Diagnosing Hirschsprung's disease is a clinical challenge. Hirschsprung's disease should be considered in any child who has a history of constipation dating back to the newborn period. We examined diagnostic methods and their results retrospectively in 37 neonates and infants who underwent both barium enema and anorectal manometry for the diagnosis of Hirschsprungs disease at Asan Medical Center between January 1999 and April 2001. Two radiologists and a surgeon repeatedly reviewed both of the diagnostic results. In anorectal manometry, thirty-four studies were in agreement with the definitive diagnosis, giving an overall diagnostic accuracy of 91.9 % (neonate; 100 %, infant; 85.7 %). The accuracy and specificity of barium enema was lower than those of anorectal manometry, but sensitivity was higher. There was no significant difference between the two methods. Both studies showed findings consistent with the final diagnosis. However, discordant results needed further evaluation or close observation to diagnose accurately. We conclude that Hirschsprungs disease should not be diagnosed by only one diagnostic method.

To clarify the necessity of surgery for fistula-in-ano (FIA) in infant, a retrospective analysis of 82 cases FIA in infant were performed for 11 years period from 1987 to 1998. Group A included 44 cases in the period of surgery oriented treatment to 1992, and group B contained 38, period of feeding control oriented management (FC) after 1993. FC, that was indicated in case with loose stool and eczematoid perianal skin (LSES), composed of quit breast feeding, change of cow milk or complete weaning. Surgical decision was made after improvement of stool condition. Fistulectomy was performed in 29 cases (65.9 %) of group A. However, in group B, only 15 cases (39.5 %) required surgery (p=0.0036). Thirty-four cases in B had the history of LSES, and FC was applied in 25, and was effective in 24. Among 24 cases, 21 showed complete healing with FC, 3 had surgery because of the persistent FIA, and 1 got operation due to no improvement of stool condition. In conclusion, FC has to be applied to FIA in infant before surgery, particularly in cases with history of LSES. As one of the etiologic factors for FIA, LSES could be considered to this particular age group.

This is a 20 year analysis of the problems associated with enterostomy formation, and closure. Forty-three stomas were established in 43 patients: 23 for anorectal malformations, 11 for Hirschsprung's diseases, 4 for necrotizing enterocolitis, 3 for multiple ileal atresias, 1 for volvulus neonatorum with perforation, and 1 for diaphragmatic hernia with colon perforation. Thirty boys and 13 girls were included (mean age 4.8 months). Stoma complications were encountered in 13 patients (30.2 %): stomal prolapse, stenosis, obstruction, paracolic hernia, retraction, dysfunction, and skin excoriation. Four patients (9.3 %) required stomal revision. Occurrence of complications was not related to age and primary disease, but sigmoid colostomy showed lower complication rate than transverse colostomy (20.0 % vs 42.9 %, p<0.05). There were five deaths but, only one (2.3 %) was directly related to the enterostomy complication. Twenty-one stomas were closed in our hospital and complications occurred in seven patients (33.3 %). The most common complication was wound sepsis in 5 children. In conclusion, because the significant morbidity of stomal formation still exists, refinements of the surgical technique seem to be required. Sigmoid loop colostomy is preferred whenever possible.

Between March 1999 and January 2000, 82 boys with the diagnosis of inguinal hernias (12 bilateral and 70 unilateral hernias), underwent Ultrasound (US) examination of both sides of the groin, a total of 164 inguinal imaging prior to surgery. The patients ages ranged from 3 days to 12 years with a mean of 32.6 months. Ninty four examinations were on the clinically symptomatic side and 70 were on the asymptomatic side. The US criteria for the diagnosis of an inguinal hernia were as follows: 1) visceral hernia, the presence of bowel loops, or omentum in the inguinal canal, 2) communicating hydrocele, the presence of fluid in the processus vaginalis, 3) widening of patent processus vaginalis at the level of nternal inguinal ring. The width of patent processus vaginalis at the level of internal inguinal ring over 4 mm is onsidered an occult hernia. Among the 94 symptomatic groins, US findings showed 31 (33 %) visceral hernias, 18 (19 %) communicating hydroceles, and 38 (41 %) widening of the internal inguinal ring, and 7 (7 %) groins without abnormalities. In 70 asymptomatic groins, there were 4 (6 %) visceral hernias, 5 (7 %) communicating hydroceles, 11 (16 %) widening of the internal inguinal ring, and 50 (70 %) groins without abnormalities. Among the 70 asymptomatic groins there were US abnormalities in 20 (28 %). One hundred and seven groins with positive US findings were surgically explored. Among 107 operated sites, the operative findings were compatible with the US diagnosis in 104, a sensitivity for US of 97.2 %. In patients with US findings of widening of internal inguinal ring (>4 mm), there was patent processus vaginalis in 36 out of 38 symptomatic groins and 10 of 11 asymptomatic groins. The sensitivity of US to the operative findings in widening of internal inguinal ring was 93.8 %. For visceral hernia and communicating hydrocele, the sensitivity of positive US findings was 100 %. Ultrasonography for inguinal hernias appears to be a rapid, reliable, and noninvasive screening diagnostic tool with high positive specificity. Therefore, we recommend the use of US as a routine diagnostic tool in pediatric patients with inguinal hernias and hydroceles.

Infantile hypertrophic pyloric stenosis (IHPS) a common childhood disorders characterized by nonbilious projectile vomiting, an olive shaped mass in the right upper quadrant of the abdomen and visible gastric peristaltic wave in the upper abdomen. Its etiology and pathogenesis are not clear but abnormal nerve distribution of the pylorus has been postulated^2-6. We performed immunocytochemical staning to the pyloric muscle from 10 IHPS and 3 controls patients, utilizing specific monoclonal antibody to NCAM(neural cell adhesion molecule). In IHPS patients, the number of NCAM protein immunoreactive nerve fibers were less than that in normal subjects. Auerbach myenteric plexuse was well developed and interbundle nerve plexuse was present but nerve fibers supplying individual muscle cells in smooth muscle bundles were poorly developed. These results indicate reduction of innervation in smooth muscles in IHPS patients that possibly contributes to the pathogenesis of IHPS.

Meckel's diverticulum (MD) occurs in approximately 2 % of the population. The major complications of MD are bleeding, intestinal obstruction, infection and perforation. Perforation is the least common but most serious complication, the incidence od which is about 5-10 %. The causes of perforation are inflammatory diverticulitis and peptic ulceration. the purpose of study is to review the characteristics of perforated MD in children. Six patients with perforated MD who had been operated upon at the Department of Pediatric Surgery, Yeungnam University Hospital from April 1984 to July 2001 were included. Male predominated in a ratio of 5:1 and there were 2 neonates. The chief complaints were abdominal pain and distension. Half of the children showed a past history of bloody stools. The average age was 4 year and 9 months. The mean distance from the ileocecal valve to the diverticulum was 60 cm. Average length of the diverticulum was approximately 3 cm and width was 1.7 cm. The perforation site was the tip of the diverticulum in 3 cases, the base in 2 cases and along the lateral border in one. In two patients, ectopic gastric mucosa was found in the specimen. All of the patients were operated upon with a diagnosis of peritonitis of unknown etiology. In conclusion, when a child shows symptoms of acute abdomen or peritonitis, especially in boys, with the history of bloody stools and episodic abdominal pain, perforated MD should be suspected.

The purpose of this study is to evaluate the applicability of intravenous atropine sulfate therapy in infantile hypertrophic pyloric stenosis (IHPS). From 1998 to 2000 among 35 cases of IHPS, pyloromytomy was performed in 13 (Group A), and intravenous atropine was given as a primary therapy in 22 cases (Group B). In group A, all cases were cured completely. In group B, 13 (59 %) out of 22 cases were successfully treated with atropine, but 9 were failed therapy, and required operation. The recovery period to normal feeding and the hospital stay of the successful atropine group were longer than those of pyloromyotomy, 8.6 days vs. 2.9 days and 13.2 days vs. 4.1 days, respectively. In conclusion, intravenous atropine therapy did not replace pyloromyotomy, but it might be an alternative for the selected patients with contraindications for operation.

No abstract available.

Abnormal distribution of the enteric nerves such as adrenergic, cholinergic and peptidergic nerves may cause the functional obstruction in Hirschsprung's disease (HD). Although the sustained contraction of the aganglionic segment is the main pathophysiology of HD, the etiology and pathogenesis is not thoroughly understood. With the recent progress of molecular biology and genetics,a more detailed approach to the pathogenesis of the HD can be undertaken. In this review, the roles of the nitric oxide, nitric oxide synthase and interstitial cells of Cajal on smooth muscle relaxation, the effects of extracellular matrix, cell adhesion molecules, neurotrophic factors on the migration and maturation of the neural crest cells are described. In the section of genetic factors, familial occurrences, association of chromosomal abnormalities, RET gene, glial cell line-derived neurotrophic factor gene, endothelin-3 gene and endothelin-B receptor gene and their relationships to HD is briefly reviewed.

Diagnosing Hirschprungs disease (HD) is a clinical challenge to pediatric surgeons. The cardinal symptoms are failure of passage of meconium within first 24 hours of life, abdominal distension, and vomiting. The severity of these symptoms and the degree of consitpation vary considerably between patients. HD is suspected on the basis of history and clinical findings and the diagnosis is established by radiological examination, anorectal manometry, and histochemical analysis of biopsy specimens. In this review, the advantages and pitfalls of each diagnostic modality are discussed. And a diagnostic approach utilizing these diagnostic modalities in children with suspicious HD is presented.

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 contrastenema 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.

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.

Since Swenson and Bill established the treatment principles of Hirshsprung's disease in 1948, there have been significant advances in the management of this disease. During the last decade, one-stage correction of Hirshsprung's disease without colostomy and primary laparoscopic pull-through procedure became popular with recent advances in the technology and refinement of the equipments. But the outcomes for Hirshsprung's disease are not always successful, and long-term follow-up isessential. Most children after corrective surgery show significant improvement in respect to fecal continence and constipation, that may not be apparent until late adolescence. The purpose of this study was to review the postoperative problems of Hirschsprung's disease and it's management.

Enterocolitis associated with Hirschsprung's disease has been a major cause of morbidity and even mortality, and before and after definitieve surgical treatment. It shows typical clinical characteristics, however, its pathogenesis has been poorly understood. Treatment is diverse, and consists of conservative tertment with intravenous hydration, antibiotics and rectal wash out, and surgical tertment with temporatory enterostomy, and other surgical procedures.