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 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

Fifty-one infants with limited abduction of the hip and acetabular dysplasia were, between 1969 and 1975, treated with abduction-adduction exercises, administered by the parents; no abduction devices were used. In 1983 a follow-up examination was carried out on 41 of these patients. Although at birth these children had characteristics similar to patients with congenital dislocation of the hip, none of their hips dislocated. At birth acetabular measurements showed that half the children had severe dysplasia and the other half slight dysplasia; the difference between the affected and the healthy hips was significant. At follow-up the gait was normal in all the patients. Movements at the hips were symmetrical and within normal limits in all but one patient. The acetabular angle, the centre-edge angle, the shaft-neck angle, the hip ratio, and the size of the femoral head were the same on the previously affected side as on the normal side. Nineteen of the children followed up had preferred lying on one side and dysplasia of the upper hip had developed. The significance of the sleeping position on the development of acetabular dysplasia is discussed

A brief review of the literature on malignant change occurring in fibrous dysplasia is given and a further case of a sarcoma arising in a patient with polyostotic fibrous dysplasia is reported

Tibial dysplasia is a rare congenital deformity which must be distinguished from the more common fibular dysplasia. We have reviewed 24 patients with 35 affected legs. The classification system of Kalamchi and Dawe (1985) was found to be preferable to that of Jones, Barnes and Lloyd-Roberts (1978) as a guide to prognosis and management. We discuss the outcome of surgical treatment, recommending selective amputation for most cases

Aims. The influence of identifiable pre-operative factors on the outcome of eccentric rotational acetabular osteotomy (ERAO) is unknown. We aimed to determine the factors that might influence the outcome, in order to develop a scoring system for predicting the prognosis for patients undergoing this procedure. Patients and Methods. We reviewed 700 consecutive ERAOs in 54 men and 646 women with symptomatic acetabular dysplasia or early onset osteoarthritis (OA) of the hip, which were undertaken between September 1989 and March 2013. The patients’ pre-operative background, clinical and radiological findings were examined retrospectively. Multivariate Cox regression analysis was performed using the time from the day of surgery to a conversion to total hip arthroplasty (THA) as an endpoint. A risk score was calculated to predict the prognosis for conversion to THA, and its predictive capacity was investigated. Results. The congruity of the hip, age, the pre-operative minimum width of the joint space and range of abduction were identified as factors predicting conversion to THA. For three groups of patients (scoring 0 to 5, 6 to 7, and 8 to 9 points), the Kaplan-Meier event-free rates of survival at 15 years post-operatively for conversion to THA were 99.6%, 85.2% and 67.3%, respectively. Conclusion. These four pre-operative factors are easily measured and predict the prognosis for patients following ERAO. They may be used for decision making when offering surgical treatment to patients with acetabular dysplasia and early onset osteoarthritis. Cite this article: Bone Joint J 2016;98-B:1326–32

We examined clinically and radiologically the knees of 46 patients (27 females and 19 males) with diastrophic dysplasia. The age of the patients varied from newborn to 38 years. A total of 18 patients was followed during their growth until adolescence. The knees of two legally aborted fetuses appeared on examination to be macroscopically normal and congruous. Excessive valgus deformity of the tibiofemoral weight-bearing angle with a mean of 14° was noted in infancy. Most of the patients had marked instability of the knees. The range of movement of the knee began to decrease before the age of five years. There were signs of early degeneration and deformation of the bony epiphyses before the age of six years. The patellofemoral joint was abnormal from an early age. A marked patella infera, often associated with a lateral position of the patella with bony fragmentation, was noted. The knee in diastrophic dysplasia is basically unstable, showing early deformation of the subchondral bone and degeneration of the joint

Three amputated legs with tibial dysplasia were studied by radiography, arteriography and anatomical dissection. The radiographic appearances were the same as the Type 1b tibial dysplasia described by Jones, Barnes and Lloyd-Roberts (1978) in that the tibiae were absent but the lower femoral epiphyses were normal. However, our anatomical findings differed from those of Jones et al. since no bony or cartilaginous anlage of the proximal tibia was found in any of the three legs. The pattern of vascular anomaly was identical in the three legs and similar to the findings of Hootnick et al. (1980) in congenital short fibula. Congenital fusion of the subtalar joint was a constant finding. These results support the hypothesis that the arterial and skeletal systems are vulnerable to a teratogenic insult in the fifth week of embryonic life. The bony and arterial anomalies should be borne in mind by the surgeon attempting reconstructive surgery for this condition

We describe the clinical and radiological results of cementless primary total hip replacement (THR) in 25 patients (18 women and seven men; 30 THRs) with severe developmental dysplasia of the hip (DDH). Their mean age at surgery was 47 years (23 to 89). In all, 21 hips had Crowe type III dysplasia and nine had Crowe type IV. Cementless acetabular components with standard polyethylene liners were introduced as close to the level of the true acetabulum as possible. The modular cementless S-ROM femoral component was used with a low resection of the femoral neck. A total of 21 patients (25 THRs) were available for review at a mean follow-up of 18.7 years (15.8 to 21.8). The mean modified Harris hip score improved from 46 points pre-operatively to 90 at final follow up (p < 0.001). . A total of 15 patients (17 THRs; 57%) underwent revision of the acetabular component at a mean of 14.6 years (7 to 20.8), all for osteolysis. Two patients (two THRs) had symptomatic loosening. No patient underwent femoral revision. Survival with revision of either component for any indication was 81% at 15 years (95% CI 60.1 to 92.3), with 21 patients at risk. . This technique may reduce the need for femoral osteotomy in severe DDH, while providing a good long-term functional result. Cite this article: Bone Joint J 2014;96-B:1449–54

We performed rotational acetabular osteotomy in order to treat dysplasia of the hip in five ambulatory adults with cerebral palsy. There was one man and four women, with a mean age of 21 years (16 to 27) who were followed up for a mean of 12 years and two months. The mean Sharp angle improved from 52° to 43°, the mean acetabular index from 30.2° to 2.8°, the mean centre-edge angle from −5.6° to 29.2°, and the mean acetabular head index from 49.2 to 88.2. There was no progression of joint degeneration and relief from pain was maintained. Our results suggest that rotational acetabular osteotomy is a valuable option for the treatment of acetabular dysplasia in adults with cerebral palsy who have incapacitating pain in the hip

We describe a new technique of reconstruction of the deficient acetabulum in cementless total hip arthroplasty. The outer iliac table just above the deficient acetabulum is osteotomised and slid downwards. We have termed this an iliac sliding graft. Between October 1997 and November 2001, cementless total hip arthroplasty with an iliac sliding graft was performed on 19 patients (19 hips) with acetabular dysplasia. The mean follow-up was 3.4 years (2 to 6). The mean pre-operative Harris hip score was 45.1 which improved significantly to 85.3 at the time of the final follow-up. No patient had post-operative abductor dysfunction. Incorporation of the graft was seen after two to three months in all patients. Resorption of the graft and radiolucencies were infrequent. This technique is a useful alternative to femoral head autografting when the patient’s own femoral head cannot be used

Hereditary multiple exostoses is an autosomal dominant disorder characterised by multiple osteochondromata, most commonly affecting the forearm, knee and ankle. Osteochondromata of the proximal femur have been reported to occur in 30% to 90% of affected patients with coxa valga in 25%. Acetabular dysplasia is rare but has been described. This is the first report of a patient requiring surgical intervention. A girl was seen at the age of nine with hereditary multiple exostoses and when 12 developed bilateral pain in the groin. Radiographs showed severely dysplastic acetabula with less than 50% coverage of the femoral heads and widening of the medial joint space. Large sessile osteochondromata were present along the medial side of the femoral neck proximal to the lesser trochanter, with associated coxa valga. The case illustrates the importance of obtaining initial skeletal surveys in children with hereditary multiple exostoses to identify potential problems such as acetabular dysplasia and subluxation of the hip

We investigated the variables which determine the outcome after triple osteotomy of the pelvis for the treatment of congenital dysplasia of the hip. We reviewed 51 patients (61 hips) with a median age at operation of 23 years who were treated with a Tönnis triple osteotomy. The median follow-up was six years with a minimum of two years. Eight patients (eight hips) required a revision procedure. Of the remaining 53 hips, the results were good or excellent in 36 (68%) when evaluated according to the Harris hip score (median 90 points), and 33 patients (65%) were satisfied with the procedure. Logistic regression analysis indicated that the incidence of complications such as nonunion at an osteotomy site influenced patient satisfaction (p = 0.079). The incidence of complications correlated positively with increasing patient age at operation (p = 0.004). The amount of acetabular correction did not correlate with patient satisfaction. In univariate analysis, the groups of ’satisfied’ and ‘not satisfied’ patients differed significantly in Harris hip score, age, incidence of nonunion at the osteotomy sites, complications and late revisions. In conclusion, the patient’s age at operation and the incidence of complications influence patient satisfaction after triple osteotomy, but the amount of radiologically evident acetabular correction shows no correlation to outcome

1. Two cases are reported showing the syndrome of chondrodysplasia, manual polydactyly, ectodermal dysplasia affecting the teeth and nails, and congenital heart disease. 2. Particulars of thirty-eight cases are tabulated, and the features of the syndrome are discussed. 3. The syndrome is regarded as showing an autosomal recessive mode of inheritance

We report the case of a 12-year-old boy with polyostotic fibrous dysplasia of the upper femur in whom a massive customised polyethylene prosthesis functioned successfully for more than fifty years

The exceptionally high prevalence of diastrophic dysplasia in Finland has enabled us to analyse the foot deformities of 102 patients at their first orthopaedic evaluation and classify 204 feet into five categories. The most common finding (43%) was a foot with tarsal valgus deformity and metatarsus adductus; 37% showed either equinovarus adductus (29%) or equinus (8%) deformities. At the first examination 13% showed metatarsus adductus deformity alone, and 7% were clinically normal. The expression 'club foot', generally used for the foot deformity in diastrophic dysplasia is a misnomer. There is a wide spectrum of deformities, some of them specific for the condition

1. A survey of genetic and other etiological factors has been carried out in 589 index patients with congenital dislocation of the hip and their families, with special investigation of acetabular dysplasia, familial joint laxity and a comparison of neonatal and late-diagnosis cases. 2. It is believed that there are two etiological groups with congenital dislocation of the hip, i) a group with acetabular dysplasia which is inherited as a multiple gene system and is responsible for a high proportion of cases diagnosed late, and ii) a group with joint laxity which is responsible for a high proportion of neonatal cases. 3. Evidence is presented to show that acetabular dysplasia is a separate heritable system in some families. 4. Other findings relating to the genetic aspects of the survey are summarised

The acetabular rim syndrome is a pathological entity which we illustrate by reference to 29 cases. The syndrome is a precursor of osteoarthritis of the hip secondary to acetabular dysplasia. The symptoms are pain and impaired function. All our cases were treated by operation which consisted in most instances of re-orientation of the acetabulum by peri-acetabular osteotomy and arthrotomy of the hip. In all cases, the limbus was found to be detached from the bony rim of the acetabulum. In several instances there was a separated bone fragment, or 'os acetabuli' as well. In acetabular dysplasia, the acetabular rim is subject to abnormal stress which may cause the limbus to rupture, and a fragment of bone to separate from the adjacent bone margin. Dysplastic acetabuli may be classified into two radiological types. In type I there is an incongruent shallow acetabulum. In type II the acetabulum is congruent but the coverage of the femoral head is deficient

Malignant transformation of fibrous dysplasia to chondrosarcoma is rare. We report a case in which malignancy developed in an area of fibrous dysplasia in the ilium. We believe this to be the second reported case at this site. Treatment was by excision of the hemipelvis including the ala of the sacrum. A review of the literature is presented