title: A Case of a Central Conducting Lymphatic Anomaly Responsive to Sirolimus.
abstract: The study of vascular anomalies is a rapidly progressing field in medicine. The development of new knowledge in the pathology and management of these disease processes are exemplified in the treatment of hemangiomas with propranolol and generalized lymphatic malformations with sirolimus. Central conducting lymphatic anomalies have traditionally been refractory to medical and surgical interventions. We report a case of a central conducting lymphatic anomaly that was responsive to sirolimus. A 14-year-old boy presented with chylothorax and chyluria with a lymphangiogram demonstrating abnormal lymphatic flow and reflux along the entire course of the central channels. Traditionally, medical management has been limited to somatostatin and low-fat diet with poor response and surgical interventions that are palliative. Sirolimus allows a new medical option that could improve management of this unresponsive population.
authors.affiliation: Department of Pediatrics, Vascular Anomalies Center, and mccormickaa@upmc.edu.
authors.forename: Andrew
authors.initials: A
authors.lastname: McCormick
authors.affiliation: Department of Pediatrics, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania.
authors.forename: Stacy
authors.initials: S
authors.lastname: Rosenberg
authors.affiliation: Department of Pediatrics, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania.
authors.forename: Katherine
authors.initials: K
authors.lastname: Trier
authors.affiliation: Department of Pediatrics, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania.
authors.forename: Arcangela
authors.initials: A
authors.lastname: Balest
mesh_terms: D000293:Adolescent; D006801:Humans; D008206:Lymphatic Diseases / Q000188:drug therapy*; D008297:Male; D012074:Remission Induction; D020123:Sirolimus / Q000627:therapeutic use*
chemical_list: D020123:Sirolimus