Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder and the most disabling condition of heterotopic (extraskeletal) ossification in humans. Extraskeletal bone formation associated with inflammation preceding the osseous conversion usually begins in the first decade, predominantly in the head, neck and shoulders. All patients have malformed great toes. Most patients have a spontaneous mutation of the ACVR1 gene. We report a 17-year-old girl with malformed great toes who had her first episode of heterotopic ossification and impaired mobility of the left hip at the age of 13 years. No inflammatory fibroproliferative masses preceded the onset of heterotopic ossification. Radiographic studies demonstrated myositis ossificans, but failure to associate the great toe malformation with heterotopic ossification led to a failure to diagnose FOP. She underwent repeated and unnecessary operative procedures to remove a recurrent lesion. FOP was finally suspected when the great toe malformation was correlated with the trauma-induced heterotopic ossification. Genetic analysis confirmed the presence of the classic FOP mutation (ACVR1 c.617G>A; R206H). Conclusion This case highlights the importance of examining the great toes in anyone with heterotopic ossification. The association of malformations of the great toe with heterotopic ossification in all cases of classic FOP will lead to prompt clinical diagnosis and the prevention of iatrogenic harm.

We analyzed the time-dependent results after Coventry osteotomy in 118 patients (129 cases) with uni-compartmental osteoarthrosis of the knee. The median follow-up was 11.6 years (range 0.7-17 years). Data were noted according to the time since surgery. Group I (> 2 years) consisted of all 129 cases, group II (> 4 years) of 41 cases and group III (> 8 years) of 15 cases. The HSS knee score (max. 100 points) improved from 33.2 +/- 20.4 (range 17-60) to 68.3 +/- 25.3 (range 30-90) in group I, to 54.7 +/- 18.9 (range 29-90) in group II and to 43.7 +/- 20.9 (range 23-85) in group III. The improvement started 4.6 +/- 7.8 months (range 0-60 months) after the operation and persisted for 4 years +/- 37.4 months (range 0-125 months). The functional knee score (max. 100 points) changed from 61.7 +/- 14.1 (range 41-70) to 71.7 +/- 13.1 (range 53-87) in group I, to 70.0 +/- 11.8 (range 54-88) in group II and to 64.2 +/- 8.0 (range 42-90) in group III. The initial loss in knee flexion was 5.6 degrees (range 0 degree-20 degrees) and for extension 1.0 degree (range -5 degrees-25 degrees). Anteroposterior ligament stability (max. 10 points) decreased from 9.2 +/- 2.1 (range 2-10) to 5.6 +/- 1.7 (range 2-9) in group III. Lateral ligament stability (max. 15 points) was relatively constant, from 12.6 +/- 1.9 (range 4-15) to 9.7 +/- 1.9 (range 2-14). Complications included one tibia fracture, one infection, six peroneus pareses, four haematomas and one pseudarthrosis. The mechanical axis was corrected to an average knee valgu2 of 5.2 degrees +/- 7.4 degrees, which deteriorated over time. Radiographic evidence of arthrosis appeared independent of the operation.

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Adequate diagnostic measures are mandatory in families with ascending aortic aneurysms or type-A aortic dissections to identify or exclude family members at risk for aortic diseases. Even in the absence of identifiable mutations causing isolated aortic aneurysms or aortic dissections, we recommend standardised examinations of all first-degree relatives of affected families. An indication for prophylactic aortic root replacement should be considered for patients at risk.

In a cadaver dissection study the relation of the arthroscopic portals to the neurovascular structures was documented. In six cadaveric elbows the capsule was distended with 35-40cc fluid of 0.9% NaCl by using the direct lateral portal. An anterolateral and anteromedial approach to the elbow joint were established. The distance of the arthroscopic portals to the neurovascular bundles were measured at empty and filled joint after performing an anatomic dissection. The influence of flexion and extension of the joint as well as pronation and supination of the forearm on the distance of the arthroscopic sheath to the neural structures was documented. Lesions of the superficial cutaneous nerves were not seen. Using the anterolateral portal in the best position of the joint (90 degrees flexion and not distended joint at max. pronation of the forearm) we measured a proximity of 4.5 mm (range 2-10 mm) to the radial nerve. For the anteromedial approach the mean distance of the median nerve to the arthroscopic sheath was 15.5 mm (range 8-27 mm), when the optimal joint position was used (90 degrees flexed joint, distended, max. supination of the forearm).