| Popis: |
Background: Vanishing bone disease / Gorham-Stout disease (GSD) is a condition that produces deformity and instability of bone. The fibro lympho-vascular tissue replaces the bone leading to massive osteolysis and its sequelae, but the exact cause is yet unknown. The disease involves the spine infrequently, but due to the proximity of the spinal cord it can seriously affect the patient. The aim of this study is to report as a review to contribute to the diagnosis, and treatment modalities in GSD affection of spine with the reported literature available from 1983 till March 2022. Materials & Method: This metanalysis study is focused on GSD involving the spine. The search was done in two databases PubMed and Google scholar from 1983 up to March 2022. The Study selection was done to study the demographic pattern of GSD in spine and its outcome with conservative and surgical treatment and to determine the best suitable medical treatment for stopping disease progression and achieving remission. Results: We retrieved 72 articles from Google scholar and PubMed out of which 5 articles were excluded (90 reported cases). Heffez criteria was followed for diagnosis in all these cases (n=86, 95.5%). 57 patients (64%) were operated and 33 patients (36%) were managed conservatively. Per-operative failure to achieve a fixation/reconstruction were reported in 2 (2.53%) cases. Number of surgeries till follow-up were average 1.70+ 1.23 (1-5) surgeries. The average follow-up of cases reported was 47.1+ 48.9 (3-240 months). Union was documented in 10 cases (3.4%). 9 of these cases needed additional bone graft/substitute. Bisphosphonates(n=40), sirolimus (n=5), interferon (n=17), radiotherapy (n= 31) and beta-blockers (n=4) were given in medications. 23 patients had remission. Death occurred in 17 patients (18.88%). Conclusion: Surgery is needed frequently. Failure of fixations, achieving union and remission are daunting and ’off label” therapies are the dictum. Radiotherapy has been used more frequently with or without bisphosphonates. Though promising medical treatment are evolving and focus of treatment is directed towards anti-angiogenic, anti-osteolytic and anabolic therapy, but no standard treatment recommendations can be made out from existing literature. Keywords: Vanishing bone disease, Gorham stout, Osteolysis, Spine, Deformity, Sirolimus, TNF |
