Lymphangiomas or cystic hygromas are quite commonly seen in children. During a 22-year period, from January 1980 to December 2001, 117 patients with lymphangioma were treated and followed in the Department of Pediatric Surgery at Hanyang University Hospital. The male-to-female ratio was 1.9:1 (77:40) with a male preponderance. As for the age incidence at time of diagnosis, 10 (8.5 %) patients were noted under 1 month of age, 37 (31.6 %) were between 1 month and 1 year of age, 12 (10.3 %) between 1 and 2 years of age, so 59 (50.4 %) were under 2 years of age. Sixty one (52.1 %) lymphangioma-cases were located in the neck, including one case at the nape. Axilla was the second in frequency and the rest were scattered at various sites. The intrascrotal lymphangioma is very rare but we have experienced one case of primary intrascrotal lymphangioma. Eighteen (15.4 %) cases were located in the intraabdominal area, 10 in the mesentery, 2 in the greater omentum and 6 in the retroperitoneum. The chief complaints of intraabdominal lymphangioma were abdominal pain, intestinal obstruction, inguinal hernia, palpable mass, and/or abdominal distension. Among 77 histologically proven cases, 14 cases were cavernous lymphangiomas and the rest were cystic lymphangiomas. Bleeding in the lymphangioma was noted in 20 (17.1 %) cases of all. As for the treatment, a complete excision was performed in 77 (65.8 %) patients and AgNO₃ sclerotherapy after incomplete excision was performed in 23 (19.7 %). Picibanil (OK-432) sclerotherapy was performed in 17 (14.5 %) patients. Recurrence rate was 7.7 % and mortality occurred in one case who had a large neck lymphangioma extending into the mediastinum.

The antiangiogenic effects of novel agent KJ3, Betulinic acid, and Fumagillin on the neovascularization were studied by examining ultrastructural alterations in the vasculature of synthetic gelform and mouse neuroblastoma C1300. Small pieces of gelform with 0.4 % agar were introduced subcutaneously (s.c.) in 7 week old male CH3/HeJ mice. After the LD₅₀s were determined by FACS analysis, a third of LD₅₀ of three drugs were injected either locally or intraperitoneally every other day for 14 days. A/J mice were inoculated s.c. with the C1300 neuroblastoma cell line, then either saline or three drugs were injected in the same manner. The antiangiogenic effects of three drugs were studied by measuring the histologic changes in tumors, and immunostaining for CD34, VIII/vWF, CD105, and thymidine phosphorylase. In the drug treated groups, the number of vessels in gelform experiments and C1300 neuroblastoma experiments were lower than the corresponding values in the control. The histologic findings were significantly different in drug treated groups on day 7, but these were not significant on day 14. These results imply that antiangiogenic agents were effective when the tumor burden is minimal.

Branchial anomaly is a frequently occurring congenital abnormality in childhood. It is important for the pediatric surgeon alike to be familiar with the embryology and differentiation of head and neck structure to accurately diagnose and treat these lesions. Eighty-five patients with branchial anomaly treated at Hanyang University Hospital between 1980 and 2001 were reviewed to determine relative frequency, clinical classification and appropriate treatment. The male to female ratio of branchial anomaly was 1.2:1. The most commonly presenting age was before 1 year (32 %) and the age group between 1 and 3 year (22 %) followed it. According to the classification of branchial anomalies, 73 of 85 cases were second branchial anomaly, 9 had the first type and 3 did fourth type. One patient showed combined anomalies of the first and the second type. Infection sign were seen in 70 % of patients at the time of the first visit to our hospital and also patients' symptoms were frequently related with the infection. Forty-one cases (48 %) were fistula, 21 (25 %) were cysts, 21 (25 %) were sinuses, and two were only cartilage remnants. The most common type of the branchial anomalies is the second branchial fistula and the most common symptoms of the anomalies are related with infection. Initial proper diagnosis and anatomical classification of the anomalies are very important in managing the lesions. The efforts to find the exact anatomical location of the fistula or sinus tract are necessary because total excision of the lesions including those tracts is the only way to prevent recurrence.

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.

The aim of this study was to evaluate the posterior sagittal anorectoplasty (PSARP) as a re-do operation in patients who failed initial repair of anorectal malformation. Nine patients (4 boys and 5 girls) who had previous failed surgery for anorectal malformation underwent secondary operations through posterior sagittal approach. The main reasons of surgery were constipation (n=3) and persistent anatomical derangement in spite of previous correction surgery (n=6). In addition to constipation, the former group (n=3) had various anatomical defects, and the latter group (n=6), of course, had constipation in some degrees. Patients ranged in age from 2 to 19 years (median 3 years) with only one over the age of 6 years. The primary procedures included PSARP (n=8) and anoplasty (n=1). The rectum was mobilized from surrounding structures through posterior sagittal approach and anatomical defects were corrected. The rectum underwent reconstruction, which involved relocation of the rectum and anus within the limits of the intact muscle complex. Patients underwent follow-up for periods ranging from 6 to 77 months (mean 37 months) after surgery. Anatomical corrections of all the defects were successfully fulfilled in 9 patients. All the patients were satisfied with the functional results after redo-PSARP compared with the preoperative defecatory function. This study suggests that (1) some of the patients with troublesome constipation may have anatomical defects, prominent or hidden, (2) surgeons should suspect the possibility of anatomical defect as the cause of incontinence and (3) preoperative thorough investigation to reveal the anatomical defects should be included in estimating patients with severe incontinence after previous surgery and planning the correction for failed previous surgery as well.

Inguinal hernia is a major surgical disease in pediatric surgery, occurring in 3.5% to 5% of all mature newborns and 9% to 11% of all premature babies. The
objective
of this study is to analyze the predisposing factors in association with recurrences of inguinal hernias in infants and children. In the period from January 1995 to September 2001, 1,575 infants and children who had primary inguinal hernias and recurrent inguinal hernias operated on at the Department of Pediatric Surgery at Seoul National University Hospital were evaluated retrospectively. We evaluated the data by medical records and by telephone interview. The sex, age, location of hernia, comorbidity, prematurity, incarceration, interval to operation after incarceration, postoperative complications were analyzed as predisposing factors in associated with hernia recurrence. Operative findings of recurrent inguinal hernia were reviewed. The data were statistically analyzed with Pearson Chi-Square test and Fisher-exact test. A total of eighteen (1.14%) out of 1,575 patients underwent an operation due to recurrent inguinal hernia. In 5 (27.8%) out of 18 recurred patients, institution of the primary herniorrhaphy was our hospital and in the other 13 (72.2%) was outside hospital. No impact on the development of recurrences was seen for sex, age, interval to operation after incarceration, and postoperative complications. The significant predisposing factors of recurrent inguinal hernias were left inguinal hernias (p=0.002), comorbidity (p=0.002), prematurity (p=0.006), incarceration (p=0.017) and technical error of first herniorrhaphy. We expect that knowledge for predisposing factors of recurrent inguinal hernias and experienced skill of pediatric surgeons will decrease recurrence rate in primary inguinal hernia.

To evaluate the clinical characteristics and results of treatment of fistula-in-ano and perianal abscess in childhood, we analyzed 95 cases of fistula-in-ano and/or perianal abscess seen in childhood, between January 1995 and June 2001 at the Department of General Surgery of Ewha Womans University Mokdong Hospital. Perianal abscess was in 25 patients, anal fistula in 62 and combined disease (perianal fistula and abscess) in 8. Male predominance was noted (95 %). Median age was 8 months and 78 % of cases were presented under the age of 1 year. Median duration of symptoms was 60 days. Twenty four abscesses (77 %) and 18 lesions (72 %) of combined disease (n=25) were located on both lateral sides of the anus, and fistulas located on both lateral sides were 33 (53 %). Multiplicity of the lesion was noted in 25 % of cases. Sixteen percent of abscess, 81 % of fistula and 88 % of combination group have had previous perianal abscesses. The perianal abscesses were treated with incision and curreTage and fistulas were treated with fistulotomy or fistulectomy. There were no recurrent diseases and no complications after surgical treatment. Although the progresses of the perianal abscess and fistula in ano in childhood may be self-limitied, surgical management was safe and curable.

Mesenteric and omental cysts are rare intra-abdominal lesions in childhood, and may present various clinical features such as an asymptomatic mass or an acute abdomen. Therefore, these entities are frequently misdiagnosed preoperatively or are found only incidentally at operation for other conditions. We analyzed our experiences of 19 cases in a 19 year period from 1981 to 1999, at College of Medicine, Catholic University of Korea. There were 12 boys and 7 girls with a mean age of 4.8 years (range, 3 days to 15 years). Common presenting symptoms were abdominal pain (47%), abdominal distension (31%), abdominal mass (24%), vomiting (15%) and fever (10%). Ultrasonography was the most preferred method of diagnosis. Other diagnostic modalities include CT, MRI, and abdominal ascites tapping in selected patients. Location of the mesenteric cysts was small bowel mesentery in nine, the right mesocolon and retroperitoneum in one, the left mesocolon in one, and the jejunum, sigmoid-colon mesentery in one. Most of the patients underwent cyst excision, but six patients required concomitant bowel resection for complete removal of the lesions, and two patients underwent unroofing and simple aspiration respectively. There was one mortality case due to sepsis.

A survey on biliary atresia was made among 26 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and a case registration form for each patient during the twentyone-year period of 1980-2000. Three hundred and eighty patients were registered from 18 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1 : 1.3. The age of patients on diagnosis with biliary atresia was on average 65.4 ±36.2 days old. The national distribution was 32.8% in Seoul, 25.3% in Gyoungki-Do, 21.6% in Gyoungsang-Do, 9.27% in Choongchung-Do, etc. in order. The most common clinical presentation was jaundice (98.4%) and change of stool color (86.2%) was second. Two hundred eighty (74.7%) of 375 patients were operated by 80 days of age. Three hundred thirty six (91.9%) of 366 patients were operated on by the original Kasai procedure, and 305 (84.3%) of 362 patients were observed by bile-drainage postoperatively. The overall postoperative complication rate was 18.5% and the overall postoperative mortality rate was 6.8%. The associated anomalies were observed in 72 cases (22.5%). One hundred ninty five (64.7%) of 302 patients have been alive in follow-up and 49 (25.1%) have survived over 5 years without problem after operation. Ascending cholangitis, varices and ascites affected survival significantly, and the important long-term prognostic factor was the occurrence of complications.

When jaundice persists for more than 14 days postnatally, the early diagnosis of surgical jaundice is important for the prognosis in extrahepatic biliary atresia after draining procedure. The role of diagnostic laparoscopy to differenctiate medical causes of jaundice from biliary atresia is evaluated in this report. Four patients with prolonged jaundice have been included in this study. When the gallbladder was not visualized we proceeded to laparotomy. In patients with enlarged gallbladder visualized at laparoscopy, laparoscopic guided cholangiogram was performed, and laparoscopic liver biopsy was done for those who had a patent biliary tree. Two patients had small atretic gallbladder and underwent a Kasai hepato-portoenterostomy. One patients showed a patent gallbladder and common bile duct with atresia of the common hepatic and intrahepatic ducts, and they underwent a Kasai hepatic-portoenterostomy. One patient showed an enlarged gallbladder and laparoscopic-guided cholangiogram were normal. Laparoscopic liver biopsy was performed. There were no complications. Laparoscopy wth laparoscopic-guided cholangiogram may be a valuable method in accurate and earlier diagnosis in an infant with prolonged jaundice.

This is a case of tracheomalacia associated with esophageal atresia. An 11-month-old- male boy presented with a life-threatening apneic spell after correction of esophageal atresia (Gross type C). After complete exclusion of the other possible causes of the apneic spell, the presumptive diagnosis of tracheomalacia was made with fluoroscopy and 3-dimensional chest CT. The final diagnosis was made with rigid bronchoscopy under spontaneous respiration. The aortopexy was performed with intraoperative bronchoscopic examination. The postoperative period was unremarkably uneventful. The patient was discharged 9 days after the aortopexy and has remained well to date (5 months after the aortopexy).