Hirschsprung’s disease

A congenital condition where parts of the lower colon lack nerve cells, making peristalsis impossible, so that food accumulates in the upper colon which becomes swollen.


Hirschsprung disease (also known as congenital intestinal aganglionosis) is characterized by an absence of nerve cells in a segment of the bowel. This disease interferes with the peristalsis action of the bowel and results in chronic bowel obstructions. For newborn infants this includes the inability to pass meconium, the infant’s dark-green first bowel movement. The classic symptom for all children with this disorder is chronic constipation. For older infants, symptoms include abdominal distention and chronic diarrhea. Although usually first evident in infancy, symptoms can be manifested throughout childhood and adolescence


A congenital disorder characterized by absence of autonomic parasympathetic ganglion cells in the colon that prevents peristalsis at the diverticulum causing inadequate gastric motility and mechanical obstruction of the intestine.


A genetic disorder in which a lack of nerve cells in a portion of the colon prevents normal bowel movements. Hirschsprung disease is caused by a failure of nerve cells to develop in a small segment of the end part of the colon, usually near the anus. The absence of nerve cells in the colon inhibits peristalsis, the rhythmic contractions that move stool through the colon. In the newborn, symptoms include the failure to pass meconium, the first stools of the newborn, within 24 hours of birth; a distended belly; dehydration; and weight loss. Hirschsprung disease is suspected in older children who have histories of constipation and bowel problems from birth. The disease occurs in one of every 5,000 live births and is 4 to 5 times more common in boys than girls.


A congenital condition in which the rectum and sometimes part of the lower colon have failed to develop a normal nerve network. The affected portion does not expand or conduct the contents of the bowel, which accumulate in and distend the upper colon. Symptoms, which are usually apparent in the first weeks of life, are abdominal pain and swelling and severe or complete constipation. Diagnosis is by X-ray and by microscopic examination of samples of the bowel wall, which shows the absence of nerve cells. Treatment is by surgery to remove the affected segment and join the remaining (normal) colon to the anus.


Hirschsprung’s disease, or megacolon, is a rare congenital disorder characterized by great hypertrophy and dilatation of the colon. The rectum and lower colon have failed to develop a normal nerve network, thus disturbing normal contraction and expansion of these structures. This results in severe, chronic constipation, usually from birth. Treatment is surgical removal of the affected sections, with the remaining colon being joined to the anus.


The most common cause of lower gastrointestinal obstruction in neonates. Patients with this disease exhibit signs of an extremely dilated colon and accompanying chronic constipation, fecal impaction, and overflow diarrhea. It occurs in 1 in 5000 children, with a male-to-female ratio of 4:1. About 15% of cases are diagnosed in the first month of life, 64% by the third month, and 80% by age 1 year. Only 8% remain undiagnosed by 3 years of age.


Hirschsprung disease is an innate anatomical anomaly or birth defect affecting the gastrointestinal tract, wherein the ganglia (nerve clusters) within the bowel wall are absent. This absence of nerves initiates from the anal region and extends to varying lengths along the bowel. Consequently, a condition known as megacolon develops, characterized by a substantial enlargement of the bowel above the affected area. The nerves play a vital role in facilitating the coordinated muscular contractions of the intestinal lining, which propel the contents of the bowel forward.


Hirschsprung’s disease is a congenital disorder in which the ganglion cells that regulate the rhythmic contractions of the intestine are absent in the rectum and potentially the lower part of the colon. This results in a narrowed section of the colon that impedes the movement of waste material.


This disease is relatively rare and has a tendency to be familial. It appears approximately four times more frequently in boys. Symptoms such as constipation and bloating typically manifest within the initial few weeks of life. Affected children often exhibit a lack of appetite and may experience inadequate growth.


A barium X-ray test can help identify the constricted part of the intestine. Additionally, a biopsy might be performed. The treatment for Hirschsprung’s disease typically involves surgically removing the narrowed segment and reattaching the healthy part of the intestine to the anus.


A condition observed in children where the lower part of the colon becomes significantly enlarged and can accumulate up to a week’s worth of feces. The primary symptom is constipation. It is believed to result from an excessive activity of the sympathetic nerves, which leads to colon paralysis and increased tension in the intestinal valves. Cutting the sympathetic nerves or administering a high spinal anesthetic can bring about a cure, and interestingly, the improvement in the colon persists even after the anesthetic wears off, though the reason for this phenomenon remains unclear. Surgical removal of the enlarged colon is rarely performed nowadays, as most cases respond well to the aforementioned treatments.


 


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