title: Use of somatostatin analogues to treat chylothorax in a child with Generalised Lymphatic Dysplasia.
abstract: Generalised Lymphatic Dysplasia is a rare condition that may be associated with significant chylothoraces. The management of such effusions is often challenging. We present the case of a 15-year-old girl with bilateral chylothoraces and lymphoedema of her limbs. A clinical diagnosis of Generalised Lymphatic Dysplasia was made and long-term treatment with somatostatin analogues (somatostatin initially followed by monthly octreotide) was initiated. Over 12 months there was symptomatic benefit with some objective improvement in lung function and no adverse effects. After a year of treatment there was some reaccumulation of fluid, however this did not require any intervention. This is the first paediatric report of the use of somatostatin analogues to manage chylothorax in Generalised Lymphatic Dysplasia and we conclude that they represent a potentially useful treatment modality. Experience is only anecdotal however and further studies are required to establish an evidence base with regard to efficacy and safety.
authors.affiliation: Department of Respiratory Paediatrics, Great North Children's Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne NE1 4LP, United Kingdom ; Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne NE2 4HH, United Kingdom.
authors.forename: Malcolm
authors.initials: M
authors.lastname: Brodlie
authors.affiliation: Department of Respiratory Paediatrics, Great North Children's Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne NE1 4LP, United Kingdom.
authors.forename: Sara
authors.initials: S
authors.lastname: Abdelgalil
authors.affiliation: South West Thames Regional Genetics Unit, St George's, University of London, Cranmer Terrace, London SW17 0RE, United Kingdom.
authors.forename: Sahar
authors.initials: S
authors.lastname: Mansour
authors.affiliation: Department of Respiratory Paediatrics, Great North Children's Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne NE1 4LP, United Kingdom.
authors.forename: David A
authors.initials: DA
authors.lastname: Spencer
keywords: Children; Chylothorax; Generalised Lymphatic Dysplasia; Octreotide; Paediatrics; Somatostatin