Only two cases have been reported of congenital dislocation of the hip in infants born after extrauterine pregnancies. We report a further two and discuss the management and the variable outcome. These cases seem to confirm that congenital dislocation of the hip is associated with moulding forces rather than being a teratological abnormality

Aims. It is well established that there is a strong association between Perthes’ disease and worsening socioeconomic deprivation. It has been suggested that the primary determinant driving this association is exposure to tobacco smoke. This study aimed to examine this hypothesis. Patients and Methods. A hospital case-control study (n = 149/146) examined the association between tobacco smoke exposure and Perthes’ disease, adjusting for area-level socioeconomic deprivation. Tobacco smoke exposure was assessed by parental questionnaire of smoking habits during pregnancy, and by quantitative assay of current exposure using the urinary cotinine-creatinine ratio, which is a widely used and validated measure of tobacco smoke exposure. Results. The odds of Perthes’ disease significantly increased with reported in utero exposure after adjustment for socioeconomic deprivation (maternal smoking odds ratio (OR) 2.06, 95% confidence interval (CI) 1.17 to 3.63; paternal smoking OR 2.09, 95% CI 1.26 to 3.46). The cotinine-creatinine ratio was significantly greater in cases, OR 1.63 (95% CI 1.09 to 2.43), suggesting a greater ‘dose’ of current tobacco exposure. Conclusion. An association exists between tobacco smoke exposure and Perthes’ disease but we remain unable to disentangle the association with socioeconomic deprivation. Cite this article: Bone Joint J 2017;99-B:1102–8

Aims

Developmental dysplasia of the hip (DDH) can be managed effectively with non-surgical interventions when diagnosed early. However, the likelihood of surgical intervention increases with a late presentation. Therefore, an effective screening programme is essential to prevent late diagnosis and reduce surgical morbidity in the population.

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

Aims

We investigated the prevalence of late developmental dysplasia of the hip (DDH), abduction bracing treatment, and surgical procedures performed following the implementation of universal ultrasound screening versus selective ultrasound screening programmes.

Aims

To assess if congenital foot deformity is a risk factor for developmental dysplasia of the hip (DDH).

Aims

To describe and analyze the mid-term functional outcomes of a large series of patients who underwent the Hoffer procedure for brachial plexus birth palsy (BPBP).

Aims

A clicky hip is a common referral for clinical and sonographic screening for developmental dysplasia of the hip (DDH). There is controversy regarding whether it represents a true risk factor for pathological DDH. Therefore a 20-year prospective, longitudinal, observational study was undertaken to assess the relationship between the presence of a neonatal clicky hip and pathological DDH.

Twins are often considered to be at an increased risk of developmental dysplasia of the hip (DDH); we therefore investigated whether multiple births have a higher incidence of DDH, and if selective ultrasound scanning should be considered for these infants.

We reviewed our records of all live births between 1 January 2004 and 31 December 2008 and included 25 246 single and 990 multiple births.

Multiple births did not have a significantly higher incidence of DDH compared with single births (0.0030 vs 0.0023, p = 0.8939). Of the 990 multiple births, 267 had neonatal ultrasound scans and one case of DDH was diagnosed and treated successfully with a Pavlik harness. There were two late-presenting cases at eight and 14 months of age, neither of whom had risk factors for DDH and consequently had not had a neonatal scan. Whereas selective ultrasound scanning of multiple births would have led to earlier detection and treatment of the late-presenting cases, they did not have a significantly higher incidence of DDH compared with single births.

We conclude that being a twin or triplet in itself is not a risk factor for DDH and that selective ultrasound scanning is not indicated for this population.

Cite this article: Bone Joint J 2013;95-B:132–4.

Congenital Talipes Equinovarus (CTEV) is one of the most common congenital limb deformities. We reviewed the records of infants who had received treatment for structural CTEV between 1 January 2007 and 30 November 2012. This was cross-referenced with the prenatal scans of mothers over a corresponding period of time. We investigated the sensitivity, specificity, and positive and negative predictive values of the fetal anomaly scan for the detection of CTEV and explored whether the publication of Fetal Anomaly Screening Programme guidelines in 2010 affected the rate of detection.

During the study period there were 95 532 prenatal scans and 34 373 live births at our hospital. A total of 37 fetuses with findings suggestive of CTEV were included in the study, of whom 30 were found to have structural CTEV at birth. The sensitivity of screening for CTEV was 71.4% and the positive predictive value was 81.1%. The negative predictive value and specificity were more than 99.5%. There was no significant difference between the rates of detection before and after publication of the guidelines (p = 0.5).

We conclude that a prenatal fetal anomaly ultrasound screening diagnosis of CTEV has a good positive predictive value enabling prenatal counselling. The change in screening guidance has not affected the proportion of missed cases. This information will aid counselling parents about the effectiveness and accuracy of prenatal ultrasound in diagnosing CTEV.

Cite this article: Bone Joint J 2014;96-B:984–8.

Aims

To explore the of age of onset distribution for Perthes’ disease of the hip, with particular reference to gender, laterality and conformity to the lognormal distribution.

Club foot was diagnosed by ultrasonography in 91 feet (52 fetuses) at a mean gestational age of 22.1 weeks (14 to 35.6). Outcome was obtained by chart review in 26 women or telephone interview in 26. Feet were classified as normal, positional deformity, isolated club foot or complex club foot.

At initial diagnosis, 69 feet (40 fetuses) were classified as isolated club foot and 22 feet (12 fetuses) as complex club foot. The diagnosis was changed after follow-up ultrasound scan in 13 fetuses (25%), and the final ultrasound diagnosis was normal in one fetus, isolated club foot in 31 fetuses, and complex club foot in 20 fetuses.

At birth, club foot was found in 79 feet in 43 infants for a positive predictive value of 83%. Accuracy of the specific diagnosis of isolated club foot or complex club foot was lower; 63% at the initial ultrasound scan and 73% at the final scan. The difference in diagnostic accuracy between isolated and complex club foot was not statistically significant. In no case was postnatal complex club foot undiagnosed on fetal ultrasound and all inaccuracies were overdiagnoses. Karyotyping was performed in 25 cases. Abnormalities were noted in three fetuses, all with complex club foot and with additional findings on ultrasound.

This prospective cohort study aimed to investigate the relationship between developmental dysplasia of the hip and mode of delivery in 571 consecutive breech infants using a modified Graf’s static morphological method to grade the severity of dysplasia.

In this group, 262 infants were born by planned Caesarian section, 223 by emergency section and 86 vaginally. Taking all grades of hip dysplasia into account (Graf types II, III and IV), there was no statistical difference in the incidence of dysplasia between the groups (elective section 8.4%, emergency section 8.1% and vaginal delivery 7.0%). However, when cases with Graf type II dysplasia, which may represent physiological immaturity, were excluded, the rate of type III and IV hips, which we consider to be clinically relevant, increased in the vaginally delivered group (4.7%) compared with the elective section group (1.1%), with a relative risk of approximately 1:4 (95% confidence interval 1.03 to 15.91). No difference was observed between the emergency and elective section groups, or between the emergency section and vaginally delivered groups.

This study supports previous published work, with the added value that the diagnoses were all confirmed by ultrasound.

Between 1978 and 1997 all newborns in the Austrian province of Tyrol were reviewed regarding hip dysplasia and related surgery. This involved a mean of 8257 births per year (7766 to 8858). Two observation periods were determined: 1978 to 1982 (clinical examination alone) and 1993 to 1997 (clinical examination and universal ultrasound screening). A retrospective analysis compared the number and cost of interventions due to hip dysplasia in three patient age groups: A, 0 to < 1.5 years; B, ≥ 1.5 to < 15 years; and C, ≥ 15 to < 35 years.

In group A, there was a decrease in hip reductions from a mean of 25.2 (sd 2.8) to 7.0 (sd 1.4) cases per year. In group B, operative procedures decreased from a mean of 17.8 (sd 3.5) to 2.6 (sd 1.3) per year. There was a 75.9% decrease in the total number of interventions for groups A and B.

An increase of €57 000 in the overall cost per year for the second period (1993 to 1997) was seen, mainly due to the screening programme. However, there was a marked reduction in costs of all surgical and non-surgical treatments for dysplastic hips from €410 000 (1978 to 1982) to €117 000 (1993 to 1997). We believe the small proportional increase in costs of the universal ultrasound screening programme is justifiable as it was associated with a reduction in the number of non-surgical and surgical interventions. We therefore recommend universal hip ultrasound screening for neonates.

The medial rotation contracture caused by weak external rotation secondary to obstetric brachial plexus injury leads to deformation of the bones of the shoulder. Scapular hypoplasia, elevation and rotation deformity are accompanied by progressive dislocation of the humeral head. Between February and August 2005, 44 children underwent a new surgical procedure called the ‘triangle tilt’ operation to correct this bony shoulder deformity. Surgical levelling of the distal acromioclavicular triangle combined with tightening of the posterior glenohumeral capsule (capsulorrhaphy) improved shoulder function and corrected the glenohumeral axis in these patients. The posture of the arm at rest was improved and active external rotation increased by a mean of 53° (0° to 115°) in the 40 children who were followed up for more than one year.

There was a mean improvement of 4.9 points (1.7 to 8.3) of the Mallet shoulder function score after surgical correction of the bony deformity.

Congenital unilateral anterolateral tibial bowing in combination with a bifid ipsilateral great toe is a very rare deformity which resembles the anterolateral tibial bowing that occurs in association with congenital pseudarthrosis of the tibia. However, spontaneous resolution of the deformity without operative treatment and with a continuously straight fibula has been described in all previously reported cases. We report three additional cases and discuss the options for treatment. We suggest that this is a specific entity within the field of anterolateral bowing of the tibia and conclude that it has a much better prognosis than congenital pseudarthrosis of the tibia, although conservative treatment alone may not be sufficient.

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.