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

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

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

In hepatoblastoma, encouraging cure rates have been achieved with recent advances in chemotherapy and surgical techniques. The aim of this study is to evaluate the role of combined therapeutic modalities and surgical resection in hepatoblastoma. Fifteen cases of hepatoblastoma were treated from January 1993 to August 2000. Six patients had resectable tumors at initial diagnosis. All underwent surgical resection and in four patients postoperative adjuvant chemotherapy was needed. Nine out of 15 patients had unresectbale tumors at initial diagnosis, and preoperative chemotherapy was applied. There was one operative mortality and 14 patients showed good prognosis after surgery. Although various treatment modalities should be combined for the unresectable hepatoblastoma, surgical resection remains the major curative procedure.
Chemotherapy, Adjuvant ; Diagnosis ; Drug Therapy ; Hepatoblastoma* ; Humans ; Mortality ; Prognosis

PURPOSE: The results of surgical treatment for acute suppurative cervical lymphadenitis in children were evaluated with literature reviews. METHODS: Children under 15-year old who required operative management for acute suppurative cervical lymphadenitis between January 1996 and December 2000 were evaluated with retrospective manner. The clinical characteristics, pathologic results and microbiologic studies were reviewed. RESULTS: 37 patients were treated with surgical methods. 36 patients recovered fully after surgical intervention without any recurrence and one patient expired of sepsis. CONCLUSION: The classical treatment, incision and drainage, is one of the treatment of choice for the recovery from suppurative cervical lymphadenitis in children.
Adolescent ; Child* ; Drainage ; Humans ; Lymphadenitis* ; Recurrence ; Retrospective Studies ; Sepsis

Femoral hernias are very uncommon in children and very easily misdiagnosed. During a period of three years, three children of femoral hernia were treated by one pediatric surgeon at Severance Hospital. Only one case was diagnosed correctly before surgery; the other two were thought to be either an indirect inguinal hernia or groin mass. Only one patient had curative hernioplasty (McVay hernioplasty) at the first operation; the other two did not have curative hernioplasty at the first operation. Femoral hernia in childhood is a challenging clinical problem because of its rarity and similar clinical presentation as indirect inguinal hernia. The frequency with which an incidental indirect inguinal hernia sac or patent processus vaginalis can be found at surgery can perpetuate a misdiagnosis. The absence of an expected indirect inguinal hernia sac or an apparent recurrence of an indirect inguinal hernia should lead to consideration of possible femoral hernia.
Child* ; Diagnostic Errors ; Groin ; Hernia, Femoral* ; Hernia, Inguinal ; Herniorrhaphy ; Humans ; Recurrence

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

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