Selective sonographic screening for developmental dysplasia of the hip - increasing trends in late diagnosis.

There are concerns that selective sonographic screening for developmental dysplasia of the hip (DDH) may be suboptimal. Our aim was to test this hypothesis by identifying trends in presentation and surgical treatment in patients with DDH. This is a retrospective review of children born between 1997-2018 who were treated surgically for DDH at our sub- regional paediatric orthopaedic unit. Demographic data, risk factors, age of diagnosis and surgical treatments were analysed. Late diagnosis was defined as greater than 4 months. 103 children (14 male, 89 female) underwent surgery. 93 hips were operated for dislocation and 21 for dysplasia. 13 patients presented with bilateral hip dislocations. The median age at diagnosis was 10 months (95% CI: 4-15). 62/103 (60.2%) were diagnosed late (after 4 months) and the median age for diagnosis in this group was 18.5 months (95% CI: 16-20.5). Significantly more patients were referred late (p=0.0077). The presence of risk factors (breech presentation or family history) was associated with early diagnosis. Over the duration of our study the operation rate per 1000 live births gradually increased, and on Poisson regression analysis there was a statistically significant increasing trend towards late diagnosis in recent years (p=0.0237), which necessitated more aggressive surgical management. In the UK, the current selective sonographic screening programme for DDH has shown a deterioration over the years of this study and this questions its current effectiveness. It appears that the majority of irreducible hip dislocations are diagnosed late, with an increased need for surgical management.

Neonatal screening and selective sonographic imaging in the diagnosis of developmental dysplasia of the hip.

Aims: The aim of this prospective cohort study was to evaluate the effectiveness of the neonatal hip instability screening programme. Patients and Methods: The study involved a four-year observational assessment of a neonatal hip screening programme. All newborns were examined using the Barlow or Ortolani manoeuvre within 72 hours of birth; those with positive findings were referred to a 'one-stop' screening clinic for clinical and sonographic assessment of the hip. The results were compared with previous published studies from this unit. Results: A total of 124 newborns with a positive Barlow or Ortolani manoeuvre, clunk positive, or 'unstable' were referred. Five were found to have clinical instability of the hip. Sonographically, 92 newborns had Graf Type I hips, 12 had Graf Type II hips, and 20 had Graf Type IV hips. The positive predictive value (PPV) of clinical screening was 4.0% and the PPV of sonography was 16.1%. This has led to an increased rate of surgery for DDH. Conclusion: Compared with previously published ten-year and 15-year studies, there has been a marked deterioration in the PPV in those referred with potential instability of the hip. There appears to be a paradox, with rising referrals and a decreasing PPV combined with an increasing rate of surgery in newborns with developmental dysplasia of the hip. Cite this article: Bone Joint J 2018;100-B:806-10.

The positive predictive value of asymmetrical skin creases in the diagnosis of pathological developmental dysplasia of the hip.

Aims: The aim of this study was to identify the association between asymmetrical skin creases of the thigh, buttock or inguinal region and pathological developmental dysplasia of the hip (DDH). Patients and Methods: Between 1 January 1996 and 31 December 2016, all patients referred to our unit from primary or secondary care with risk factors for DDH were assessed in a "one stop" clinic. All had clinical and sonographic assessment by the senior author (RWP) with the results being recorded prospectively. The inclusion criteria for this study were babies and children referred with asymmetrical skin creases. Those with a neurological cause of DDH were excluded. The positive predictive value (PPV) for pathological DDH was calculated. Results: A total of 105 patients met the inclusion criteria. There were 71 girls and 34 boys. Only two were found to have pathological DDH. Both also had unilateral limited abduction of the hip in flexion and a positive Galeazzi sign with apparent leg-length discrepancy. Thus, if the specialist examination of a patient with asymmetrical skin creases was normal, the PPV for DDH was 0%. Conclusion: Isolated asymmetrical skin creases are an unreliable clinical sign in the diagnosis of pathological DDH. Greater emphasis should be placed on the presence of additional clinical signs to guide radiological screening in babies and children. Cite this article: Bone Joint J 2018;100-B:675-9.

Clicky hip alone is not a true risk factor for developmental dysplasia of the hip.

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. PATIENTS AND METHODS: A total of 362 infants from 1997 to 2016 were referred with clicky hips to our 'one-stop' paediatric hip screening clinic. Hips were assessed clinically for instability and by ultrasound imaging using a simplified Graf/Harcke classification. Dislocated or dislocatable hips were classified as Graf Type IV hips. RESULTS: The mean age at presentation was 13.8 weeks (12.8 to 14.7). In all 351 out of 362 children (97.0%) had Graf Type I hips (normal) that required no treatment. Nine children (2.5%) had Graf Type II hips but all resolved to Graf Type I hips on follow-up scans. One child (0.3%) had Graf Type III hip dysplasia and one child (0.3%) had an irreducible hip dislocation. The two pathological hips were associated with unilateral limited hip abduction. Mean referrals increased from 12.9 to 23.3 each year (p = 0.002) from the first decade of the study to the second, driven by increasing primary care referrals (5.5 versus 16.7 per year, p < 0.001). CONCLUSION: Most clicky hips required no treatment other than reassurance to parents. Clicky hips with a normal hip examination should be considered a variant of normal childhood and not a risk factor for DDH. However, an abnormal hip examination including unilateral limited hip abduction should prompt urgent further investigations. Cite this article: Bone Joint J 2017;99-B:1533-6.

'The psychological problem is looking at you, are you looking for it?': psychological associations with symptomatic musculoskeletal referrals.

It is important that paediatric orthopaedic surgeons recognise that psychological conditions may present with musculoskeletal symptoms. Identification through careful and sensitive history taking is vital. There is a lack of training in this regard in paediatric orthopaedics. We present a series of cases initially referred for a musculoskeletal complaint. Further probing revealed an underlying psychological problem which was neither picked up by the referring physician nor volunteered by the patient. In our opinion, it is important that this training omission is addressed as such psychological problems may be devastating for these individuals and their families.

Neonatal clinical screening of the hip in the diagnosis of developmental dysplasia of the hip: a 15-year prospective longitudinal observational study.

Over a 15-year prospective period, 201 infants with a clinically unstable hip at neonatal screening were subsequently reviewed in a 'one stop' clinic where they were assessed clinically and sonographically. Their mean age was 1.62 weeks (95% confidence interval (CI) 1.35 to 1.89). Clinical neonatal hip screening revealed a sensitivity of 62% (mean, 62.6 95%CI 50.9 to 74.3), specificity of 99.8% (mean, 99.8, 95% CI 99.7 to 99.8) and positive predictive value (PPV) of 24% (mean, 26.2, 95% CI 19.3 to 33.0). Static and dynamic sonography for Graf type IV dysplastic hips had a 15-year sensitivity of 77% (mean, 75.8 95% CI 66.9 to 84.6), specificity of 99.8% (mean, 99.8, 95% CI 99.8 to 99.8) and a PPV of 49% (mean, 55.1, 95% CI 41.6 to 68.5). There were 36 infants with an irreducible dislocation of the hip (0.57 per 1000 live births), including six that failed to resolve with neonatal splintage. Most clinically unstable hips referred to a specialist clinic are female and stabilise spontaneously. Most irreducible dislocations are not identified from this neonatal instability group. There may be a small subgroup of females with instability of the hip which may be at risk of progression to irreducibility despite early treatment in a Pavlik harness. A controlled study is required to assess the value of neonatal clinical screening programmes.

Is congenital talipes equinovarus a risk factor for pathological dysplasia of the hip? : a 21-year prospective, longitudinal observational study.

There is controversy whether congenital foot abnormalities are true risk factors for pathological dysplasia of the hip. Previous United Kingdom screening guidelines considered congenital talipes equinovarus (CTEV) to be a risk factor for hip dysplasia, but present guidelines do not. We assessed the potential relationship between pathological dysplasia of the hip and fixed idiopathic CTEV. We present a single-centre 21-year prospective longitudinal observational study. All fixed idiopathic CTEV cases were classified (Harrold and Walker Types 1 to 3) and the hips clinically and sonographically assessed. Sonographic Graf Type III, IV and radiological irreducible hip dislocation were considered to be pathological hip dysplasia. Over 21 years there were 139 children with 199 cases of fixed idiopathic CTEV feet. Sonographically, there were 259 normal hips, 18 Graf Type II hips, 1 Graf Type III hip and 0 Graf Type IV hip. There were no cases of radiological or sonographic irreducible hip dislocation. Fixed idiopathic CTEV should not be considered as a significant risk factor for pathological hip dysplasia. This conclusion is in keeping with the current newborn and infant physical examination guidelines in which the only risk factors routinely screened are family history and breech presentation. Our findings suggest CTEV should not be considered a significant risk factor in pathological dysplasia of the hip.

Is limitation of hip abduction a useful clinical sign in the diagnosis of developmental dysplasia of the hip?

AIM: The relationship between the presence and severity of sonographically diagnosed developmental dysplasia of the hip (DDH) and the clinical abnormality of limitation of hip abduction (LHA) was investigated. METHODS: A prospective, longitudinal, selective 'at risk' and neonatal instability hip ultrasound programme between 1 January 1996 and 31 December 2005. 2876 neonates/infants were initially screened for DDH by clinical examination and by hip ultrasound imaging. Pathological sonographically evaluated DDH was considered to be Graf Type III, IV and irreducible hip dislocation. Inclusion criteria were cases of unilateral or bilateral limitation of hip abduction hip. EXCLUSION CRITERIA: syndromal, neuromuscular and skeletal dysplasia cases. RESULTS: 492 children presented with LHA (55 unilateral LHA). The mean age of neonates/infants with either unilateral or bilateral LHA was significantly higher than those without (p<0.001). In the sonographic diagnosis of Graf Type III and IV dysplasias, unilateral LHA had a PPV of 40% compared with only 0.3% for bilateral LHA. The sensitivity of unilateral LHA increased to 78.3% and a PPV 54.7% after the age of 8 weeks for Graf Types III, IV and irreducible hip dislocation. CONCLUSIONS: This study identifies a time-dependent association with unilateral LHA in the diagnosis of 'pathological' DDH after the age of 8 weeks. The presence of bilateral LHA in the young infant may be a normal variant and is an inaccurate clinical sign in the diagnosis of pathological DDH. LHA should be actively sought after 8 weeks of age and if present should be followed by a formal ultrasound or radiographic examination to confirm whether or not the hip is developing in a satisfactory manner.

The inter-relationship of clinical parameters in congenital talipes equinovarus: relevance to pathological anatomy and clinical classification.

BACKGROUND: The clinical features that define congenital talipes equinovarus (CTEV) are the presence of four principal components, equinus, varus, adductus and cavus. Classification systems in CTEV often include a form of assessment of these components and also other concurrent clinical parameters which feature in the condition. METHODS: Over a 14-year period from 1992 to 2006, 95 consecutive cases of CTEV were prospectively assessed and data recorded in order to investigate the relationships between the clinical parameters in CTEV and to compare these relationships with those that one would expect from our knowledge of the pathological anatomy and mechanics of the condition, relating these findings to the commonly used systems for classification. RESULTS: Ninety-five cases of CTEV had failed conservative treatment and had undergone surgical release. The mean age at surgical release and assessment was 9 months. Cluster analysis demonstrated that there were, broadly, two groups of patients. The first group was those patients with a greater equinus deformity (greater than 31°). This group had a greater adductus deformity and the presence of other parameters indicating increased severity (multiplanar stiffness with the presence of cavus and medial skin crease). The second group was those patients with a smaller equinus deformity (less than 31°) who were more heterogenous with regards to the other parameters. CONCLUSIONS: We analysed on a statistical basis the relevant aspects of the deformity in CTEV. We have demonstrated that there are certain parameters, namely, equinus and adductus, whose severity can reasonably predict the severity of other components of the deformity. With regards to hindfoot parameters, increased equinus is related to less sagittal plane reducibility and to stiff hindfoot varus (coronal plane stiffness). In terms of midfoot parameters, the degree of adductus is related to the presence of cavus deformity and the presence of a medial skin crease is associated with less reducibility of the adductus (axial plane stiffness). This is consistent with our current understanding of the pathological anatomy of CTEV and bears implications with regards to treatment and the design of proposed classification systems that are in use.

The relationship between mode of delivery and developmental dysplasia of the hip in breech infants: a four-year prospective cohort study.

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.

Neonatal foot deformities and their relationship to developmental dysplasia of the hip: an 11-year prospective, longitudinal observational study.

In a prospective study over 11 years we assessed the relationship between neonatal deformities of the foot and the presence of ultrasonographic developmental dysplasia of the hip (DDH). Between 1 January 1996 and 31 December 2006, 614 infants with deformities of the foot were referred for clinical and ultrasonographic evaluation. There were 436 cases of postural talipes equinovarus deformity (TEV), 60 of fixed congenital talipes equinovarus (CTEV), 93 of congenital talipes calcaneovalgus (CTCV) and 25 of metatarsus adductus. The overall risk of ultrasonographic dysplasia or instability was 1:27 in postural TEV, 1:8.6 in CTEV, 1:5.2 in CTCV and 1:25 in metatarsus adductus. The risk of type-IV instability of the hip or irreducible dislocation was 1:436 (0.2%) in postural TEV, 1:15.4 (6.5%) in CTCV and 1:25 (4%) in metatarsus adductus. There were no cases of hip instability (type IV) or of irreducible dislocation in the CTEV group. Routine screening for DDH in cases of postural TEV and CTEV is no longer advocated. The former is poorly defined, leading to the over-diagnosis of a possibly spurious condition. Ultrasonographic imaging and surveillance of hips in infants with CTCV and possibly those with metatarsus adductus should continue.

Developmental dysplasia of the hip: ultrasound screening and treatment. How are they related?

An assessment of the current clinical and ultrasound screening programmes. Early treatment by splintage is advocated for instability and Graf type III dysplasia of the hip.

Bone and joint tuberculosis: cases in Blackburn between 1988 and 2005.

We report 79 cases of bone and joint tuberculosis between 1988 and 2005, eight of which were in the Caucasian population and 71 in the non-white population. The diagnosis was made in the majority (73.4%) by positive bacteriology and/or histology. The mean age at the time of diagnosis was higher in the Caucasian group at 51.5 years (28 to 66) than in the South Asian group at 36.85 years (12 to 93). Only one patient had previous BCG immunisation. The spine was the site most commonly affected (44.3%). Surgical stabilisation and/or decompression was performed in 23% of these cases because of cord compression on imaging or the presence of neurological signs. A six-month course of chemotherapy comprising of an initial two months of rifampicin, isoniazide, pyrazinamide and sometimes ethambutol followed by four months treatment with rifampicin and isoniazide, was successful in all cases without proven drug resistance.

Non traumatic dislocation of the patella in children: the case for a dysplastic aetiology.

Congenital dislocation of the patella may be a phenotype, which has several genotypes, i.e. it may be congenital or acquired. This hypothesis is suggested with three illustrative case studies. Risk factors predictive of the development of irreducible dislocation of the patella in infancy are suggested. As there is a spectrum of possible aetiologies, 'late' diagnosis and a number of 'risk' factors but a common pathology, the word 'congenital' is not always appropriate. We suggest 'Developmental dysplasia and dislocation of the patella (DDDP)' is a more appropriate term for this rare condition.

The significance of at-risk factors in ultrasound surveillance of developmental dysplasia of the hip. A ten-year prospective study.

Of the 34,723 infants born between 1 June 1992 and 31 May 2002, the hips of 2578 with clinical instability or at-risk factors for developmental dysplasia of the hip were imaged by ultrasound. Instability of the hip was present in 77 patients, of whom only 24 (31.2%) had an associated risk factor. From the 'at-risk' groups, the overall risk of type-III dysplasia, instability and irreducibility was 1:15 when family history, 1:27 when breech delivery and 1:33 when foot deformity were considered as risk factors. Of those hips which were ultrasonographically stable, 88 had type-III dysplasia. A national programme of selective ultrasound screening of at-risk factors for the diagnosis of hip dislocation or instability alone cannot be recommended because of its low predictive value (1:88). However, the incidence of type-III dysplasia and hip dislocation or dislocatability in the groups with clinical instability, family history, breech position and possibly postural foot deformity as risk factors could justify a programme of selective ultrasound imaging.

Instability of the neonatal hip: the role of early or late splintage.

From January 1992 to December 1997, 59 dislocatable hips (37 children), confirmed by ultrasound, were splinted by Pavlik bracing within 2 weeks of birth. The majority were splinted within 1 week. From January 1998 to December 1999, 16 dislocatable hips (11 children) were evaluated by ultrasound and were not initially splinted but were followed up by serial ultrasound. They were splinted if they did not spontaneously stabilise on ultrasound. None of those patients treated by immediate splintage between 1992 and 1997 have subsequently required surgical intervention. Two of the infants treated between 1998 and 1999 (12% of the hips) later required a surgical procedure on the hip joint or proximal femur (p=0.049, Fisher's exact test). Nine out of 16 hips required splintage at a later date. We would not advocate delayed observation by ultrasound of unstable hips after 2 weeks of age. A clinically unstable hip should be assessed by ultrasound within 2 weeks of birth. If it is unstable on ultrasound, it should be splinted for 6 weeks. There is no need to wean off the splint.

Congenital and irreducible non-traumatic dislocation of the patella--a modified soft tissue procedure.

Many procedures have been described for the correction of congenital dislocation of the patella. Most operations entail extensive soft tissue releases through curved skin incisions. A modification of the Langenskiold and Ritsila procedure is described. The main alterations include a limited and straight anterior skin incision, a fashioning of a 'buckle' of the transferred distal-patellar tendon to a distally based flap and the transposition of an extensive medial rotation flap. This report describes the early results (mean=19 months) of the modified procedure in three cases of congenital lateral dislocation of the patella.