A Rare Case of Progressive Gorham's Disease of Right Shoulder Girdle and Cervical Spine in A Child: 10 Year Follow-up and A Review of Literature.

Abstract

Introduction:‘Vanishing Bone Disease’ or Gorham’s disease is a very rare form of primary idiopathic osteolysis with only around 200 cases being reported till date. We present in this case report a ten year follow-up of a patient who had progressive osteolysis of the right shoulder girdle and eventually involved the spine inspite of supplementary calcium and bisphosphonate therapy. We also report the utility of using an intra-medullary device is to attain acceptable union in case of pathological long bone fracture in Gorham’s disease.Case:A four year old child presented to us with a spontaneous osteolysis of the right scapula ten years ago in 2003. Within two years, the osteolysis progressed to the entire scapula, clavicle and he had a clavicular fracture. The patient was treated conservatively, oral calcium and bisphosphonate therapy was initiated after ruling out any metabolic bone disease. The patient again sustained a pathological fracture humerus five years later, which was treated with open reduction and internal fixation with an intra-medullary K wire. The fracture united without any other supplementary treatment but osteolysis continued to progressively involve the entire scapula, proximal humerus, clavicle, upper two ribs and the cervical spine.Conclusion:Gorham’s disease is a rare disease with an unpredictable course without any satisfactory treatment. There is still a role for surgical intervention to treat long bone pathological fractures secondary to Gorham’s disease with an intra-medullary device. The fractures unite without any other supplementary radio or chemotherapy.