1/143. A male newborn with colonic atresia and total colonic aganglionosis.A case of colonic atresia (CA) and aganglionosis is presented, which is probably the tenth in the English literature. The boy presented on day 3 of life with delayed passage of meconium, milk intolerance, and progressive abdominal distension. A barium enema study was complicated by barium peritonitis. Emergency peritoneal lavage was done and the intestinal obstruction was relieved by a proximal defunctioning ileostomy; type III CA (Grosfeld classification) was identified. The proximal atretic end was the blind-ending caecum and the distal atretic end commenced in the splenic area as a microcolon extending to the pelvis and replacing the normal colon. Total colonic aganglionosis (TCA) was confirmed by biopsies. This is the first case in the literature of TCA with CA documented before any attempts at colonic resection and anastomosis were made. This association highlights the recommendation for biopsy of the micro-colon and rectum when Type III CA (Grosfeld classification) is encountered in a newborn. The infant underwent a cholecystectomy and Duhamel-Martin operation at 14 months that was complicated by abdominal wound dehiscence; the ileostomy was closed at 22 months of age.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/143. A laparoscopic pull-through operation for Hirschsprung's disease: report of two infant cases.We describe herein a primary laparoscopic pull-through procedure that was successfully employed to treat two infants with Hirschsprung's disease. Mobilization of the rectum and sigmoid colon was performed laparoscopically, and the rectal mucosa was removed via a transanal submucosal resection. After inducing the rectal prolapse intussusceptically, the rectum was circumferentially transected, and the mobilized colon was pulled down through the rectal muscle cuff. Resection of the aganglionic bowel and the coloanal anastomosis, using the Soave-Denda method, was performed outside the anus. Both infants had an uneventful postoperative course with early recovery of bowel movement. Our experience demonstrates that this minimally invasive surgical procedure is feasible for young infants, and we believe that Hirschsprung's disease may be a definite indication for laparoscopic surgery.- - - - - - - - - - ranking = 0.16666666666667keywords = ganglion (Clic here for more details about this article) |
3/143. Repeat pull-through surgery for complicated Hirschsprung's disease: indications, techniques, and results.BACKGROUND: Most children with Hirschsprung's disease (HD) do well after a pull-through procedure. In the occasional child in which the operation fails, a repeat procedure may be necessary. methods: Nine children with HD aged 20 months to 9 years underwent repeat pull-through over a 4-year period. Original pull-throughs (six Soave, two Swenson, one Duhamel) were performed elsewhere 12 to 95 months earlier (median, 36 months). Indications for revision were stricture unresponsive to dilatation (n = 3) and acquired aganglionosis (n = 6). One of the latter had associated segmental intestinal neuronal dysplasia. One child with a stricture after a Swenson procedure underwent a repeat Swenson. The other eight underwent reconstruction using a Duhamel technique. Five had a defunctioning stoma before or at the time of repeat surgery. RESULTS: Median follow-up was 15 months (range, 4 to 40 months). Complications included wound infection (n = 2), anastomotic bleeding (n = 2), stoma leak (n = 1) or stenosis (n = 1), "kinking" at the top of the Duhamel (n = 1), and persistent septum (n = 1). Three patients have had a good outcome with normal stool patterns. One has intermittent soiling, and one has what is believed to be stool-holding behavior. Four have persistent obstructive symptoms caused by sphincter hypertonicity, which are being successfully managed nonoperatively. CONCLUSIONS: Repeat pull-through surgery can be performed safely in appropriately selected patients. Duhamel reconstruction usually is preferred for technical reasons, and a stoma is not always necessary. Outcome is generally favorable, but anal sphincter hypertonicity may cause persistent symptoms in some patients.- - - - - - - - - - ranking = 0.16666666666667keywords = ganglion (Clic here for more details about this article) |
4/143. Total colonic aganglionosis: diagnosis and management in a 12-year-old boy.Total colonic aganglionosis (TCA) is an unusual form of Hirschsprung's disease, and most of these present within the first few weeks and nearly all by the end of the first year of life. Very few cases presenting in older childhood or adulthood have been reported. Without a high index of suspicion these cases can easily be missed and, therefore, patients undergo repeated laparotomies for subacute intestinal obstruction without relief of symptoms. The diagnosis is suspected on operative findings and confirmed by histological evaluation of seromuscular biopsy specimens. Proper diagnosis will prevent loss of bowel length caused by inadvertent resection and will help in performing an optimal procedure to treat these patients. Discussed herein is another case of TCA in a 12-year-old boy with review of relevant literature.- - - - - - - - - - ranking = 0.83333333333333keywords = ganglion (Clic here for more details about this article) |
5/143. Near total intestinal aganglionosis with extreme short-bowel syndrome--a difficult surgical dilemma.Forty cases of total or near-total intestinal agangliosis (NTIA) were described to date in the English literature. Most cases had a lethal outcome. We describe the 41st case--a Beduin male neonate--who had only 30 cm of proximal hypoganglionic jejunum. He is presently almost one-year-old and thriving on home TPN, receiving one quarter of his caloric requirements orally using pregestamil, an MCT formula. The initial intricate course, diagnosis and several operative procedures, are elaborated. A review of the scant literature is discussed. The elusiveness of the correct diagnosis is pointed out and means to overcome these errors are described. Various surgical procedures have been suggested, none of which offer the perfect solution to the severe basic problem of short bowel. Long-term parenteral hyperalimentation is still the main modality of treatment. Based on our modest experience, we suggest saving every possible length of jejunum, even if hypoganglionic, since this bowel, following a few weeks of adaptation, starts to function fairly well, suggesting perhaps some neuro-muscular maturation. The best surgical approach is still pending. We present a report of a child with this disease and discuss the therapeutic dilemma.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
6/143. Failure to pass meconium: diagnosing neonatal intestinal obstruction.Timely passage of the first stool is a hallmark of the well-being of the newborn infant. Failure of a full-term newborn to pass meconium in the first 24 hours may signal intestinal obstruction. Lower intestinal obstruction may be associated with disorders such as Hirschsprung's disease, anorectal malformations, meconium plug syndrome, small left colon syndrome, hypoganglionosis, neuronal intestinal dysplasia and megacystis-microcolon-intestinal hypoperistalsis syndrome. Radiologic studies are usually required to make the diagnosis. In addition, specific tests such as pelvic magnetic resonance imaging, anorectal manometry and rectal biopsy are helpful in the evaluation of newborns with failure to pass meconium.- - - - - - - - - - ranking = 0.16666666666667keywords = ganglion (Clic here for more details about this article) |
7/143. Near-total intestinal aganglionosis in the Waardenburg-Shah syndrome.Both pigmentation and otic defects of waardenburg syndrome and Hirschsprung's disease have a common origin in neural crest cells and were described in 1951 and 1887, respectively. The clinical manifestations of both in the same patient were described in 1981 in 12 infants so afflicted. The authors present such a case of long segment aganglionosis in a 15-day-old Marshallese girl with Waardenburg-Shah syndrome and discuss diagnosis, treatment, and prognosis.- - - - - - - - - - ranking = 0.83333333333333keywords = ganglion (Clic here for more details about this article) |
8/143. Ondine's curse and Hirschsprung's disease: neurocristopathic syndrome.We report a female newborn with Ondine's curse and Hirschsprung's disease--neurocristopathic syndrome. The female infant required endotracheal intubation and mechanical ventilation due to apnea which developed soon after birth. She had abdominal distension with bilious vomiting. A barium enema revealed a caliber change at the rectum and rectal biopsies showed no ganglion cells. colostomy was performed at the age of 17 days. Hypoxemia with hypercapnia was noted during her sleep, and tracheostomy was performed at the age of 55 days. In addition, deafness and pupillary autonomic dysfunction were observed. The definitive surgery for Hirschsprung's disease was performed at the age of 4 months. She is now 2 years old with normal growth but needs ventilator support at home. In this case, we detected no mutation in the RET gene and EDNRB gene.- - - - - - - - - - ranking = 0.16666666666667keywords = ganglion (Clic here for more details about this article) |
9/143. Aganglionosis of the small intestine: a rare form of Hirschsprung's disease.Absence of ganglion cells in the small intestine, a rare form of Hirschsprung's disease, is a condition found in newborns and associated with increased morbidity and mortality. Families are faced with numerous challenges, including maintenance of nutrition, the need for surgical interventions, and the potential for complications associated with the medical technologies necessary to maintain children with aganglionosis. Home care needs are not unlike those of other children with severe intestinal dysfunction such as short bowel syndrome. This article gives an overview of Hirschsprung's disease and presents three case studies of small bowel Hirschsprung's. One center's experience with Hirschsprung's disease supports the need for a multidisciplinary approach to long-term management and home care.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
10/143. An ileostomy at the aganglionic ileum for total colon aganglionosis.For the initial management of total colon aganglionosis (TCA), an ileostomy is usually created at the distal end of the ganglionic bowel, which is associated with profuse diarrhea resulting in a water and electrolyte imbalance as well as malnutrition during the ileostomy period. An ileostomy at the aganglionic ileum several centimeters toward the anal side of the transition for TCA is thus expected to cause bowel retention without any bowel obstruction and to facilitate water reabsorption in the small bowel. We performed this procedure in 2 babies with TCA. The length of the aganglionic ileum was 50 cm and 10 cm, respectively. The ileostomies were made 5 cm distal to the transition at 1 month and 1 day after birth, respectively. Oral feedings were well tolerated, resulting in adequate weight gain and the stools were semisolid during the ileostomy periods. Definitive operations using the Duhamel procedure with a right colon patch graft without the creation of a new ileostomy were successfully performed at 7 and 3 months of age, respectively. The postoperative courses were satisfactory. An ileostomy at the aganglionic ileum appears to be the method of choice as initial management for patients with TCA.- - - - - - - - - - ranking = 2.1666666666667keywords = ganglion (Clic here for more details about this article) |
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