Hirschsprungs disease 109

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D: Congenital absence of myenteric ganglion cells in the rectum extending for a variable distance proximally, resulting in impaired motility and functional obstruction. ^^

A: Failure of migration of the neural crest derived parasympathetic ganglion cell, several genes have been implicated, e.g. NCAM and RET proto-oncogene.

A/R: Familial history (5-10% cases), MEN 2A, Down's syndrome (trisomy 21).

E: Uncommon (1/5000 live births). Presents in infants or young children. Male: female is 4:1 in rectosigmoid disease with females tending to have longer aganglionic segments.

H: Most present under the age of 5 years.

Failure to pass meconium within 48 h of life. Abdominal distension and vomiting.

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Older children present with failure to thrive, chronic constipation, abdominal distension.

Abdominal distension with tinkling bowel sounds if obstruction. PR examination may reveal a tight anal sphincter and hard stools in the rectum, and in the infant may result in sudden passage of stool and flatus, decompressing the abdomen.

May become very unwell, pyrexial with abdominal tenderness if enterocolitis develops.

Macro: 10% have short segment aganglionosis involving the terminal rectum. It involves the sigmoid colon in 75%, proximal colon in 10%, whole colon in 5%, with small bowel involvement being rare.

Micro: Histology shows characteristic lack of ganglion cells and proliferation of nerve trunks in Meissner's plexus.

Imaging: Abdominal radiographs may show multiple loops of dilated small and large bowel and an empty rectum. A barium contrast enema shows a transition zone where dilated colon becomes narrow in the aganglionic segment (see Fig. 19).

Rectal manometry: Shows absence of internal sphincter relaxation with a reduction in intraluminal pressure in the anal canal following distension of the rectum with a balloon (rarely performed except in older children). Rectal biopsy: For definitive diagnosis, mucosal/submucosal suction biopsies without need for anaesthesia or full-thickness biopsy under anaesthesia.

Surgical: Definitive removal of the aganglionic segment is often delayed until the child is well nourished and the dilated proximal bowel has returned to normal. The bowel is deflated by defunctioning colostomy or regular rectal washouts. Alternatively, a single stage procedure without colostomy is performed in the neonatal period. Operations include Swenson, Duhamel or Soave procedures. The aganglionic segment is excised, the normal bowel pulled through and anastomosed to the anus. Presence of ganglia in the segment must be confirmed by frozen section. If present, a colostomy is closed once the anastomosis has healed.

Constipation, bowel obstruction, enterocolitis, caecal perforation. Of surgery: anastomotic leak with perirectal or pelvic abscess.

Good with appropriate management. Enterocolitis is the principle cause of mortality in untreated disease.

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