Search

Congenital diaphragmatic hernia (CDH) is a major congenital malformation with high mortality. Outcome data on larger unselected patient groups in Germany are unavailable as there is no registry for CDH. Therefore, routine data from the largest German health insurance fund were analyzed for the years 2009-2013. Main outcome measures were incidence, survival and length of hospital stay. Follow-up was 12 months. 285 patients were included. The incidence of CDH was 2.73 per 10,000 live births. Overall mortality was 30.2%. A total of 72.1% of the fatalities occurred before surgery. Highest mortality (64%) was noted in patients who were admitted to specialized care later as the first day of life. Patients receiving surgical repair had a better prognosis (mortality: 10.8%). A total of 67 patients (23.5%) were treated with ECMO with a mortality of 41.8%. The median cumulative hospital stay among one-year survivors was 40 days and differed between ECMO- and non-ECMO-treated patients (91 vs. 32.5 days, p < 0.001). This is the largest German cohort study of CDH patients with a one-year follow-up. The ECMO subgroup showed a higher mortality. Another important finding is that delayed treatment in specialized care increases mortality. Prospective clinical registries are needed to elucidate the treatment outcomes in detail.

Introduction:  Congenital malformations are associated with substantial neonatal morbidity and mortality. Furthermore, only sparse data are available on the modalities of care provided to and the associated clinical outcomes in affected neonates. In this study, we focused on five malformations that require surgery during the neonatal period: duodenal stenosis and atresia (DA), gastroschisis (GA), omphalocele (OM), congenital diaphragmatic herniation (CDH), and esophageal atresia (EA)., Materials and Methods:  We reviewed the Hessian neonatal registry (2010-2015) to identify records including the ICD-10 (International Classification of Diseases, Tenth Edition) codes for the aforementioned diagnoses and identified 283 patients who were affected by at least one of these conditions. Multiple regression analyses were performed to further identify risk factors for mortality and extended length of hospital stay., Results:  The incidence rates per 10,000 live births and inhospital mortality rates were as follows: DA: 1.79 and 3.6%; GA: 1.79 and 1.8%; OM: 1.60 and 24%; CDH: 1.32 and 27.5%; and EA: 2.67 and 11.1%, respectively. Thirty-three percent of the patients had not been born in a perinatal center in which corrective surgeries were performed. The following risk factors were significantly associated with early mortality: trisomy 13 and 18, congenital heart defects, prematurity, and high-risk malformations (OM and CDH). The predictors of length of stay were as follows: gestational age, number of additional malformations, and treatment in the center with the highest patient volume., Conclusion:  Epidemiology and outcome of major congenital malformations in Hesse, Germany, are comparable to previously published data. In addition, our data revealed a volume-outcome association with regard to the length of hospital stay., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Autologous stem cell transplantation remains an integral treatment tool for certain childhood malignancies. In children, a central venous catheter is typically necessary to provide adequate flow rates for preparative apheresis. In this study, the feasibility and efficiency of collecting CD34+ cells via an indwelling Hickman catheter, preimplanted for chemotherapy, instead of placing an additional temporary central venous catheter was evaluated., Study Design and Methods: Forty-eight pediatric leukaphereses for autologous hematopoietic stem cell transplantation using Spectra Optia MNC, Version 3.0 were reviewed. We compared preimplanted Hickman catheters with a temporary Shaldon catheter, inserted for apheresis. Apheresis was considered successful if a dose of 2 × 10 6 CD34+ peripheral blood stem cells/kg BW was achieved., Results: In 43 (89.6%) of the 48 patients, a Hickman catheter was used for leukapheresis. Only 5 patients (10.4%) received a temporary Shaldon catheter. In both groups, apheresis was performed without apparent adverse reactions. The dose of collected CD34+ peripheral blood stem cells was 12.7 × 10 6 (range, 2.3-70.7 × 10 6 ) cells/kg BW in the Hickman group and 16.2 × 10 6 (range, 3.8-48.4 × 10 6 ) cells/kg BW in the Shaldon group, showing no statistically significant difference (p = 0.58). In both groups, the primary endpoint of a minimal CD34+ cell concentration of 2 × 10 6 cells/kg BW was achieved at a maximum of two leukapheresis sessions. Apheresis efficacy was further confirmed by the collection efficiency of 40.2% in the Hickman group and 27.8% in the Shaldon group (p = 0.32)., Conclusion: These data indicate the reliable feasibility and efficacy of mobilized apheresis via an indwelling Hickman catheter. In light of this, the routine insertion of a dialysis catheter for the purpose of leukapheresis should be critically reconsidered., (© 2019 AABB.)

Background: The impact of general anesthesia is considered a risk factor for developmental delay. Very few studies have been performed to measure the neurodevelopmental outcome of patients with selected malformations., Objectives: The purpose of this ambidirectional cohort study was to measure the neurodevelopmental outcome of patients with congenital gastrointestinal-tract malformations (GIM)., Methods: Forty patients with relevant congenital GIM born in the period from June 2008 to April 2011 were identified. The inclusion criteria were a gestational age >32 completed weeks and surgery that required a general anesthetic within the first 28 days of life. The neonatal characteristics and anesthesia data were retrospectively collected. Based on information about the neonatal characteristics and socioeconomic background, a matched pair was found. All participants were tested at the corrected age of 24 months with the Bayley Scales of Infant Development II assessment., Results: The outcome was split into the psychomotor index (PDI) and mental developmental index (MDI). The patient group achieved a mean PDI of 103 and the control group achieved 106, i.e. these values were not significantly different. The mean MDI was 102 in the patient group and 110 in the control group. This difference was significant (p = 0.022). Detailed analysis of the items showed no significance for nonverbal items but a significant difference for verbal items (p = 0.029). Further analysis showed no correlation between relevant anesthesia data and the neurodevelopmental outcome., Conclusions: We found lower MDI scores due to worse verbal abilities in the patient group. Children born with GIM should be considered a risk group with respect to language development., (© 2016 S. Karger AG, Basel.)