Loeys-Dietz syndrome with bilateral radial head dislocations: a case report.

Loeys-Dietz syndrome is characterised by vascular aneurysms, hypertelorism, and a bifid uvula. We report on an 11-year-old boy with Loeys-Dietz syndrome who presented with bilateral radial head dislocations and severe osteopaenia with changes of avascular necrosis in both hips causing an out-toeing, wide gait. Considering the poor prognosis for elbow movement and possible radial head dysplasia, surgical reduction of the radial heads was deferred. A subtrochanteric de-rotation osteotomy of the left hip was performed to improve the gait.

Qualitative assessment of the challenges to the treatment of idiopathic clubfoot by the Ponseti method in urban India.

The Ponseti method of clubfoot treatment has been shown to be a very safe, effective and minimally invasive technique when performed in medical centers in Europe and North America. However, only a limited number of studies have helped identify the challenges for effective treatment with the Ponseti method in India. In this study a qualitative approach was used through distribution of questionnaires, personal interviews and focus groups with orthopedic surgeons (in urban centers) and parents of patients with clubfoot. The following factors were evaluated: (i) physician education, (ii) alternative methods of treatment/modification of the Ponseti technique, (iii) compliance by parents, (iv) treatment in underserved areas, (v) culture, (vi) community knowledge of clubfoot, and (vii) the health care system in India. The results showed that all of the factors evaluated hindered outcomes for patients; however, parent's compliance with bracing, lack of proper rural clubfoot treatment clinics, poverty and physician education were the most prominent challenges. The results of this study can be used to implement specific strategies to improve the diffusion and implementation of the Ponseti method for treating clubfoot throughout India.

Convergent radioulnar translocation with radial head fracture in a child: a case report and review of the literature.

SUMMARY: Convergent type radioulnar translocation is an extremely rare form of injury occurring in a growing child. We report a case of convergent radioulnar translocation along with a radial head fracture in a young child. Open reduction of the elbow and internal fixation of the radial head fragment helped in achieving concentric reduction and good functional outcome at 2 years of follow-up. This case has been shown to highlight the diagnostic and management issues of this rare condition, which may be easily missed initially.

Congenital postero-medial bowing of the tibia and fibula: is early surgery worthwhile?

We report the results of surgical treatment of congenital postero-medial bowing of the tibia and fibula. Twenty patients with congenital postero-medial bowing were seen with nine patients treated surgically (corrective osteotomy or lengthening and deformity correction with Ilizarov fixator) and 11 patients managed conservatively. The angles of medial and posterior angulation and limb length discrepancy were recorded serially and compared. Surgical complications were recorded. The mean follow-up was 9.5 and 6.1 years after surgery. Although there was a reduction in angulation and correction of limb length discrepancy, we encountered complications in the surgically treated patients. There was no statistically significant difference between the surgically treated and conservatively managed groups with respect to mean angulation, though there was a significant difference in the mean limb length discrepancy. In conclusion, we advocate a one-stage lengthening and correction of the residual deformity closer to skeletal maturity.

Infantile tibia vara: treatment of Langenskiold stage IV.

An eight year old girl presented with a progressively increasing deformity of the left proximal tibia since last 2 years. She had no history of trauma, fever and swelling of left knee. There were no obvious signs of rickets/muscular dystrophy. She had 25 degrees of tibia vara clinically with lateral thrust and a prominent fibular head. The radiograph of left knee revealed tibia vara with medial beaking and a significant depression of the medial tibial epiphysis and metaphysis. A computed tomography (CT) scan revealed significant depression of the medial tibial epiphysis but no bony bar in the physis or fusion of the medical tibial epiphysis. There was a posterior slope in addition to the medial one. She was treated with elevation of the medial tibial hemiplateau with subtuberosity valgus derotation dome osteotomy. She also underwent a lateral proximal tibial hemiphysiodesis (temporary stapling). A prophylactic subcutaneons anterolateral compartment fasciotomy was also performed. All osteotomies united in 2 months. All deformities were corrected and she regained a knee range of 0 to 130 degrees. At final followup (4 years), there was no recurrence of varus deformity, knee was stable, with 1cm of leg length discrepancy. In Langenskiold stage IV tibia vara, elevation of medial tibial plateau, a subtuberosity valgus derotation osteotomy and a concomitant lateral hemiephiphysiodesis has given good results.

Epiphyseal separations in spastic cerebral palsy.

Metaphyseal and diaphyseal fractures have been reported in children with severe spastic cerebral palsy, but there is no reference to epiphyseal separations in these patients. We report nine epiphyseal separations involving the distal femur and proximal humerus in four severely affected children with spastic quadriplegic cerebral palsy. The clinico-radiological features confirmed the cause to be scurvy. These epiphyseal separations have a good prognosis unlike the physeal injuries reported in children with myelodysplasia. Treatment with vitamin C and splintage resulted in rapid healing with excellent remodeling. Scurvy should be considered a potential cause for an epiphyseal slip in a child with severe spastic cerebral palsy. Routine dietary supplementation of vitamin C is recommended for these malnourished and nonambulant children.

Congenital vertical talus in arthrogryposis and other contractural syndromes.

No practical classification system exists to identify which patients may have successful outcome following treatment of congenital vertical talus in arthrogryposis. We classified 229 patients into five distinct groups: Group I had amyoplasia or so-called classic arthrogryposis, Group II had distal arthrogryposis, Group III had a specific syndrome as a diagnosis, Group IV had severe systemic or neurologic involvement, and Group V had unclassifiable contracture syndromes. No patient with amyoplasia (Group I) had congenital vertical talus. The congenital vertical talus seen in distal arthrogryposis (Group II) was milder than that seen in Groups III or IV and feet in this group responded well to early one-stage surgical correction. Congenital vertical talus that occurred in association with a generalized syndrome or with extensive systemic and neurologic involvement (Groups III and IV) was severe and refractory to treatment. Most children in these groups were unable to walk; therefore, the goal of treatment should be to achieve a pain-free foot to allow fitting of normal shoes. Most children in Group V were able to walk and responded well to operative correction; they should be treated before walking age.