Cases reported "Hirschsprung Disease"

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11/51. Hirschsprung's disease, associated rare congenital anomalies.

    OBJECTIVE: Hirschsprung's disease may be associated with a number of congenital anomalies of which Down's syndrome and intestinal atresias are commonly encountered. The study aimed to assess the impact of rare associated anomalies on the diagnosis and management of Hirschsprung's disease. methods: A retrospective review of the clinical presentation, diagnosis and outcome of thirty five consecutive newly diagnosed cases of Hirschsprung's disease encountered over two years was performed. RESULTS: Besides Down's syndrome (two), intestinal atresia (one) and pigmentary ocular defects (two), three rare anomalies (Occipital meningocele, Calcific meconium cyst with anal stenosis, Malrotation) were encountered in four of thirty five cases. The clinical features, radiologic anatomy and gross morphology of the bowel were unconventional and the diagnosis was supported by intraoperative acetylcholinesterase staining of biopsies. Though the diagnosis was relatively delayed in these cases, the outcome has been comparable to the rest. The dilemma in their diagnosis and management and their possible pathoembryology is discussed. CONCLUSION: awareness of such associations and a specific investigative protocol is imperative for timely diagnosis and minimal morbidity in complex presentations of Hirschsprung's disease.
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12/51. 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.
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13/51. Severe anaphylactic shock without exanthema in a case of unknown latex allergy and review of the literature.

    We describe a case of severe anaphylactic shock without exanthema in an 8-year-old boy who was undergoing elective surgery for an ileostomy. The boy had a history of four anorectal operations in the newborn period, but no history of allergies. Sudden, profound bronchospasm and cardiorespiratory collapse occurred 30 min after the beginning of the operation. No signs of exanthema or urticaria were seen. The patient was resuscitated successfully and remained ventilated for 25 h. During this time, he needed epinephrine in dosages of 0.3-0.05 microg.kg-1.min-1. Radioallergosorbent (RAST) tests for the patient were positive Cap Class 3 and 4, and specific immunoglobulin e was highly positive, suggesting a natural rubber latex allergy as the cause of the anaphylactic shock. ethylene oxide was negative. Eleven days later, a further operation was performed with a strict latex-free protocol in the presence of histamine 1 and 2 receptor antagonists. On this occasion, the intraoperative course was uneventful. incidence of anaphylactic reactions, prevalence of latex sensitization, special risk groups and management are discussed.
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14/51. 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.
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15/51. Combined abdominal and posterior sagittal approach for redo pull-through operation in Hirschsprung's disease.

    BACKGROUND/PURPOSE: In Hirschsprung's disease (HD) redo pull-through (PT) is indicated for anastomotic complications and retained aganglionosis after a previous operation. Duhamel or Swenson method is used commonly for redo operations. The pelvic dissection may be difficult, especially in Swenson's type of operation, because of fibrosis resulting from previous surgery or its complications. To overcome this, the authors used a combined abdominal and posterior sagittal approach to perform redo pull-through of Swenson's type in 4 children. methods: Four boys (2.5 to 12 years) underwent redo pull-through for failed endorectal pull through (n = 2), persistent symptoms after 2 myectomies (n = 1) and late anastomotic disruption after Swenson's PT (n = 1). Abdominal dissection was done first to mobilize colon and resect aganglionic segment as far as the mid pelvis. The mobilized ganglionic colon was tacked to the pelvic rectal stump, hemostasis checked, and the abdomen closed. The lower pelvic dissection was done through the posterior sagittal route, under direct vision. The remainder of diseased rectum was excised, and the pull-through colon was retrieved and anastomosed to the anal stump. No covering colostomy was done. RESULTS: A rectocutaneous fistula developed in one patient, which healed spontaneously. All patients had increased stool frequency in the early postoperative period but improved with time. All patients have attained normal voluntary bowel actions, but one child has infrequent minor soiling. There was no anastomotic narrowing in any case. CONCLUSIONS: Posterior sagittal approach is a useful alternative in difficult redo pull-through surgery. It offers excellent exposure, precise dissection, and direct anastomosis. There are minimal chances of complications, and continence is retained.
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16/51. Staged laparoscopic-assisted pull-through for Hirschsprung's disease.

    The authors report 3 cases of Hirschsprung's disease that were treated by laparoscopic-assisted transanal pull-through after a colostomy already had been performed. Two of these patients presented with severe enterocolitis, and a primary laparoscopic-assisted single-stage transanal pull through was not feasible. The third patient had a colostomy performed and was referred to us for a definitive procedure. Many centers over the world now perform laparoscopic-assisted single-stage pull-through as a primary modality of management for Hirschsprung's disease. But for a country like india, where patients with Hirschsprung's disease present or are referred late and frequently with enterocolitis, performing a primary procedure is not possible in all cases. However, this has been used as the definitive procedure after performing a diverting colostomy and histopathologic determination of the length of the aganglionic bowel. The procedure gives excellent results and permits early postoperative feeding, early hospital discharge, and good cosmetic results.
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17/51. Endorectal pull-through with posterior sagittal approach to the repair of postoperative rectourethral and rectovaginal fistula.

    BACKGROUND/PURPOSE: Rectourethral (RUF) or rectovaginal fistula (RVF) is a troublesome complication after anorectal surgery because of dense adhesions around the fistula. The authors applied a new technique for the redo surgery. methods: Case 1 is Hirschsprung's disease in a 1-year-old boy who underwent modified Duhamel's procedure and had RUF. Case 2 is rectovestibular fistula in an 11-year-old girl who had anterior sagittal anorectoplasty complicated by RVF. Case 3 is multiple urogenital anomalies including rectovesical fistula in a 4-year-old boy in whom transvesical repair was unsuccessful. The colon was mobilized as far as possible at laparotomy. The rectum was opened via a posterior sagittal approach leaving 1 cm of the anal canal. Extended endorectal mucosectomy was performed to the dentate line, and the fistula was closed from inside of the rectum. The remaining mucosal cuff was everted out of the anus and the intact colon was pulled through the rectum and anastomosed to the cuff extraanally. RESULTS: The postoperative contrast enema showed no recurrent fistula, and defecation was not impaired. CONCLUSIONS: Endorectal pull-through of the intact colon can spare troublesome mobilization of the fistula and can prevent the recurrence of fistula. Rectal incision via a posterior sagittal approach provides a direct view of the fistula.
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18/51. Plasticity of the enteric nervous system in patients with intestinal neuronal dysplasia associated with Hirschsprung's disease: a report of three patients.

    Intestinal neuronal dysplasia is a controversial form of dysganglionosis that has been described both as an isolated disorder and in association with Hirschsprung's disease. It has been blamed for the bad outcome of bowel function in patients operated on for Hirschsprung's disease. According to various authors, intestinal neuronal dysplasia could be a primary disorder or secondary to other diseases of the bowel. The aim of this study was to assess the plasticity of the enteric nervous system in patients with Hirschsprung's disease-associated intestinal neuronal dysplasia and its ability to evolve spontaneously to normal innervation patterns. Since we prospectively introduced the assessment of the enteric nervous system of the ganglionated bowel in patients operated on for Hirschsprung's disease, 31 patients have been operated on for Hirschsprung's disease in our institution between 1995 and 2002. Among these patients, nine suffered postoperatively from severe constipation and five from bouts of entocolitis. IND was found in eight of them. We studied the evolution of the innervation in three of these patients by repeated serial full-thickness biopsies of the colon. All three patients underwent a colostomy before or after the pull-through procedure. Histopathological assessment of the enteric nervous system was made with conventional acetylcholinesterase histochemistry, rapid acetylcholinesterase histochemistry and immunohistochemistry for the Protein Gene Product 9.5 and the antigen CD56. This evolution was compared with the clinical outcome of bowel function. In our three patients with Hirschsprung's disease-associated intestinal neuronal dysplasia, this form of dysganglionosis evolved to normal innervation patterns within a period ranging from 9 to 18 months. This evolution was accompanied by an improvement of bowel function in all. We conclude that Hirschsprung's disease-associated intestinal neuronal dysplasia can evolve to a normal innervation, at least under certain circumstances such as a colostomy. Specific histopathological techniques are required to assess accurately the enteric nervous system.
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19/51. Laparoscopic relief of obstructing folded muscular cuff after transanal pull-through for aganglionosis.

    Transanal endorectal resection and colonic pull-through (TERPT) is a good technique for the management of Hirschsprung's disease. This procedure is feasible in the vast majority of patients and is associated with excellent results, early postoperative recovery, and no visible scars. We report the case of a patient who developed early postoperative severe constipation after TERPT due to unusual folding of the muscular cuff rim, which tightly narrowed the pulled-through colon. This complication was diagnosed and treated by laparoscopy. To prevent it, we recommend splitting of the aganglionic muscular cuff during TERPT.
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20/51. Stapled anopexy for short segment Hirschprungs disease in adults.

    Traditional treatment of short segment Hirschprungs disease in adult consists of major resectional procedures, often with the use of a temporary stoma. patients with this disease may have significant morbidities that increase their risk of post-operative morbidity and mortality. In an attempt to minimize the procedural related morbidity, we describe the application of the stapled anopexy technique to treat short segment Hirschprungs disease.
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