It has been widely accepted that complete surgical resection of hepatoblastoma is essential for long-term survival. But unfortunately less that 50% of hepatic tumors in children can be totally removed at the time of diagnosis. This report is to present the experience of successful resection of hepatoblastoma after concurrent radiotherapy with transarterial chemoinfusion in a child. We believe this modality of treatment enables complete resection of unresectable hepatoblastoma, which is resistant to the systemic chemotherapy.
Child ; Diagnosis ; Drug Therapy ; Hepatoblastoma* ; Humans ; Radiotherapy*

PURPOSE: The megacolon after repairing an anorectal malformation is not a rare complication, and there is much controversiy on the causes, the treatment of choice and the results after a longterm follow-up. We present 5 cases of a megacolon after the repair of an anorectal malformation, which were controlled with either a surgical resection or conservative treatment. METHODS: Five patients with a megacolon after the repair of an anorectal malformation were studied. A retrospective chart review was done and fecal continence was evaluated with an individual interview. RESULTS: All five patients initially underwent conservative treatment with laxatives and/or enemas. One Patient responded well to conservative treatment and the diameter of the bowel reduced to normal size. Another patient responded to conservative treatment after correcting the location of the anus. Three patients needed a surgical resection and one of those needed a further procedure to correct the anal location. After the surgical resection of the megacolon and/or correction of the anus (one out of the three patients), they soon reported an almost normal bowel habit. CONCLUSION: The first step in treating a megacolon after repairing an anorectal malformation was conservative treatment. However patients without an adequate response to conservative treatment are best managed with a surgical resection. The cause of the megacolon is now under investigation and the lack of adequate management after repair is one of the subjects.
Anal Canal ; Enema ; Follow-Up Studies ; Humans ; Laxatives ; Megacolon* ; Retrospective Studies

Pseudoaneurysm of splenic artery may arise from a vascular erosion by the inflammatory processes around the splenic artery, particularly in acute pancreatitis and chronic pancreatitis, which may cause rupture of pseudoaneurysm and life threatening hemorrhage. Collective experience with this massive hemorrhage is attended by a high mortality rate even with prompt therapy, and conservative management is associated with an almost 100 per cent of mortality rate. Identification of the bleeding site at laparotomy may be exceedingly difficult, which makes the preoperative detection of bleeding source desirable. Peripancreatic vascular lesions can be identified by angiography, and in selected cases the risk of urgent operation to control massive hemorrhage may be obviated by embolization. The authors have recently experienced a case of ruptured splenic artery pseudoaneurysm combined with a pancreatic pseudocyst in a 6 years old boy. A bolus enhanced CT scan and angiography were essential to confirm these complications of pancreatic pseudocyst. We managed this child successfully with an urgent procedure of transcatheter arterial embolization and another elective surgery of pancreatic pseudocyst.
Aneurysm, False* ; Angiography ; Child* ; Hemorrhage ; Humans ; Laparotomy ; Male ; Mortality ; Pancreatic Pseudocyst ; Pancreatitis ; Pancreatitis, Chronic ; Rupture ; Splenic Artery* ; Tomography, X-Ray Computed

The prognostic factors for extrahepatic biliary atresia (EHBA) after Kasai portoenterostomy include the patient's age at portoenterostomy (age), size of bile duct in theporta hepatis (size), clearance of jaundice after operation (clearance) and the surgeon's experience. The aim of this study is to examine the most significant prognostic factor of EHBA after Kasai portoenterostomy. This retrospective study was done in 51 cases of EHBA that received Kasai portoenterostomy by one pediatric surgeon. For the statistical analysis, Kaplan-Meier method, Logrank test and Cox regression test were used. A p value of less than 0.05 was considered to be significant. Fifteen patients were regarded as dead in this study, including nine cases of liver transplantation. There was no significant difference of survival to age. The age is also not a significant risk factor for survival in this study (Cox Regression test; p = 0.63). There was no significant difference in survival in relation to the size of bile duct. However, bile duct size was a significant risk factor for survival (Cox Regression test; p = 0.002). There was a significant difference in relation to survival and clearance (Kaplan-Meier method; p = 0.02). The clearing was also a significant risk factor for survival (Cox Regression test; p = 0.001). The clearance of jaundice is the most significant prognostic factor of EHBA after Kasai portoenterostomy.
Bile Ducts ; Biliary Atresia* ; Humans ; Jaundice ; Kaplan-Meier Estimate ; Liver Transplantation ; Prognosis ; Retrospective Studies ; Risk Factors

Subarachnoid hemorrhage from a ruptured intracranial aneurysm during pregnancy is rare but results in significant maternal and fetal mortality. The authors report 5 cases of anesthetic experience with pregnant patients undergoing a surgical aneurysmal clipping out of 2,100 patients with subarachnoid hemorrhages due to a ruptured cerebral aneurysm from 1972 until May 2001. All of the patients were diagnosed with a subarachnoid hemorrhage by a brain CT and cerebral angiography. Anesthetic modality and surgical timing should be adjusted by gestational age and the physiologic changes which accompany the pregnancy and the potential risks to the fetus from investigating and treating the mother. Anesthetic goals for this patient include maintenance of uteroplacental perfusion, and fetal as well as maternal well-being. We gave a general anesthesia with isoflurane-nitrous oxide and fentanyl. The patients were monitored with standard monitorings for surgical repair of a cerebral aneurysm and perioperative fetal heart monitoring. All of the five patients recovered well from the surgical aneurysmal repair. After surgical aneurysmal repair, 4 patients maintained their pregnancies and had their babies delivered at term through a cesarean section in 3 patients and transvagina in 1 patient. However, 1 patient who presented persisting hypertension experienced an intrauterine fetal death at 25 weeks of gestational age.
Anesthesia, General ; Aneurysm ; Brain ; Cerebral Angiography ; Cesarean Section ; Female ; Fentanyl ; Fetal Death ; Fetal Heart ; Fetal Mortality ; Fetus ; Gestational Age ; Humans ; Hypertension ; Intracranial Aneurysm* ; Mothers ; Perfusion ; Pregnancy* ; Rupture* ; Subarachnoid Hemorrhage

PURPOSE: Prenatal diagnosis of congenital anomalies provides the information for the perinatal treatment, which can be beneficial to the patients. Yonsei University is one of the largest tertiary referral centers in Korea and its achievement in pediatric surgery is representative figure of pediatric surgery in Korea. This achievement is used to assess the impacts of prenatal ultrasonograms on the doutcomes of prenally diagnosed anomalies in the neonates. METHODS: Between 1991 and 2000, 41,458 prenatal ultrasonograms were performed on the pregnant women and the fetal abdominal abnormalities were suspected in 165 fetuses. Of these, 87 fetuses were delivered and the abnormalities were finally confirmed. Theses 87 fetuses was the basis of this study in terms of their prenatal and final diagnosis with the outcomes. RESULTS: Among 87 fetuses, 17 cases were terminated in relation to the maternal health or multiple anomalies. Of the remaining 70 fetuses, 55 patients survived. Among the prenatal diagnoses of 87 fetuses, final diagnosis were made from 75 fetuses and the accuracy of the prenatal diagnosis was found to be 60.0% (45/75). Surgical correction was necessary in 44 cases to confirm the diagnosis and of these, 40 patients survived after the surgical correction. CONCLUSION: Prenatal diagnosis of the congenital anomalies will improve postnatal outcomes by proper surgical management. However, it can affect the rate terminations of pregnancies even though its accuracy is not so high. To advance the knowledge of the fetal pathophysiology, pediatric surgeons must play an important role in the prenatal diagnosisin relation to the postnatal treatment of the anomalies.
Diagnosis ; Female ; Fetus ; Humans ; Infant, Newborn ; Korea ; Maternal Health ; Perinatal Care ; Pregnancy ; Pregnant Women ; Prenatal Diagnosis ; Tertiary Care Centers ; Ultrasonography*

Neuroblastoma arises from the embryonic tissue of adrenergic rest. It is commonly found in children but mostly in nonrenal tissue. We present a case of intrarenal neuroblastoma which was initially mistaken as Wilms' tumor in a 18 months-old girl treated with radical nephrectomy with postoperative adjuvant chemotherapy. The location of mass within the kidney cannot always indicate that it is Wilms' tumor. Sometimes neuroblastoma from adrenal or retroperitoneal space often compresses or invades directly the kidney and it can also origanate in the kidney. Important clinical aspects in differenctialting neuroblastoma and Wilms' tumor along with review of literatures are discussed.
Chemotherapy, Adjuvant ; Child ; Female ; Humans ; Infant ; Kidney ; Nephrectomy ; Neuroblastoma* ; Retroperitoneal Space ; Wilms Tumor

Giant Meckel's diverticulum is a very rare lesion and its association with a congenital diaphragmatic hernia has not been reported previously. We report a case of newborn with a giant Meckel's diverticulum and congenital diaphragmatic hernia. A large round atypical air-filled bowel segment was found by chest radiography preoperatively, and a giant Meckel's diverticulum was located within the left hemithorax during surgery.
Hernia, Diaphragmatic/*complications/congenital/*pathology ; Human ; Infant, Newborn ; Male ; Meckel Diverticulum/*complications/*pathology/surgery

A quantitative assessment of the density of the protein gene product 9.5 (PGP9.5), the neural cell adhesion molecule (NCAM), and the low-affinity nerve growth factor receptor (NGFR) expressing nerve fibers in the circular muscle layer in the colon was carried out by morphometric analyses from 13 patients with Hirschsprung's disease (HD). The difference in the nerve fiber density between the ganglionic and aganglionic segments was compared by calculating the ratio of the sum of the areas occupied by positively stained nerve fibers per unit area of the muscle after immunohistochemical staining on paraffin embedded tissue sections using computer software. There was an obvious difference in the density of the PGP9.5 stained nerve fibers between the ganglionic (0.0380 +/- 0.0171) and aganglionic segments (0.0143 +/- 0.01661). The NCAM-positive nerve fibers were fewer in number than those of both the PGP9.5-positive fibers and NCAM-positive fibers, which were also markedly lower in number in the aganglionic segment (0.0066 +/- 0.0076) than in the ganglionic segment (0.0230 +/- 0.0195). Immunostaining for low-affinity NGFR revealed much fainter staining in the ganglionic and aganglionic segment without a statistically significant difference in their density. Considering the fact that PGP9.5 is a very sensitive marker for nerve fibers, the results of this study reaffirm the innervation failure of the proper muscle in HD. The decreased NCAM expression level in the aganglionic segment appears to be caused not by the selective down-regulation of NCAM expression among the nerve fibers but by a markedly reduced number of nerve fibers.
Colon/*innervation ; Hirschsprung Disease/*pathology ; Human ; Muscle, Smooth/*innervation ; Nerve Fibers/*chemistry/pathology ; Neural Cell Adhesion Molecules/*analysis ; Receptor, Nerve Growth Factor/analysis ; Thiolester Hydrolases/*analysis

BACKGROUND: Balloon kyphoplasty is the new technique that helps to decrease the pain and improve mobility as well as restore the vertebral body height and kyphotic curve in fractured vertebrae. We evaluated the outcome of balloon kyphoplasty in the reduction of vertebral body height, kyphotic curve and clinical improvement in the patients with painful vertebral compression fractures. METHODS: From July 2002 to February 2005, 84 levels of vertebral compression fractures in 66 patients were treated with balloon kyphoplasty. The assessment criteria were the changes over time in visual analogue scale (VAS) and mobility score. We evaluated the vertebral body height and kyphotic curve at preoperative 1 day and postoperative 1 day. RESULTS: Procedures were performed in 66 patients with a total of 84 affected vertebral bodies. The anterior wall height was restored in 74 / 84 (88%) levels with a mean increment of 2.9 mm, and the mid-vertebral body height was restored in 79 / 84 (94%) levels with a mean increment of 4.2 mm. Kyphosis correction was achieved in 60 / 84 (71.4%) from 10.1 degrees to 7.5 degrees. Pain intensity reduced by 60% in one day after operation and by 75-85% in later time. Mobility scores of all patients were improved immediately after the procedure. Cement leakage occurred in 3 levels but there was no clinical problem. CONCLUSIONS: Kyphoplasty is an efficient and safe treatment of painful vertebral compression fracture in pain relief, mobility improvement, and reduction of deformity.
Body Height ; Congenital Abnormalities ; Fractures, Compression* ; Humans ; Kyphoplasty* ; Kyphosis ; Spine