Fibrous Dysplasia / McCune Albright syndrome (FD/MAS) represents a wide spectrum of diseases due to somatic gain-of-function mutations of the GNAS gene. The mutation leads to overactivity in the target tissues and to a wide phenotype of clinical features that vary in severity and age of onset. The rarity of the disease and its variable presentation to multiple specialities often leads to misdiagnosis and inappropriate variability in investigations and treatments. To address this, our international consortium of clinicians, researchers, and patients’ advocates has developed pragmatic clinical guidelines for best clinical practice for the definition, diagnosis, staging, treatment and monitoring for FD/MAS to empower patients and support clinical teams in both general and specialised healthcare settings. With the lack of strong evidence to inform care, the guidelines were developed based on review of published literature, long-standing extensive experience of authors, input from other healthcare professionals involved in the care of FD/MAS patients and feedback from patients and patient groups across the globe. This has led to the formulation of a set of statements to inform healthcare professionals, patients, their families, carers and patient groups of the best practice of care. It is anticipated the implementation of these recommendations will lead to improvement in the care of patients with FD/MAS internationally. Electronic supplementary material The online version of this article (10.1186/s13023-019-1102-9) contains supplementary material, which is available to authorized users.

An optimal intravertebral cement volume was identified for accomplishment of pain relief through PVP in painful OVCFs. Appropriate thresholds were provided to guide the operator.

This retrospective study describes the long-term results of core decompression and placement of a non-vascularised bone graft in the management of avascular necrosis of the femoral head. We treated 80 hips in 65 patients, 18 by a cortical tibial autograft and 62 by a fibular allograft. The mean age of the patients was 36 years (SD 13.2). A total of 78 hips were available for evaluation of which pre-operatively six were Ficat-Arlet stage 0, three stage I, 31 stage IIA, 16 stage IIB, 13 stage III and nine stage IV. A total of 34 hips (44%) were revised at a mean of four years (SD 3.8). Survivorship analysis using a clinical end-point showed a survival rate of 59% five years after surgery. We found a significant difference (p = 0.002) in survivorship, when using a clinical and radiological end-point, between the two grafts, in favour of the tibial autograft. We considered this difference to be the result of the better quality and increased volume of tibial bone compared with that from the trochanteric region used with the fibular allograft. This is a relatively simple, extra-articular and reproducible procedure. In our view core decompression, removal of the necrotic tissue and packing of the cancellous grafts into the core track are vital parts of the procedure.

Osteoarticular allograft reconstructions are associated with high rates of mechanical complications. Although comparative studies with alternative techniques are scarce, the risk of mechanical failure in our opinion does not justify routine employment of osteoarticular allografts for reconstruction of large joints after tumour resection. Cite this article: 2017;99-B:522-30.

In a prospective study between August 2002 and August 2005, we studied the quantitative clinical and radiological outcome 36 months after percutaneous vertebroplasty for intractable type-II osteoporotic vertebral compression fractures which had been unresponsive to conservative treatment for at least eight weeks. We also examined the quality of life (QoL). The clinical follow-up involved the use of a pain intensity numerical rating scale (PI-NRS, 0 to 10), the Short-Form 36 (SF-36) QoL questionnaire and an anamnestic questionnaire before and at seven days (PI-NRS only), and one, three, 12 and 36 months post-operatively. A total of 30 consecutive patients received percutaneous vertebroplasty for 62 vertebral compression fractures with a mean time between fracture and treatment of 7.7 months (2.2 to 39). An immediate, significant and lasting reduction in the average and worst back pain was found, represented by a decrease of 3.1 and 2.7 points after seven days and 3.1 and 2.8 points after 36 months, respectively (p < 0.00). Comparison of the pre- and post-vertebroplasty scores on the various SF-36 domains showed an ultimate significant increase in six of eight domains and both summary scores. Asymptomatic leakage of cement was found in 47 of 58 (81%) of treated vertebrae. Two minor complications occurred, an asymptomatic pulmonary cement embolism and a cement spur along the needle track. Percutaneous vertebroplasty in the treatment of chronic vertebral compression fractures results in an immediate, significant and lasting reduction in back pain, and overall improvement in physical and mental health.