Branchial cleft anomalies are the second most common head and neck congenital lesions seen in children. Amongst the branchial cleft malformations, second cleft lesions account for 95 % of the branchial anomalies. This article analyzes all the cases of second branchial cleft anomalies operated on at Seoul National University Hospital from September 1995 to February 2011. We analyzed sex, age, symptom and sign, accompanied anomaly, diagnosis, treatment, pathologic report and outcome via retrospective review of medical records. In this series, we had 61 patients (27 female and 34 male). The mean age at the time of operation was 38 months. 31 lesions were on the right, 20 were on the left and 10 were bilateral. The most frequent chief complaints at presentation were non-tender mass and cervical opening without any discharge. According to anatomic type, 29 patients had branchial cleft sinuses, 14 had cysts, 14 had fistulas and 4 had skin tags. Complete excision was attempted if possible and antibiotics challenged when infection was suspected. Complete excision was achieved in 96.7 % of cases. Incision and drainage was done in 2 cases due to severe inflammation, and both recurred. Postoperative complications included wound infection in 2 cases. Microscopic examonation revealed squamous epithelium in 90.2 % and squamous metaplasia in one case in the branchial cleft cyst wall. In summary, second branchial anomaly is found more frequently on right side of neck. Fistulas are diagnosed earlier than cystic forms. Most cases could be diagnosed by physical examination. The definitive treatment is complete excision and sufficient antibiotics coverage for cases with inflammation. After drainage of infected lesions, follow up excision after 1 year might be beneficial for preventing recurrence.

A vitelline duct (VD) anomaly is a relatively common congenital abnormality of the umbilical area. The anomalies include patent vitelline duct (PVD), cyst, fistula or sinus. The incidence is approximately 2% of the populations, but development of symptoms is rare. Recently, we experienced two cases; PVD accompanied by a smallomphalocele and intestinal volvulus due to mesenteric band between Meckel's diverticulum and the mesentery. Thereafter,we evaluated the data of vitelline duct anomalies for 27 years. From 1980 to 2006, 18 cases of VD anomalies were reviewed based on the hospital records retrospectively. There were 15 boys and 3 girls and age ranged from 2 days to 15 years. Among the 18 cases, 15 cases were symptomatic and consisted of Meckel's diverticulum (10 cases), PVD (4 cases) and umbilical polyp (1 case). Three asymptomatic cases of Meckel's diverticulum were found incidentally were and were observed without resection. Ten cases of Meckel's diverticulum were presented with intestinal bleedings (4 cases), intestinal obstructions (5 cases) and perforation (1 case). Wedge resections and segmental resections of ileum were performed in 8 patients and 2 patients, respectively. Postoperative complications were adhesive ileus (1 case) and wound seroma (1 case). Small omphaloceles were accompanied in two of 4 PVD patients. There was 1 small omphalocele case which was accompanied by a prolapse of ileum. In summary, VD anomalies were more common in male and more than half of them were found in patients less than 1 year of age. PVD was diagnosed most frequently in neonates. Meckel's diverticulum presented with intestinal obstruction more frequently than bleeding.

VATER association is defined as a combination of 3 or more anomalies-vertebra(V), imperforate anus (A), esophageal atresia with or without tracheoesophageal fistula (TE), renal and radial anomaly(R). We reviewed our experiences in one center to determine etiology, prevalence, clinical manifestation, other associated anomaly and prognosis. Two hundred and twenty-three cases that underwent operations for imperforate anus or esophageal atresia were analyzed retrospectively through medical records at Department of Pediatric Surgery, Asan Medical Center from June, 1989 to July, 2005. The total number of neonates who had been admitted during period of study were 46,773 and VATER association was 9 (0.019 %, 1.92 persons per 10,000 neonates). Median gestational age and birth weight were 37⁺⁴wk (35⁺¹ – 41⁺⁴) and 2,594 g (1,671–3,660), respectively and median age of mother was 32 years (23–38). There was no family history. Three patients were twins but their counterparts had no anomalies. Patients who have 3 anomalies were 6, 4 anomalies in two and 5 anomalies in one patient. Vertebra anomalies were detected in 7(77.7 %), imperforate anus in 8(88.9 %), esophageal atresia in 5 patients (55.6 %), renal anomaly in 6(66.7 %), and radial anomaly in 5(55.6 %), respectively. Four patients are alive, 2 patients were lost during follow up period. Three patients died due to neonatal sepsis, respiratory dysfunction and cardiac failure. VATER association did not appear to be a definite risk factor, but merely a randomized combination of 5 anomalies. The prognosis was dependent on the other associated anomalies, appropriateness of management and operation. Careful follow-up and aggressive treatmentare required for improving survival and quality of life.

No abstract available.

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.

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.

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.

Seventy neonates with congenital intestinal atresia and stenosis who were treated at pediatric surgical service, Hanyang University Hospital from September 1979 to December 1996 were analyzed retrospectively. The lesion occurred in 27 cases at the duodenum, in 26 cases at the jejunum, in 13 cases at the ileum and in 2 cases at the pylorus and colon each. There were 10 multiple atresias and 7 apple-peel anomaly cases. The atresia predominated over the stenosis by the ratio of 4 : 1. Male to female ratio was 1.3 : 1. The average gestational age was 38 weeks, and the average birth weight was 2,754 grams. Though 22.9 % were borne prematurely and 34.3 % had low birth weight, 92.3 % of them had a weight appropriate for gestational age. Polyhydramnios(40 %) was more frequently observed in duodenal and jejunal atresia while microcolon in ileal atresia(58.3 %). Weight loss and electrolyte imbalance occurred more frequently in the duodenal stenosis cases because of delayed diagnosis. Twenty(55.6 %) of 37 jejunoileal atresia cases had evidence of intrauterine vascular accident: 4 intrauterine intussusception, 3 intrauterine volvulus and 3 strangulated intestine in gastroschisis, and 10 cases of intrauterine peritonitis. There were one or more associated anomalies in 45 patients(64.3 %). Preoperatively proximal loop volvulus developed in 3 cases and proximal loop perforation in 5 cases and one case each of distal loop perforation, duodenal perforation and midgllt volvulus occurred in the jejunoileal atresia. Overall mortality rate was 20 %.

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.

The survey on branchial anomalies was conducted by Korean Association of Pediatric Surgeons. A total of 173 cases were reported, which were managed by 36 members and cooperators during the three years from January 1, 1993 through December 31, 1995. The following results were obtained by retrospective analysis of the 173 cases of branchial anomalies. The presenting symptoms were cervical mass in 101 cases, pit with or without discharge in 71, cervical abscess in 47 and respiratory difficulty in 3. The average age of the patients with cervical abscess was 52 months. Seventy(79%) of 89 patients with branchial anomalies and a cystic mass had their first clinical manifestations by 1 year of age, while 40(51%) of 78 patients with only a branchial cyst had their first clinical manifestation in first year of life. Radiologic studies were carried out in 77 patients(43%). The preferred diagnostic modalities were ultrasonography(47 patients), simple neck radiogram(l9) and CT scan(17). Preoperative diagnosis was correctly made in 156(91%) of 173 patients. Seventeen patients were incorrectly diagnosed as thyroglossal duct cyst in 5 patients, cystic hygroma in 4, dermoid cyst in 3, and lymphadenopathy in 3. There were no remarkable difference in sex and laterality of presentation but bilateral lesions were found in 9(5%) patients and unusual locations of the anomalies were the manubrium, left subclavicular area, median cervial area, preauricular and parotid area. There were 78(45%) patients with cyst, 52(30%) patients with sinus, 35(20%) patients with fistula and 8(5%) patient with skin tag. Embryological classification was possible in only 64(37%) patients. The 2nd branchial anomaly was present in 50 (78%), the 1st branchial anomaly in 10(18%), and the 3rd or 4th branchial anomaly in 4(6%). Histopathological study of the lining epithelium(N=134) is recorded that 45% were lined with squamous epithelium, 17% with respiratory epithelium, 6% with. squamous and respiratory epithelium, 14% with inflammatory change. Lymphoid tissue was common(62%) in the wall of the lesions. Twelve(7%) of 158 patients had postoperative complications including wound complication, recurrence and facial nerve palsy.

Congenital duplication of the gallbladder, including true duplication and septated gallbladder, is an uncommon but potentially complicated malformation. It presents biliary colic associated with acute or chronic cholecystitis. It can be diagnosed preoperatively by various imaging tools such as real-time ultrasonography, biliary scintigraphy, oral cholecystography, or endoscopic retrograde cholangiography. It can be safely managed by either laparoscopic or open procedures. This report describes a 6-year-old girl with true duplication of gallbladder diagnosed preoperatively by real-time ultrasonography and treated by open cholecystectomy.