Searchable abstracts of presentations at key conferences on calcified tissues
Bone Abstracts (2017) 6 P175 | DOI: 10.1530/boneabs.6.P175

ICCBH2017 Poster Presentations (1) (209 abstracts)

Management of Gorham disease in the cervicothoracic spine with mobile gravity traction and Sirolimus

Paul Foster 1 , M. Zulf Mughal 2 , Julian Leong 1 & Benjamin Jacobs 1

1Royal National Orthopaedic Hospital, Stanmore, UK; 2Royal Manchester Childrens Hospital, Manchester, UK.

Background: Gorham Disease is a rare condition characterised by massive osteolysis. The pathophysiology is related to angio/lymphatic proliferation within bone. No genetic transmission has been identified and onset occurs in patients of all ages. Surgical fixation of the spine may be unsuccessful due to progressive osteolysis of bone surrounding the metalwork, or of the bone graft.

Presenting problem: An 11 year-old boy presented with a 2 year history of back and shoulder pain. He then suffered weakness and paraesthesia in both legs for a few minutes after performing a forward roll. He was noted to have scoliosis, but was otherwise well. Blood bone and inflammatory markers were normal. Imaging demonstrated a severe kyphosis of the thoracic spine with extensive marrow hyperintensity and fatty signal within the bone. Biopsy of the spine was consistent with Gorham disease, transiliac bone biopsy was normal.

Clinical management: A Halo traction system was applied and a customised wheelchair constructed, connecting the Halo to weights via a pulley system. This provided continuous traction to the spine, but allowed him to mobilise in a chair. At night he was transferred to bed traction. Traction weight was gradually increased to a maximum of 1/3 body weight over 7 weeks. Pharmacological therapy with intravenous Zoledronate and Sirolimus was initiated to attempt to improve bone quality ahead of spinal surgery.

Discussion: Mechanical traction and pharmacological treatment were used to prepare this patient’s spine for surgery. Pre-operative traction has provided a minor improvement in the kyphotic deformity and it is hoped that this combination will improve the post-operative outcome.

Disclosure: The authors declared no competing interests.


Volume 6

8th International Conference on Children's Bone Health


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