Gorham–Stout disease with life-threatening pleural effusion treated with a pleuro-peritoneal shunt: a case report. (1st June 2022)

• Record Type: Journal Article
• Authors: Toivonen, Leevi A; Neva, Marko H; Sioris, Thanos; Isomäki, Pia; Metso, Saara
• Is Part Of: Endocrinology, diabetes & metabolism case reports. (2022)
• Publication Date: 2022-06-01
• Subjects: Adolescent/young adult -- Male -- White -- Finland; Bone -- Bone; Surgery; Novel treatment -- June -- 2022; Endocrinology -- Periodicals; Diabetes -- Periodicals; Diabetes Mellitus; Endocrinology; Diabetes; Endocrinology; Case Reports; Periodicals; Periodicals; 616.4
• DOI: 10.1530/EDM-21-0101
• Languages: English
• ISSNs: 2052-0573

Abstract

Abstract : Summary: Gorham–Stout disease (GSD) is a rare bone disease characterized by massive osteolysis and lymphatic proliferation. The origin of the condition is unknown, and no established treatment protocol exists. Massive pleural effusion is a frequent complication of GSD in the thoracic region. We present the case of a 23-year-old male with thoracic GSD, subsequent paraparesis, and life-threatening pleural effusion. The patient was managed by a multidisciplinary team with a good recovery. The pleural effusion was successfully treated with a pleuro-peritoneal shunt. This is the first report of the use of this mini-invasive technique in the management of pleural effusion related to GSD. Further, we present the potential role of interleukin-6 and bone resorption markers in the measurement of the disease activity. Learning points: Multidisciplinary approach is important in the management of rare and severe disorders such as Gorham-Stout disease. Pleuro-peritoneal shunting is a valuable option in the treatment of pleural effusion related to GSD. Interleukin-6 and bone resorption markers appear useful in measuring the disease activity of GSD.