[Alveolar capillary dysplasia with misalignment of pulmonary veins: a fatal cause of refractory neonatal cyanosis]. / Dysplasie alvéolo-capillaire avec mésalignement des veines pulmonaires : une cause de cyanose néonatale réfractaire létale.

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare neonatal pathology that combines refractory hypoxemia with severe pulmonary arterial hypertension, and leads to death every time. Histologic examination of lung tissue confirms the diagnosis and is characterized by a decreased number of pulmonary capillaries, immature lobular development, and abnormal proximity between pulmonary arteries and veins. This abnormal proximity is responsible for an arteriovenous shunt. We report five cases confirmed by postmortem histology, which occurred over 14 years in Lower Normandy (France). The cumulative incidence is therefore of 1.8 for 100,000 births. In these five cases, the first symptoms appeared during the first hour of life and death occurred before 24h in four of five cases. The patient with the longest survival had mild histological lesions and delayed emergence of the first symptoms. Genitourinary and gastrointestinal anomalies were associated with ACD/MPV in two cases, and bilateral pulmonary hypoplasia in three cases. Optimized invasive ventilation, pulmonary vasodilators, vasoactive drugs, and pulmonary surfactant did not improve survival. Extracorporeal membrane oxygenation (ECMO) was not used. We present a review of the literature on ACD/MPV, a clinical and histological entity little known to both clinicians and pathologists, whereas a premortem diagnosis is possible and genetic counseling in affected families can be suggested.

Three dimensional echocardiographic assessment of multiple rhabdomyoma in newborn.

BACKGROUND: Cardiac rhabdomyomas represent the most common primary cardiac tumour in children and are strongly associated with tuberous sclerosis complex. RESULTS: We reported a newborn for whom three-dimensional echocardiogram, with multiplane mode, real-time imaging, full volume and i-slice view, allowed detailed visualisation of multiple highly echogenic and well-circumscribed cardiac rhabdomyoma. CONCLUSIONS: Three-dimensional imaging allowed a better definition of the tumour characteristics and provided a better delineation of the spatial relationship of the mass with a tomographic perspective. Three dimensional imaging may facilitate a possible operative planning and should be included in cardiac mass evaluation and follow-up.

[Medical and economic evaluation of neonatal hospital at home structure]. / Évaluation médico-économique d'une structure d'hospitalisation à domicile de néonatologie.

BACKGROUND: Hospital at home (HAH) shortens hospitalization time by providing at-home hospital-level care. The aim of this study was to describe and assess the cost of the neonatal HAH stay and compare it to the incomes produced by activity-based payments during the 1st year of a neonatal HAH program. METHOD: Medical and economic cost study from the hospital's point of view. For children admitted to the neonatal HAH unit between May 2010 and May 2011, sociodemographic characteristics were identified, consumed resources evaluated, and costs compared to the incomes produced by activity-based payments. RESULTS: Over 75% of children admitted to neonatal HAHs were former preterm infants and 67% of stays included nutritional support. The average length of stay was 16.5 days (SD, 11). The 85 stays produced 152,582 euros of income, the median income was 1531 euros. The median cost of the HAH stay was 1945 euros, resulting in a loss of 45,518 euros for the hospital, but the filling rate was not at its maximum during this period of scalability (77%). Personnel was the most costly item (73% of the total cost) followed by general management and structural costs (20%). CONCLUSION: Economic aspects must be considered to preserve the financial viability of a HAH unit, but the secondary human benefits must be highlighted. A 100% occupation rate would nearly balance the neonatal HAH budget. However, fees must be adjusted to ensure the sustainability and development of these structures.