Aims

Children with spinal dysraphism can develop various musculoskeletal deformities, necessitating a range of orthopaedic interventions, causing significant morbidity, and making considerable demands on resources. This systematic review aimed to identify what outcome measures have been reported in the literature for children with spinal dysraphism who undergo orthopaedic interventions involving the lower limbs.

In a prospective trial we performed MRI of the spine and hind brain in 31 patients with scoliosis of onset between the ages of four and 12 years. In eight patients (26%) there was a significant neuroanatomical abnormality; there were six cases of Chiari-1 malformation associated with a syrinx, one isolated Chiari-1 malformation and one astrocytoma of the cervical spine. Four of these patients had left-sided curves. There were no clinical features which could reliably identify those patients with abnormalities on MRI. In particular, the unilateral absence of abdominal reflexes was found to be non-specific (1 of 8 of patients with neuroanatomical abnormalities (12.5%) v 2 of 23 with normal scans (8.7%)). In view of the established risks of surgical correction of scoliosis in the presence of undecompressed syringomyelia and the possible improvement that may follow decompression of the foramen magnum, we feel that MRI of all patients with scoliosis of juvenile onset should be obligatory

Somatosensory evoked potentials (SSEPs) measure the conduction pathways from the periphery to the brain and can demonstrate the site of neurological impairment in a variety of locomotor conditions. SSEPs were studied in 44 children (64 feet) with surgically corrected club feet. Four children had unreproducible responses, 18 showed abnormal recordings and 22 showed normal responses. In a further 31 feet (21 children) subjected to motor electrophysiological tests, 16 (52%) were abnormal. Overall, 44 of 95 feet (46%) showed abnormal SSEPs or motor electrophysiological tests. Neurological abnormality was related both to the severity of the deformity and the surgical outcome. It was seen in 38% of feet with grade-2 and in 53% of feet with grade-3 deformity. A fair surgical result was obtained in 36% of feet with a conduction deficit and in only 6% with no abnormality. These results suggest an association between neurological abnormality as demonstrated by SSEPs or motor electrophysiological studies and the severity of deformity in club foot and its response to surgical treatment

Aims

Hip displacement, common in patients with cerebral palsy (CP), causes pain and hinders adequate care. Hip reconstructive surgery (HRS) is performed to treat hip displacement; however, only a few studies have quantitatively assessed femoral head sphericity after HRS. The aim of this study was to quantitatively assess improvement in hip sphericity after HRS in patients with CP.

Aims

Although atlantoaxial rotatory fixation (AARF) is a common cause of torticollis in children, the diagnosis may be delayed. The condition is characterised by a lack of rotation at the atlantoaxial joint which becomes fixed in a rotated and subluxed position. The management of children with a delayed presentation of this condition is controversial. This is a retrospective study of a group of such children.

Aims

Single event multilevel surgery (SEMLS) has been shown to improve gait in children with cerebral palsy (CP). However, there is limited evidence regarding long-term outcomes and factors influencing them.

The results of a functional, clinical and radiological study of 30 children (60 hips) with whole-body cerebral palsy were reviewed at a mean follow-up of 10.2 years (9.5 to 11). Correction of windsweep deformity of the hips was performed by bilateral simultaneous combined soft-tissue and bony surgery at a mean age of 7.7 years (3.1 to 12.2).

We were able to recall 22 patients; five had died of unrelated causes and three were lost to follow-up. Evaluation involved interviews with patients/carers and clinical and radiological examination.

The gross motor functional classification system was used to assess overall motor function and showed improvement in seven patients. Of the 12 patients thought to have pain pre-operatively, only one had pain post-operatively. Improved handling was reported in 18 of 22 patients (82%). Those with handling problems were attributed by the carers to growth of the patients. All patients/carers considered the procedure worthwhile. The range of hip movements improved, and the mean windsweep index improved from 50 pre-operatively to 36 at follow-up.

The migration percentage and centre-edge angle were assessed on plain radiographs. Radiological containment improved, the mean migration percentage improved from 50 pre-operatively to 20 at follow-up and the mean centre-edge angle improved from −5° to 29°.

No statistical difference was noted between the three-year and ten-year follow-up results, indicating that the improvements in clinical and radiological outcome had been maintained.

In 1994, a register for cerebral palsy and a health-care programme were started in southern Sweden with the aim of preventing dislocation of the hip in children with cerebral palsy. It involved all children with cerebral palsy born in 1992 or later.

None of the 206 affected children born between 1992 and 1997 has developed a dislocation following the introduction of the prevention programme. Another 48 children moved into the area and none developed any further dislocation. Of the 251 children with cerebral palsy, aged between five and 11 years, living in the area on January 1, 2003, only two had a dislocated hip. One boy had moved into the area at age of nine with a dislocation and a girl whose parents chose not to participate in the programme developed bilateral dislocation. One boy, whose condition was considered to be too poor for preventative surgery, developed a painful dislocation of the hip at the age of five years and died three years later.

Eight of 103 children in a control group, consisting of all children with cerebral palsy living in the area between 1994 and 2002, and born between 1990 and 1991, developed a dislocation of the hip before the age of six years.

The decreased incidence of dislocation after the introduction of the prevention programme was significant (p < 0.001). Dislocation of the hip in cerebral palsy remains a serious problem, and prevention is important. Our screening programme and early intervention when lateral displacement of the femoral head was detected appear to be successful.