The aim of this study was to investigate the incidence of dysplasia in the ‘normal’ contralateral hip in patients with unilateral developmental dislocation of the hip (DDH) and to evaluate the long-term prognosis of such hips. A total of 48 patients (40 girls and eight boys) were treated for late-detected unilateral DDH between 1958 and 1962. After preliminary skin traction, closed reduction was achieved at a mean age of 17.8 months (4 to 65) in all except one patient who needed open reduction. In 25 patients early derotation femoral osteotomy of the contralateral hip had been undertaken within three years of reduction, and later surgery in ten patients. Radiographs taken during childhood and adulthood were reviewed. The mean age of the patients was 50.9 years (43 to 55) at the time of the latest radiological review. In all, eight patients (17%) developed dysplasia of the contralateral hip, defined as a centre-edge (CE) angle < 20° during childhood or at skeletal maturity. Six of these patients underwent surgery to improve cover of the femoral head; the dysplasia improved in two after varus femoral osteotomy and in two after an acetabular shelf operation. During long-term follow-up the dysplasia deteriorated to subluxation in two patients (CE angles 4° and 5°, respectively) who both developed osteoarthritis (OA), and one of these underwent total hip replacement at the age of 49 years. In conclusion, the long-term prognosis for the contralateral hip was relatively good, as OA occurred in only two hips (4%) at a mean follow-up of 50 years. Regular review of the ‘normal’ side is indicated, and corrective surgery should be undertaken in those who develop subluxation. Cite this article: Bone Joint J 2014; 96-B:1161–6

Aims. The aim of this study was to investigate the changes in femoral trochlear morphology following surgical correction of recurrent patellar dislocation associated with trochlear dysplasia in children. Patients and Methods. A total of 23 patients with a mean age of 9.6 years (7 to 11) were included All had bilateral recurrent patellar dislocation associated with femoral trochlear dysplasia. The knee with traumatic dislocation at the time of presentation or that had dislocated most frequently was treated with medial patellar retinacular plasty (Group S). The contralateral knee served as a control and was treated conservatively (Group C). All patients were treated between October 2008 and August 2013. The mean follow-up was 48.7 months (43 to 56). Axial CT scans were undertaken in all patients to assess the trochlear morphological characteristics on a particular axial image which was established at the point with the greatest epicondylar width based on measurements preoperatively and at the final follow-up. Results. Preoperatively, there were no statistically significant differences between the trochlear morphology in the two groups (sulcus angle, p 0.852; trochlear groove depth, p 0.885; lateral trochlear inclination, p 0.676; lateral-to-medial facet ratio, p 0.468; lateral condylar height, p 0.899; medial condylar height, p 0.816). Many radiological parameters of trochlear morphology were significantly different between the two groups at the final follow-up, including well-known parameters, such as the mean sulcus angle (Group S, 146.27° (. sd. 7.18); Group C, 160.61° (. sd. 9.29); p < 0.001), the mean trochlear groove depth (Group S, 6.25 mm (. sd. 0.41); Group C, 3.48 mm (. sd. 0.65); p < 0.001) and the mean lateral trochlear inclination (Group S, 20.99° (. sd. 3.87); Group C, 12.18° (. sd. 1.85); p < 0.001). Lesser known parameters such as the ratio of the lateral to medial trochlear length (Group S, 1.46 (. sd. 0.19); Group C, 2.14 (. sd. 0.42); p < 0.001), which is a measurement of facet asymmetry, and the lateral and medial condylar height were also significantly different between the two groups (p < 0.001). Conclusion. The femoral trochlear morphology can be improved by early (before epiphyseal closure) surgical correction in children with recurrent patellar dislocation associated with femoral trochlear dysplasia. Cite this article: Bone Joint J 2018;100-B:811–21

1. The examination of fifty-one infants presenting signs of unilateral dysplasia (preluxation) of the hip disclosed the existence of an abduction-contracture in the opposite "normal" hip. 2. The abduction-contracture was not infrequently found in the newborn, but seemed to attract attention mostly between the second and fifth month of life. It was seldom found after the sixth month. 3. The dysplasia in the contralateral hip showed the usual features. 4. The relationship between the degree of abduction-contracture and the degree of dysplasia was a direct one only occasionally; in most cases they seemed unrelated to each other. 5. Observation of these patients showed that the abduction-contracture followed a constant course towards gradual and spontaneous regression. The dysplasia progressed in one of the following directions: spontaneous recovery, rapid or slow recovery with treatment by abduction splint, subluxation, or true dislocation. 6. It is submitted that the coexistence of unilateral dysplasia of the hip and abduction-contracture in the opposite hip is not fortuitous. It is believed that the abduction-contracture determines the development of the dysplasia in the opposite hip through the faulty mechanics caused by "fixed pelvic obliquity.". 7. The discovery of unilateral abduction-contracture soon after birth should be a warning that dysplasia may develop in the opposite hip. A careful watch should therefore be kept for signs of abduction-contracture and for the later development of dysplasia

In Japan, osteoarthritis (OA) of the hip secondary to acetabular dysplasia is very common, and there are few data concerning the pathogeneses and incidence of femoroacetabular impingement (FAI). We have attempted to clarify the radiological prevalence of painful FAI in a cohort of Japanese patients and to investigate the radiological findings. We identified 176 symptomatic patients (202 hips) with Tönnis grade 0 or 1 osteoarthritis, whom we prospectively studied between August 2011 and July 2012. There were 61 men (65 hips) and 115 women (137 hips) with a mean age of 51.8 years (11 to 83). Radiological analyses included the α-angle, centre–edge angle, cross-over sign, pistol grip deformity and femoral head neck ratio. Of the 202 hips, 79 (39.1%) had acetabular dysplasia, while 80 hips (39.6%) had no known aetiology. We found evidence of FAI in 60 hips (29.7%). Radiological FAI findings associated with cam deformity were the most common. There was a significant relationship between the pistol grip deformity and both the α-angle (p < 0.001) and femoral head–neck ratio (p = 0.024). Radiological evidence of symptomatic FAI was not uncommon in these Japanese patients. Cite this article: Bone Joint J 2013;96-B:172–6

We investigated the clinical and radiological outcome of trochleaplasty for recurrent patellar dislocation in association with trochlear dysplasia in 38 consecutive patients (45 knees) with a mean follow-up of 8.3 years (4 to 14). None had recurrence of dislocation after trochleaplasty. Post-operatively, patellofemoral pain, present pre-operatively in only 35 knees, became worse in 15 (33.4%), remained unchanged in four (8.8%) and improved in 22 (49%). Four knees which had no pain pre-operatively (8.8%) continued to have no pain. A total of 33 knees were available for radiological assessment. Post-operatively, all but two knees (93.9%) had correction of trochlear dysplasia radiologically but degenerative changes of the patellofemoral joint developed in 30% (10) of the knees. We conclude that recurrent patellar dislocation associated with trochlear dysplasia can be treated successfully by trochleaplasty, but the impact on patellofemoral pain and the development of patellofemoral osteoarthritis is less predictable. Overall, subjective patient satisfaction with restored patellofemoral stability after trochleaplasty appeared to outweigh its possible sequelae

Patients with skeletal dysplasia are prone to developing advanced osteoarthritis of the knee requiring total knee replacement (TKR) at a younger age than the general population. TKR in this unique group of patients is a technically demanding procedure owing to the deformity, flexion contracture, generalised hypotonia and ligamentous laxity. We retrospectively reviewed the outcome of 11 TKRs performed in eight patients with skeletal dysplasia at our institution using the Stanmore Modular Individualised Lower Extremity System (SMILES) custom-made rotating-hinge TKR. There were three men and five women with mean age of 57 years (41 to 79). Patients were followed clinically and radiologically for a mean of seven years (3 to 11.5). The mean Knee Society clinical and function scores improved from 24 (14 to 36) and 20 (5 to 40) pre-operatively, respectively, to 68 (28 to 80) and 50 (22 to 74), respectively, at final follow-up. Four complications were recorded, including a patellar fracture following a fall, a tibial peri-prosthetic fracture, persistent anterior knee pain, and aseptic loosening of a femoral component requiring revision. Our results demonstrate that custom primary rotating-hinge TKR in patients with skeletal dysplasia is effective at relieving pain, with a satisfactory range of movement and improved function. It compensates for bony deformity and ligament deficiency and reduces the likelihood of corrective osteotomy. Patellofemoral joint complications are frequent and functional outcome is worse than with primary TKR in the general population

We followed up 76 consecutive hips with symptomatic acetabular dysplasia treated by acetabular shelf augmentation for a mean period of 11 years. Survival analysis using conversion to hip replacement as an end-point was 86% at five years and 46% at ten years. Forty-four hips with slight or no narrowing of the joint space pre-operatively had a survival of 97% at five and 75% at ten years. This was significantly higher (p = 0.0007) than that of the 32 hips with moderate or severe narrowing of the joint-space, which was 76% at five and 22% at ten years. There was no significant relationship between survival and age (p = 0.37) or the pre- and post-operative centre-edge (p = 0.39) and acetabular angles (p = 0.85). Shelf acetabuloplasty is a reliable, safe procedure offering medium-term symptomatic relief for adults with acetabular dysplasia. The best results were achieved in patients with mild and moderate dysplasia of the hip with little arthritis

Since this paper was submitted for publication three additional reports have appeared, two of which represent typical examples of the syndrome. Husson and Parkman (1961) reported the case of a female infant dying at the age of four months with chondroectodermal dysplasia, obliteration of the upper labiogingival sulcus, and congenital heart disease. The heart lesions included anomalous pulmonary venous return, single atrium and a persistent left superior vena cava. There was no known consanguinity. In reviewing the cardiac lesions described in chondroectodermal dysplasia they include another case with anomalous pulmonary venous return described by Darling, Rothney and Craig (1957), which also showed chondroectodermal dysplasia and polydactyly, though details of the extracardiac malformations are not given. Nabrady (1961) described a four-year-old Hungarian girl with ectodermal defects involving the teeth and nails but not the hair, polydactyly, distal shortening of the extremities producing dwarfing, and typical radiological appearances of the long bones. There was presumptive evidence of a septal defect of the heart. There was no consanguinity, but the mother was considered to show a "forme fruste" of the condition. A necropsy report by Meitner (1961) of a newly born premature infant with multiple congenital malformations of organs of ectodermal, mesodermal and endodermal origin is of interest because these malformations included extreme polydactyly of hands and feet, absence of nails, chondrodysplasia, and trilocular heart. In many respects, however, the case is atypical of chondroectodermal dysplasia

1 . Twenty patients with fibrous dysplasia, confirmed histologically, are reported and discussed in regard to classification, etiology, pathogenesis and treatment. The various fibrous or fibrocystic lesions of bone are characterised briefly for purpose of contrast, and the position of fibrous dysplasia in this heterogeneous collection is suggested. 2. The classification of fibrous dysplasia based on the degree of skeletal involvement is used, and the diagnostic, therapeutic and prognostic implications of this classification emphasised. The authors endorse the opinion that fibrous dysplasia is a developmental defect. Clinical, histological and radiographic evidence is presented to point to the distinct evolution of the monostotic lesions, to which a positive and aggressive approach is recommended

Aims. Acetabular dysplasia is frequently associated with intra-articular pathology such as labral tears, but whether labral tears should be treated at the time of periacetabular osteotomy (PAO) remains controversial. The purpose of this study was to compare the clinical outcomes and radiographic corrections of PAO for acetabular dysplasia between patients with and without labral tears pre-operatively. Patients and Methods. We retrospectively reviewed 70 hips in 67 patients with acetabular dysplasia who underwent PAO. Of 47 hips (45 patients) with labral tears pre-operatively, 27 (25 patients) underwent PAO alone, and were classified as the labral tear alone (LT) group, and 20 (20 patients) underwent combined PAO and osteochondroplasty, and were classified as the labral tear osteochondroplasty (LTO) group. The non-labral tear (NLT) group included 23 hips in 22 patients. Results. There were no significant differences between groups for post-operative Harris hip scores, degree of progression of osteoarthritis or rate of reoperation. The pre-operative alpha angle was significantly larger in the LTO group compared with the other groups (p < 0.0001). Conclusion. PAO provides equivalent short-term relief of pain and functional outcome in patients with or without labral tears. The rate of progression of osteoarthritis and reoperation was not significantly increased in patients with labral tears. Take home message: PAO provides equivalent short-term pain relief and functional outcomes in patients with acetabular dysplasia with and without labral tears. We did not find significantly increased risks of progression of osteoarthritis or re-operation in those with labral tears. Cite this article: Bone Joint J 2016;98-B:741–6

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. Cite this article: Bone Joint J 2014;96-B:1553–5

Our aim was to determine whether abnormalities noted on MRI immediately after reduction for developmental dysplasia of the hip could predict the persistance of dysplasia and aid surgical planning. Scans of 13 hips in which acetabular dysplasia had resolved by the age of four years were compared with those of five which had required pelvic osteotomy for persisting dysplasia. The scans were analysed by two consultant musculoskeletal radiologists who were blinded to the outcome in each child. The postreduction scans highlighted a number of anatomical abnormalities secondary to developmental dysplasia of the hip, but statistical analysis showed that none were predictive of persisting acetabular dysplasia in the older child, suggesting that the factors which determine the long-term outcome were not visible on these images

Untreated acetabular dysplasia following treatment for developmental dysplasia of the hip (DDH) leads to early degenerative joint disease. Clinicians must accurately and reliably recognise dysplasia in order to intervene appropriately with secondary acetabular or femoral procedures. This study sought early predictors of residual dysplasia in order to establish empirically-based indications for treatment. DDH treated by closed or open reduction alone was reviewed. Residual hip dysplasia was defined according to the Severin classification at skeletal maturity. Future hip replacement in a subset of these patients was compared with the Severin classification. Serial measurements of acetabular development and subluxation of the femoral head were collected, as were the age at reduction, type of reduction, and Tonnis grade prior to reduction. These variables were used to predict the Severin classification. The mean age at reduction in 72 hips was 16 months (1 to 46). On the final radiograph, 47 hips (65%) were classified as Severin I/II, and 25 as Severin III/IV (35%). At 40 years after reduction, five of 43 hips (21%) had had a total hip replacement (THR). The Severin grade was predictive for THR. Early measurements of the acetabular index (AI) were predictive for Severin grade. For example, an AI of 35° or more at two years after reduction was associated with an 80% probability of becoming a Severin grade III/IV hip. This study links early acetabular remodelling, residual dysplasia at skeletal maturity and the long-term risk of THR. It presents evidence describing the diagnostic value of early predictors of residual dysplasia, and therefore, of the long-term risk of degenerative change

Objectives. To determine the pattern of mutations of the WISP3 gene in clinically identified progressive pseudorheumatoid dysplasia (PPD) in an Indian population. Patients and Methods. A total of 15 patients with clinical features of PPD were enrolled in this study. Genomic DNA was isolated and polymerase chain reaction performed to amplify the WISP3 gene. Screening for mutations was done by conformation-sensitive gel electrophoresis, beginning with the fifth exon and subsequently proceeding to the remaining exons. Sanger sequencing was performed for both forward and reverse strands to confirm the mutations. Results. In all, two of the 15 patients had compound heterozygous mutations: one a nonsense mutation c.156C>A (p.C52*) in exon 2, and the other a missense mutation c.677G>T (p.G226V) in exon 4. All others were homozygous, with three bearing a nonsense mutation c.156C>A (p.C52*) in exon 2, three a missense mutation c.233G>A (p.C78Y) in exon 2, five a missense mutation c.1010G>A (p.C337Y) in exon 5, one a nonsense mutation c.348C>A (p.Y116*) in exon 3, and one with a novel deletion mutation c.593_597delATAGA (p.Y198*) in exon 4. Conclusion. We identified a novel mutation c.593_597delATAGA (p.Y198*) in the fourth exon of the WISP3 gene. We also confirmed c.1010G>A as one of the common mutations in an Indian population with progressive pseudorheumatoid dysplasia. Cite this article: V. Madhuri, M. Santhanam, K. Rajagopal, L. K. Sugumar, V. Balaji. WISP3 mutational analysis in Indian patients diagnosed with progressive pseudorheumatoid dysplasia and report of a novel mutation at p.Y198* Bone Joint Res 2016;5:301–306. DOI: 10.1302/2046-3758.57.2000520

1. The occurrence of bilateral double-layer patellae in association with multiple epiphysial dysplasia is described in three siblings. 2. Twelve cases of bilateral double-layer patellae have been reported previously, and in all there was an accompanying skeletal dystrophy which, although not diagnosed as such, is likely to have been multiple epiphysial dysplasia. 3. It is suggested that although they occur only in a minority of cases, double-layer patellae when present are a feature of considerable diagnostic value in multiple epiphysial dysplasia. Lateral radiography of the knees may therefore be of assistance in the diagnosis of multiple epiphysial dysplasia

Aims. This study aimed to explore whether intraoperative nerve monitoring can identify risk factors and reduce the incidence of nerve injury in patients with high-riding developmental dysplasia. Patients and Methods. We conducted a historical controlled study of patients with unilateral Crowe IV developmental dysplasia of the hip (DDH). Between October 2016 and October 2017, intraoperative nerve monitoring of the femoral and sciatic nerves was applied in total hip arthroplasty (THA). A neuromonitoring technician was employed to monitor nerve function and inform the surgeon of ongoing changes in a timely manner. Patients who did not have intraoperative nerve monitoring between September 2015 and October 2016 were selected as the control group. All the surgeries were performed by one surgeon. Demographics and clinical data were analyzed. A total of 35 patients in the monitoring group (ten male, 25 female; mean age 37.1 years (20 to 46)) and 56 patients in the control group (13 male, 43 female; mean age 37.9 years (23 to 52)) were enrolled. The mean follow-up of all patients was 13.1 months (10 to 15). Results. The two groups had no significant differences in preoperative data. In the monitoring group, ten nerve alerts occurred intraoperatively, and no neural complications were detected postoperatively. In the control group, six patients had neural complications. The rate of nerve injury was lower in the monitoring group than in the control group, but this did not achieve statistical significance. The degree of leg lengthening was significantly greater in the monitoring group than in the control group. In further analyses, patients who had previous hip surgery were more likely to have intraoperative nerve alerts and postoperative nerve injury. Conclusion. Nerve injury usually occurred during the processes of exposure and reduction. The use of intraoperative nerve monitoring showed a trend towards reduced nerve injury in THA for Crowe IV DDH patients. Hence, we recommend its routine use in patients undergoing leg lengthening, especially in those with previous hip surgery. Cite this article: Bone Joint J 2019;101-B:1438–1446

The aim of this study was to review the early outcome of the Femoro-Patella Vialla (FPV) joint replacement. A total of 48 consecutive FPVs were implanted between December 2007 and June 2011. Case-note analysis was performed to evaluate the indications, operative histology, operative findings, post-operative complications and reasons for revision. The mean age of the patients was 63.3 years (48.2 to 81.0) and the mean follow-up was 25.0 months (6.1 to 48.9). Revision was performed in seven (14.6%) at a mean of 21.7 months, and there was one re-revision. Persistent pain was observed in three further patients who remain unrevised. The reasons for revision were pain due to progressive tibiofemoral disease in five, inflammatory arthritis in one, and patellar fracture following trauma in one. No failures were related to the implant or the technique. Trochlear dysplasia was associated with a significantly lower rate of revision (5.9% vs 35.7%, p = 0.017) and a lower incidence of revision or persistent pain (11.8% vs 42.9%, p = 0.045). . Focal patellofemoral osteoarthritis secondary to trochlear dysplasia should be considered the best indication for patellofemoral replacement. Standardised radiological imaging, with MRI to exclude overt tibiofemoral disease should be part of the pre-operative assessment, especially for the non-dysplastic knee. Cite this article: Bone Joint J 2013;95-B:793–7

To confirm whether developmental dysplasia of the hip has a risk of hip impingement, we analysed maximum ranges of movement to the point of bony impingement, and impingement location using three-dimensional (3D) surface models of the pelvis and femur in combination with 3D morphology of the hip joint using computer-assisted methods. Results of computed tomography were examined for 52 hip joints with DDH and 73 normal healthy hip joints. DDH shows larger maximum extension (p = 0.001) and internal rotation at 90° flexion (p < 0.001). Similar maximum flexion (p = 0.835) and external rotation (p = 0.713) were observed between groups, while high rates of extra-articular impingement were noticed in these directions in DDH (p < 0.001). Smaller cranial acetabular anteversion (p = 0.048), centre-edge angles (p < 0.001), a circumferentially shallower acetabulum, larger femoral neck anteversion (p < 0.001), and larger alpha angle were identified in DDH. Risk of anterior impingement in retroverted DDH hips is similar to that in retroverted normal hips in excessive adduction but minimal in less adduction. These findings might be borne in mind when considering the possibility of extra-articular posterior impingement in DDH being a source of pain, particularly for patients with a highly anteverted femoral neck. Cite this article: Bone Joint J 2014;96-B:580–9

The dysplasia cup, which was devised as an adjunct to the Birmingham Hip Resurfacing system, has a hydroxyapatite-coated porous surface and two supplementary neutralisation screws to provide stable primary fixation, permit early weight-bearing, and allow incorporation of morcellised autograft without the need for structural bone grafting. A total of 110 consecutive dysplasia resurfacing arthroplasties in 103 patients (55 men and 48 women) performed between 1997 and 2000 was reviewed with a minimum follow-up of six years. The mean age at operation was 47.2 years (21 to 62) and 104 hips (94%) were Crowe grade II or III. During the mean follow-up of 7.8 years (6 to 9.6), three hips (2.7%) were converted to a total hip replacement at a mean of 3.9 years (2 months to 8.1 years), giving a cumulative survival of 95.2% at nine years (95% confidence interval 89 to 100). The revisions were due to a fracture of the femoral neck, a collapse of the femoral head and a deep infection. There was no aseptic loosening or osteolysis of the acetabular component associated with either of the revisions performed for failure of the femoral component. No patient is awaiting a revision. The median Oxford hip score in 98 patients with surviving hips at the final review was 13 and the 10th and the 90th percentiles were 12 and 23, respectively

1. Familial metaphysial dysplasia occurring in two families is recorded. 2. One case with unusual skull changes and webbing of the digits is described in detail. 3. The similarity between the shape of the bones in familial metaphysial dysplasia and other bone disease is discussed