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.

It has been widely accepted that complete surgical resection of hepatoblastoma is essential for long-term survival. But unfortunately less that 50% of hepatic tumors in children can be totally removed at the time of diagnosis. This report is to present the experience of successful resection of hepatoblastoma after concurrent radiotherapy with transarterial chemoinfusion in a child. We believe this modality of treatment enables complete resection of unresectable hepatoblastoma, which is resistant to the systemic chemotherapy.

Slide tracheoplasty, as a treatment for congenital tracheal stenosis, has been recently reported to have good results and quite a number of advantages as compared with conventional tracheoplasties. The aim of this study is to report a new surgical technique modified from the slide tracheoplasty, “the slide cricotracheoplasty” for the congenital cricotracheal stenosis. A girl was born by Cesarean section and the diagnosis of esophageal atresia (Gross type C) and cricotracheal stenosis (30% of total length of trachea) was established. Esophageal atresia was successfully corrected at the 8th day of life. At the 31st day of life, corrective surgery for congenital cricotracheal stenosis, the slide cricotracheoplasty, was performed with success. Slide cricotracheoplasty is almost the same procedure as slide tracheoplasty except for two technical features. First the cricoid cartilage was split on its anterior surface. Second the split cricoid cartilage was fixed to pre vertebral fascia to maintain enough space to accommodate the sliding caudal segment of trachea because of the stiffness of the cricoid cartilage. We believe that the sliding cricotracheoplasty is a new surgical technique for congenital cricotracheal stenosis that has similar results and advantages as the sliding tracheoplasty.

In hepatoblastoma, encouraging cure rates have been achieved with recent advances in chemotherapy and surgical techniques. The aim of this study is to evaluate the role of combined therapeutic modalities and surgical resection in hepatoblastoma. Fifteen cases of hepatoblastoma were treated from January 1993 to August 2000. Six patients had resectable tumors at initial diagnosis. All underwent surgical resection and in four patients postoperative adjuvant chemotherapy was needed. Nine out of 15 patients had unresectbale tumors at initial diagnosis, and preoperative chemotherapy was applied. There was one operative mortality and 14 patients showed good prognosis after surgery. Although various treatment modalities should be combined for the unresectable hepatoblastoma, surgical resection remains the major curative procedure.

Neuroblastoma arises from the embryonic tissue of the adrenergic rest. It is commonly found in children and mostly in nonrenal tissue. We present a case of intrarenal neuroblastoma which was initially thought to be a Wilms' tumor. The patient was a 18 months-old girl treated with radical nephrectomy and adjuvant chemotherapy after operation. The neoplasm within the kidney in children cannot always indicate Wilms' tumor. Neuroblastoma of the adrenal gland or retroperitoneal tissue may often compress or invade the kidney directly or arise from the kidney. Clinical aspects that differentiate between neuroblastoma and Wilms' tumor are discussed with a review of the literatures.

Pseudoaneurysm of splenic artery may arise from a vascular erosion by the inflammatory processes around the splenic artery, particularly in acute pancreatitis and chronic pancreatitis, which may cause rupture of pseudoaneurysm and life threatening hemorrhage. Collective experience with this massive hemorrhage is attended by a high mortality rate even with prompt therapy, and conservative management is associated with an almost 100 per cent of mortality rate. Identification of the bleeding site at laparotomy may be exceedingly difficult, which makes the preoperative detection of bleeding source desirable. Peripancreatic vascular lesions can be identified by angiography, and in selected cases the risk of urgent operation to control massive hemorrhage may be obviated by embolization. The authors have recently experienced a case of ruptured splenic artery pseudoaneurysm combined with a pancreatic pseudocyst in a 6 years old boy. A bolus enhanced CT scan and angiography were essential to confirm these complications of pancreatic pseudocyst. We managed this child successfully with an urgent procedure of transcatheter arterial embolization and another elective surgery of pancreatic pseudocyst.

Femoral hernias are very uncommon in children and very easily misdiagnosed. During a period of three years, three children of femoral hernia were treated by one pediatric surgeon at Severance Hospital. Only one case was diagnosed correctly before surgery; the other two were thought to be either an indirect inguinal hernia or groin mass. Only one patient had curative hernioplasty (McVay hernioplasty) at the first operation; the other two did not have curative hernioplasty at the first operation. Femoral hernia in childhood is a challenging clinical problem because of its rarity and similar clinical presentation as indirect inguinal hernia. The frequency with which an incidental indirect inguinal hernia sac or patent processus vaginalis can be found at surgery can perpetuate a misdiagnosis. The absence of an expected indirect inguinal hernia sac or an apparent recurrence of an indirect inguinal hernia should lead to consideration of possible femoral hernia.

Evaluation of the sacrum in anorectal malformations (ARMs) is important because of the frequent association with ARMs and functional outcome after correction of ARMs. Sacral defects are not easily detected because of immaturity of sacrum in children and overlooking of pediatric surgeons. The authors utilized the sacral ratio in normal children and patients with ARMs. In normal children, the mean true sacral ratio and mean sacrococcygeal ratio were 0.60±0.08 and 0.72±0.13 respectively. Sacral ratio was not correlated with age and did not changed with age in same patient. However, true sacral ratio and sacrococcygeal ratio were significantly lower in patients with high type ARMs than those of normal children (p<0.001). There was no difference between patients with low type ARMs and normal children. These results suggest that abnormal sacrums are more frequently encountered in patients with high type ARMs than in normal children, and that true sacral ratio and sacrococcygeal ratio can be used in the evaluation of the abnormal sacrum.