A previously healthy 29-year-old woman presented to our orthopedic outpatient clinic with a 6-month history of pain in the right sacroiliac joint. The initially intermittent symptoms had become exacerbated after recent childbirth. Radiography of the pelvis showed symphyseal separation and a prominent bony defect of the right ilium (Figure). Magnetic resonance imaging confirmed advanced osteolysis and revealed T2-hyperintense proliferation of angiomatous structures. Subsequent biopsy showed blood and lymph vessels within the affected rarefied lamellar bone and confirmed the diagnosis of Gorham–Stout syndrome (GSS), a rare mono- or polyostotic disease in which idiopathic intraosseous angiomatous proliferation leads to progressive resorption of bone. In accordance with recent research showing clinical amelioration of GSS on administration of bisphosphonates, we began off-label treatment with zoledronate (4 mg i.v. every 4 weeks). Two years of this treatment stabilized the local findings. 11 years after initial diagnosis the patient remains well and pain-free at her annual follow-up visits.
Dr. med. Kristian Nikolaus Schneider, Prof. Dr. med. Georg Gosheger, PD Dr. med. Dimosthenis Andreou,
Universitätsklinikum Münster, Klinik für Allgemeine Orthopädie und Tumororthopädie, Albert-Schweitzer-Campus 1, firstname.lastname@example.org
Conflict of interest statement: The authors declare that no conflict of interest exists.
Translated from the original German by David Roseveare.
Cite this as: Schneider KN, Gosheger G, Andreou D: Gorham–Stout syndrome. Dtsch Arztebl Int 2019; 116: . DOI: 10.3238/arztebl.2019.0507a