Congenital anomalies in the head and neck region such as preauricular sinus and skin tag, thyroglossal duct cyst, branchial anomaly, cystic hygroma and dermoid cyst are common in pediatric population. It is important for pediatricians and pediatric surgeons to be familiar with the embryology and the anatomical characterics of these lesions in order to diagnose and treat properly. Three hundred nineteen patients with congenital head and neck anomalies treated at Hanyang University Hospital between 1980 and 1999 were reviewed to determine the relative frequency of the anomalies and to analyze the method of management. Eight-four patients(25.1%) of 335 lesions had preauricular sinus and skin tag, 81 patients(24.2%) had thyroglossal duct cyst, 81 patients(24.2%) had branchial anomaly, 58 patients(17.3%) had cystic hygroma, 31 patients(9.2%) had dermoid cyst. The male-to-female ratio was 1.4:1. The thyroglossal duct cyst was most commonly present at 3-5years, however branchial anomaly was commonly diagnosed in children younger than 1 year. Preauricular sinus shoeed familial tendency in three patients and bilaterality is 33.8%. Most head and neck anomalies in children had clinical and anatomical characterics. A careful history and physical examination were very useful for diagnosis and proper management. The initial surgery should be done by experienced pediatric surgeonsl since the recurrence rate after incomplete surgical excision could be high.

Congenital diaphragmatic hernia (CDH) in the past was considered a surgical emergency requiring immediate operation. Several groups now advocate preoperative stabilization and delayed surgery. The treatment strategy for CDH in this institution is delayed surgery after preoperative stabilization. The aim of this study was to evaluate the results of delayed surgery. A retrospective review of 16 neonates with CDH was performed. Surfactant, conventional mechanical ventilation, high frequency oscillation, and nitric oxide were utilized for preoperative stabilization as necessary. The difference in outcome between two groups differentiated by the duration of the preoperative stabilization periods with mechanical ventilation (≤ 8 hours and >8 hours) was determined. Chi-square test was used to analyze the data. There were 7 right-sided hernias and 9 left. The average duration of stabilization was 32.4 hours. Hepatic herniation through the defect was found in 6 cases and all died. The most common postoperative complication was pneumothorax. The mortality rate of the right side hernia was higher than the left (85.7% vs. 33.3%, p=0.036). Mortality rate of the group (N=8) whose preoperative stabilization period was 8 hours or less was better than that (N=6) whose preoperative stabilization period was more than 8 hours (25.0% vs. 83.3%, p=0.031). The overall mortality rate was 56.3%. The better prognosis was noticed in left side hernia, no liver herniation, or shorter preoperative stabilization period.

Gastrointestinal duplications are rare congenital malformation that may require surgical intervention in the neonate, infant, and occasionally the older child. Symptoms produced by duplications vary according to their location, size, type and histology. We report the clinical characteristics and the surgical results of 9 cases of the gastrointestinal duplications treated at at Asan Medical Center between 1989 and 2000. Five patients were boys and four were girls; age of patients ranged from 5 days to 10 years. Eight duplications were cystic and one was tubular. One involved the stomach; five were in the ileum, and two in the cecum. The most common presentation was intestinal obstruction. There was associated anomaly in one patient, pulmonary sequestration and double ureter. Ectopic gastric mucosa was found in two. All patients underwent surgical resection. There was no perioperative mortality or morbidity. Although gastrointestinal duplication is a rare entity, consideration of associated anomalies and being familiar with the anatomy and clinical features are required for adequate management. In cystic form, complete excision is recommended but planned surgery is required for long segment tubular lesion.

Mesenchymal hamartoma of the liver is a rare benign tumor, usually presenting in early childhood. Five children with mesenchymal hamartoma of the liver pathologically verified at Seoul National University Children's Hospital between 1978 and 2000 were analyzed retrospectively. There were two girls and three boys, and their mean age at the operation was 16.0months (range, 4 - 32 months). Three patients presented with abdominal distension. A patient was detected incidentally, and another was detected by prenatal ultrasongraphic examination. Tumor size ranged from 10x8.5cm to 34x29cm. Three tumors were located in the right lobe and two in the left lobe. Four cases underwent complete surgical resection, and the other one underwent incomplete surgical resection and marsupialization. Recurrence or malignant transformation was not noted. Five patients survived without evidence of disease for 35, 36, 38, 142 and 228 months. In conclusion, although mesenchymal hamartoma of the liver is benign lesion, it may be confused, and mixed with embryonal sarcoma. A recent report showed recurrence or malignant transformation after partial excision of the tumor. Therefore, complete excision of the tumor with surrounding normal liver tissue is recommended.

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.

The major complications of Meckel's diverticulum(MD) are bleeding, intestinal obstruction, infection, umbilical fistula and perforation. Although the relative incidences vary between authors, bleeding is the most common complication in children. The aim of our study is to show the symptomatic guideline for the diagnosis of the bleeding MD. Eight cases with bleeding MD which were operated upon at the department of Pediatric Surgery, Yeungnam University Hospital from April 1985 to April 2001 were reviewed. Half of the patients were under 2 years of age and all patients were male. All patients were preoperatively diagnosed by previous history of intestinal bleeding (melena, hematochezia) and 99mTc pertechnetate MD scan. Segmental resection and end-to-end anastomosis was performed in 6 patients and diverticulectomy in 2 patients. Heterotropic gastric mucosa was found in 6 patients. Postoperative complication was not observed in any cases. In conclusion, in any male children with obscure intestinal bleeding, especially less than 2 years of age, bleeding MD must be suspected. It seems to us that 99mTc pertechnetate MD scan is a useful tool to diagnose bleeding MD.

To understand the current status of neonatal surgery in Korea, a survey was made among the 36 members of the Korean Association of Pediatric Surgeons. The response rate was 75% (26 surgeons in 20 hospitals). Five hundred fifty three neonatal surgical patients treated in 1999 were analyzed. Regional numbers of patients were closely related to the regional population in most areas. Ano-rectal malformations (17%), pyloric stenosis (16%), Hirschsprung's disease (13%), atresia/stenosis of the gut (11%), esophageal atresia (8%) were the most common anomalies treated. The majority of operations were done within the first week of life. Seventy one per cent of cases were major life threatening or so-called neonatal index cases. Over-all mortality was 8 per cent. Higher mortality was observed in patients with diaphragmatic hernia (26%), gastro-intestinal perforation (18%), NEC (18%), and esophageal atresia(14%). Higher mortality was observed in patients with extremely low birth weight (33%) and low birth weight (18%). Associated anomalies were observed in 20%. Prenatal ultrasound was performed in 36 per cent with sensitivity of 20%. Result of this study was compared to the previous report (1994) and that of Japan (1998).

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.

The authors, over the last 6 months, have treated 2 patients with perforated typhlitis complicating acute lymphocytic leukemia (ALL) with good outcome. The first patient was a 13-year-old male who developed intermittent high fever, abdominal pain, abdominal distention and diarrhea during the course of maintenance chemotherapy. The peripheral leukocyte ranged from 230-470/mm³. Serial ultra sonograms and CT scans demonstrated irregular thickening of the cecal and ascending colonic walls and subsequent ragged perforation of the posterior wall of the cecum. He survived after treatment by right hemicolectomy and aggressive supportive measures. The patient case was a 3 year-old female who developed intermittent high fever , right lower abdominal pain, a mass, and watery diarrhea during the course of maintenance chemotherapy. Serial ultra sonograms and CT scans demonstrated irregular thickening of the cecal wall (6-15mm in thickness) and subsequent small perforation of the posterior wall of the cecum with thick-walled localized abscess. She has recovered completely after aggressive medical management. We learned two lessons from our experience treating these patients: 1) early diagnosis provided by a high index of suspicion and the use of ultra sonogram or CT scan is essential. And 2) although perforation is one of the surgical indications for the treatment of typhlitis, it is possible to manage the perforation nonoperatively in selected cases with localized abscess.

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.

Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years. The vast majority are in favorable stages of the disease (stage I, II, IV-S). The authors experienced one case of stage IV-S neuroblastoma of the adrenal gland with liver metastasis, which regressed spontaneously after removal by adrenalectomy. This patient was noticed to have an abdominal mass at prenatal ultrasonography performed at 36weeks of gestation. This tumor was a neuroblastoma of the left adrenal gland with multiple liver metastases. Left adrenalectomy and liver biopsy were performed at 3 months of age. Thirty-eight months after surgery, an MRI demonstrated that the hepatic metastatic lesions had completely regressed without chemotherapy or radiation.