Chylothorax and chylous ascites: Management and pitfalls

Aim Chylothorax is a rare but life-threatening condition in newborns. Octreotide, a somatostatin analogue, is widely used as a therapeutic option in neonates with congenital and acquired chylothorax, but its therapeutic role has not been clarified yet. Methods We performed a systematic review to assess the efficacy and safety of octreotide in the treatment of congenital and acquired chylothorax in newborns. Comprehensive research, updated till 31 October 2017, was performed by searching in PubMed, MEDLINE, EMBASE and the Cochrane Central Register of Controlled Trials (CENTRAL) databases using the MeSH terms 'octreotide' and 'chylothorax'. Both term and preterm newborns with congenital or acquired chylothorax treated with octreotide within the 30th day of life were included. Octreotide treatment was considered effective if a progressive reduction/ceasing in drained chylous effusion occurred. Results A total of 39 articles were included. Octreotide was effective in 47% of patients, with a slight but not significant difference between congenital (30/57; 53.3%) and acquired (9/27; 33.3%) chylothorax (P = 0.10). Marked variation in octreotide regimen was observed. The most common therapeutic scheme was intravenous infusion at a starting dose of 1 μg/kg/h, gradually increasing to 10 μg/kg/h according to the therapeutic response. Side effects were reported in 12 of 84 patients (14.3%). Only case reports were included in this review due to the lack of randomised controlled trials. Conclusion Octreotide is a relatively effective and safe treatment option in neonates with chylothorax, especially for the congenital forms.

Octreotide for congenital and acquired chylothorax in newborns: A systematic review.

Background Congenital chylothorax (CCT) is a rare disease of unknown aetiology. Treatment approaches vary; none has been evaluated prospectively. Objective To prospectively determine incidence, treatment and outcome of infants with CCT born in Germany in 2012. Design CCT was defined as non-traumatic chylous pleural effusion within 28 days after birth. As part of the Surveillance Unit for Rare Pediatric Conditions in Germany (Erhebungseinheit fur seltene padiatrische Erkrankungen in Deutschland), all paediatric departments (n=432) received monthly reporting cards to notify the study centre of CCT cases, which were analysed based on anonymised questionnaires and discharge summaries. Data are shown as median (range) or n/N. Results Of 37 cases reported, 28 met inclusion criteria. Questionnaires and/or discharge summaries were available for 27/28. Assuming complete reporting, the incidence of CCT was 1:24 000. Nine infants suffered from proven or suspected syndromal anomalies, most frequently Noonan syndrome (5/9). Postnatally, 23 required mechanical ventilation, 3 continuous positive airway pressure; only 1 had no respiratory support. 17 infants were treated with inotropes/vasopressors, 25 required pleural drainage for 11 (1–36) days. In 13 infants, enteral feeds were withheld initially; 25 received medium-chain triglyceride diet at some time, 9 were treated with octreotide or somatostatin. 18 infants survived without, 6 with sequelae attributable to the underlying disorder; 3 infants died (median age at death 37 (2–144) days). Duration of hospital stay in survivors was 51 (20–127) days. Infants treated with octreotide or somatostatin had similar outcomes compared with those not treated. Conclusions Based on this small observational study, CCT seems to have a favourable prognosis if not associated with genetic disorders.

https://fn.bmj.com/content/fetalneonatal/early/2014/12/05/archdischild-2014-307274.full.pdf

Congenital chylothorax: a prospective nationwide epidemiological study in Germany.

Guidelines are fundamental in addressing everyday clinical indications and in reporting the current evidence-based data of related scientific investigations. At the same time, a spatial and tempora...

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Global guidelines trends and controversies in lower limb venous and lymphatic disease: Narrative literature revision and experts' opinions following the vWINter international meeting in Phlebology, Lymphology & Aesthetics, 23-25 January 2019.

Corticosteroids are the mainstay of therapy for many pediatric disorders and sometimes are life-saving. Both endogenous and synthetic derivatives diffuse across the cell membrane and, by binding to their cognate glucocorticoid receptor, modulate a variety of physiological functions, such as glucose metabolism, immune homeostasis, organ development, and the endocrine system. However, despite their proved and known efficacy, corticosteroids show a lot of side effects, among which growth retardation is of particular concern and specific for pediatric age. The aim of this review is to discuss the mechanism of action of corticosteroids, and how their genomic effects have both beneficial and adverse consequences. We will focus on the use of corticosteroids in different pediatric subspecialties and most common diseases, analyzing the most recent evidence.

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Clinical Use and Molecular Action of Corticosteroids in the Pediatric Age

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.

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N. Vercellino

Papers

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

Vascular Anomalies Guidelines by the Italian Society for the study of Vascular Anomalies (SISAV).