Use of Paley Classification and SUPERankle Procedure in the Management of Fibular Hemimelia.

BACKGROUND: Fibular hemimelia is the most common deficiency involving the long bones. Paley classification is based on the ankle joint morphology, identifies the basic pathology, and helps in planning the surgical management. Reconstruction surgery encompasses foot deformity correction and limb length equalization. The SUPERankle procedure is a combination of bone and soft tissue procedures that stabilizes the foot and addresses all deformities. METHODS: We retrospectively reviewed 29 consecutive patients (29 limb segments), surgically treated between December 2000 and December 2014. Among the 29 patients, 27 were treated with reconstructive procedures. Type 1 (8 patients) cases were treated with only limb lengthening, and correction of tibial deformities. Type 2 (7 patients) cases were treated by distal tibial medial hemiepiphysiodesis or supramalleolar varus osteotomy. In type 3 (10 patients) cases, the foot deformity was corrected using the SUPERankle procedure. Type 4 (2 patients) cases were treated with supramalleolar osteotomy along with posteromedial release and lateral column shortening. In a second stage, limb lengthening was performed, using the Ilizarov technique. In the remaining 2 patients (type 3A and type 3C), amputation was performed using Syme technique as a first choice of treatment. RESULTS: The results were evaluated using Association for the Study and Application of Methods of Ilizarov scoring. Excellent results were obtained in 15 of 27 (55%) patients. Six (22%) patients had good results, 4 (14.8%) had fair results, and 2 (7%) had poor results. Mean limb length discrepancy at initial presentation was 3.55 cm (range: 2 to 5.5 cm) which significantly improved to 1.01 cm (range: 0 to 3 cm) after treatment (P=0.015). CONCLUSIONS: Our results and a review of the literature clearly suggest that limb reconstruction according to Paley classification, is an excellent option in the management of fibular hemimelia. Our 2-staged procedure (SUPERankle procedure followed by limb lengthening) helps in reducing the complications of limb lengthening and incidence of ankle stiffness. Performing the first surgery at an earlier age (below 5 y) plays a significant role in preventing recurrent foot deformities. LEVEL OF EVIDENCE: Level IV.

Correction of coronal plane deformities around the knee using a tension band plate in children younger than 10 years.

BACKGROUND: Guided growth through temporary hemiepiphysiodesis has gained acceptance as the preferred primary treatment in treating pediatric lower limb deformities as it is minimally invasive with a lesser morbidity than the traditional osteotomy. The tension band plate is the most recent development in implants used for temporary hemiepiphysiodesis. Our aim was to determine its safety and efficacy in correcting coronal plane deformities around the knee in children younger than 10 years. MATERIALS AND METHODS: A total of 24 children under the age of 10 were operated for coronal plane deformities around the knee with a single extra periosteal tension band plate and two nonlocking screws. All the children had a pathological deformity for which a detailed preoperative work-up was carried out to ascertain the cause of the deformity and rule out physiological ones. The average age at hemiepiphysiodesis was 5 years 3 months (range: 2 years to 9 years 1 month). RESULTS: The plates were inserted for an average of 15.625 months (range: 7 months to 29 months). All the patients showed improvement in the mechanical axis. Two patients showed partial correction. Two cases of screw loosening were observed. In the genu valgum group, the tibiofemoral angle improved from a preoperative mean of 19.89° valgus (range: 10° valgus to 40° valgus) to 5.72° valgus (range: 2° varus to 10° valgus). In patients with genu varum the tibiofemoral angle improved from a mean of 28.27° varus (range: 13° varus to 41° varus) to 1.59° valgus (range: 0-8° valgus). CONCLUSION: Temporary hemiepiphysiodesis through the application of the tension band plate is an effective method to correct coronal plane deformities around the knee with minimal complications. Its ease and accuracy of insertion has extended the indication of temporary hemiepiphysiodesis to patients younger than 10 years and across a wide variety of diagnosis including pathological physis, which were traditionally out of the purview of guided growth.

Management pitfalls of fractured neck of femur in osteogenesis imperfecta.

INTRODUCTION: Fractured neck of femur in osteogenesis imperfecta is rarely reported. Its management is always difficult because of bone fragility and the outcome is not well known. We, therefore, aimed to study the management pitfalls in this group of patients. METHODS: We retrospectively reviewed five cases of fractured neck of femur in four patients treated in our hospital between 2006 and 2009. The demographic data, mode of injury, fracture configuration, treatment, complications, clinical and radiological outcome were reviewed. RESULTS: According to the Sillence classification, one patient was type I, two were type III and one was type IV. There were two children (aged 8 and 15 years) and two adults (aged 21 and 22 years), with the 8 year-old girl suffering from hip fracture on different sides in two accidents. All five hip fractures were the result of low-energy injury and were associated with other fractures. Two undisplaced fractures required intra-operative fluoroscopic confirmation to demonstrate movement at the fracture site. The interval between the injury and fixation ranged from 6 h to 3 days. One hip required secondary surgery to openly reduce the fracture due to inadequate primary fixation and reduction. Two hips were fixed with paediatric dynamic hip screws and three hips were fixed with cannulated screws. All patients were immobilised in hip spica for 6 weeks. The average follow-up was 4 years (3-5 years). All patients had satisfactory union and none of them developed radiological evidence of avascular necrosis at the latest follow-up. All patients returned to their pre-injury functional level. CONCLUSION: Fractured neck of femur is rare given the high prevalence of long bone fractures in osteogenesis imperfecta. They all have characteristic associated fractures of the extremity at the time of injury and neck of femur fractures could be easily missed. Fracture fixation is a great challenge to the orthopaedic surgeons because of the small size of the patients, poor bone quality with suboptimal imaging intra-operatively and compromised purchase of fixation devices. The choice of implants should be determined by the size of the patients and the presence of prior instrumentation close to the hip joint.

Genitopatellar syndrome: a case report of a rare entity with 11 years of follow-up.

Genitopatellar syndrome is one of the syndromes described in the last decade. It is characterized by agenesis of the corpus callosum, absent or hypoplastic patellae, extremity contractures, skeletal anomalies, urogenital anomalies, and facial dysmorphic features. While writing this report, only 15 cases have been reported in the literature. The etiology, clinical features, management, and natural history of this syndrome are not yet well established. Past reports in the literature have not been able to identify the exact genetic etiology but it somewhat coincides with nail patella syndrome and short patella syndrome. We would like to introduce this terminology to the orthopedic community and highlight the clinical features of the genitopatellar syndrome. To the best of our knowledge, this is a single case report with the longest follow-up of 11 years in the literature.