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.

Hickman catheters are tunneled central venous catheters used for long-term venous access in children with malignancies. The appropriate management for various kinds of catheter related complications has become a major issue. We retrospectively analyzed the clinical, demographic, and surgical characteristics in 154 pediatric hemato-oncology patients who underwent Hickman catheter insertion between January 2005 and December 2009. There were 92 boys and 62 girls. The mean age at surgery was 7.6±5.1 years old. The mean operation time was 67.4±21.3 minutes and C-arm fluoroscopy was used in 47(30.5%). The causes of Hickman catheter removal were termination of use in 82 (57.3%), catheter related bloodstream infection in 44(30.8%), mechanical malfunction in 11(7.7%), and accidents in 6(4.2%). Univariate and multivariate analysis for associated factors with catheter related bloodstream infection showed that there were no statistically significant associated factors with catheter related infection complications. All cases except two showed clinical improvement with catheter removal and relevant antibiotics treatment. The mean catheter maintenance period in patients of catheter removal without complications was 214.9±140.2 days. And, The mean catheter maintenance period in patients of late catheter related bloodstream infection was 198.0±116.0 days. These data suggest that it is important to remove Hickman catheter as soon as possible after the termination of use. When symptoms and signs of complications were noticed, prompt diagnostic approach and management can lead to clinical improvements.

Pancreatic tumors in children are relatively rare, and their prognosis differs from that in adults. The purpose of this study is to examine the clinical characteristics, treatment, and prognosis for children with pancreatic tumors. We retrospectively reviewed the medical records of children under 15 years of age with pancreatic tumors who were treated surgically at Asan Medical Center between January 1992 and November 2009. There were 16 patients, fourteen of whom were pathologically diagnosed with solid pseudopapillary tumor. The other two patients were diagnosed with pancreatoblastoma and acinar cell carcinoma, respectively. Six patients of the 16 patients (38%) were male, and there was a male-to-female ratio of 1:1.6. The initial presentations were upper abdominal pain in eight patients (50%), palpable abdominal mass in three, and vomiting in one. Four patients were diagnosed incidentally. Six patients' tumors were located in the pancreatic head, six in the pancreatic body, and four in the pancreatic tail, respectively. The surgical procedures performed included distal pancreatectomy (n=7, 44%), median segmentectomy (n=3), enucleation (n=3), pancreaticoduodenectomy (n=2), and pylorus-preserving pancreaticoduodenectomy (n=1). Three patients underwent laparoscopic surgery. The median tumor size was 6.5cm (1.8~20 cm). Early surgical complications included pancreatic fistula (n=4), bile leakage (n=1), and delayed gastric emptying (n=1). A late complication in one patient was diabetes. The median follow-up period was five years and four months, and all patients survived without recurrence. While pancreatic tumors in adults have a poor prognosis, pancreatic tumors of childhood are usually curative with complete resection and thus have a favorable prognosis.