Early detection of developmental dysplasia of the hip (DDH) is associated with improved outcomes of conservative treatment. Therefore, we aimed to evaluate a novel screening programme that included both the primary risk factors of breech presentation and family history, and the secondary risk factors of oligohydramnios and foot deformities. A five-year prospective registry study investigating every live birth in the study’s catchment area (n = 27,731), all of whom underwent screening for risk factors and examination at the newborn and six- to eight-week neonatal examination and review. DDH was diagnosed using ultrasonography and the Graf classification system, defined as grade IIb or above or rapidly regressing IIa disease (≥4o at four weeks follow-up). Multivariate odds ratios were calculated to establish significant association, and risk differences were calculated to provide quantifiable risk increase with DDH, positive predictive value was used as a measure of predictive efficacy. The cost-effectiveness of using these risk factors to predict DDH was evaluated using NHS tariffs (January 2021).Aims
Methods
Aims. To investigate the risk factors for unsuccessful radial head reduction (RHR) in children with chronic Monteggia fractures (CMFs) treated surgically. Methods. A total of 209 children (mean age 6.84 years (SD 2.87)), who underwent surgical treatment for CMFs between March 2015 and March 2023 at six institutions, were retrospectively reviewed. Assessed risk factors included age, sex, laterality, dislocation direction and distance, preoperative proximal radial metaphysis width, time from injury to surgery, reduction method, annular ligament reconstruction, radiocapitellar joint fixation, ulnar osteotomy, site of ulnar osteotomy, preoperative and postoperative ulnar angulation, ulnar fixation method, progressive ulnar distraction, and postoperative cast immobilization. Independent-samples t-test, chi-squared test, and
Aims. Medial humeral epicondyle fractures (MHEFs) are common elbow fractures in children. Open reduction should be performed in patients with MHEF who have entrapped intra-articular fragments as well as displacement. However, following open reduction, transposition of the ulnar nerve is disputed. The aim of this study is to evaluate the need for ulnar nerve exploration and transposition. Methods. This was a retrospective cohort study. The clinical data of patients who underwent surgical treatment of MHEF in our hospital from January 2015 to January 2022 were collected. The patients were allocated to either transposition or non-transposition groups. Data for sex, age, cause of fracture, duration of follow-up, Papavasiliou and Crawford classification, injury-to-surgery time, preoperative ulnar nerve symptoms, intraoperative exploration of ulnar nerve injury, surgical incision length, intraoperative blood loss, postoperative ulnar nerve symptoms, complications, persistent ulnar neuropathy, and elbow joint function were analyzed. Binary
Aims. Radiological residual acetabular dysplasia (RAD) has been reported in up to 30% of children who had successful brace treatment of infant developmental dysplasia of the hip (DDH). Predicting those who will resolve and those who may need corrective surgery is important to optimize follow-up protocols. In this study we have aimed to identify the prevalence and predictors of RAD at two years and five years post-bracing. Methods. This was a single-centre, prospective longitudinal cohort study of infants with DDH managed using a published, standardized Pavlik harness protocol between January 2012 and December 2016. RAD was measured at two years’ mean follow-up using acetabular index-lateral edge (AI-L) and acetabular index-sourcil (AI-S), and at five years using AI-L, AI-S, centre-edge angle (CEA), and acetabular depth ratio (ADR). Each hip was classified based on published normative values for normal, borderline (1 to 2 standard deviations (SDs)), or dysplastic (> 2 SDs) based on sex, age, and laterality. Results. Of 202 infants who completed the protocol, 181 (90%) had two and five years’ follow-up radiographs. At two years, in 304 initially pathological hips, the prevalence of RAD (dysplastic) was 10% and RAD (borderline) was 30%. At five years, RAD (dysplastic) decreased to 1% to 3% and RAD (borderline) decreased to < 1% to 2%. On
Aims. The Uppföljningsprogram för cerebral pares (CPUP) Hip Score distinguishes between children with cerebral palsy (CP) at different levels of risk for displacement of the hip. The score was constructed using data from Swedish children with CP, but has not been confirmed in any other population. The aim of this study was to determine the calibration and discriminatory accuracy of this score in children with CP in Scotland. Methods. This was a total population-based study of children registered with the Cerebral Palsy Integrated Pathway Scotland. Displacement of the hip was defined as a migration percentage (MP) of > 40%. Inclusion criteria were children in Gross Motor Function Classification System (GMFCS) levels III to V. The calibration slope was estimated and Kaplan-Meier curves produced for five strata of CPUP scores to compare the observed with the predicted risk of displacement of the hip at five years. For discriminatory accuracy, the time-dependent area under the receiver operating characteristic curve (AUC) was estimated. In order to analyze differences in the performance of the score between cohorts, score weights, and subsequently the AUC, were re-estimated using the variables of the original score: the child’s age at the first examination, GMFCS level, head shaft angle, and MP of the worst hip in a
Aims. The most important complication of treatment of developmental dysplasia of the hip (DDH) is avascular necrosis (AVN) of the femoral head, which can result in proximal femoral growth disturbances leading to pain, dysfunction, and eventually to early onset osteoarthritis. In this study, we aimed to identify morphological variants in hip joint development that are predictive of a poor outcome. Methods. We retrospectively reviewed all patients who developed AVN after DDH treatment, either by closed and/or open reduction, at a single institution between 1984 and 2007 with a minimal follow-up of eight years. Standard pelvis radiographs obtained at ages one, two, three, five, and eight years, and at latest follow-up were retrieved. The Bucholz-Ogden classification was used to determine the type of AVN on all radiographs. Poor outcome was defined by Severin classification grade 3 or above on the latest follow-up radiographs and/or the need for secondary surgery. With statistical shape modelling, we identified the different shape variants of the hip at each age.
Aims. The aim of this study was to clarify the factors that predict the development of avascular necrosis (AVN) of the femoral head in children with a fracture of the femoral neck. Patients and Methods. We retrospectively reviewed 239 children with a mean age of 10.0 years (. sd. 3.9) who underwent surgical treatment for a femoral neck fracture. Risk factors were recorded, including age, sex, laterality, mechanism of injury, initial displacement, the type of fracture, the time to reduction, and the method and quality of reduction. AVN of the femoral head was assessed on radiographs.
We have reviewed our experience of the removal of deep extremity orthopaedic implants in children to establish the nature, rate and risk of complications associated with this procedure. A retrospective review was performed of 801 children who had 1223 implants inserted and subsequently removed over a period of 17 years. Bivariate analysis of possible predictors including clinical factors, complications associated with implant insertion and indications for removal and the complications encountered at removal was performed. A
Aims. The aim of this study was to assess whether supine flexibility predicts the likelihood of curve progression in patients with adolescent idiopathic scoliosis (AIS) undergoing brace treatment. Methods. This was a retrospective analysis of patients with AIS prescribed with an underarm brace between September 2008 to April 2013 and followed up until 18 years of age or required surgery. Patients with structural proximal curves that preclude underarm bracing, those who were lost to follow-up, and those who had poor compliance to bracing (<16 hours a day) were excluded. The major curve Cobb angle, curve type, and location were measured on the pre-brace standing posteroanterior (PA) radiograph, supine whole spine radiograph, initial in-brace standing PA radiograph, and the post-brace weaning standing PA radiograph. Validation of the previous in-brace Cobb angle regression model was performed. The outcome of curve progression post-bracing was tested using a
We examined differences in the rate of open reduction, operating time, length of hospital stay and outcome between two groups of children with displaced supracondylar fractures of the humerus who underwent surgery either within 12 hours of the injury or later. There were 77 children with type-3 supracondylar fractures. Of these, in 43 the fracture was reduced and pinned within 12 hours and in 34 more than 12 hours after injury. Both groups were similar in regard to gender, age and length of follow-up. Bivariate and
Redisplacement is the most common complication
of immobilisation in a cast for the treatment of diaphyseal fractures
of the forearm in children. We have previously shown that the three-point
index (TPI) can accurately predict redisplacement of fractures of
the distal radius. In this prospective study we applied this index
to assessment of diaphyseal fractures of the forearm in children
and compared it with other cast-related indices that might predict
redisplacement. A total of 76 children were included. Their ages,
initial displacement, quality of reduction, site and level of the
fractures and quality of the casting according to the TPI, Canterbury
index and padding index were analysed.
Hip displacement, defined in this study as a
migration percentage (MP) of more than 40%, is a common, debilitating complication
of cerebral palsy (CP). In this prospective study we analysed the
risk of developing hip displacement within five years of the first
pelvic radiograph. . All children with CP in southern and western Sweden are invited
to register in the hip surveillance programme CPUP. Inclusion criteria
for the two groups in this study were children from the CPUP database
born between 1994 and 2009 with Gross Motor Function Classification
System (GMFCS) III to V. Group 1 included children who developed
hip displacement, group 2 included children who did not develop
hip displacement over a minimum follow-up of five years. A total
of 145 children were included with a mean age at their initial pelvic
radiograph of 3.5 years (0.6 to 9.7). The odds ratio for hip displacement was calculated for GMFCS-level,
age and initial MP and head-shaft angle. A risk score was constructed
with these variables using multiple
Aims. Our aim was to investigate the predictive factors for the development
of a rebound phenomenon after temporary hemiepiphysiodesis in children
with genu valgum. Patients and Methods. We studied 37 limbs with idiopathic genu valgum who were treated
with hemiepiphyseal stapling, and with more than six months remaining
growth at removal of the staples. All children were followed until
skeletal maturity or for more than two years after removal of the
staples. Results. On multivariate
There is a lack of high-quality research investigating outcomes of Ponseti-treated idiopathic clubfeet and correlation with relapse. This study assessed clinical and quality of life (QoL) outcomes using a standardized core outcome set (COS), comparing children with and without relapse. A total of 11 international centres participated in this institutional review board-approved observational study. Data including demographics, information regarding presentation, treatment, and details of subsequent relapse and management were collected between 1 June 2022 and 30 June 2023 from consecutive clinic patients who had a minimum five-year follow-up. The clubfoot COS incorporating 31 parameters was used. A regression model assessed relationships between baseline variables and outcomes (clinical/QoL).Aims
Methods
The gold standard for percutaneous Achilles tendon tenotomy during the Ponseti treatment for idiopathic clubfoot is a tenotomy with a No. 15 blade. This trial aims to establish the technique where the tenotomy is performed with a large-bore needle as noninferior to the gold standard. We randomized feet from children aged below 36 months with idiopathic clubfoot on a 1:1 basis in either the blade or needle group. Follow-up was conducted at three weeks and three months postoperatively, where dorsiflexion range, Pirani scores, and complications were recorded. The noninferiority margin was set at 4° difference in dorsiflexion range at three months postoperatively.Aims
Methods
The aim of this trial was to assess the cost-effectiveness of a soft bandage and immediate discharge, compared with rigid immobilization, in children aged four to 15 years with a torus fracture of the distal radius. A within-trial economic evaluation was conducted from the UK NHS and personal social services (PSS) perspective, as well as a broader societal point of view. Health resources and quality of life (the youth version of the EuroQol five-dimension questionnaire (EQ-5D-Y)) data were collected, as part of the Forearm Recovery in Children Evaluation (FORCE) multicentre randomized controlled trial over a six-week period, using trial case report forms and patient-completed questionnaires. Costs and health gains (quality-adjusted life years (QALYs)) were estimated for the two trial treatment groups. Regression was used to estimate the probability of the new treatment being cost-effective at a range of ‘willingness-to-pay’ thresholds, which reflect a range of costs per QALY at which governments are typically prepared to reimburse for treatment.Aims
Methods
We have reviewed, retrospectively, all children with a lower limb deformity who underwent an acute correction and lengthening with a monolateral fixator between 1987 and 1996. The patients were all under the age of 19 years and had a minimum follow-up of eight months after removal of the fixator. A total of 41 children had 57 corrections and lengthening. Their mean age was 11.3 years (3.2 to 18.7) and there were 23 girls and 18 boys. The mean maximum correction in any one plane was 23° (7 to 45). In 41 bony segments (either femur or tibia) a uniplanar correction was made while various combinations were carried out in 16. The site of the osteotomy was predominantly diaphyseal, at a mean of 47% (17% to 73%) of the total bone length and the mean length gained was 6.4 cm (1.0 to 17.0). Univariate analysis identified a moderately strong relationship between the bone healing index (BHI), length gained, maximum correction and grade-II to grade-III complications. For
The aim of this study was to evaluate the epidemiology and treatment of Perthes’ disease of the hip. This was an anonymized comprehensive cohort study of Perthes’ disease, with a nested consented cohort. A total of 143 of 144 hospitals treating children’s hip disease in the UK participated over an 18-month period. Cases were cross-checked using a secondary independent reporting network of trainee surgeons to minimize those missing. Clinician-reported outcomes were collected until two years. Patient-reported outcome measures (PROMs) were collected for a subset of participants.Aims
Methods
The aim of this study was to inform the epidemiology and treatment of slipped capital femoral epiphysis (SCFE). This was an anonymized comprehensive cohort study, with a nested consented cohort, following the the Idea, Development, Exploration, Assessment, Long-term study (IDEAL) framework. A total of 143 of 144 hospitals treating SCFE in Great Britain participated over an 18-month period. Patients were cross-checked against national administrative data and potential missing patients were identified. Clinician-reported outcomes were collected until two years. Patient-reported outcome measures (PROMs) were collected for a subset of participants.Aims
Methods
Torus fractures of the distal radius are the most common fractures in children. The NICE non-complex fracture guidelines recently concluded that bandaging was probably the optimal treatment for these injuries. However, across the UK current treatment varies widely due to a lack of evidence underpinning the guidelines. The Forearm Fracture Recovery in Children Evaluation (FORCE) trial evaluates the effect of a soft bandage and immediate discharge compared with rigid immobilization. FORCE is a multicentre, parallel group randomized controlled equivalence trial. The primary outcome is the Wong-Baker FACES pain score at three days after randomization and the primary analysis of this outcome will use a multivariate linear regression model to compare the two groups. Secondary outcomes are measured at one and seven days, and three and six-weeks post-randomization and include the Patient Reported Outcome Measurement Information System (PROMIS) upper extremity limb score, EuroQoL EQ-5D-Y, analgesia use, school absence, complications, and healthcare resource use. The planned statistical and health economic analyses for this trial are described here. The FORCE trial protocol has been published separately.Aims
Methods
We aimed to address the question on whether there is a place for shoulder stabilization surgery in patients who had voluntary posterior instability starting in childhood and adolescence, and later becoming involuntary and uncontrollable. Consecutive patients who had an operation for recurrent posterior instability before the age of 18 years were studied retrospectively. All patients had failed conservative treatment for at least six months prior to surgery; and no patients had psychiatric disorders. Two groups were identified and compared: voluntary posterior instability starting in childhood which became uncontrollable and involuntary (group VBI); and involuntary posterior instability (group I). Patients were reviewed and assessed at least two years after surgery by two examiners.Aims
Methods
Slipped capital femoral epiphysis (SCFE) is one of the most common hip diseases of adolescence that can cause marked disability, yet there is little robust evidence to guide treatment. Fundamental aspects of the disease, such as frequency, are unknown and consequently the desire of clinicians to undertake robust intervention studies is somewhat prohibited by a lack of fundamental knowledge. The study is an anonymized nationwide comprehensive cohort study with nested consented within the mechanism of the British Orthopaedic Surgery Surveillance (BOSS) Study. All relevant hospitals treating SCFE in England, Scotland, and Wales will contribute anonymized case details. Potential missing cases will be cross-checked against two independent external sources of data (the national administrative data and independent trainee data). Patients will be invited to enrich the data collected by supplementing anonymized case data with patient-reported outcome measures. In line with recommendations of the IDEAL Collaboration, the study will primarily seek to determine incidence, describe case mix and variations in surgical interventions, and explore the relationships between baseline factors (patients and types of interventions) and two-year outcomes.Aims
Methods
Displaced fractures of the lateral condyle of the humerus are
frequently managed surgically with the aim of avoiding nonunion,
malunion, disturbances of growth and later arthritis. The ideal
method of fixation is however not known, and treatment varies between
surgeons and hospitals. The aim of this study was to compare the
outcome of two well-established forms of surgical treatment, Kirschner
wire (K-wire) and screw fixation. A retrospective cohort study of children who underwent surgical
treatment for a fracture of the lateral condyle of the humerus between
January 2005 and December 2014 at two centres was undertaken. Pre,
intraoperative and postoperative characteristics were evaluated. A total of 336 children were included in the study. Their mean
age at the time of injury was 5.8 years (0 to 15) with a male:female
patient ratio of 3:2. A total of 243 (72%) had a Milch II fracture
and the fracture was displaced by > 2 mm in 228 (68%). In all, 235
patients underwent K-wire fixation and 101 had screw fixation. Aims
Patients and Methods
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. 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.Aims
Patients and Methods
The incidence of clinically significant avascular
necrosis (AVN) following medial open reduction of the dislocated
hip in children with developmental dysplasia of the hip (DDH) remains
unknown. We performed a systematic review of the literature to identify
all clinical studies reporting the results of medial open reduction
surgery. A total of 14 papers reporting 734 hips met the inclusion
criteria. The mean follow-up was 10.9 years (2 to 28). The rate
of clinically significant AVN (types 2 to 4) was 20% (149/734).
From these papers 221 hips in 174 children had sufficient information
to permit more detailed analysis. The rate of AVN increased with
the length of follow-up to 24% at skeletal maturity, with type 2
AVN predominating in hips after five years’ follow-up. The presence
of AVN resulted in a higher incidence of an unsatisfactory outcome
at skeletal maturity (55% Cite this article:
In 1994 a cerebral palsy (CP) register and healthcare
programme was established in southern Sweden with the primary aim
of preventing dislocation of the hip in these children. The results from the first ten years were published in 2005 and
showed a decrease in the incidence of dislocation of the hip, from
8% in a historical control group of 103 children born between 1990
and 1991 to 0.5% in a group of 258 children born between 1992 and
1997. These two cohorts have now been re-evaluated and an additional
group of 431 children born between 1998 and 2007 has been added. By 1 January 2014, nine children in the control group, two in
the first study group and none in the second study group had developed
a dislocated hip (p <
0.001). The two children in the first study
group who developed a dislocated hip were too unwell to undergo
preventive surgery. Every child with a dislocated hip reported severe pain,
at least periodically, and four underwent salvage surgery. Of the
689 children in the study groups, 91 (13%) underwent preventive
surgery. A population-based hip surveillance programme enables the early
identification and preventive treatment, which can result in a significantly
lower incidence of dislocation of the hip in children with CP. Cite this article:
We present the clinical and radiographic outcome of 81 children
with Gartland type I to III supracondylar humeral fractures at a
minimum follow-up of ten years (mean 12.1 years; 10.3 to 16.1) following
injury. The clinical and functional outcomes are compared with normal
age- and gender-matched individuals. The population-based study
setting was first identified from the institutional registries;
the rate of participation was 76%. Controls were randomly selected
from Finnish National Population Registry.Aims
Patients and Methods
Forearm fractures in children have a tendency
to displace in a cast leading to malunion with reduced functional
and cosmetic results. In order to identify risk factors for displacement,
a total of 247 conservatively treated fractures of the forearm in
246 children with a mean age of 7.3 years ( Fractures of both forearm bones in children have a strong tendency
to displace even in an above-elbow cast. Severe fractures of the
non-dominant arm are at highest risk for displacement. Radiographs
at set times during treatment might identify early displacement,
which should be treated before malunion occurs, especially in older children
with less potential for remodelling. Cite this article:
This nationwide prospective study was designed to determine prognostic factors and evaluate the outcome of different treatments of Perthes’ disease. A total of 28 hospitals in Norway were instructed to report all new cases of Perthes’ disease over a period of five years and 425 patients were reported and followed for five years. Of these, 368 with unilateral disease were included in the present study. The hips were classified radiologically according to a modified two-group Catterall classification and the lateral pillar classification. A total of 358 patients (97%) attended the five-year follow-up, when a modified three-group Stulberg classification was used as a radiological outcome measure. For patients over six years of age at diagnosis and with more than 50% necrosis of the femoral head (152 patients), the surgeons at the different hospitals had chosen one of three methods of treatment: physiotherapy (55 patients), the Scottish Rite abduction orthosis (26), and proximal femoral varus osteotomy (71). Of these hips, 146 (96%) were available for the five-year follow-up. The strongest predictor of outcome was femoral head involvement of more or less than 50% (odds ratio (OR) = 7.76, 95% confidence interval (CI) 2.82 to 21.37), followed by age at diagnosis (OR = 0.98, 95% CI 0.92 to 0.99) and the lateral pillar classification (OR = 0.62, 95% CI 0.40 to 0.98). In children over six years at diagnosis with more than 50% of femoral head necrosis, proximal femoral varus osteotomy gave a significantly better outcome than orthosis (p = 0.001) or physiotherapy (p = 0.001). There was no significant difference between the physiotherapy and orthosis groups (p = 0.36), and we found no difference in outcome after any of the treatments in children under six years (p = 0.73). We recommend proximal femoral varus osteotomy in children aged six years and over at the time of diagnosis with hips having more than 50% femoral head necrosis. The abduction orthosis should be abandoned in Perthes’ disease.
The aim of this prospective study was to investigate
prematurity as a risk factor for developmental dysplasia of the hip
(DDH). The hips of 221 infants (88 female, 133 male, mean age 31.11
weeks; standard deviation ( These results suggests that prematurity is not a predisposing
factor for DDH. Cite this article:
A small proportion of children with Gartland
type III supracondylar humeral fracture (SCHF) experience troubling limited
or delayed recovery after operative treatment. We hypothesised that
the fracture level relative to the isthmus of the humerus would
affect the outcome. We retrospectively reviewed 230 children who underwent closed
reduction and percutaneous pinning (CRPP) for their Gartland type
III SCHFs between March 2003 and December 2012. There were 144 boys
and 86 girls, with the mean age of six years (1.1 to 15.2). The
clinico-radiological characteristics and surgical outcomes (recovery
of the elbow range of movement, post-operative angulation, and the
final Flynn grade) were recorded. Multivariate analysis was employed
to identify prognostic factors that influenced outcome, including
fracture level. Multivariate analysis revealed that a fracture below
the humeral isthmus was significantly associated with poor prognosis
in terms of the range of elbow movement (p <
0.001), angulation
(p = 0.001) and Flynn grade (p = 0.003). Age over ten years was also
a poor prognostic factor for recovery of the range of elbow movement (p
= 0.027). This is the first study demonstrating a subclassification system
of Gartland III fractures with prognostic significance. This will
guide surgeons in peri-operative planning and counselling as well
as directing future research aimed at improving outcomes. Cite this article:
Two types of fracture, early and late, have been
reported following limb lengthening in patients with achondroplasia (ACH)
and hypochondroplasia (HCH). We reviewed 25 patients with these conditions who underwent 72
segmental limb lengthening procedures involving the femur and/or
tibia, between 2003 and 2011. Gender, age at surgery, lengthened
segment, body mass index, the shape of the callus, the amount and
percentage of lengthening and the healing index were evaluated to determine
predictive factors for the occurrence of early (within three weeks
after removal of the fixation pins) and late fracture (>
three weeks
after removal of the pins). The Mann‑Whitney U test and Pearson’s
chi-squared test for univariate analysis and stepwise regression
model for multivariate analysis were used to identify the predictive factor
for each fracture. Only one patient (two tibiae) was excluded from
the analysis due to excessively slow formation of the regenerate,
which required supplementary measures. A total of 24 patients with
70 limbs were included in the study. There were 11 early fractures in eight patients. The shape of
the callus (lateral or central callus) was the only statistical
variable related to the occurrence of early fracture in univariate
and multivariate analyses. Late fracture was observed in six limbs
and the mean time between removal of the fixation pins and fracture
was 18.3 weeks (3.3 to 38.4). Lengthening of the tibia, larger healing
index, and lateral or central callus were related to the occurrence
of a late fracture in univariate analysis. A multivariate analysis
demonstrated that the shape of the callus was the strongest predictor
for late fracture (odds ratio: 19.3, 95% confidence interval: 2.91
to 128). Lateral or central callus had a significantly larger risk
of fracture than fusiform, cylindrical, or concave callus. Radiological monitoring of the shape of the callus during distraction
is important to prevent early and late fracture of lengthened limbs
in patients with ACH or HCH. In patients with thin callus formation,
some measures to stimulate bone formation should be considered as
early as possible. Cite this article:
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 evaluated radiological hip remodelling after shelf acetabuloplasty and sought to identify prognostic factors in 25 patients with a mean age of 8.9 years (7.0 to 12.3) who had unilateral Perthes’ disease with reducible subluxation of the hip in the fragmentation stage. At a mean follow-up of 6.7 years (3.2 to 9.0), satisfactory remodelling was observed in 18 hips (72%). The type of labrum in hip abduction, as determined by intra-operative dynamic arthrography, was found to be a statistically significant prognostic factor (p = 0.012). Shelf acetabuloplasty as containment surgery seems to be best indicated for hips in which there is not marked collapse of the epiphysis and in which the extruded epiphyseal segment slips easily underneath the labrum on abduction, without imposing undue pressure on the lateral edge of the acetabulum.
The role of heritable thrombophilic risk factors in the pathogenesis of the Perthes’ disease is controversial. The clinical and radiological findings of Perthes’ disease may be indistinguishable from those of Gaucher’s disease, and the most common Jewish N370S Gaucher mutation is threefold greater in patients with Perthes’ disease. Familial osteonecrosis of the femoral head is associated with variant mutations of collagen type II (COL2A1 mutations). We therefore studied the potential role of genetic thrombophilia and the Gaucher and COL2A1 mutations in children with Perthes’ disease. Genomic DNA of 119 children with radiologically-confirmed Perthes’ disease diagnosed between 1986 and 2005 was analysed for the thrombophilic polymorphisms Factor V Leiden, 677T-MTHFR and FIIG20210A. The results were compared with those of a group of 276 children without Perthes’ disease. DNA was also analysed for the Gaucher mutations N370S, G insertion (84GG), L444P, Intron 2 (IVS2+1G>
A) and R496H. Enzymic assays confirmed the Gaucher disease status. Collagen (COL2A1) mutations of the 12q13 gene were also analysed. The prevalence of thrombophilic markers was similar among the 119 patients with Perthes’ disease and the 276 control subjects. The prevalence of the Gaucher mutation was consistent with Israeli population carriership data and did not confirm an earlier-claimed association with Perthes’ disease. All 199 patients were negative for the studied COL2A1 mutations. We found no genetic association between Perthes’ disease and either Gaucher’s disease or COL2A1 mutations or increased genetic thrombophilia among our patients compared with the control group. A systematic review of case-control studies suggested that there was a positive association between Perthes’ disease and Factor V Leiden. The impact of this association upon the disease, although not consistent across the studies, remains unclear.