Congenital aganglionic megacolon in Nigerian adults: two case reports and review of the literature.

Congenital aganglionic mega colon (Hirschsprung's disease) is a motor disorder in the gut, due to a defect in the craniocaudal migration of the neuroblast originating from the neural crest that occurs during the first twelve weeks of gestation, causing a functional intestinal obstruction, with its attendant complications, in infants. Despite modern pediatric practice, with emphasis on early diagnosis, Hirschsprung's disease is seen in adults in regions where perinatal care is limited. We report two cases of Nigerian adults with longstanding, recurrent constipation, getting relieved by laxatives and herbal enemata, and then presented to our Emergency Department with a history of progressive abdominal distention, colicky pain, occasional vomiting, and weight loss. Per rectal examination revealed a gripping sensation in the rectum, 10 cm from the anal verge, with rectal fecal load. Barium enema showed a grossly distended proximal large colon, with high fecal retention, with the transition zone at the middle one-third of the rectum. Due to difficulty in bowel preparation of these patients, emergency laparotomy was done. The first case had a diverting sigmoid colostomy and later had a low anterior resection. The second case had a one-stage procedure. Histology of both the cases showed aganglionosis of the stenotic segment and a normal distal rectum. Both patients had complete resolution of the symptoms, without complications, in a three-year follow-up. The related literatures were reviewed. Hirschsprung's disease should be considered in adults patient presenting with chronic constipation. Low anterior resection of the rectum would be a surgical option for the treatment of short and zonal segment of adult Hirschsprung's disease.

Modified Soave procedure for the treatment of vascular malformations involving anorectum and sigmoid colon.

PURPOSE: The objective of this study is to introduce a modified Soave procedure for the treatment of vascular malformations involving the anorectum and sigmoid colon (VMARS) in children. METHODS: Cases of 12 children with VMARS between 2000 and 2008 were reviewed and analyzed. The confirmed diagnosis was established by barium enema, colonoscopy, computed tomography, and magnetic resonance imaging. All the patients underwent a modified Soave procedure. In 11 patients, Sarasola-Klose hemorrhoidectomy was used for the distal part of endorectal dissection. RESULTS: The mean length of resected bowel was 22.5 cm, ranging from 17 to 28 cm. Histologically, the surgical specimens showed that the lesions were venous malformation. Postoperative recovery was uneventful. The patients had good continence with no rectal bleeding, but intermittent fecal soiling was noted in one case. CONCLUSIONS: VMARS forms a unique subset of patients with vascular malformations who have rectal bleeding. The combination of Soave procedure and Sarasola-Klose hemorrhoidectomy is a safe and effective procedure for VMARS.

Transanal one-stage endorectal pull-through for Hirschsprung's disease in infants and children.

PURPOSE: This report presents the technique and results of transanal one-stage endorectal pull-through procedure in children with rectosigmoid lesions from Hirschsprung's disease. METHODS: Eight children aged one month to 6 years with frozen section biopsy-proven Hirschsprung's disease underwent transanal one-stage endorectal pull-through procedures during a 12-month period. A rectosigmoid transitional zone was suggested by contrast enema in 7 patients; rectal manometry was done to confirm the diagnosis in one patient. Preoperative colonic irrigation to evacuate feces out of the dilated colon was done in the hospital. Bowel preparation was the same as conventional colorectal surgery. Full-thickness rectal biopsy at 1 to 2 cm above the dentate line was submitted for pathologic diagnosis. A rectal mucosectomy dissection was started 0.5 cm proximal to the dentate lines and was extended into the intraperitoneal rectum. The muscular sleeve was divided circumferentialy at 3 to 4 cm proximal to the dentate line, exposing the intraperitoneal rectum and allowing full-thickness mobilization of the rectosigmoid colon out of the anus. Aganglionic colon segment was resected, and the normal colon was pull down to anastomose with the distal end of anorectal mucosa. RESULTS: Operating time, including taking frozen sections, ranged from 110 to 180 minutes. The length of bowel resections ranged from 9 to 25 cm. The length of hospital stay depended on the amount of fecal impaction in the colon. Older children with substantial fecal impaction required 2 weeks of preoperative saline enema. One infant needed 3 days for bowel preparation, the same as for conventional colorectal surgery. The hospital stay ranged from 6 to 7 days in children younger than 2 years and 10 to 28 days in older children. There were no intraoperative or postoperative complications related to the pull-through procedure. One case of colitis occurred in the 6-year-old child, which required rectal tube decompression one week after the operation. Seven patients passed stool within 24 hours after surgery. All patients had normal bowel movements within 3 weeks. There was no rectal cuff stricture or enterocolitis during one year of follow-up. CONCLUSIONS: Transanal one-stage endorectal pull-through operations for rectosigmoid lesions from Hirschsprung's disease can be performed successfully in all ages of children with good results, avoiding transabdominal exploration. The early postoperative enterocolitis in the older children might occur and should be treated urgently. The partial coloanal anastomosis obstruction found in older children could be treated by placing a rectal tube into the anus to decompress the dilated pull-through colon. The limitation of this approach is that retroperitoneal fixation of the descending colon could not be dissected by the transanal route.

[Colonic varices; a case report].

Colonic varices is a rare entity that is caused by portal hypertension in most instances. In the case reported here, barium enema and colonoscopy showed multiple tortuous polypoid lesions with intact mucosal surface. Inferior mesenteric angiography showed arteriovenous malformation in the sigmoid colon, complete obstruction of the inferior mesenteric vein, and tortuous dilated marginal veins. We assume that the colonic varices of this case was secondary to complete obstruction of the inferior mesenteric vein following AVM of the sigmoid colon. Close follow-up should is warranted in cases of colonic varices because of the risk of bleeding.

Sonographic diagnosis of ischemic colitis.

An abdominal ultrasound examination performed on two patients with abdominal pain showed segmental wall thickening of the colon that proved to be due to ischemic colitis. In one case, the typical "target lesion" was recognized. These findings could be confirmed with computed tomography, whereas a single contrast enema was unable to demonstrate the ischemic disease. In the second case, a typical five-layered structure of the bowel wall could be demonstrated. It is concluded that US can be useful in the early diagnosis of colonic ischemia.

Vascular occlusion and stricture of the sigmoid colon secondary to trauma from a pneumatic hammer.

A patient with stricture of the descending colon secondary to use of a pneumatic hammer is presented. Barium enema and angiographic studies suggested that the etiology was a traumatic vascular injury. Barium granulomata, found in the resected specimen, were presumably due to barium having entered the devitalized tissues during an air contrast study performed during an acute ischemic colitis. The mechanisms of traumatic strictures of the colon are discussed.