title: Pregnancy Complicated by Gorham-Stout Disease and Refractory Chylothorax.
abstract: IntroductionGorham-Stout Disease (GSD) is a rare disorder of bony destruction due to lymphangiomatosis, and is often triggered by hormones. One complication of GSD is the development of chylothorax, which carries a high mortality rate. Very little experience has been published to guide management in GSD during pregnancy to optimize both fetal and maternal health.Case StudyA 20-year-old woman with known GSD presented with shortness of breath at 18 weeks of pregnancy, due to bilateral chylothoraces which required daily drainage. To minimize chylous fluid formation, she was placed on bowel rest with total parenteral nutrition (limiting lipid intake) and received octreotide to decrease splanchnic blood flow and chylous fluid drainage. Treatment options were limited due to her pregnancy. Twice daily home chest tube drainage of a single lung cavity, total parenteral nutrition, octreotide, and albumin infusions allowed successful delivery of a healthy 37 weeks' gestation infant by cesarean delivery.DiscussionThis case illustrates the management of a rare clinical disease of bone resorption and lymphangiomatosis complicated by bilateral, refractory chylothoraces, triggered by pregnancy, in whom treatment options are limited, and the need for a multidisciplinary health care team to ensure successful maternal and fetal outcomes.
authors.affiliation: Department of Medicine, Stanford University, Palo Alto, California.
authors.forename: Jessica
authors.initials: J
authors.lastname: Hellyer
authors.affiliation: Department of Surgery, University of California, San Francisco, California.
authors.forename: Hunter
authors.initials: H
authors.lastname: Oliver-Allen
authors.affiliation: Department of Medicine, Stanford University, Palo Alto, California.
authors.forename: Majid
authors.initials: M
authors.lastname: Shafiq
authors.affiliation: Department of Medicine, Stanford University, Palo Alto, California.
authors.forename: Alisha
authors.initials: A
authors.lastname: Tolani
authors.affiliation: Department of Pediatrics, Stanford University, Palo Alto, California; Departments of Obstetrics and Gynecology, Stanford University, Palo Alto, California.
authors.forename: Maurice
authors.initials: M
authors.lastname: Druzin
authors.affiliation: Department of Pediatrics, Stanford University, Palo Alto, California.
authors.forename: Michael
authors.initials: M
authors.lastname: Jeng
authors.affiliation: Division of Cardiovascular Medicine, Stanford Center for Lymphatic and Venous Disorders, Stanford University School of Medicine, Stanford, California.
authors.forename: Stanley
authors.initials: S
authors.lastname: Rockson
authors.affiliation: Department of Medicine, Stanford University, Palo Alto, California.
authors.forename: Robert
authors.initials: R
authors.lastname: Lowsky
keywords: Gorham–Stout disease; chylothorax; octreotide; pregnancy; total parenteral nutrition