Gorham Disease of Distal Humerus Successfully Treated by Slight Shortening and Pin and Plate Fixation

Keivan Rahbari; Kian Zohrabi; Reza Shahriar Kamrani

doi:10.18502/jost.v8i4.10459

Keivan Rahbari Assistant Professor, Department of Orthopedics, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
Kian Zohrabi Clinical Fellowship Candidate, Department of Orthopedics, Shariati Hospital, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Reza Shahriar Kamrani Professor, Department of Orthopedic and Trauma Surgery, Shariati Hospital, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

DOI: https://doi.org/10.18502/jost.v8i4.10459

Keywords: : Gorham Disease; Bone Resorption; Osteolysis; Humerus

Abstract

Background: Gorham's disease (GD) is one of the scarce and idiopathic skeletal diseases which causes osteolysis following the proliferation of blood vessels. Progressive osteolysis GD of distal humerus with articular involvement and pathologic fracture has not been reported and our case is the first report of this disease involving distal humerus and its joints’ surfaces.

Case Report: A 9-year-old boy, case of nonunion of medial condyle of humerus and pathologic fracture of distal humerus after minor trauma, was referred to our clinic and treatment started by casting but due to displacement and nonunion, we decide to operate him. Intraoperative finding was in favour of aneurysmal bone cyst (ABC) near fracture site; therefore, wide resection and fixation by medial tension band wiring (TBW), lateral plating, and fibular allograft application was done (post-operation pathologic result did not show microscopical features of this tumor) and 6 weeks later, he developed stress riser fracture above lateral plate; thus, plate removal was done and severe bone resorption was revealed. Therefore, another specimen was sent for pathology that showed hamartomatous and hemangiomatous lesion of bone. All findings were in favour of GD. He was operated another time and fixed by Persian fixation with small pins and plate, and early plate removal was done for prevention of stress riser fracture.

Conclusion: In cases of GD of distal humerus and pathologic fracture, Persian fixation is a good option for fixation and we suggest early device removal for prevention of stress riser fracture.