1/38. 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 = area (Clic here for more details about this article) |
2/38. Sigmoid volvulus in children and adolescents.BACKGROUND: Sigmoid volvulus is an exceptionally rare and potentially life-threatening condition in the pediatric age group. STUDY DESIGN: We report our experience with three children treated for sigmoid volvulus and review the cases reported in the medical literature since 1940. RESULTS: Since 1940, 63 cases of sigmoid volvulus in children (including this series) have been reported. The median age was 7 years and the male to female ratio was 3.5:1. Two distinct presentations (acute and recurrent) were identified. Abdominal symptoms dominated the clinical picture. barium enemas either confirmed or were highly suggestive of sigmoid volvulus. Reduction by barium enema was successful in 77% (10 of 13) of the attempts. Forty-nine patients underwent operative treatment, with sigmoidectomy (with or without primary anastomosis) being the most common. The overall mortality rate was 6%, operative mortality was 8.1%, and neonatal mortality was 14%. Associated conditions were frequent. Particular emphasis should be placed on ruling out Hirschsprung's disease (present in 11 of 63 patients). CONCLUSIONS: Sigmoid volvulus remains a rare occurrence in children, but it should be included in the differential diagnosis of pain in children when colonic distention is present. An algorithm for treatment is proposed.- - - - - - - - - - ranking = 0.26989292088043keywords = pain (Clic here for more details about this article) |
3/38. Abdominal compartment syndrome in a patient with congenital megacolon.A 13-year-old male with a history of chronic congenital megacolon presented to the emergency department with a 1-day history of increasing abdominal pain, distension, and emesis. The patient was admitted for bowel disimpaction and irrigation. The patient rapidly developed an acute abdominal compartment syndrome because of his massive colonic dilation. Surgical decompression resulted in a reperfusion phenomenon and ultimately resulted in coagulopathy and patient demise. This case presents a unique cause of the abdominal compartment syndrome and discusses the implications to the emergency physician.- - - - - - - - - - ranking = 0.26989292088043keywords = pain (Clic here for more details about this article) |
4/38. Clinical letter: epidural analgesia in a newborn with Hirschsprung's disease, associated with congenital central hypoventilation syndrome.A case is presented of a neonate with Hirschsprung's disease, associated with congenital central hypoventilation syndrome. After an ileostomy (at 2 days) and a stoma revision (at 10 days), postoperative pain management was established by continuous intravenous infusion of morphine, which caused severe postoperative respiratory depression. At 6 weeks a re-exploration and stoma revision was performed using postoperative epidural analgesia with bupivacaine. This caused no respiratory depression. A colectomy under epidural analgesia at 8 months was also uneventful. Respiratory difficulties in children with congenital central hypoventilation syndrome associated with Hirschsprung's disease are discussed in relation to the technique of choice for postoperative pain management.- - - - - - - - - - ranking = 0.53978584176086keywords = pain (Clic here for more details about this article) |
5/38. Three-dimensional morphology of gut innervation in total intestinal aganglionosis using whole-mount preparation.BACKGROUND: Total intestinal aganglionosis (TIA) is a rare form of Hirschsprung's disease (HD). The aim of this study was to examine the 3-dimensional morphology of the myentric plexus of the entire gastrointestinal tract in a newborn with total intestinal aganglionosis. methods: Whole-mount preparations were made of the entire gastrointestinal tract using NADPH-diaphorase histochemistry and c-kit (a marker of interstitial cells of cajal) immunohistochemistry. RESULTS: Whole-mount preparations of the esophagus, stomach, and duodenum showed 3-dimensional morphology of the myenteric plexus forming a meshlike network of nerve fibers, connected to each other and to ganglia. There were large numbers of NADPH-diaphrase-positive nerve fibers between the muscle fibers in the circular muscle layer. In esophagus, stomach, and duodenum, c-kit-positive interstitial cells of cajal (ICC) formed a 3-dimensional network between the two muscle layers and also were abundant within the circular muscle layer. In the jejunum, ileum, and colon, the myenteric plexus was absent and was replaced by hypertrophic nerve bundles that stained weakly with NADPH-diaphrase. Circular muscle layer completely lacked NADPH-diaphrase-positive nerve fibers. The c-kit-positive ICCs in the jejunum, ileum, and colon were sparse and localized mainly around the nerve trunks between the circular and longitudinal muscle layers. CONCLUSIONS: Whole-mount preparation is an elegant 3-dimensional technique in which the relationship of branching and interconnecting nerve fibers to each other and to muscle can be seen clearly. Absence of myenteric plexus, lack of nitrergic innervation, and depletion of interstitial cells of cajal in the bowel wall throughout the small and large bowel contribute to the inability of the smooth muscle to relax, thereby causing lack of peristalsis in TIA.- - - - - - - - - - ranking = 4726.730184414keywords = plexus (Clic here for more details about this article) |
6/38. Laparoscopic full-thickness intestinal biopsies in children.BACKGROUND: laparoscopy may reduce postoperative pain and hospital stay, compared with laparotomy. The use of laparoscopic surgery to obtain full-thickness intestinal biopsies in children has not been previously reported. methods: Eleven children aged 1.6 to 19 years (median, 4.5 years) underwent laparoscopic full-thickness biopsy of the stomach, small bowel, colon, or a combination thereof. Each procedure used one 12-mm and two 5-mm ports. RESULTS: Eight children with obstructive symptoms after a pull-through for hirschsprung disease underwent multiple colon and small bowel biopsies (range, 3-6; median, 5); intestinal neuronal dysplasia was found in two. Two patients with cystic fibrosis had diffuse colonic narrowing; a diagnosis of enzyme-induced fibrosing colonopathy was made in one and nonspecific inflammation was found in the other. One child had a thickened stomach, and a gastroscopic-directed full-thickness biopsy revealed plasmacytoma. Nine of the 11 patients had a previous laparotomy, and ports were placed through preexisting scars. Median hospital stay was 2 days. No patient required more than 24 hours of narcotics. There were no leaks, and no other morbidity or mortality occurred. None of the patients required conversion to an open procedure. biopsy results significantly affected treatment for each patient. CONCLUSIONS: Laparoscopic full-thickness intestinal biopsy is safe and effective for a variety of gastrointestinal problems in children. This technique is associated with a short hospital stay, minimal pain, and a very low risk of complications and can be performed even in patients who have had a previous laparotomy.- - - - - - - - - - ranking = 0.53978584176086keywords = pain (Clic here for more details about this article) |
7/38. Total colonic manometry as a guide for surgical management of functional colonic obstruction: Preliminary results.BACKGROUND/PURPOSE: Functional colonic obstruction (pseudo-obstruction) encompasses a broad group of motility disorders. Medical management of colonic pseudo-obstruction is complex and often fails, leading to surgical referral. In most cases (excepting Hirschsprung's disease) the surgeon is unable to precisely localize the area of functional obstruction. Total colonic manometry can directly measure intraluminal pressures and contractile function along the entire length of the colon. The authors propose that total colonic manometry can be used by the pediatric surgeon to guide the timing and extent of surgical therapy in refractory functional colonic obstruction. methods: Four patients were evaluated for functional colonic obstruction. All underwent barium enema and rectal biopsy with a diagnosis of Hirschsprung's disease in one patient. All patients underwent colonoscopy and total colonic manometry. Manometric tracings were obtained while fasting, after feeding, and after pharmacologic stimulation both preoperatively (n = 4) and postoperatively (n = 3). RESULTS: Total colonic manometry identified an abrupt end of normal peristalsis in 2 of the non-Hirschsprung's patients (one in the proximal colon and one in the transverse colon). Medical therapy failed in both of these patients, and they underwent diverting ostomy proximal to the loss of normal peristalsis. The third non-Hirschsprung's patient essentially had normal manometry and was able to have her colon decompressed successfully on a laxative regimen. Repeat manometry after colonic decompression showed return of normal peristalsis in 2 of these patients and continued abnormal peristaltic activity in the third. Definitive surgical intervention based on the results of total colonic manometry was performed on the latter. All 3 patients achieved normal continence. A fourth patient had Hirschsprung's disease confirmed by rectal biopsy and underwent a 1-stage neonatal modified Duhamel procedure, which was complicated by postoperative functional obstruction. manometry showed a lack of peristaltic function beginning in the right colon. An ileostomy was performed, and timing of ileostomy closure was guided by the return of normal colonic peristalsis seen on manometry. CONCLUSIONS: These initial cases show the utility of total colonic manometry in the management of colonic pseudo-obstruction syndromes. In addition to its diagnostic utility, direct measurement of colonic motor activity can be valuable in deciding the need for and timing of diversion, the extent of resection, and the suitability of the patient for restoring bowel continuity. In Hirschsprung's disease, total colonic manometry can potentially be used to determine suitability for primary neonatal pull-through versus a staged approach. J Pediatr Surg 36:1757-1763.- - - - - - - - - - ranking = 1keywords = area (Clic here for more details about this article) |
8/38. Submucosal hypoganglionosis causing chronic idiopathic intestinal pseudo-obstruction.A 39-year-old woman presented with recurrent symptoms suggestive of intestinal obstruction. She was put on total parenteral nutrition (TPN) and consequently developed sepsis and endocarditis. TPN was stopped and a venting enterostomy was performed. Biopsies of mucosa and submucosa were taken at surgery; immunohistochemistry for neuronal proteins, protein gene product 9.5 (PGP 9.5) and the glial S-100-protein was done. Many enlarged nerve fiber strands were found in the submucosa. Few small ganglia containing a small number of nerve cells could be observed, suggesting hypoganglionosis. This patient with chronic idiopathic intestinal pseudoobstruction of neurogenic type had a defect in the submucous plexus, whereas visceral neuropathies are usually characterized by defects of the myenteric plexus with normal submucous plexus.- - - - - - - - - - ranking = 3545.0476383105keywords = plexus (Clic here for more details about this article) |
9/38. Disseminated intestinal hypoganglionosis treated by colectomy and tapering of the small intestine. A case report.A girl suffering from chronic constipation and abdominal distension from her first year of life underwent internal anal sphincter myectomies at 5 and 7 years of age without resolution of her symptoms. At the age of 8, an ileostomy was performed because of excessive colonic dilation and hypomotility. Biopsies from the colon and distal ileum showed intestinal neuronal dysplasia TYPE B (INDB) with hypoganglionic areas. colectomy and ileorectal anastomosis were done at the age of 10. Three years later, however, an ileostomy was re-established because of recurrent episodes of pseudo-obstruction. In the hope of improving intestinal motility, the dilated small intestine was tapered over its entire length of 3.6 meters. Histological findings still demonstrated oligoneuronal hypoganglionosis and INDB all along the resected strip of bowel wall. After 6 months, the stoma was closed. At the age of 15 years, tapering of the distal 80 cm of the ileum was repeated in combination with cholecystectomy for cholecystolithiasis. Intestinal transit time decreased from 55 hours before the first to 18 hours after the second tapering procedure. Now, 7 years after the last operation, the patient passes 3 - 4 soft stools daily, is physically active, on a normal diet and not on any regular medication.- - - - - - - - - - ranking = 1keywords = area (Clic here for more details about this article) |
10/38. Total colonic aganglionosis: a case study.Total colonic aganglionosis (TCA) is a rare, hybrid form of Hirschsprung's disease. It is a functional rather than mechanical obstruction, characterized by the absence of intrinsic ganglion cells in the myenteric and submucosal plexuses of the bowel wall. Ganglion cells regulate normal colonic peristaltic activity. Paucity of ganglion cells results in an aganglionic segment of bowel that is functionally abnormal and does not propagate the normal peristaltic wave that moves to it from the proximal ganglionic bowel. The lesion originates in the rectum and extends proximally over a variable distance of the bowel. The further the lesion extends, the more difficult the management becomes. Clinical and radiologic findings can be useful in diagnosis, but they are not pathognomonic. The definitive diagnosis is made following suction biopsy of the rectum, colon, and ileum. Ultimate treatment for TCA is surgical, although no single surgical procedure has been proven superior. Total parenteral nutrition during the postoperative period is essential to ensure appropriate fluid and electrolyte status. Improvements in supportive care and earlier recognition and diagnosis of TCA in infants have led to a significantly increased rate of survival since the lesion was first recognized. The embryology, pathogenesis, clinical presentation, diagnosis, management prognosis, and outcome of TCA are discussed. A case study is presented.- - - - - - - - - - ranking = 1181.6825461035keywords = plexus (Clic here for more details about this article) |
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