Biliary atresia (BA) is an infantile cholestatic disease of progressive obliterative cholangiopathy with varying degrees of damage to both extra and intrahepatic bile ducts due to unknown causes. The diagnostic studies should be done to diagnose or exclude BA without unnecessary delay. Kasai portoenterostomy is the first choice of treatment for bile drainage from microscopic bile ductules present in the portal fibrous mass. The medical management after Kasai portoenterostomy should be done carefully to maintain bile excretion and prevent and treat complications including cholangitis, hepatic fibrosis, portal hypertension and nutritional problem. The reported five years-survival rates after Kasai portoenterostomy range from 30 to 60%. About 20% of all patients undergoing Kasai portoenterostomy during infancy survive into adulthood with their native liver. Even if Kasai portoenterostomy remains as the first line of treatment in BA, liver transplantation serves as a good salvage treatment when portoenterostomy fails or liver function gradually deteriorates after initially successful establishment of bile flow. Overall 5-year survival rate in BA is about 90% in recent series.

Meconium obstruction (MO) in neonates arises from highly viscid meconium and the poor motility of the premature gut. Recently the incidence of the MO in neonates has been increasing, but, the diagnosis and treatment of this disease have not yet been clarified. Between March 2004 and April 2010, 24 neonates were treated for MO at Severance Children's Hospital. Their clinical characteristics and treatment were reviewed retrospectively. Twenty neonates were diagnosed with MO and 4 neonates were diagnosed with Hirschsprung's disease (HD). The mean birth weight and gestational age of the 20 neonates with MO were 1.45±0.90kg and 31.1±4.6 weeks, respectively. Thirteen neonates (65%) diagnosed with MO weighed less than 1.5kg and 10 neonates (50%) weighed less than 1kg. Half of the neonates with MO were treated by non-operative methods and the other half were treated by operative methods. Compared with the group that weighed over 1.5kg, the group that weighed less than 1.5kg were more frequently operated upon (61.5% vs. 28.5%), and contrast enemas were performed later and more frequently. Also the group that weighed less than 1.5kg had a higher mortality rate (15.4% vs. 0%). Three of the four neonates with HD were diagnosed with long-segment aganglionosis. In conclusion, MO occurred in very low birth weight neonates more often and must be differentiated from HD. Also, MO in very low birth weight neonates should be treated with special attention due to more a complicated clinical course.

We analyzed the clinical characteristics and outcome of ileocecal and small bowel intussusceptions (ICI and SBI) in the pediatric patients. From August 2003 to July 2010, 144 children with intussusception were included in this study. We retrospectively reviewed the clinical records and imaging study findings. A total of 86 children with ICI and 58 children with SBI were diagnosed. Children with SBI were older than ICI (36.6±24.6 months vs. 24.2±21.6 months, p=0.002). Typical symptoms such as irritability, abdominal mass, bloody stool were more frequent in ICI than SBI (p<0.05) patients. In the ICI group, intussusceptums were reduced with air reduction (84.5%), surgery (17.4%), and spontaneity (1.2%). All patients in the SBI group were reduced spontaneously. SBI occurred in older age and was reduced spontaneously more frequently than ICI. Conservative management with close observation with follow-up by ultrasonography is recommended for SBI.

Thyroglossal duct cysts (TGDC) are the most common type of congenital developmental anomaly encountered in the anterior midline of the neck in childhood. The aim of the study was to evaluate the clinical characteristics of TGDC and identify any factors that could be related to recurrence after surgery. This study consisted of a retrospective chart review of 45 patients treated at Kyungpook National University Hospital for TGDC between 1990 and 2008. All records were reviewed for age and sex, length of history, presentation, diagnostic methods, sizes and locations of cyst, surgical management, histopathology of the lesion and recurrences. The statistical analysis of risk factors for recurrence was made using the Fisher's exact test with a significance level of p < 0.05. The male to female ratio was 2.2:1 with a male preponderance. The mean age at operation was 5 years and 2 months (4 months – 17 years). The most common presenting symptom was a nontender cervical mass (78%). Most TGDC were found in the midline position. Twenty four were infrahyoid, 17 were hyoid, and 4 were suprahyoid level. Forty one (91%) patients received the Sistrunk operation, and 4(9%) patients received cyst excision. Postoperative a seroma developed in six patients in the early postoperative days. There were a total of 3(6.6%) recurrences, 2 in patients who had excision only and in one patient who had the Sistrunk operation. Univariate analysis for risk factors with recurrence showed that there was no statistical relationship between the presence of preoperative infection and the development of recurrence. The removal of hyoid bone along with TGDC was a statistically significant risk factor for recurrent disease. This study suggests that the Sistrunk operation is the treatment of choice for TGDC in order to reduce recurrence.

Meckel's diverticulum (MD) has various clinical presentations and due to the limitation of imaging studies, pre-operative diagnosis is a challenge in pediatric patients. Recently, laparoscopic exploration has been suggested as a favorable method for the diagnosis and treatment of complicated MD. We investigated the results of laparoscopic-assisted surgery compared with open technique. We retrospectively studied patients who underwent resection of complicated MD at our institute from 1997 to 2010 and compared 11 treated by laparoscopic-assisted diverticulectomy (LD) with 11 treated by open diverticulectomy (OD) for complicated MD. Operation time was not significantly different in the two groups. Hospital stay and time to diet were not significantly different. Two patients were re-admitted due to mechanical ileus in the LD group. None of patients in either group needed re-operation. Considering the possibility of false-positive results with imaging studies and the cosmetic benefit, laparoscopic-assisted surgery is a safe and effective treatment modality to diagnose and treat complicated Meckel's diverticulum.

Use of laparoscopic appendectomy (LA) for perforated appendicitis (PA) in children remains controversial because of the development of postoperative intra-abdominal abscess formation. We developed the irrigation method for the prevention of abscess formation after LA performed for PA in children with severe panperitonitis. We called it ‘the shaking irrigation’. The object of this study was to analyze the efficacy of this irrigation method. All cases of PA with severe panperitonitis in children that underwent LA with massive shaking irrigation and drainage between June 2003 and December 2007 were studied retrospectively. We included only PA with panperitonitis and large amounts of purulent ascites throughout the abdomen as well as an inflamed small bowel with ileus. Thirty-four children were involved in this study. The mean patient age was eight years. The mean amount of irrigation fluid was 8.2L (range: 4-15L). The mean operative time was 89.5 min. The mean length of the hospital stay was 5.1 days. There were no postoperative intra-abdominal abscesses. There was no conversion to open surgery. In conclusion, Use of LA in PA with severe panperitonitis in children is safe and effective. Massive shaking irrigation and abdominal drainage appears to prevent intra-abdominal abscesses after LA for PA with panperitonitis.

Minimally invasive techniques for pediatric inguinal hernia repair have been evolving in recent years. We applied the laparoscopic method to repair pediatric inguinal hernia using the techniques of sac transection and intra-corporeal ligation. Between November 2008 and August 2010, 67 pediatric patients (47 boys and 20 girls) with inguinal hernias were included in this study. Postoperative activities, pain, and complication were checked prospectively at regular follow-up. One patient presented with clinically bilateral hernia, and three patients had metachronous hernias. Thirty-two cases out of 63 patients with unilateral hernias had a patent processus vaginalis on the contralateral side. Mean operation time was 35±11.4 minutes for unilateral hernias and 43±11 minutes for bilateral hernias. There were no intra-operative complications. One patient had a small hematoma on the groin postoperatively, which subsided spontaneously in a week. Recurrence and metachronous hernia were not found at follow up. In summary, laparoscopic inguinal repair in children is safe, easy to perform and has an additional advantage of contralateral exploration. Further studies should include long term follow-up.

Currarino syndrome is a hereditary syndrome characterized by the triad of a sacral bony defect, presacral mass and anorectal malformation. We retrospectively reviewed 13 Currarino syndrome patients who were treated in our center between 1997 and 2010. Demographic data, initial symptoms, initial diagnosis, pathologic diagnosis of presacral mass, associated anomalies and managements were analyzed. There were 8 boys and 5 girls. Four patients were diagnosed as Currarino syndrome immediately after birth with failure of passage of meconium and abdominal distension. Four patients underwent surgery for imperforate anus immediately after birth and were diagnosed as Currarino syndrome later and underwent reoperation. Three patients were diagnosed during work-up and management with of the tentative diagnosis of Hirschsprung's disease. Diagnosis of the remaining two patients was at the age of 26 months and 9 years and anorectal malformation was not associated. Twelve patients showed hemi-sacrum and one patient showed bilateral sacral subtotal agenesis. Two patients without anorectal malformation underwent presacral mass excision, untethering of spinal cord and repair of myelomeningocele. Six out of 8 patients, excluding 3 that expired or were lost to follow up, with anorectal malformation underwent colostomy, presacral mass excision, untethering of spinal cord, repair of myelomeningocele, posterior sagittal anorectoplasty and colostomy repair. One patient underwent only posterior sagittal anorectoplasty after colostomy. One waits the scheduled operation only with Hegar dilatation. Pathologic examation of presacral masses showed myelomeningoceles in 4 patients, lipomyelomeningoceles in 3 patients and dermoid cyst in one patient. Teratoma was combined in 2 patients. Eight patients needed neurosurgical operation for spinal cord problems. Seven patients had urologic anomalies and two of them underwent operation. Currarino syndrome should be considered as a differential diagnosis in pediatric patients with abdominal distension, constipation and anorectal malformation. For proper evaluation and treatment, a multi-disciplinary approach is recommended.

Although laparoscopic surgery for hepatobiliary disease in children is possible, it is technically challenging. In an attempt to overcome these difficulties, the da Vinci Robotic Surgical System(R) was used to facilitate the minimally invasive treatment of choledochal cyst in six children. In early consecutive three cases, we experienced three complications; a case of laparotomy conversion, a case of late stenosis of the hepaticojejunostomy, and a case of leakage from a hepaticojejunostomy. However, in the last three cases the complete resection of the choledochal cyst and Roux-en-Y hepaticojejunostomy were performed using the robotic surgical system without complication.

We think robot-assisted choledochal cyst resection in children appears safe and feasible, and may increase the variety of complex procedures in pediatric surgical fields.

This study was aimed to evaluate associated congenital anomalies in the patients with esophageal atresia with tracheoesophageal fistula (EA/TEF). Forty-two neonates with the diagnosis of EA/TEF treated over a 10 year period in a single institution were included in this study. The demography of EA/TEF was analyzed. Major associated anomalies including vertebral, anal, cardiac, renal, limb, neurologic and chromosome were reviewed and categorized. Males were slightly more dominant than females (1.47:1) and all patients had Gross type C EA/TEF. Only 19% of the patients had solitary EA/TEF without associated anomalies. Cardiac anomalies were the most common associated congenital anomaly in patients with EA/TEF (73.8%). But 47.6% were cured spontaneously or did not affect patients' life. Atrial septal defect (ASD) was the most common cardiac anomaly followed by patent ductus arteriosus (PDA) and ventricular septal defect (VSD). Among gastrointestinal anomalies (23.8%), anorectal malformations were the most frequent, 70% Vertebral and limb abnormalities accounted for 11.9% and urogenital malformations 9.5% of the anomalies in patients with EA/TEF. VACTERL associated anomalies were 23.8% and 4.8% had full VACTERL. Almost 12% of EA/TEF had neurologic anomalies. Patients with EA/TEF require preoperative evaluation including neurologic evaluation to detect anomalies not related to VACTERL. Though associated cardiac anomaly occurred in 73.8% of patients in our study, only 21.42% needed surgical correction. The authors suggesrs further studies with large numbers of patients with EA/TEF.

The onset of hypertrophic pyloric stenosis in the postoperative course of esophageal atresia with tracheoesophageal fistula is rarely reported. The diagnosis could be delayed due to its mimicking symptoms of other postoperative complications including gastroesophageal reflux or anastomotic stricture. We present an infant who had surgery for esophageal atresia with tracheoesophageal fistula. He had never fed since birth. The infant presented with an increased amount of orogastric tube drainage and consistently distended gastric air on simple abdominal X-ray. Abdominal ultrasonography showed hypertrophic thick pyloric muscle. The diagnosis of pyloric stenosis was confirmed d is rarely reported. The diagnosis could be delayed due to its mimicking symptoms of other postoperative complications including gastroesophageal reflux or anastomotic stricture. We present an infant who had surgery for esophageal atresia with tracheoesophageal fistula. He had never fed. The infant presented with uring surgery. After pyloromyotomy, the patient's condition improved.