Congenital diaphragmatic hernia (CDH) is characterized by failure of diaphragmatic development with lung hypoplasia and persistent pulmonary hypertension of the newborn (PPHN). If conventional treatment with gentle ventilation and optimized vasoactive medication fails, extracorporeal membrane oxygenation (ECMO) may be considered. The benefits of ECMO in CDH are still controversial, since there are only few randomized trials demonstrating the advantages of this therapeutic option. At present, there is no precise prenatal and/or early postnatal prognostication parameter to predict reversibility of PPHN in CDH patients. Indications for initiating ECMO include either respiratory or circulatory parameters, which are also undergoing continuous refinement. Centers with higher case numbers and the availability of ECMO published promising survival rates, but data on long-term results, including morbidity and quality of life, are rare. Survival might be influenced by the timing of ECMO initiation and the timing of surgical repair. In this regard a trend toward early initiation of ECMO and early surgery on ECMO exists. The results concerning the cannulation modes are similar and a consensus on time limit for ECMO runs does not exist. The use of ECMO in CDH will continue to be evaluated, and prospective randomized trials and registry network are necessary to help answering the addressed questions of patient selection and management.

The causative agent of legionellosis is the Gram-negative intracellular bacteria Legionella spp. Its clinical presentation varies from a mild febrile illness called Pontiac fever to the severe and possible fatal pneumonia, Legionnaires’ disease. Immunocompromised patients, in particular, are affected. Only a small number of infected neonates are described in the literature. Most of them have been associated with water birth or the use of air humidifiers. In the last five years, a growing number of cases have been reported in Germany by the national institute of disease surveillance and prevention (Robert-Koch Institute). Here, we describe a fatal case report of pulmonary legionellosis with acute respiratory distress syndrome (ARDS), sepsis, associated cutaneous manifestation, and extracorporeal membrane oxygenation in a full-term neonate. Moreover, we present a review of the literature discussing the epidemiology, risk factors, clinical features, diagnostics, treatment options, and prevention for this rare condition in neonates.

Background: Severe respiratory failure of the newborn requires adjunctive therapies as application of surfactant, inhalation of nitric oxide (iNO), high frequency oscillatory ventilation (HFOV), or extracorporeal membrane oxygenation (ECMO). We designed this study to analyze the the usage and effectiveness of adjunctive therapies and the mortality of severe respiratory failure. Patients and Methods: The survey in Germany was done in collaboration with the “Erhebungseinheit für seltene pädiatrische Erkrankungen” (ESPED). 397 patients within 2 years were included into the study. Effectiveness of each adjunctive therapy was judged by the treating physician. Results: The most frequent diagnosis was respiratory distress syndrome (RDS) with 36.8%, followed by pneumonia sepsis (16.4%), meconium aspiration syndrome (MAS) and congenital diaphragmatic hernia (CDH). Surfactant was applied in 77.3% of all cases with a reported effectiveness of 71.6%. More than 40% of all patients were treated with iNO, which led to an improvement in every second case. HFOV was used in every third case with a response rate of about 60%. ECMO was performed on one in 7 patients and was sucessful with a survival rate of nearly 80%. The overall mortality was 10.3%. 29 patients in total died without ECMO. 10 of them might actually have been contraindicated, but 19 cases with a potential benefit from ECMO were not transferred for ECMO. Conclusion: Our study-data suggests that more newborns suffering from respiratory failure should be transferred to centers offering ECMO. Hintergrund: Ein schweres neonatales Lungenversagen führt zum Einsatz von additiven Therapien, wie der Applikation von Surfactant, inhalativem Stickstoffmonoxid (iNO), der Hochfrequenz-Oszillationsbeatmung oder der extrakorporalen Membranoxygenierung (ECMO). Ziel dieser Studie war es Anwendung und Effektivität der additiven Therapien zu dokumentieren, und die Mortalität des schweren Lungenversagens zu analysieren. Patienten und Methodik: Über die Erhebungseinheit für seltene pädiatrische Erkrankungen in Deutschland (ESPED, Düsseldorf) wurden 397 Patienten innerhalb von 2 Jahren in die Studie aufgenommen. Die Effektivität der jeweiligen additiven Therapie wurde durch den behandelnden Arzt beurteilt. Ergebnisse: Die häufigste Diagnose war der Surfactantmangel mit 36,8%, gefolgt von Pneumonie/Sepsis (16,4%), Mekoniumsaspirationssyndrom (MAS) und der kongenitalen Zwerchfellhernie (CDH). Surfactant wurde in 77,3% aller Fälle appliziert mit einer Effektivität von 71,6%. Mehr als 40% aller Patienten wurden mit iNO behandelt, mit einer Wirksamkeit von etwa 50%. HFOV wurde bei einem Drittel aller Fälle mit einer Wirksamkeit von 60% angewendet. Die ECMO-Therapie kam bei etwa jedem siebten Patienten zum Einsatz und war in fast 80% erfolgreich. Die Gesamtsterblichkeit innerhalb der Kohorte lag bei 10,3%. 29 Patienten starben ohne ECMO-Therapie. Davon konnten wir 10 Fälle ermitteln, welche für ECMO kontraindiziert gewesen wären. Die restlichen 19 Patienten, welche womöglich von einer ECMO-Therpaie profitiert hätten, wurden nicht an ein entsprechendes Zentrum überwiesen. Schlussfolgerung: Die Daten legen nahe, dass mehr Neugeborene mit Lungenversagen an ECMO Zentren verlegt werden sollten.

Background Despite recent studies failing to prove beneficial effects of iNO therapy in patients with CDH, its use is still widespread. The aim of this work was to analyze iNO use in a retrospective cohort focusing on outcome parameters. Patients 378 CDH patients born and treated in Mannheim University Medical Center, Department for Neonatology, between 2010 and 2017 constituted our cohort. Therapy was based on the standardized protocol of the CDH EURO Consortium. Method General data (sex, birth weight, gestational age etc.) and therapy-related data (duration of iNO application, OI after 60 mins, need for ECMO support etc.) were collected from clinical reports and then conducted using SAS for both mono- and multivariate analyses. Results Out of 378 newborns with CDH, 265 received iNO (70.1%), of whom 82 (30.9%) showed a significant OI reduction of ≥5 pts after 60 mins (=responders), median OI improved by 1.85 pts overall. Among initial responders iNO, application reduced the need for ECMO support (p=0.0054), increased the time to ECMO initiation (p=0.005) and reduced mortality (p=0.0396). Discussion A group of 43 patients considerably benefited from iNO and thererfore as they did not need ECMO support. Even though iNO therapy has failed to prove significant beneficial effects for non-responders, the application is still to be considered an essential treatment method in the transitional period of CDH patients. Conclusions A more critical approach towards iNO application in nonresponders should be promoted. Further extensive multicenter studies on treatment alternatives for CDH-PAH are desirable.