Necrotizing Enterocolitis following Gastroschisis Repair: An Update

Purpose: Necrotizing enterocolitis (NEC) is a known complication of gastroschisis with an incidence above the expected rate in the neonatal population. While many physicians today are aware of this association, the last publication to explore this association in detail and identify possible risk factors of NEC in gastroschisis patients was published over twenty years ago. From our large database of patients with gastroschisis managed by a single group of pediatric surgeons, we reviewed our experience and the recent literature to update what is known about gastroschisis and NEC. Methods: From 2001 to 2017, a gastroschisis registry was maintained. Data from 218 gastroschisis patients were reviewed. Patient demographics and hospital course were reviewed. Patients with confirmed NEC were compared to those without NEC. Results: Two hundred eighteen patients were born with gastroschisis during the time frame of this study. We observed a 5% rate (11 of 218) of NEC. Five patients (45%) developed recurring NEC and 4 patients (36%) were readmitted for NEC development following initial discharge. Variables associated with NEC included low gestational age (P=0.016) and low birth weight (P=0.003). Patients born prior to 37 weeks gestation had a 4.8 times greater risk of developing NEC than those born at term. Rates of IUGR were not statistically different between NEC and non-NEC patients. The method of delivery (cesarean vs vaginal), use of a silo, and form of nutrition were not significantly associated with NEC development. Conclusions: The overall incidence of NEC has decreased compared to earlier reports. NEC does complicate the hospital course for patients, significantly increasing duration of in-hospital treatment. NEC in gastroschisis differs in comparison to traditional NEC, presenting later in life. Risk factors identified include low gestational age and low birth weight. Avoiding elective preterm deliveries may decrease the rate of NEC in gastroschisis

The Global Retinoblastoma Outcome Study: a prospective, cluster-based analysis of 4064 patients from 149 countries

Background Retinoblastoma is the most common intraocular cancer worldwide. There is some evidence to suggest that major differences exist in treatment outcomes for children with retinoblastoma from different regions, but these differences have not been assessed on a global scale. We aimed to report 3-year outcomes for children with retinoblastoma globally and to investigate factors associated with survival. Methods We did a prospective cluster-based analysis of treatment-naive patients with retinoblastoma who were diagnosed between Jan 1,2017, and Dec 31,2017, then treated and followed up for 3 years. Patients were recruited from 260 specialised treatment centres worldwide. Data were obtained from participating centres on primary and additional treatments, duration of follow-up, metastasis, eye globe salvage, and survival outcome. We analysed time to death and time to enucleation with Cox regression models. Findings The cohort included 4064 children from 149 countries. The median age at diagnosis was 23.2 months (IQR 11.0-36.5). Extraocular tumour spread (cT4 of the cTNMH classification) at diagnosis was reported in five (0.8%) of 636 children from high-income countries, 55 (5.4%) of 1027 children from upper-middle-income countries, 342 (19. 7%) of 1738 children from lower-middle-income countries, and 196 (42.9%) of 457 children from low-income countries. Enudeation surgery was available for all children and intravenous chemotherapy was available for 4014 (98.8%) of 4064 children. The 3-year survival rate was 99.5% (95% CI 98.8-100.0) for children from high-income countries, 91.2% (89.5-93.0) for children from upper-middle-income countries, 80.3% (78.3-82.3) for children from lower-middle-income countries, and 57.3% (524-63-0) for children from low-income countries. On analysis, independent factors for worse survival were residence in low-income countries compared to high-income countries (hazard ratio 16.67; 95% CI 4.76-50.00), cT4 advanced tumour compared to cT1 (8.98; 4.44-18.18), and older age at diagnosis in children up to 3 years (1.38 per year; 1.23-1.56). For children aged 3-7 years, the mortality risk decreased slightly (p=0.0104 for the change in slope). Interpretation This study, estimated to include approximately half of all new retinoblastoma cases worldwide in 2017, shows profound inequity in survival of children depending on the national income level of their country of residence. In high-income countries, death from retinoblastoma is rare, whereas in low-income countries estimated 3-year survival is just over 50%. Although essential treatments are available in nearly all countries, early diagnosis and treatment in low-income countries are key to improving survival outcomes. Copyright (C) 2022 The Author(s). Published by Elsevier Ltd.Y