Sirolimus Therapy as Perioperative Treatment of Gorham-Stout Disease in the Thoracic Spine: A Case Report.

Autor:	Mo AZ; Department of Orthopaedics, Lenox Hill Hospital, New York, NY., Trenor CC 3rd; Division of Hematology/Oncology and Vascular Anomalies Center (C.C.T) and Department of Orthopedics (D.J.H.), Boston Children's Hospital, Boston, Massachusetts., Hedequist DJ; Division of Hematology/Oncology and Vascular Anomalies Center (C.C.T) and Department of Orthopedics (D.J.H.), Boston Children's Hospital, Boston, Massachusetts.
Jazyk:	angličtina
Zdroj:	JBJS case connector [JBJS Case Connect] 2018 Jul-Sep; Vol. 8 (3), pp. e70.
DOI:	10.2106/JBJS.CC.17.00287
Abstrakt:	Case: Gorham-Stout disease (GSD) is a rare entity that is marked by progressive osteolysis and bone resorption. A 14-year-old boy who was being followed for scoliosis presented with a marked curve progression and kyphoscoliosis. Imaging revealed osteolysis of the posterior elements and the ribs, suggestive of GSD. The structural compromise threatened spinal cord compression. Preoperative sirolimus therapy was initiated to stabilize the disease prior to corrective instrumentation. A biopsy specimen that was obtained at the time of instrumentation showed lymphatic vascular spaces consistent with GSD. Sirolimus therapy with the addition of bisphosphonate therapy was continued postoperatively. Conclusion: To our knowledge, this case report is the first to describe sirolimus therapy combined with surgery for GSD of the spine. The patient did well with consecutive medical optimization and surgical intervention, including postoperative sirolimus and bisphosphonate therapy.
Databáze:	MEDLINE
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