The etiology of several motility disorders, including persistent megacolon after definitive surgery for Hirschsprung's disease, meconium ileus which is not associated with cystic fibrosis and idiopathic megacolon, is still unclear. Interstitial cells of Cajal (ICC) are thought to modulate gut motility as gastrointestinal pace maker cells. The aim of this study was to evaluate the role of ICC in the bowel walls of the patients (n=15) who had variable motility disorders. The ICC were identified by immunohistochemical staining using an anti-C-Kit antibody and the results were compared with control specimens (n=2). The control group (G1) showed evenly distributed ICC in their bowel walls. The second group (G2, n=5) who had normal bowel movements after Duhamel procedures and the third group (G3, n=4) who had persistent megacolon after Duhamel procedures showed absent or scarcely distributed ICC in their aganglionic bowels. The ICC were identified by immunohistochemical staining using an anti-C-Kit antibody and the results were compared with control specimens (n=2). The control group (G1) showed evenly distributed ICC in their bowel walls. The second group (G2, n=5) who had normal bowel movements after Duhamel procedures and the third group (G3, n=4) who had persistent megacolon after Duhamel procedures showed absent or scarcely distributed ICC in their aganglionic bowels. Whereas ICC were evenly distributed in the ganglionic bowels of G2, they were not seen or scarecely distributed in the ganglionic bowels of G3. Two patients (G4) who suffered from idiopathic megacolon showed absence or decrease of ICC in spite of presence of ganglion cells in their colons. Four neonates (G5) who underwent ileostomy because of meconium obstruction showed absent or markedly decreased ICC in the colon at the time of ileostomy and the distribution of ICC was changed to a normal pattern at the time of ileostomy closure between 39-104 days of age and their bowelmotility were restored after that. The results suggest that lack of ICC caused reduce motility in the ganglionic colons and it may be responsible for the development of various motility disorders. Delayed maturity of ICC may also play a role in the meconium obstruction of neinates.

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.

Injured spleens have been successfully managed without operation in a number of children; however, splenectomy or splenic-conserving surgery may not be avoided because of exsanguinating hemorrhage. This study was performed to evaluate the efficacy of splenic arterial embolization (SAE) to control hemorrhage from injured spleens in children. We compared the outcomes of two groups of children with splenic injury. The first group (G1) consisted of eighteen children who were managed with conventional selective nonoperative treatment between 1993 and 1994. The second group (G2) consisted of 23 children prospectively studied from 1996 to 1997 after SAE was added in the management protocol of splenic injury. The criteria for SAE were grade III or IV injury, extravasation of contrast material revealed by CT, or unstable vital signs without evidence of associated injuries. Laparotomy was performed in 6 patients of G1 (33.3%), 2 of whom had associated injuries. Five underwent splenectomy and the overall salvage rate in G1 was 72.2% (13/18). In G2, eight patients (34.8%) had SAE, which stopped bleeding successfully in all patients. Two of G2 (8.7%) had laparotomy because of associated injuries. Only one patient underwent splenectomy and the salvage rate was 95.6% (22/23). No patients required transfusion after SAE. In conclusion, the SAE effectively controlled hemorrhage from injured spleens. More spleens were salvaged with a reduced laparotomy rate after application of SAE in splenic injury.

Anal endosonography has recently been popularized in adult patients in order to access staging of rectal cancer and other lesions of the anorectum. This study is not familiar to pediatric surgeons. We performed anal endosonography in 30 children without anorectal disease in order to determine the standard morphology of the anorectum. The internal anal sphincter(IAS) was clearly identified as a homogeneous hypoechoic circular band, extending caudally to a level just proximal to the anal verge. The external anal sphincter(EAS) showed mixed echogenicity and different architecture along the anal canal; the EAS was U-shape in the upper canal and it had a circular pattern in the lower canal. In the upper canal of girls, the perineal body and the vagina were found just anterior of the anal canal, which made the U shape of the EAS. In the male, the sphincter tapered anteriorly into two arcs that met in the midline. The perineal body was prominent in the female. Posteriorly, the anococcygeal ligament was represented by a triangular shadow in both sex. The thickness of the lAS was measured in 3 directions, left, right and posterior, at 3 levels, upper, middle and lower areas of the anal canal. The average thickness was 0.86-2.40 mm between 6 and 18 months of age(Group 1), 0.88-3.20 mm between 19 and 36(Group 2), 1.07-2.20 mm between 37 and 54(Group 3) and 1.18-2.42 mm more than 54-month-old(Group 4). The thickness was correlated wtih the age of the children only in the right upper(p= 0.008) and the left middle portion(p=0.015). We were able to obtain standard morphologic features of the normal anal canal in children with anal endosonography. We believe that this technique is a safe and an effective procedure to evaluate anorectal lesions in children.

During a 6-year period, from January1990 to December 1995, 101 neonates with congenital anomalies were admitted to the division of Pediatric Surgery of Y oungdong Severance Hospital. All of them had prenatal screening more than once with ultrasound. Fifty eight of them had prenatally detectable anomalies by ultrasonography. However abnormalities were prenatally detected in 24 neonates( 41 %). The detection rate was 70% in patientws who had the prenatal screening at our hospital, whereas, the rate was 24% when it was performed at other medical facilities. Duodenal and jejunoileal atresia showed the highest detection rate(86%) followed by abdominal mass. Esophageal atresia was suggested by maternal polyhydramnios in 3 patients(25%). Only one patient with diaphragmatic hernia ( 1.75%) was prenatally detected and none with gastroschisis. The mean interval from birth to operation was 32 hours in the prenatally detected patients and 50 hours in the nondetected. The complication rate and the mortality after emergency operation were 20% and 7% in the detected group, and 58% and 23% in the nondetected, respectively. The average period of the hospitalization was 20 days in the detected group and 39 days in the nondetected. We conclude that the prenatal detection of anomalies is necessary to ensure adequate care for the mothers and the babies with congenital anomalies. This includes early transfer, timing of optimal delivery and operation.