• Advances in pediatric surgery
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• v.8 no.1
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• pp.62-63
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• 2002

Various surgical techniques for Hirschsprung's disease including total colonic aganglionosis have been performed with similar results. The type of redo pull-through procedure is determined by the cause of failure and the type of primary pull-through.

• Advances in pediatric surgery
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• v.4 no.1
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• pp.74-78
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• 1998

Early recognition and surgical treatment of Hirschsprung's disease prevents serious mortality and morbidity from enterocolitis and obstruction. Usually this disease is characterized by a single aganglionic segment of the colon extending distally to the anal margin. In surgical treatment, the surgeon performs a frozen section biopsy to confirm whether there are ganglion cells. If there are intervening ganglionic sites in aganglionic bowel, there may be confusion in diagnosis and treatment. The authors experienced one case of total colonic aganglionosis with skip area. A transverse loop colostomy was performed on a 7 day-old male baby with colon perforation due to Hirschsprung's disease. But intestinal obstruction persisted and required two more operations to find the true nature of the disease. There were aganglionic segments from the anal margin to the terminal ileum 3.7cm proximal to the ileocecal valve. The entire transverse colon and appendix were normally ganglionated.

• Advances in pediatric surgery
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• v.1 no.2
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• pp.186-189
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• 1995

Neonatal intestinal perforation is mainly caused by necrotizing enterocolitis, intestinal atresia, meconium ileus or unknown etiology. Occasionally, Hirschsprung's disease presents with neonatal intestinal perforation, of which, it is known that total colonic aganglionosis is common. Therefore, Hirschsprung's disease should be considered as a cause of neonatal intestinal perforation. The authors have experienced 3 cases of neonatal Hirschsprung's disease associated with colonic perforations. Cecal perforations were noted in 2 cases with aganglionosis from descending colon and sigmoid perforation in a case with aganglionosis in rectum. These cases will be discussed with literature review.

• Advances in pediatric surgery
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• v.13 no.1
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• pp.61-65
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• 2007

Conventional treatment of Hirschsprung's disease consists of initial colostomy followed by pull-through operation. But, the treatment of Hirschsprung's disease has been changed along with the development of new surgical technique. Since 1995, endo-GIA has been available at our hospital and one stage Duhamel operation has been performed for neonatal Hirschsprung's disease. Between May 1995 and April 2006, 26 neonates have been treated with one stage pull-through operation by one pediatric surgeon at HanYang University Hospital. The sex ratio was 4.2:1 with male predominance. Clinical findings included abdominal distension (96.2 %), vomiting (50.0 %), delayed passage of meconium (46.2 %), constipation (23.1 %), and enterocolitis (15.4 %). Twenty two cases (84.6 %) were short-segment and 4 cases (15.4 %) were long-segment disease, of which 2 cases were total colon aganglionosis. One of the two patients with total colonic aganglionosis had double transition zones - distal ileum and hepatic flexure of the colon. The average age at operation was $14.56{\pm}8.77$ days and the average weight at operation was $3.26{\pm}0.66kg$. Primary Duhamel operations were performed in 25 patients and Soave-Boley operations was performed in one patient. The endo-GIA 35 (Ethicon, USA) was used from 1995 until 1997, and after that endo-GIA 60 (USSC, USA) was used. The average Duhamel operation time was $88.57{\pm}22.80$ minutes. Wound abscess (n = 2) and septum formation (n =1) occurred after Duhamel operation. Bowel function was normalized in 59 % within 3 months and in 95% within 1 year after operation. There was no mortality after one stage pull-through operation in neonate.

• Advances in pediatric surgery
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• v.8 no.1
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• pp.54-61
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• 2002

Hirschsprung's disease (HD) is usually diagnosed in the newborn period and early infancy. The common presentation of HD in newborns consists of a history of delayed passage of meconium within the first 48 hours of life. The differential diagnosis in newborns is one of the clinical challenges of this disorder. A number of medical conditions which cause functional obstruction of the intestines are easily excluded. Neonates with meconium ileus, meconium plug syndrome, distal ileal atresia and low imperforate anus often present in a manner similar to those with HD in the first few days of life. Abdominal radiographs may help to diagnose complete obstruction such as intestinal atresia. Microcolon on contrast enema can be shown in cases with total colonic aganglionosis, ileal atresia or meconium ileus. Suction rectal biopsy or frozen section biopsy at operation is essential for differential diagnosis in such cases. HD is also considered in any child who has a history of constipation regardless of age. Older children with functional constipation may have symptoms that resemble those of HD and contrast enema is usually diagnostic. However, children with other motility disorders generally referred to as chronic idiopathic intestinal pseudoobstruction present with very similar symptoms and radiographic findings. These disorders are classified according to their histologic characteristics.; visceral myopathy, visceral neuropathy, intestinal neuronal dysplasia (IND), hypoganglionosis, immature ganglia, internal sphincter achalasia. Therefore, the workup for motility disorders should include rectal biopsy not only to confirm the presence of ganglion cells but also evaluate the other pathologic conditions.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.202-207
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• 2003

Intestinal neuronal dysplasia (IND) is a disorder of abnormal intestinal innervation resulting in dysfunctional colonic motility. IND shares clinical features with Hirschsprung's disease but differentiated by histological findings such as hyperplasia of submucosal and myenteric plexuses, giant ganglia, ectopic ganglion cell and increased acetylcholinesterase activity in lamina propria. Although IND may exist as an isolated condition, more commonly, it occurs in association with Hirschsprung's disease. We report a case of twins affected with IND. Both children manifested with delayed passage of meconium and severe abdominal distention after birth. Barium enema in both patients showed microcolon. They underwent emergency ileostomy under the impression of total aganglionosis. But surgical biopsy specimens showed hyperganglionosis in submucosa with formation of giant ganglia. Both neonates suffers from several episodes of peudo-obstruction after the repair operation of colostomy.

• 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.