Hirschsprungs Disease

Hirschsprungs disease is a congenital anomaly that results in mechanical obstruction from inadequate motility of part of the intestine. HD occurs when there is blockage in the large intestine that is caused by missing nerve cells in the colon. The colon stays contracted and no digested food can be pushed through allowing intestinal content to build up. HD develops in children before they are born. It is not caused by anything the mother did while pregnant. HD affects 1 in 5000 live birth. The lack of colorectal innervation inhibits peristalsis, and the affected portion of intestine becomes spastic and contracted. The internal rectal sphincter fails to relax, which prevents evacuation of fecal material and gas and causes severe abdominal distention and constipation. The most common site affected is the rectosigmoid colon (short segment disease), and the less common is the upper descending colon and possibly the transverse colon are affected (long segment disease).

Causes Muscle contractions in the gut help digested materials move through the intestine. This is called peristalsis. Nerves in between the muscle layers trigger the contractions. In Hirschsprung's disease, the nerves are missing from a part of the bowel. Areas without such nerves cannot push material through. This causes a blockage. Intestinal contents build up behind the blockage, causing the bowel and abdomen to become swollen. Hirschsprung's disease causes about 25% of all newborn intestinal blockages. It occurs five times more often in males than in females. Hirschsprung's disease is sometimes associated with other inherited or congenital conditions, such as Down syndrome. Signs and Symptoms: Symptoms that may be present in newborns and infants include:

Difficulty with bowel movements Failure to pass meconium shortly after birth Failure to pass a first stool within 24 - 48 hours after birth Infrequent but explosive stools Jaundice Poor feeding Poor weight gain Vomiting Watery diarrhea (in the newborn)

Symptoms in older children:

Constipation that gradually gets worse Fecal impaction Malnutrition

Slow growth Swollen belly

Diagnostic Evaluation: 1. Rectal examination demonstrate absence of fecal material in the long segment disease; in short segment disease, rectal impaction may be present and, when the finger is removed, it may cause a rush of stool as the obstruction is temporary relieved. 2. X-rays shows severe gaseous distention of the bowel, with absence of gas in the rectum. 3. Radiopaque markers, when ingested, measure intestinal transit time. Children with short segment disease retain the markers in the rectum for long periods. 4. Barium enema shows narrowed intestine proximal to anus and dilated intestine proximal to narrow segment. 5. Rectal biopsy may be done to demonstrate absent or reduced number of ganglion nerve cells, and confirm diagnosis. 6. Anorectal manometry may be done to record the reflex response to anal sphincter, requires cooperation of the child. 7. Ultrasonogram may be done to demonstrate dilated colon. Therapeutic Interventions: 1. Enema or colonic irrigation with physiologic saline solution. 2. Older child whose symptoms are chronic but not severe may be treated with isotonic enemas, stool softeners, and a low residue diet. Surgical Interventions: 1. Initially, a colostomy or ileostomy is performed to decompress intestine, divert fecal stream, and rest the normal bowel. 2. Definitive surgery is done to remove the non-functioning bowel segment with various pull-through procedures (abdominoperineal, endorectal, or rectorectal).

Nursing Interventions: Preoperative 1. Assist in emptying the bowel by giving repeated enemas and colonic irrigations. 2. If abdominal distention is not relieved by enemas, discomfort is significant, and rectal tube insertion fails to give relief, consult doctor for a nasogastric (NG) tube. 3. Offer pacifier for infant to suck if on parenteral fluids. 4. Encourage parents to hold and rock the infant. 5. Maintain position of comfort with head elevated. Offer soothing stimulation (eg. music, touch, play therapy). 6. Offer small frequent feedings. Low residue diet will aid in keeping the stool soft. 7. Administer parenteral nutrition if feeding causes additional discomfort because of distention and nausea. 8. For older child, provide demonstration and written and verbal instruction to family for saline enema administration and use of stool softeners. Postoperative 1. Change wound dressing using sterile technique. 2. Prevent wound contamination from diaper. 3. Prevent perianal and anal excoriation by thorough cleaning and use of ointments after the infant soils (postoperative stools can number 7 to 10 per day). 4. Use careful handwashing technique. 5. Report any wound redness, swelling or drainage, evisceration, or dehiscence immediately. 6. Suction oral secretions frequently to prevent infection of the tracheobronchial tree and lungs. 7. In older child, encourage frequent coughing and deep breathing to maintain respiratory status. 8. Allow the infant to cry for a short period to prevent atelectasis.

9. Change position of infant frequently to increase circulation and allow for aeration of all lung areas. 10. Maintain patency of NG tube immediately postoperatively. 11. Maintain NPO status until bowel sounds return and the bowel is ready for feedings as determined by the physician. 12. Provide frequent oral hygiene while on NPO status. 13. Administer fluids to maintain hydration and replace lost electrolytes. Begin oral feedings as ordered. 14. Support the parents when teaching them to care for their childs colostomy. Reassure parents that colostomy will not cause delay in the childs normal development. 15. Involve the entire family in teaching colostomy care to enhance acceptance of body change of the child.

Reference http://nursingcrib.com/nursing-notes-reviewer/hirschsprungs-disease/ http://www.nlm.nih.gov/medlineplus/ency/article/001140.htm http://www.scribd.com/doc/25905546/Hirschsprung%E2%80%99s-DiseaseAganglionic-Megacolon http://www.i-am-pregnant.com/Birth/Birth-defects/Hirschsprung%60s-Disease