Prevalence and orthopedic management of foot and ankle deformities in Charcot-Marie-Tooth disease.

INTRODUCTION: Foot deformities are frequent complications in Charcot-Marie-Tooth disease (CMT) patients, often requiring orthopedic surgery. However, there are no prospective, randomized studies on surgical management, and there is variation in the approaches among centers both within and between countries. METHODS: In this study we assessed the frequency of foot deformities and surgery among patients recruited into the Inherited Neuropathies Consortium (INC). We also designed a survey addressed to orthopedic surgeons at INC centers to determine whether surgical approaches to orthopedic complications in CMT are variable. RESULTS: Foot deformities were reported in 71% of CMT patients; 30% of the patients had surgery. Survey questions were answered by 16 surgeons working in different specialized centers. Most of the respondents were foot and ankle surgeons. There was marked variation in surgical management. DISCUSSION: Our findings confirm that the approaches to orthopedic management of CMT are varied. We identify areas that require further research. Muscle Nerve 57: 255-259, 2018.

The hip in arthrogryposis.

PATHOLOGY: Hip dislocation is seen in approximately 30 % of children with amyoplasia and approximately 50 % of these will be bilateral. TREATMENT: Closed reduction is rarely successful. Open reduction is indicated for unilateral dislocations and for the majority of bilateral dislocations. Reduction is recommended via a medial approach. RESULTS: A long-term satisfactory outcome can be achieved but with some loss of hip range of movement.

Achondroplasia and limb lengthening: Results in a UK cohort and review of the literature.

AIMS: We aim to review the results, complications and outcomes of a single surgeon's series of lower limb lengthening in patients with achondroplasia. METHODS: Ten achondroplastic children underwent limb lengthening. The patients, medical records and radiographs were reviewed. RESULTS: The average age at the time of the index operation was 7.8 years. A single surgeon undertook all procedures. The average total length gain was 20.5 cm. The commonest complication was a fractured femur after removal of the frame. CONCLUSION: Although complication rates were high (70%), none were left with any long-term sequelae and all were pleased with the results.

Late recurrence of developmental dysplasia of the hip following Pavlik harness treatment until normal ultrasound appearance.

PURPOSE: Establish whether recurrent dysplasia once a dysplastic hip has been treated to ultrasonographic normality is possible. METHODS: 370 babies were referred to a hip ultrasound clinic from June 2005 to 2007 to assess for dysplasia. 96 dysplastic hips underwent appropriate treatment until normal hip morphology achieved on follow-up ultrasounds. Minimum further 12 months follow-up. RESULTS: 3 children (4%) developed late recurrence of dysplasia. Two required a plaster hip spica. One had an additional adductor tenotomy. One required late pelvic osteotomy. CONCLUSION: This study highlights the need for long-term follow-up of dysplastic hips with an early pelvic X-ray at around six months.

Compass elbow hinge: short-term results in five adolescents.

The Compass Elbow Hinge (Smith and Nephew, Memphis, Tennessee, USA) was developed in the late 1990s as a mean of treating elbow contractures, utilizing Ilizarov's principles of distraction histeogenesis and a circular frame external fixator. Subsequent reports were published on its use for treating elbow ankylosis and instability in adults. Since 1999 five paediatric patients (aged from 12 to 15 years) have been treated by this device in our institute. The indications were post-traumatic elbow stiffness in four patients and elbow ankylosis following septic arthritis in one patient. Application of the elbow hinge was accompanied by open soft tissue contracture release, both anteriorly and posteriorly. Elbow mobilization and physiotherapy were commenced immediately postoperatively. The hinge was removed after 6-8 weeks. Follow-up ranged from 5 to 36 months. Complications were limited to simple pin track infection in three patients, and transient ulnar neuropraxia in one. The Compass Elbow Hinge (Smith & Nephew) application was successful in improving the arc of motion in three patients out of five.

Recurrent plexiform neurofibromatosis of the foot with extensive bone destruction.

Plexiform neurofibromatosis is uncommon in the foot. No case of neurofibromatous involvement of the bone in the foot has been reported. We report the first case of plexiform neurofibromatosis with destruction of bones in the foot.