• Advances in pediatric surgery
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• v.7 no.2
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• pp.105-111
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• 2001

To investigate the diagnostic accuracy and applicability of barium enema(BE) and rectal suction biopsy with acetyl cholinesterase(AChE) histochemistry in the diagnosis of neonatal Hirschsprung's disease(HD), we retrospectively reviewed the findings of BE and AChE staining in 96 neonates with suspected HD during a 10-year period from January 1991 to December 2000. Sixty-nine cases of HD(58 males and 11 females) and 27 cases of non-HD are included in this study. In regard to BE, HD was based on definite transitional zone, suspicious HD on reversed rectosigmoid index(RSI <1), and non-HD on normal RSI(RSI>1). The histochemical criterion used for the diagnosis of HD was that of Chow et al(1977), i.e., the presence of many coarse discrete cholinergic nerve fibers in the muscularis mucosae and in the immediately subjacent submucosa regardless of infiltration of cholinergic nerve fibers in the lamina propria. Of 66 neonates with HD who underwent BE, transitional zone was identified in 33 cases(50 %) and reversed RSI in 19 cases(21 %), microcolon in 4 cases and normal finding in 10 cases(15 %) while of 27 neonates with non-HD, there was normal finding in 16 cases and reversed RSI in 9 cases(41 %). Thus diagnostic accuracy based on transitional zone was 64 %. The positive predictive value of reversed RSI for the diagnosis of HD was 68 %. Of 42 neonates with HD who underwent AChE histochemistry, there were 41 AChE-positive reactions and one AChE-negative reaction in a neonate with total colonic aganglionosis, while of 27 cases of non-HD, there were one equivocal AChE-positive reaction and 26 AChE-negative reactions. Thus AChE histochemical study showed a 97 % diagnostic accuracy with a 98 % sensitivity and a 96 % specificity. In conclusion, we believe that BE is valuable as a first diagnostic step since about 80 % of neonates with HD show significant radiologic findings such as a transitional zone or reversed RSI. AChE histochemical study was a more reliable diagnos tic tool showing a 97 % diagnostic accuracy, and is part.

• Advances in pediatric surgery
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• v.19 no.2
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• pp.122-129
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• 2013

Immature ganglion cell (IGC) is known for its relationship with intestinal motility and its impact on postoperative functional outcomes of Hirschsprung's disease (HD). There are few studies on the relationship between intestinal dysmotility and IGC in HD patients. 67 patients pathologically diagnosed with HD and who received definitive operation in Seoul National University Children's Hospital from 2010 to 2011 were included. 10 patients were excluded due to inadequate immunohistochemical staining results. The proximal end of resected ganglionic segment was evaluated with immunohistochemistry examination with MAP-2, a marker of ganglionic cells and bcl-2, a marker of IGCs The median age at operation was 155 (15-4678) day-old. 55 (96.5%) patients positive for bcl-2, were regarded as having IGC, and 2 (3.5%) patients positive for MAP-2 but negative for bcl-2, were regarded as having only mature ganglion cells. In the bcl-2 positive group, there were 7 patients (12.7%) with constipation, 15 patients (27.3%) with soiling, 3 patients (5.5%) with perianal excoriation and 6 patients (10.9%) with medication use. In bcl-2 negative group, intestinal dysmotility was not seen. There was no statistical significance in the two groups. Considering that HD is diagnosed at a young age, the rate of IGC present is very high and it might be inappropriate to relate IGC to functional outcome at young ages.

• Advances in pediatric surgery
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• v.6 no.1
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• pp.27-31
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• 2000

Fecal incontinence is not rare in children who have been operated upon for anorectal malformation, Hirschsprung's disease, and meningomyelocele. Incontinence can negatively impact on the emotional and social development of these children. Antegrade continence enema (ACE) was utilized in 9 patients with meningomyelocele from January 1998 to June 1999. Cleanliness and post-operative complications were evaluated. Three operative techniques were employed; reversed appendicocecostomy, in situ appendicostomy and neoappendicostomy with cecal flap. All of the patients achieved complete fecal cleanliness. Four patients had post-operative complications; two with stoma leakage, one with abdominal pain on irrigation and one with stoma stenosis. The leakage was minimal and the pain on irrigation improved spontaneously. The stoma stenosis required revision 6th month postoperatively. The ACE has been shown to be safe and highly effective.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.2
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• pp.137-145
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• 2020

Purpose: The Oral-anal Transit Test (OTT) is a simple method of obtaining information about colonic transit. We aim to assess the correlation of OTT with the neuromuscular integrity of the colon determined by colonic manometry (CM). Methods: All patients who had OTT followed by CM were evaluated. Less than 6 of 24 markers remaining on OTT was considered normal. CM was performed per previously published guidelines. A normal CM was defined as at least one High Amplitude Propagating Contraction progressing from the most proximal sensor through the sigmoid colon. Results: A total of 34 patients underwent both OTT and CM (44% male, age 4-18 years, mean 11.5 years, 97% functional constipation +/- soiling, Hirschsprung's Disease). Of normal and abnormal OTT patients, 85.7% (6/7) and 18.5% (5/27) respectively had normal CM. When all markers progressed to at least the sigmoid colon, this was 100% predictive against colonic inertia. Greater than 50% of patients with manometric isolated sigmoid dysfunction had markers proximal to the recto-sigmoid. Conclusion: OTT and CM are both valuable studies that assess different aspects of colonic function. OTT can be used as a screening test to rule out colonic inertia. However, the most proximal extent of remaining markers does not predict the anatomical extent of the manometric abnormality, particularly in isolated sigmoid dysfunction.