Management of Patella Dislocation in Say-Barber-Biesecker-Young-Simpson’s Syndrome: A Report of Two Cases

Say-Barber-Biesecker-Young-Simpson’s syndrome is one of the Ohdo-like syndromes. It is a very rare congenital condition that is commonly defined by its main clinical features that are blepharophimosis, ptosis, mental retardation, and delayed motor development. They are often associated with skeletal manifestations that are joint laxity, long thumbs and toes, and hypoplastic and/or dislocated patellae. To our knowledge, the available literature does not report any case where attention is drawn to management of skeletal aspect of this specific syndrome, especially surgically. We report 2 cases of SBBYS syndrome with patellar dislocation that we followed for 11 years. One case (with bilateral dislocation) was managed conservatively, and the other (with unilateral dislocation) underwent conservative and surgical treatment. Both had good functional outcome at follow-up. This experience shows that patellar abnormality in this condition can be efficiently addressed conservatively and/or surgically with satisfying results

Comparison of posteromedial and subtalar release in surgical treatment of resistant clubfoot

This preliminary study evaluated two surgical techniques for the treatment of resistant clubfoot: the posteromedical and the subtalar release. Two groups of matched patients with idiopathic clubfoot who underwent surgery between 1983 and 1995 were compared. In group 1 (12 patients, 18 feet), posteromedial release was performed. In this group, the results were satisfactory with a tendency to hypocorrection: 4 (22%) feet needed a secondary procedure (average follow-up: 97.8 months). Group 2 (12 patients, 17 feet) underwent complete subtalar release. The results were clinically better for group 2 (average follow-up: 38.8 monts). Only 1 (5.9%) patient needed a secondary procedure. These results suggest complete subtalar release yields better postoperative correction than posteromedial release

L'apport de l'analyse électromyographique de la marche dans l'étude du pied spastique de l'infirme moteur d'origine cérébrale : étude préliminaire

PURPOSE OF THE STUDY: This preliminary study describes the methodology and the results of gait analysis in cases of equinus and equino-varus deformity of the foot in cerebral palsy children. The ultimate goal was to establish an aid to decision in spastic foot surgical management. MATERIAL: A prospective series of 12 walking children (16 feet) with cerebral palsy has been evaluated prior to surgical correction of equinus or equino-varus deformity of the foot. The mean age was 8 years (range 4 to 11 years of age). METHOD: The pattern of muscle activity during gait cycle has been recorded by surface electrodes for the tibialis anterior, the triceps and the peroneae and by implanted electrodes for the tibialis posterior. Foot switches have been used to differentiate swing and stance gait phases. The results were compared with these of a series of normal children previously published. RESULTS: In four cases, the dynamic equinus was due to an extended or continuous contraction of the triceps surae. The varus deformity appeared to originate from two muscles: the tibialis posterior in 9 cases and the tibialis anterior in 1 case. Both muscles were responsible for the deformity in 2 cases. Two muscular activation patterns were observed in the tibialis posterior: inverted (2 feet) or permanent (9 feet). From the main muscle which was responsible for deformity, we have determined the surgical technique which was most appropriate to restore the muscular balance. DISCUSSION: Our observations confirm Perry's hypothesis, namely that selective and phasic control during the walking cycle does not occur for patients suffering from cerebral palsy. Different surgical procedures were chosen according to the literature on this subject. In the treatment of equinus deformity, lengthening of the Achilles tendon is a satisfactory technique for hemiplegic patients. But we prefer gastrocnemius recession described by Vulpius in spastic diplegia in order to avoid over-lengthening or calcanal gait. If it can be shown that posterior tibial muscle overactivity is the cause of equinovarus, we perform a posterior tibial tendon lengthening, as proposed by Ruda and Frost, or a split posterior tibial tendon transfer as developed by Green. If the posterior tibial muscle is active only during the swing phase, we accomplish a split posterior tibial tendon transfer through the interosseus membrane as advocated by Saji. If the anterior tibial muscle is continuously active, a split anterior tibial tendon transfer to the cuboid described by Hoffer is performed. If the activity is continuous in both the tibial posterior and the tibial anterior muscles, we add a posterior tibial myotendinous lengthening to the split anterior tibial tendon transfer. CONCLUSION: Since 1992, we have developed in our institution a gait analysis laboratory in order to bring objective data in the process of decision making for tendon transfer surgery. With 4 years experience, this objective support now seems to us compulsory in decision of type of transfer. The goal of this preliminary study was to explain how we use the data and match these to our experience and literature

Primary chronic sclerosing osteomyelitis - a case-report

A case of primary chronic sclerosing osteomyelitis of the fibula in a 14-year-old is described. This rare condition can be difficult to differentiate from a bone tumor. Its pathogenesis is controversial, although the prevalent hypothesis involves chronic osteomyelitis developing after an unrecognized phase of acute infection. The existence of primary chronic sclerosing osteomyelitis as a disease in its own right has been challenged. Recently, primary chronic sclerosing osteomyelitis has been interpreted as a localized or monofocal variant of multifocal recurrent chronic osteitis or of the bone abnormalities associated with seronegative spondyloarthropathies (SAPHO syndrome). (C) 2004 Published by Elsevier SAS