We reviewed 13 patients with congenital insensitivity to pain. A quantitative sweat test was carried out in five and an intradermal histamine test in ten. DNA examination showed specific mutations in four patients. There were three clinical presentations: type A, in which multiple infections occurred (five patients); type B, with fractures, growth disturbances and avascular necrosis (three patients); and type C, with Charcot arthropathies and joint dislocations, as well as fractures and infections (five patients, four with mental retardation). Patient education, shoeware and periods of non-weight-bearing are important in the prevention and early treatment of decubitus ulcers. The differentiation between fractures and infections should be based on aspiration and cultures to prevent unnecessary surgery. Established infections should be treated by wide surgical debridement. Deformities can be managed by corrective osteotomies, and shortening by shoe raises or epiphysiodesis. Joint dislocations are best treated conservatively

Proximal femoral resection (PFR) is a proven pain-relieving procedure for the management of patients with severe cerebral palsy and a painful displaced hip. Previous authors have recommended post-operative traction or immobilisation to prevent a recurrence of pain due to proximal migration of the femoral stump. We present a series of 79 PFRs in 63 patients, age 14.7 years (10 to 26; 35 male, 28 female), none of whom had post-operative traction or immobilisation.

A total of 71 hips (89.6%) were reported to be pain free or to have mild pain following surgery. Four children underwent further resection for persistent pain; of these, three had successful resolution of pain and one had no benefit. A total of 16 hips (20.2%) showed radiographic evidence of heterotopic ossification, all of which had formed within one year of surgery. Four patients had a wound infection, one of which needed debridement; all recovered fully. A total of 59 patients (94%) reported improvements in seating and hygiene.

The results are as good as or better than the historical results of using traction or immobilisation. We recommend that following PFR, children can be managed without traction or immobilisation, and can be discharged earlier and with fewer complications. However, care should be taken with severely dystonic patients, in whom more extensive femoral resection should be considered in combination with management of the increased tone.

Cite this article: Bone Joint J 2014; 96-B:701–6.

The aims of this study were to report functional outcomes of salvage procedures for patients with cerebral palsy (CP) who have chronic dislocation of the hip using validated scoring systems, and to compare the results of three surgical techniques.

We reviewed 37 patients retrospectively. The mean age at the time of surgery was 12.2 years (8 to 22) and the mean follow-up was 56 months (24 to 114). Patients were divided into three groups: 14 who underwent proximal femoral resection arthroplasty (PFRA group 1), ten who underwent subtrochanteric valgus osteotomy (SVO group 2), and 13 who underwent subtrochanteric valgus osteotomy with resection of the femoral head (SVO with FHR group 3). All patients were evaluated using the Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) and the Pediatric Quality of Life Inventory (PedsQL).

Significant improvements occurred in most CPCHILD and PedsQL subsection scores following surgery in all patients, without significant differences between the groups. There were 12 post-operative complications. Less severe complications were seen in group 1 than in groups 2 and 3.

Salvage surgery appears to provide pain relief in patients with CP who have painful chronic dislocation of the hip. The three salvage procedures produced similar results, however, we recommend the use of PFRA as the complications are less severe.

Take home message: Salvage surgery can be of benefit to patients with CP with chronic painful hip dislocation, but should be limited to selected patients considering complications.

Cite this article: Bone Joint J 2016;98-B:137–43.

We report the outcome of 28 patients with spina bifida who between 1989 and 2006 underwent 43 lower extremity deformity corrections using the Ilizarov technique. The indications were a flexion deformity of the knee in 13 limbs, tibial rotational deformity in 11 and foot deformity in 19. The mean age at operation was 12.3 years (5.2 to 20.6). Patients had a mean of 1.6 previous operations (0 to 5) on the affected limb. The mean duration of treatment with a frame was 9.4 weeks (3 to 26) and the mean follow-up was 4.4 years (1 to 9). There were 12 problems (27.9%), five obstacles (11.6%) and 13 complications (30.2%) in the 43 procedures. Further operations were needed in seven patients. Three knees had significant recurrence of deformity. Two tibiae required further surgery for recurrence. All feet were plantigrade and braceable.

We conclude that the Ilizarov technique offers a refreshing approach to the complex lower-limb deformity in spina bifida.

Since September 1964, neonates born in New Plymouth have undergone clinical examination for instability of the hip in a structured clinical screening programme. Of the 41 563 babies born during this period, 1639 were diagnosed as having unstable hips and 663 (1.6%) with persisting instability were splinted, five of which failed. Also, three unsplinted hips progressed to congenital dislocation, and there were four late-presenting (walking) cases, giving an overall failure rate of 0.29 per 1000 live births, with an incidence of late-walking congenital dislocation of the hip of 0.1 per 1000 live births.

This study confirms that clinical screening for neonatal instability of the hip by experienced orthopaedic examiners significantly reduces the incidence of late-presenting (walking) congenital dislocation of the hip.

We reviewed the long-term radiological outcome, complications and revision operations in 19 children with quadriplegic cerebral palsy and hip dysplasia who underwent combined peri-iliac osteotomy and femoral varus derotation osteotomy. They had a mean age of 7.5 years (1.6 to 10.9) and comprised 22 hip dislocations and subluxations. We also studied the outcome for the contralateral hip. At a mean follow-up of 11.7 years (10 to 15.1) the Melbourne cerebral palsy (CP) hip classification was grade 2 in 16 hips, grade 3 in five, and grade 5 in one. There were five complications seen in four hips (21%, four patients), including one dislocation, one subluxation, one coxa vara with adduction deformity, one subtrochanteric fracture and one infection. A recurrent soft-tissue contracture occurred in five hips and ten required revision surgery.

In pre-adolescent children with quadriplegic cerebral palsy good long-term outcomes can be achieved after reconstruction of the hip; regular follow-up is required.

Cite this article: Bone Joint J 2013;95-B:259–65.

We describe a patient with cerebral palsy, of normal intelligence, who could not walk but who by the age of 16 had been successfully managed with a staged bilateral total hip arthroplasty using a constrained liner.

In order to treat painful subluxation or dislocation secondary to cerebral palsy, 11 patients (12 hips) underwent combined femoral and Chiari pelvic osteotomies with additional soft-tissue releases at a mean age of 14.1 years (9.1 to 17.8). Relief of pain, improvement in movement of the hip, and in sitting posture, and ease of perineal care were recorded in all, and were maintained at a mean follow-up of 13.1 years (8 to 17.5). The improvement in general mobility was marginal, but those who were able to walk benefited the most.

The radiological measurements made before operation were modified afterwards to use the lateral margin of the neoacetabulum produced by the pelvic osteotomy. The radiological migration index improved from a mean of 80.6% (61% to 100%) to 13.7% (0% to 33%) (p < 0.0001). The mean changes in centre edge angle and Sharp’s angle were 72° (56° to 87°; p < 0.0001) and 12.3° (9° to 15.6°; p < 0.0001), respectively. Radiological evidence of progressive arthritic change was seen in one hip, in which only a partial reduction had been achieved, and there was early narrowing of the joint space in another. Painless heterotopic ossification was observed in one patient with athetoid quadriplegia. In seven hips the lateral Kawamura approach, elevating the greater trochanter, provided exposure for both osteotomies and allowed the construction of a dome-shaped iliac osteotomy, while protecting the sciatic nerve.