therapy is resection of the aganglionic segment followed by a pull-through of ganglionic bowel down to the anus. There are different procedures that have been used, but the three most common ones are: rectosigmoidectomy, retrorectal transanal pull-through, and endorectal pull-through. Recently the surgery is being performed in the newborn period using minimally invasive surgical techniques, like laparoscopy.NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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