title: Congenital chylothorax: a prospective nationwide epidemiological study in Germany.
abstract: 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.
  
  
  METHODS
  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 für seltene pädiatrische 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.
authors.affiliation: Department of Neonatology, University Children's Hospital of Tübingen, University of Tübingen, Tübingen, Germany.
authors.forename: Anja
authors.initials: A
authors.lastname: Bialkowski
authors.affiliation: Department of Neonatology, University Children's Hospital of Tübingen, University of Tübingen, Tübingen, Germany.
authors.forename: Christian F
authors.initials: CF
authors.lastname: Poets
authors.affiliation: Department of Neonatology, University Children's Hospital of Tübingen, University of Tübingen, Tübingen, Germany Center for Pediatric Clinical Studies, University Children's Hospital of Tübingen, University of Tübingen, Tübingen, Germany.
authors.forename: Axel R
authors.initials: AR
authors.lastname: Franz
mesh_terms: D001724:Birth Weight; D002916:Chylothorax / Q000151:congenital* / Q000453:epidemiology / Q000628:therapy; D005260:Female; D005765:Gastrointestinal Agents / Q000627:therapeutic use; D005858:Germany / Q000453:epidemiology; D005865:Gestational Age; D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D008297:Male; D015282:Octreotide / Q000627:therapeutic use; D011446:Prospective Studies; D012121:Respiration, Artificial / Q000379:methods; D013004:Somatostatin / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D005765:Gastrointestinal Agents; D013004:Somatostatin; D015282:Octreotide
keywords: Congenital Abnorm; Epidemiology; Intensive Care; Neonatology