Cord compression resulting from Fibrous Dysplasia (FD) of the spine : a case report
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1
Guy's Hospital, Osteoporosis clinic, United Kingdom
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2
Guy's Hospital, Orthopaedics, United Kingdom
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3
Guy's Hospital, Nuclear Medicine, United Kingdom
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4
Hospital Bichat, Molecular Genetics Laboratory, France
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5
Guy's Hospital, Osteoporosis clinic, France
The patient was a 59 year old man who was diagnosed with fibrous dysplasia (FD) 20 years previously. CT scan at the time showed involvement of the 3rd, 5th and 8th rib only. He remained asymptomatic until recently when he started getting pain in his chest and upper back. Neurological examination was normal. Repeat CT and MRI scan confirmed progression of the bone lesions with grossly expanded sclerotic bone and lytic lesions involving particularly T2, T4 and T5. There was complete destruction of the vertebral body of T3 with bulging of the FD lesions into his spinal canal at T3. There was also involvement of his ribs consistent with polyostotic FD. 99TC-MDP bone scan showed intense uptake involving the r-sided ribs and upper thoracic spine. An 18 F FDG PET scan showed increased FDG uptake in the expansile and lytic lesions of the spine (cervical and thoracic spine) and right ribs. The appearances were in keeping with metabolically active disease. He was referred to the metabolic bone clinic for treatment with i.v bisphosphonate. Laboratory findings confirmed increased bone turnover (alkaline phosphatase : 209 (35-129 IU/L), P1NP : 172 (28-80 ug/L) and hypophosphataemia (0.6 mmol/L). However, bisphosphonate treatment was delayed due to a planned knee operation. He developed progressive myelopathy with difficulty in walking and sensory loss at T5 sensory level and required emergency admission. MRI confirmed encroachment upon the spinal canal causing cord compression. He underwent urgent T4/T5 thoracic laminectomy and cord decompression. Histology showed bone fragments containing irregular trabeculae of woven bone and an aneurysmal bone cyst containing osteoclast giant cells. There was no evidence of malignancy. Post-operatively he made an uneventful recovery and was treated medically with i.v Zoledronate and Calcium/vitamin D supplements with improvement in his bone pain. Mutation analysis of his peripheral blood cells did not show any mutation in the GNAS gene at position R201H. {BR}FD is a skeletal disorder characterised by abnormal fibro-osseous tissue. Skeletal involvement leads to pain, deformity and fractures. Treatment with bisphosphonate reduces pain and bone turnover. The molecular cause is due to post-zygotic activating mutations in the GNAS gene.
Keywords:
Bones,
Bone Research
Conference:
2011 joint meeting of the Bone Research Society & the British Orthopaedic Research Society, Cambridge, United Kingdom, 27 Jun - 29 Jun, 2011.
Presentation Type:
Clinical Case
Topic:
Abstracts
Citation:
Scott
I,
Lucas
J,
Gnanasegaran
G,
Silve
C,
Fogelman
I and
Hampson
G
(2011). Cord compression resulting from Fibrous Dysplasia (FD) of the spine : a case report.
Front. Endocrinol.
Conference Abstract:
2011 joint meeting of the Bone Research Society & the British Orthopaedic Research Society.
doi: 10.3389/conf.fendo.2011.02.00060
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Received:
30 Sep 2011;
Published Online:
30 Sep 2011.
*
Correspondence:
Dr. I Scott, Guy's Hospital, Osteoporosis clinic, London, United Kingdom, ian.scott@gstt.nhs.uk