Congenital fetal and neonatal visceral chylous effusions: neonatal chylothorax and chylous ascites revisited. A multicenter retrospective study.

This retrospective study was carried out at eight Neonatal Intensive Care Units (NICU) Centers worldwide on 33 newborns presenting at birth with pleural, pericardial, or abdominal chylous effusions. Diagnosis of chylous effusion is based on findings of fluid with a milk-like appearance, a concentration of triglycerides in pleural effusion >1.1 mmol/l, and a total cell count >1,000 cells/ml with a predominance of >80% lymphocytes. Thirty-three newborns met the inclusion criteria and were studied. Six subjects who presented at birth with fetal effusion were treated by in-utero pleuro-amniotic shunt. Five of these patients are alive at follow-up. At birth, pleural drainage was performed in 29/33 patients and abdominal drainage was carried out in 3/33. Total parenteral nutrition (TPN) was given to 32/33 patients; 19/23 patients were fed a medium-chain triglycerides (MCT). No adverse effects were observed. Eight patients were treated with Octreotide at dosages ranging from 1 to 7 mcg/kg/hour for 8 to 35 days. All patients showed decreased chylous production. Two patients were treated by pleurodesis. Twenty-two babies are alive after at least 6 months follow-up, 9/33 are deceased, and 2 were lost to follow-up. Clinical conditions of survivors are basically good except for lung involvement [chronic lung disease (CLD) or lung lymphangiectasia] and lymphedema. All patients were using a MCT diet at follow-up with good control of chylous effusion. Visceral chylous effusions of the fetus and neonate are rare disorders, and there currently is only partial agreement on decision-making strategies. We suggest the need for an international prospective trial in an effort to establish the efficacy and effectiveness of diagnostic and therapeutic options described in this article.

[Perinatal care in the Marche region 1983. Results of an epidemiologic study]. / L'assistenza perinatale nelle Marche nel 1983. Risultati di un'indagine epidemiologica.

The purpose of this study was to offer a significant picture of perinatal care in the Region Marche. Natality, perinatal and neonatal mortality of 1983 were analysed with reference to birth weight. The obstetric and neonatal services were subdivided, according to the levels of reproductive medical care, in level III, level II, level I and sublevel. The transfers of newborn to the hospital with a service of neonatal pathology or with a neonatal intensive cure unit were taken into account. Our epidemiological data were compared with epidemiological data of Sweden in 1979 and, for very low birth weight infants, with epidemiological data of the Region Friuli Venezia Giulia in 1980. The results have shown the excessive number of obstetric services in the Region Marche, the lack of centralization of risk pregnancies, the frequent transfers of sick newborns, the positive relationship between transferred infants and a greater neonatal mortality in the services of special and intensive neonatal care. The study indicates that it is suitable a program of regionalization of obstetric and neonatal services for an early individualization and in utero referrals of risk pregnancies, for a planned on regional basis transport of sick infants.

[Cornelia de Lange syndrome. Presentation of a clinical case]. / La sindrome di Cornelia de Lange. Presentazione di un caso clinico.

A case of Cornelia de Lange syndrome is described. The infant showed the typical morphologic malformations of the syndrome, a certain resemblance with his mother and maternal grandmother and, in agreement with the most part of literature, no karyotypic anomalies. The infant developed a Staphilococcal sepsis and died at 23 days of age.