Clinical features and management of Gorham's disease:a report of 6 cases

LIAO Feng; LIU Wei-feng; XU Hai-rong; NIU Xiao-hui

doi:10.3969/j.issn.2095-252X.2018.01.013

Abstract：Objective To present patients of Gorham's disease diagnosed and / or managed in Beijing Jishuitan hospital. Methods All patients diagnosed and / or treated for Gorham's disease at our institution before 2016 were included. Clinical features, radiographic findings, pathology, treatment, and outcomes were collected by retrospective medical record review. Results Six patients ( 4 males, 2 females ) from 2003 to 2015 were included. The median age at onset was 23.5 years ( range: 14 - 33 years ). Lesion location: 3 cases around the shoulder girdle, 3 cases around the pelvic. Reasons for orthopedic referral: 4 patients were of localized pain, 2 patients were of asymptomatic skeletal lesion found incidentally. Osteolytic lesions had features of progressive, non-expansile, local osseous resorption. Biopsies of all bony lesions were reported, containing numerous thin-walled vessels and were lack of cellular atypia. Two patients did not receive any treatment, one of them was lost in the follow-up and the other lived with progressive disease for 3 years after the initial visit. Before the referral to our department, 2 patients underwent curettage and bone grafting. Four patients were treated with bisphosphonates ( 1 patient: treated for more than 2 years, stable now; 3 patients: still receiving bisphosphonate treatment and had improvement at a mean follow-up of 9.3 months ). Conclusions Diagnosis of Gorham's disease, rare to be seen, is mainly based on the imaging and pathological examination. Bisphosphonates is effective to stabilise the lesion.