Neurodevelopmental Outcome in High-Risk Congenital Diaphragmatic Hernia Patients: An Appeal for International Standardization

BACKGROUND: Since mortality in congenital diaphragmatic hernia (CDH) is decreasing, morbidity such as neurodevelopmental outcome is becoming increasingly important. OBJECTIVES: We evaluated neurodevelopmental outcome in high-risk CDH patients treated according to the CDH EURO Consortium standardized treatment protocol. METHODS: This observational, prospective cohort study was conducted in two European centers. Neurodevelopment of 88 patients (Rotterdam n = 49; Rome n = 39) was assessed at 12 and 24 months with the Bayley Scales of Infant Development (BSID)-II-NL (Rotterdam) or BSID-III (Rome). Data of the centers were analyzed separately. RESULTS: Cognition was normal in 77.8% of children from Rotterdam and in 94.8% from Rome at 12 months, and in 70.7 and 97.4%, respectively, at 24 months. Motor function was normal in 64.3% from Rotterdam and in 81.6% from Rome at 12 months and in 45.7 and 89.8%, respectively, at 24 months. Longer length of hospital stay (LoS) was associated with worse cognitive outcome and motor function; LoS, low socioeconomic status, and ethnicity were associated with lower cognition. CONCLUSIONS: At 2 years, most CDH patients have normal cognition, but are at risk for motor function delay. Due to differences in outcomes between centers, careful interpretation is needed before conclusions can be drawn for other centers. Future multicenter collaboration should not only focus on standardization of postnatal care, but also on international standardization of follow-up to identify risk factors and thereby reduce morbidity

Is counselling for CCAM that difficult? Learning from parental experience

Objective: Cystic adenomatoid malformations (CCAM) are relatively rare developmental abnormalities of the lung. Despite outcome is usually benign, parents often exhibit high anxiety level. The purpose of the present study was to collect parents’subjective experience of communication of diagnosis when expecting a fetus with CCAM

When uncertainty generates more anxiety than severity: the prenatal experience with cystic adenomatoid malformation of the lung

Aim: To assess reasons for higher levels of anxiety generated by prenatal counselling of a condition with good outcome such as cystic adenomatoid malformation (CCAM) of the lung compared to a life-threatening malformation such as congenital diaphragmatic hernia (CDH). Materials and methods: The Spielberger State-Trait Anxiety Inventory (STAI-S) was used to measure anxiety in two groups of mothers carrying a fetus with the respective malformation. Results: Forty-four mothers completed the questionnaire (CCAM, n=21 and CDH, n=23). Before consultation, the mean STAI-S scores in the CCAM group (44.80±5.92) and in CDH group (44.05±4.96) were not significantly different but was significantly reduced in both groups after consultation (CCAM 44.80 vs. 41.60, P=0.014 and CDH 44.05 vs. 34.35, P=0.0001). The groups were not significantly different regarding gestational age at diagnosis. Conclusions: After initial prenatal counselling, uncertainty about prenatal outcome and lack of defined management plans in CCAM seems to be more important than higher mortality rate occurring in CDH.Peer Reviewe