There is a close link between the embryological development of the musculoskeletal system and all other main organ systems. We report a prospective series of 202 patients with congenital vertebral abnormalities and document the associated abnormalities in other systems. There were 100 boys and 102 girls. In 153 there were 460 associated abnormalities, a mean of 2.27 abnormalities for each patient. Intravenous pyelography was carried out on 173 patients (85.6%) and ultrasonography on the remaining 29 (14.4%). Patients with genitourinary anomalies were more likely to have musculoskeletal (p = 0.002), gastrointestinal (p = 0.02) and cardiac abnormalities (p = 0.008) than those without genitourinary involvement. A total of 54 (26.7%) had at least one genitourinary abnormality, the most frequent being unilateral renal agenesis. There was urinary obstruction in six (3%). There was no association between genitourinary abnormality and the place of birth, parental age, birth order, level of spinal curvature, or the number, type and side of spinal anomaly. There was, however, a statistically significant association (p = 0.04) between costal and genitourinary abnormalities. The incidence of genitourinary abnormalities (26.7%) was similar to that of previously reported series. The diagnosis of a congenital vertebral abnormality should alert the clinician to a wide spectrum of possible associated anomalies most of which are of clinical importance

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.

Aim:. An assessment of the relationship between pathological Developmental Dysplasia of the Hip (DDH) and Congenital Talipes Equinovarus (CTEV). Introduction:. Traditional UK guidelines consider abnormalities of the foot to be a risk factor for DDH. 1,2. Currently, there is controversy whether congenital foot abnormalities are true risk factors for pathological DDH. 3,4. There is a relationship between CTCV and hip dysplasia though the relationship between CTEV and pathological DDH is less clear. 5. In a previous 11 year prospective longitudinal study no case of Graf Types III, IV or irreducible hip dislocation were associated with CTEV. 5. Subsequent correspondence and case histories have challenged this view. 6. Methods:. In order to clarify this issue, a 20-year prospective longitudinal observational study was undertaken. All cases of fixed CTEV (Harold & Walker types 1 to 3) referred to the sub-regional Paediatric Orthopaedic clinic at the Royal Blackburn Hospital were evaluated, the feet and hips clinically assessed (Ortolani & Barlow manoeuvres) and the hips ultra-sounded by the senior author (RWP). Modified Graf and Harcke hip ultrasound classification systems were employed. Graf Type III, IV and irreducible hip dislocation were considered pathological. Results:. The incidence of CTEV was 1.46 per 1000 live births (nationally quoted incidence of 1 to 2 per 1000 live births. 7. ). There was one case of Graf Type III dysplasia with no cases of clinical hip instability. Currently, the clinical significance of this type of dysplasia is uncertain. There were no cases of Graf Type IV dislocations or radiological irreducible hip dislocation. Conclusion:. Fixed CTEV should not be considered as a risk factor for pathological DDH and routine sonographic hip screening of CTEV should not be advocated. This is supportive evidence for the current English NIPE guidelines in which the only risk factors screened are family history and breech presentation. Level of evidence: II

It has been reported that there is an association between Perthes’ disease and poverty. We examined the demographic data of a group of 240 children (263 hips) who presented with Perthes’ disease in Greater Glasgow, where the mean deprivation scores are substantially greater than in the rest of Scotland, to see if this association applied and whether other clues to the aetiology of Perthes’ disease could be found. There were 197 boys and 43 girls; 39 (16.25%) had a family history of Perthes’ disease. Bone age in this series was heavily skewed towards the lower percentiles. The mean number of siblings was 1.9, with 31 (12.9%) being an only child. Maternal age at the birth of the first child showed no preponderance of older mothers. Maternal smoking during and after pregnancy was noted in 132 (55%), which compared with the 52% reported in the population of Greater Glasgow in general. Of the children in our series, 60 (25%) were in social class IV and V. However, this applies to more than half of the population of Greater Glasgow. There was no significant evidence of a preponderance of Perthes’ disease in the most deprived groups. The aetiology of Perthes’ disease is likely to be multifactorial and may include a genetic or deprivation influence resulting in delayed bone age.

We report five children who presented at the mean age of 1.5 years (1.1 to 1.9) with a progressive thoracolumbar kyphosis associated with segmental instability and subluxation of the spine at the level above an anteriorly-wedged hypoplastic vertebra at L1 or L2. The spinal deformity appeared to be developmental and not congenital in origin. The anterior wedging of the vertebra may have been secondary to localised segmental instability and subsequent kyphotic deformity.

We suggest the term ‘infantile developmental thoracolumbar kyphosis with segmental subluxation of the spine’ to differentiate this type of deformity from congenital displacement of the spine in which the congenital vertebral anomaly does not resolve. Infantile developmental kyphosis with segmental subluxation of the spine, if progressive, may carry the risk of neurological compromise. In all of our patients the kyphotic deformity progressed over a period of three months and all were treated by localised posterior spinal fusion. At a mean follow-up of 6.6 years (5.0 to 9.0), gradual correction of the kyphosis was seen on serial radiographs as well as reconstitution of the hypoplastic wedged vertebra to normality. Exploration of the arthrodesis was necessary at nine months in one patient who developed a pseudarthrosis.

A nationwide study of Perthes’ disease in Norway was undertaken over a five-year period from January 1996. There were 425 patients registered, which represents a mean annual incidence of 9.2 per 100 000 in subjects under 15 years of age, and an occurrence rate of 1:714 for the country as a whole. There were marked regional variations. The lowest incidence was found in the northern region (5.4 per 100 000 per year) and the highest in the central and western regions (10.8 and 11.3 per 100 000 per year, respectively). There was a trend towards a higher incidence in urban (9.5 per 100 000 per year) compared with rural areas (8.9 per 100 000 per year). The mean age at onset was 5.8 years (1.3 to 15.2) and the male:female ratio was 3.3:1.

We compared 402 patients with a matched control group of non-affected children (n = 1 025 952) from the Norwegian Medical Birth Registry and analysed maternal data (age at delivery, parity, duration of pregnancy), birth length and weight, birth presentation, head circumference, ponderal index and the presence of congenital anomalies. Children with Perthes’ disease were significantly shorter at birth and had an increased frequency of congenital anomalies.

Applying Sartwell’s log-normal model of incubation periods to the distribution of age at onset of Perthes’ disease showed a good fit to the log-normal curve. Our findings point toward a single cause, either genetic or environmental, acting prenatally in the aetiology of Perthes’ disease.

We report the effect of introducing a dedicated Ponseti service on the five-year treatment outcomes of children with idiopathic clubfoot.

Between 2002 and 2004, 100 feet (66 children; 50 boys and 16 girls) were treated in a general paediatric orthopaedic clinic. Of these, 96 feet (96%) responded to initial casting, 85 requiring a tenotomy of the tendo-Achillis. Recurrent deformity occurred in 38 feet and was successfully treated in 22 by repeat casting and/or tenotomy and/or transfer of the tendon of tibialis anterior, The remaining 16 required an extensive surgical release.

Between 2005 and 2006, 72 feet (53 children; 33 boys and 20 girls) were treated in a dedicated multidisciplinary Ponseti clinic. All responded to initial casting: 60 feet (83.3%) required a tenotomy of the tendo-Achillis. Recurrent deformity developed in 14, 11 of which were successfully treated by repeat casting and/or tenotomy and/or transfer of the tendon of tibialis anterior. The other three required an extensive surgical release.

Statistical analysis showed that children treated in the dedicated Ponseti clinic had a lower rate of recurrence (p = 0.068) and a lower rate of surgical release (p = 0.01) than those treated in the general clinic.

This study shows that a dedicated Ponseti clinic, run by a well-trained multidisciplinary team, can improve the outcome of idiopathic clubfoot deformity.

Cite this article: Bone Joint J 2014;96-B:1424–6

We conducted a prospective randomised controlled trial to compare the standard Ponseti plaster method with an accelerated method for the treatment of idiopathic congenital talipes equinovarus. The standard weekly plaster-change method was accelerated to three times per week. We hypothesised that both methods would be equally effective in achieving correction. A total of 40 consecutive patients (61 feet) were entered into the trial. The initial median Pirani score was 5.5 (95% confidence interval 4.5 to 6.0) in the accelerated group and 5.0 (95% confidence interval 4.0 to 5.0) in the standard control group. The scores decreased by an average 4.5 in the accelerated group and 4.0 in the control group. There was no significant difference in the final Pirani score between the two groups (chi-squared test, p = 0.308). The median number of treatment days in plaster was 16 in the accelerated group and 42 in the control group (p < 0.001). Of the 19 patients in the accelerated group, three required plaster treatment for more than 21 days and were then assigned to the standard control method. Of the 40 patients, 36 were followed for a minimum of six months.

These results suggest that comparable outcomes can be achieved with an accelerated Ponseti method. The ability to complete all necessary manipulations within a three-week period facilitates treatment where patients have to travel long distances.

We reviewed retrospectively 45 patients (46 procedures) with bladder exstrophy treated by bilateral oblique pelvic osteotomy in conjunction with genitourinary repair.

The operative technique and post-operative management with or without external fixation are described. A total of 21 patients attended a special follow-up clinic and 24 were interviewed by telephone. The mean follow-up time was 57 months (24 to 108).

Of the 45 patients, 42 reported no pain or functional disability, although six had a waddling gait and two had marked external rotation of the hip. Complications included three cases of infection and loosening of the external fixator requiring early removal with no deleterious effect. Mid-line closure failed in one neonate managed in plaster. This patient underwent a successful revision procedure several months later using repeat osteotomies and external fixation.

The percentage pubic approximation was measured on anteroposterior radiographs pre-operatively, post-operatively and at final follow-up. The mean approximation was 37% (12% to 76%). It varied markedly with age and was better when external fixation was used. The wide range reflects the inability of the anterior segment to develop naturally in spite of close approximation at operation.

We conclude that bilateral oblique pelvic osteotomy with or without external fixation is useful in the management of difficult primary closure in bladder exstrophy, failed primary closure and secondary reconstruction.

We reviewed the evidence for hip surveillance in children with cerebral palsy from the published literature.

Publications were identified using the Cochrane controlled trials register, the MEDLINE, EMBASE and CINAHL databases and by hand searching key journals and their references. Studies were included if they reported the frequency, associated risk factors or surveillance measures undertaken to identify subluxation or dislocation of the hip in children with cerebral palsy. Assessment of the quality of the methodology was undertaken independently by two researchers.

Four studies described the natural history, incidence and risk factors for dislocation of the hip. Two reported their surveillance results. Approximately 60% of children who were not walking by five years of age were likely to develop subluxation of the hip, with the greatest risk in those with severe neurological involvement. The introduction of surveillance programmes allowed earlier identification of subluxation and reduced the need for surgery on dislocated hips.

Surveillance can identify children most at risk of subluxation using radiological methods which are widely available.