ABSTRACT 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

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.

Introduction and Aims: To determine the optimum management of growth arrests secondary to meningococcal septicaemia. Method: A retrospective study of 28 children treated in children’s hospitals in the UK for long bone deformities caused by growth plate arrests secondary to meningococcal septicaemia. Results: 28 children (age range four to eight years) with growth arrests of the long bones following meningococcal septicaemia were treated for their bony deformities (a limb length discrepancy or a progressive angular deformity of the upper or lower limb) using the Ilizarov technique. Resection of bony blocks was ineffective in preventing progressive deformities. Limb length discrepancies were treated satisfactorily with equalisation of limb lengths. Angular deformities required ablation of the remaining part of the affected growth plate in order to prevent recurrence. Distal tibial deformities were treated satisfactorily with a transepiphyseal osteotomy. In the upper limb lengthening of either the radius or ulna restored alignment to the wrist. One patient with a growth arrest affecting a tibial amputation stump underwent satisfactory stump realignment and lengthening. Limb lengthening will need to be repeated in younger children, as the deformity will recur with growth until skeletal maturity. Conclusion: The Ilizarov technique enables satisfactory treatment of growth deformities secondary to meningococcal septicaemia. With peripheral growth plate arrests causing an angular deformity the remaining open growth plate needs to be ablated to prevent recurrence of the angular deformity. Any recurrence will then be a shortening only, which can be treated by further lengthening if required.

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.

Introduction: Meningococcal septicemia is a devastating illness that primarily affects children. Late orthopaedic sequelae, though rare, are being seen more frequently as acute medical management has reduced the initial mortality rate. Aims: To review the case histories and discuss the management of these children. Methods: A retrospective review of medical notes and radiographs was undertaken at the participating hospitals. Outcomes assessed included clinical & radiologic outcome, limb length equalization and correction of the mechanical axis. Results: Between 1990 and 2000, twenty patients aged 2 to7 years presented to the orthopaedic departments of the participating hospitals with late sequelae. On average presentation wasf 4 years (2 – 6) after the acute phase of the disease. The reasons for referral included angular deformity, limb length discrepancy, joint con-tracture or problems with prosthetic fitting. The lower limbs were involved more frequently than the upper limbs. In fourteen children multiple growth plates were affected. Partial growth arrest was the cause of the angular deformity and limb length discrepancy. All twenty children underwent operations for realignment of the mechanical axis and equalization of limb length. Recurrence of the angular deformity was almost universal. Conclusion: Children who survive meningococcal septicaemia are at risk for developing late orthopaedic sequelae. Lower limbs are more commonly affected with deformities of limb length and axis. We recommend complete ablation of the affected growth plates at the initial surgery to prevent recurrence of the angular deformity. Further limb length equalization procedures can be anticipated. Early recognition and orthopaedic follow-up to skeletal maturity is essential for minimizing the effects of these sequelae.

The introduction of Ilizarov leg lengthening techniques has allowed a change in philosophy in treatment of fibular hemimelia. Between January 1990 and October 1993, eight patients with 10 involved extremities underwent correction of severe grades of fibular hemimelia by the Ilizarov technique. Mean follow-up from operation was 26.7 months. Because the lengthening desired was achieved in all cases, this form of treatment is an attractive alternative to the more traditional Syme's amputation. However, complications are common. Frequency and severity of complications apparently is less in younger patients who require less extensive, staged lengthening.