Marybeth Browne

Survival after liver transplantation for hepatoblastoma: a 2-center experience.

PURPOSE Complete resection with adjuvant chemotherapy is the accepted treatment for hepatoblastoma. The aim of this study is to evaluate our results of liver transplantation (LT) for tumors still unresectable after adequate chemotherapy. METHODS All patients transplanted for hepatoblastoma from 2 institutions between 1990 and 2004 were included. Variables reviewed to determine impact on survival included the following: previous tumor resection, metastatic disease at diagnosis, microscopic vascular invasion, alpha-fetoprotein (AFP) levels at diagnosis and at transplant, tumor histology, and administration of posttransplantation chemotherapy. Effectiveness of pretransplantation chemotherapy was defined as a drop of more than 99% in peak AFP levels. RESULTS Fourteen patients were transplanted: 9 boys and 5 girls (age range, 18 months-13 years; mean age, 57 +/- 48 months). Patients were transplanted a mean of 4 +/- 1 months after diagnosis. Overall survival was 71% (10/14) with a mean follow-up of 46 months. All deaths were secondary to recurrent tumor. Of 10 patients who underwent a primary LT, 9 survived compared to only 1 of 4 transplanted for unresectable tumor recurrence after primary resection (90% vs 25%; P = .02). Decline in peak AFP of more than 99% was also associated with better survival (100% vs 56%; P = .08). Similarly, patients who received posttransplantation chemotherapy had 100% survival compared with 56% without chemotherapy (P = .08). Other variables had little effect on survival. CONCLUSIONS Liver transplantation is a successful treatment option for children with unresectable hepatoblastoma with a 90% survival rate for primary transplantation. Rescue LT for recurrent hepatoblastoma after previous resection has a poor survival outcome and should be considered a relative contraindication. Posttransplantation chemotherapy improves survival. A prospective multicenter collaboration to validate these findings with a larger patient population is necessary. Until that time, patients who receive rescue transplants should receive posttransplantation chemotherapy.

Gene Transfer of Pigment Epithelium-Derived Factor Suppresses Tumor Growth and Angiogenesis in a Hepatoblastoma Xenograft Model

Normal hepatocytes express pigment epithelium-derived factor (PEDF), an endogenous antiangiogenic factor. We hypothesized that decreased PEDF expression may be one mechanism driving hepatoblastoma growth, and in vivo gene transfer of PEDF could suppress neovascularization and limit tumor growth. PEDF functional activity was determined in vitro using endothelial cell migration assays and in vivo using a subcutaneous tumor model. HUH-6 human hepatoblastoma tumors were treated with hybrid adenoviral/adeno-associated viral expression vectors for PEDF (Hyb-PEDF, n = 4) or β-galactosidase (Hyb-βgal, n = 4) daily for 4 d. Mitotic figures, microvascular density (MVD), PEDF, and VEGF expression were assessed. Hyb-PEDF treatment inhibited in vivo tumor growth (p < 0.008) and decreased MVD (p < 0.001), the number of mitotic figures (p < 0.001), and VEGF expression when compared with Hyb-βgal-treated tumors. HUH-6 expression of PEDF was dramatically reduced when cultured under hypoxic conditions and also when grown in vivo, and the addition of neutralizing anti-PEDF antibody increased the already high baseline angiogenic activity of the HUH-6 cell secretions in vitro (p < 0.04). PEDF is an important endogenous regulator of the liver vasculature. Augmenting intra-tumoral PEDF levels inhibits tumor growth by reducing angiogenesis and VEGF expression. Potent inhibitors of angiogenesis, such as PEDF, may be an effective alternative treatment for children with hepatoblastoma.

Hazardous complications of multiple ingested magnets: report of four cases.

Foreign body ingestion is a common occurrence in the pediatric population. Frequent culprits include coins, toys, sharp objects and bones, which most often pass spontaneously. Magnet ingestion, however, can be a serious matter, especially when more than one is taken in. The extremely strong magnetic force between multiple magnets may result in numerous complications including bowel necrosis, perforation, obstruction, fistula formation, volvulus and death. We present the largest series reported to date, with four cases of multiple magnet ingestion at our institution with varied presentations and findings. We review the literature, and discuss the importance of having a high index of suspicion.

Dollars and sense of interval appendectomy in children: a cost analysis

PURPOSE Although initial nonoperative management of focal, perforated appendicitis in children is increasingly practiced, the need for subsequent interval appendectomy remains debated. We hypothesized that cost comparison would favor continued nonoperative management over routine interval appendectomy. METHODS Decision tree analysis was used to compare continued nonoperative management with routine interval appendectomy after initial success with nonoperative management of perforated appendicitis. Outcome probabilities were obtained from literature review and cost estimates from the Kids Inpatient Database. Sensitivity analyses were performed on the 2 most influential variables in the model, the probability of successful nonoperative management and the costs associated with successful observation. Monte Carlo simulation was performed using the range of cost estimates. RESULTS Costs for continued nonoperative observation were estimated at

Rapunzel syndrome: a rare cause of biliary obstruction.

3080.78 as compared to

Sirenomelia with an Angiomatous Lumbosacral Myelocystocele in a Full-term Infant

5034.58 for the interval appendectomy. Sensitivity analysis confirms a cost savings for nonoperative management as long as the likelihood of successful observation exceeds 60%. As the cost of nonoperative management increased, the required probability for its success also increased. Using wide distributions for both probability estimates as well as costs, Monte Carlo simulation favored continued observation in 75% of scenarios. CONCLUSION Continued nonoperative management has a cost advantage over routine interval appendectomy after initial success with conservative management in children with focal, perforated appendicitis.

Endoloop as the first line tool for appendiceal stump closure in children with appendicitis

522 e is a rare manifestat ands of hair extendin R apunzel syndrom ion of gastric trichobezoars with str g beyond the pylorus into the small intestine, and occasionally up to the colon. So far, only 29 cases of Rapunzel syndrome have been reported in the literature worldwide, with the first case described in 1968 (1). With the exception of 1 male, all of the other reported cases were females. Nausea, vomiting, abdominal pain, and symptoms of obstruction were the common presenting features. Trichobezoars rarely results in biliary obstruction or pancreatitis. We describe a 3-year-old girl with Rapunzel syndrome presenting with a picture of cholestasis because of biliary obstruction from a trichobezoar.

Hepatobiliary cystadenoma: a rare pediatric tumor.

Sirenomelia, also known as the mermaid syndrome, is a rare congenital malformation of uncertain etiology. It is characterized by fusion of the lower limbs and commonly associated with severe urogenital and gastrointestinal malformations. In this report, we describe the first case of an infant with sirenomelia and a massive angiomatous lumbosacral myelocystocele.

Association of Same-Day Discharge With Hospital Readmission After Appendectomy in Pediatric Patients

PURPOSE The objective of this study is to compare outcomes for pediatric patients undergoing laparoscopic appendectomy (LA) performed either (1) using an endostapler (ES) to divide the appendix and mesoappendix or (2) using endoloops (ELs) to close the appendiceal stump and electrocautery to divide the mesoappendix. METHODS We conducted a retrospective chart review of all patients who underwent LA for suspected appendicitis 4 years at a free standing childrens hospital. The use of EL and ES was compared separately in patients with perforated and nonperforated appendicitis. We compared patient characteristics and outcomes. RESULTS There were no significant differences in rate of postoperative abscess, rate of subsequent small bowel obstruction requiring operation or rate of intraperitoneal hematoma between the ES and EL groups for both nonperforated and perforated appendectomy cases. Superficial wound infection was more common in the nonperforated EL group (17/309, 5.5%) than in the nonperforated ES group (2/235, 0.9%; p = 0.007). Operative time for the EL technique (52.2 ± 15.8 minutes; p = 0.047) was shorter than for the ES technique (58 ± 23.2 minutes) for patients with perforated appendicitis. CONCLUSION EL stump closure and mesoappendix cauterization during LA is safe and effective in children with appendicitis, including perforated appendicitis.

Prevalence and Perceptions of Team Training Programs for Pediatric Surgeons and Anesthesiologists

Hepatobiliary cystadenoma is a rare hepatic neoplasm that has been reported only 10 times in the pediatric population. Although considered a benign cystic tumor of the liver, hepatobiliary cystadenoma has a high risk of recurrence with incomplete excision and a potential risk for malignant degeneration. Complete tumor excision with negative margins is the mainstay in treatment. Unfortunately, due to the paucity of cases and its vague presentation, hepatobiliary cystadenoma is rarely diagnosed preoperatively. Therefore, in patients with hepatic cystic masses without a clear diagnosis, total resection of the lesion with negative margins is indicated to adequately evaluate for malignant potential and limit the risk of recurrence. We describe a 2-year-old girl with an asymptomatic abdominal mass that was found to be hepatobiliary cystadenoma. In addition, the pathogenic, histopathologic and clinical features of hepatobiliary cystadenoma are reviewed.