Fibrodysplasia ossificans progressiva (FOP): watch the great toes!

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). 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.

Surgical correction of the true vertical talus deformity.

OBJECTIVE: Correction of skeletal deformity and restoration of muscle balance for the improvement of form and function of the foot. INDICATIONS: Developmental vertical talus deformity (idiopathic form). Vertical talus in a developmental arthrogryposis multiplex or in neurologic diseases such as cerebral palsy or spina bifida. CONTRAINDICATIONS: Serious illnesses preventing anesthesia. SURGICAL TECHNIQUE: Achilles tendon lengthening. Release of contracted parts of ankle capsule, of the talonavicular and the calcaneocuboid joints. Reduction of the hindfoot bones and fixation with Kirschner wires. Augmentation of the spring ligament. Further stabilization by an anterior transfer of the tibialis posterior tendon and a posterior transfer of the tibialis anterior tendon. In addition, in instances of severe deformities or inadequately treated feet before surgery, lengthening of extensor tendons and repositioning of anteriorly displaced peroneal tendons. For paretic feet transfer of the peroneus brevis tendon to the tendon of the peroneus longus or in instances of paralysis of the supinators transfer to the tendon of the tibialis posterior. RESULTS: This procedure was done in 74 feet of 45 patients. Follow-up of 59 feet in 35 patients after an average of 7 years and 3 months. At the time of surgery the youngest patient was 6 months old and the oldest 25 years and 6 months (average 4 years and 6 months). Assessment of results using the parameters of Walker et al. An average of 12.5 out of 16 points was reached. The loss of function was mostly due to the underlying diseases such as arthrogryposis, spina bifida or cerebral palsy. One pin site infection, one osteomyelitis, one pressure sore in the cast, and five wound healing disturbances were observed.

Clinical and electromyographic results of proximal and distal realignment procedures in young patients with recurrent patellar dislocations.

BACKGROUND: Until now, no study has been published about the electromyographic changes in the extensor apparatus after the Green procedure for proximal realignment in young patients with recurrent patellar dislocations. This electromyographic study was performed to analyze imbalances between the vastus medialis and vastus lateralis muscles after different patellar realignment procedures. HYPOTHESIS: Surgical proximal realignment leads to electromyographic changes in the extensor apparatus of the knee and to imbalances between the vastus medialis and vastus lateralis muscles. STUDY DESIGN: Cohort study; Level of evidence, 3. METHODS: A total of 28 knees of 25 patients, with a mean age of 15 years, were treated operatively for recurrent patellar dislocations. The mean follow-up was 6.3 years. Depending on the type of surgical intervention, the patients were assigned to 3 different groups: the patients of group 1 underwent only the Green vastus medialis obliquus (VMO) advancement, group 2 was treated with a combination of the Green proximal and the Roux-Goldthwait distal realignment, and group 3 was treated with a combination of the Green proximal realignment and an additional tubercle transfer. All patients underwent clinical, radiological, and electromyographic examinations. The muscle activities of a healthy control group without patellar dislocations were used for comparison. RESULTS: A combination of the Green procedure and tubercle transfer led to significantly better clinical results and a lower number of redislocations (P < .05) compared with patients who underwent only the Green proximal realignment. While ascending or descending stairs, the ratio between the electromyographic activities of the vastus medialis and vastus lateralis muscles in groups 1 and 2 was significantly smaller compared with the control group (P < .02). Only the electromyograms of group 3 revealed a balanced extensor apparatus during loading with comparable activities of the vastus medialis and vastus lateralis muscles and no significant difference to the control group (P = .37). CONCLUSION: Our electromyographic investigations showed the importance of the vastus medialis in the pathogenesis of malalignment of the extensor mechanism. The Green VMO advancement without tibial tubercle transfer is not able to balance the activities of the vastus medialis and vastus lateralis muscles and cannot be recommended for further use.