Among 60 children with teratoma, forty-three (71.7 percent) were girls and 17 (28.3 percent) boys. Primary sites were sacrococcygeal in 30 patients (50 percent), retroperitoneal in 12 (20 percent), ovarian in 11 (18.3 percent), testicular in 3 (5 percent), and one in each of nasopharyngeal, gastric, hepatic and pancreatic (1.6 percent, respectively). Fifty-five (91.7 percent) teratomas were benign and 5 (8.3 percent) malignant. Malignant teratomas were detected only at sacrococcygeal region (16.7 percent). Older than 2 months of age at diagnosis, presence of urinary and colonic obstructive symptoms, multiple masses and elevated serum alpha-fetoprotein were indicators of malignancy in sacrococcygeal region. Tumor size, presence of calcification, and gross appearance (cystic or solid) did not correlate with malignant nature. Thirteen (21.7 percent) cases were associated with other anomalies. For the immature teratoma, the operative resection without adjuvant chemotherapy was enough. Three malignant cases were survived, one with chemotheapy for 3 years and the others without chemotherapy for 5 and 10 years.

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.

We analyzed our experience of orchidopexies performed during last 10 years to evaluate results and to determine the possible approach to the treatment of undescended testes. Between 1988 and 1997, we had treated 420 undescended testes (314 palpable and 106 nonpalpable) in 356 boys. Average patient age at presentation was 4.1 years with 40.2% presenting before the age of 2 years. Of 106 nonpalpable testes, 23 testes were intraabdominal, 32 were preperitoneal and 51 were absent at the surgery. During the period of first 5 years, we had performed the surgery through 31 inguinal and 13 midline transabdominal incisions for 44 patients nonpalpable testes, while during the later 5 years, all 47 patients nonpalpable were treated through inguinal incisions. For the nonpalpable testes, the inguinal approach with or without intraperitoneal extension was successful in defining the location of testes and blind-ending vessels in all patients. Laparoscopy was not helpful in avoiding surgical exploration in all our patients with nonpalpable testes. Of 339 inguinal and midline transabdominal orchidopexies without spermatic vessels ligations, 324 testes were placed in the scrotum, 4 in the upper scrotum and 3 in the inguinal area. Eight were resulted in atrophy. Of 13 Fowler-Stephens orchidopexies, 7 were placed in the scrotum and 6 were resulted in atrophy. Testicular growths were noticed in most patients who underwent orchidopexies and the volume of fixed testes became as large as the contralateral normal testes by the mean duration of 43.3 months postoperatively. In conclusion, orchidopexies were successful in most cases of cryptorchidism in terms of testicular position and growth. However, there were more testicular atrophies in patient in whom spermatic vessels were ligated. In cases of nonpalpable undescended testis, the inguinal approach with or without intraperitoneal extension would be recommended.

Undifferentiated (embryonal) sarcoma is a rare malignancy of the liver in children and young adults. Seven cases of undifferentiated (embryonal) sarcoma of the liver pathologically verified at Seoul National University Children's Hospital between 1986 and 1999 were retrospectively analyzed. There were three girls and four boys, and their mean age at diagnosis was 12.1 years (range 7–13 years). Six patients presented with an abdominal mass or pain, and one with weight loss. Tumor size ranged from 8.0 × 8.0 cm to 15.0 × 15.0 cm. Four tumors were located in the right lobe, two in the left lobe and one in both. One patient died during chemotherapy. Initial complete resection was accomplished in three patients. Two patients underwent complete resection after chemotherapy. Five patients with complete resection survived without evidence of disease for 8, 11, 13, 28, and 84 months. A patient with partial resection and chemotherapy died of sepsis during chemotherapy 19 months after complete surgical resection. Adjuvant chemotherapy and radiotherapy were performed in all patients after complete surgical resection. In conclusion, though undifferntiated (embryonal) sarcoma of the liver is highly malignant, the combination therapy of surgery, chemotherapy and radiotherapy appears to result in a favorable prognosis

Diagnosing Hirschprung's disease is one of the clinical challenges of this disorder. In the stomach and the intestines, Cathepsin D was readily detected in cytoplasm of the rat gastric and in intestinal ganglion cells of the autonomic nervous system. The
objective
s of the present study were to examine cathepsin D expression in ganglion cells of the submucosal and myenteric plexuses of the intestine of children and to determine the utility of immunohistochemical staining of cathepsin D for detection of immature ganglion cells. Paraffin blocks of 35 intestinal segments were reviewed for immunohistochemical staining with polyclonal antibody to cathepsin D and hematoxylineosin stainings from the compatible specimens. There were 9 aganglionic segments and 9 ganglionic segments of neonates with Hirschsprung's disease, 8 intestinal segments with non-Hirschsprung's disease in neonates and 9 intestinal segments with non-Hirschsprung's disease infants over the age of 10 months. All ganglion cells showed intense granular cytoplasmic reactivity for cathepsin D regardless of maturity and all aganglionic segments had no expression for cathepsin D in the submucosal and myenteric plexuses of the intestine. However, histiocytes within the laminar propria and submucosa stained positively for cathepsin D. In conclusion, intestinal ganglion cells in children have reactivity for cathepsin D, threrfore immunohistochemical staining for cathepsin D can be used for identification of ganglion cells in neonates.

Pediatric solid tumors have many similarity among different tumors. These tumors present small round cell types, and cause frequent diagnostic problems in pediatric pathology. An important advance in the investigation of these small round cell tumors has been the identification of consistent chromosomal translocations associated with several types of tumor. Eighteen patients with soft tissue sarcoma were available for review. Seventeen cell lines were also included in this study. The RNA from the specimens were analyzed by reverse transcriptase-polymerase chain reaction (RT-PCR). PAX3-FKHR fusion was present in four of five alveolar rhabdomyosarcoma and PAX7-FKHR fusion was detected in one of five alveolar rhabdomyosarcoma. None of the specimens expressed more than one chimeric transcript. EWS-FLI1 or EWS-ERG fusions were detected in all seven Ewings' sarcoma. No specimens showed EWS-WT1 fusion. These results corresponded well to the histopathologic diagnosis. There were no differences in the histologic appearances of tumors with the more frequent PAX3-FKHR or EWS-FLI1 fusions compared with those containing the variant PAX7-FKHR or EWS-ERG fusions. RT-PCR assay for chimeric transcript is an useful tool for a rapid and
objective
diagnosis of pediatric solid tumors. Through these tools, we can approach genetically to the differential diagnosis of undifferentiated small round tumors.

Pediatric laparoscopic splenectomy has gained increasing acceptance in the surgical management of a variety of splenic disorders, in particular hematologic diseases. We report our experience with 16 patients who underwent this procedure because of hematologic disorders during the past 3 and a half years at the Department of Surgery, St. Mary's Hospital, Catholic University Medical College. The mean age was 10 years (Range 6-16 years) and the mean spleen weight was 210 gm (Range 85-500 gm). The indications for splenectomy were hereditary spherocytosis (6 cases), idiopatic thrombocytopenic purpura (8 cases), autoimmune hemolytic anemia (1 case), and idiopatic splenomegaly (1 case). All splenectomies were performed safely with mean estimate blood loss of 233 ml. Mean operative time and mean postoperative hospital stay was 157 min and 4.5 days. Respectively postoperative pain medication was needed in 3 case, just one injection in immediate postoperative period. Diet was started on posterative second day or third day. In conclusions, Laparoscopic splenectomy in pediatric patients surely is a safe procedure, offering better cosmesis, much less pain, and shorter hospital stay with lower post operative mobidity.

Neuroblastoma derived from neural crest cells of the sympathetic nervous system, is the most common extracranial solid tumor in children with many interesting features. It has the highest rate of spontaneous regression, yet has one of the poorest outcome results. Recent updates have shown much increased outcomes in multitreatment approaches and early diagnosis with screening. Authors have studied 42 patients who have been diagnosed and treated in the last decade in the Severance and Yongdong Severance Hospital respectively. They were followed up till January 1998 and was analyzed in terms of age, sexual difference, admission period, staging, diagnostic studies, clinical symptoms and physical examinations, operative time, treatment modalities, survival rate. Twenty eight patients underwent operative procedures, 16 patients had postoperative chemotherapy, and 19 patients had preoperative chemotherapy. Sexual difference was 1.33:1 in favor of males, and 43% of patients were under the age of one year. The patients were initially diagnosed symptomatically, and the abdominal mass was the most common clinical symptom. Eighty six percent of the patients were of advanced stages (>Stage III of INSS), and the 2 year survival rate showed 59.2% and the 5 year survival rate was 29.6%.

The pyloric length, diameter and muscle thickness were measured by ultrasonograms of 15 infants with infantile hypertrophic pyloric stenosis (IHPS) and were compared with the infants who came in for the routine vaccination randomly. This study analyzed the changes in pylorus for those who received pyloromyotomy using the ultrasonogram at 4 weeks and 8 weeks postoperatively, such as the time needed to become normalized, and compare with the size of the hypertrophic pylorus before the pyloromyotomy. According to Carver⁵, the pyloric muscle volume (PMV) and pyloric muscle index (PMI) were calculated in each case. The pyloric muscle volume, PMI and the thickness of pyloric muscle proved to be a more reliable guide to diagnose IHPS than length and diameter of pylorus. The pyloric muscle after pyloromyotomy was measured by ultrasound at 4 weeks and 8 weeks postoperatively. The pyloric muscle length, diameter, thickness and pyloric muscle volume were not normalized at 4 and 8 weeks postoperatively. However, pyloric muscle index was normalized at 4 weeks postoperatively which was probably due to rapid weight gain after pyloromyotomy.

Condyloma acuminata, an uncommon disease in the pediatric patients, is being reported with increasing frequency in both adults and children. During the last 10 years, we had the opportunity to treat 11 children with condyloma acuminata in anogenital area. Patient age at presentation varied from 9 months to 11 years, and boys were outnumbered girls by a ratio of 2 : 1. The lesions were found in the perianal area in 9 patients, while the remaining had lesions localized to the genitalia. No history of sexual contact or abuse could be elicited. Although the exact mode of transmission of human papillomavirus (HPV) couldn't be defined, maternal-infant transmission at birth and close nonsexual family contact were the main possible causes of HPV infection in our patients. Our experience suggest that fulguration of the warts and concomitant scissors excision for the larger ones are the satisfactory method of treatment in children. However, based on our data we feel that condyloma acuminata has a high recurrence rate despite treatment. Viral typing and careful epidemiological investigation on a larger series of patients may further clarify the causal factors and effective modalities of treatment in childhood anogenital condyloma acuminata.

A survey on intestinal atresia was made among 34 members of Korean Association of Pediatric Surgeons about the patients who were treated from the January 1, 1994 to December 31, 1996. The response rate was 82.4%. Two hundred and fifteen patients were analyzed. The lesion occurred in 73 cases at duodenum, in 72 cases at jejunum, in 71 cases at ileum and 2 cases at cecum and sigmoid colon each. There were 2 cases of combined anomalies (DA + JA + IA and DA + JA). Male to female ratio was 1:1 in duodenal atresia. and 1.8:1 in jejunoileal atresia. Seventy-four cases (34.3%) were premature babies (DA 35.2%, JA:48.6%, IA:19.2%), and 62 cases (28.7%) had low birth weight (DA:39.4%, JA 33.0%, IA:13.7%). Antenatal diagnosis was made in 92 cases (43.6%). However 22 cases (23.9%) of them were transferred to pediatric surgeon after delivery. Maternal polyhydramnios was observed in 63 cases (28.9%). Seventy-five cases (34.4%) were taken only simple abdominal film for diagnostic studies. The associated malformations were observed in 54 cases (24.8%) of intestinal aresia and more frequently observed in duodenal atresia (35 cases, 47.9%). Meconium peritonitis due to intrauterine bowel perforation was more frequently associated with ileal atresia compared to duodenum and jejunum. The overall mortality rate was 30%. (Abbreviations: DA;duodenal atersia, JA;jejunal atresia, IA;ileal atrsia, PT;p-value in total, PDJ,DI,JI;p-value between two groups among duodenal, jejunal and ileal groups)