Aim. Aim of this monocentric, prospective study was to evaluate the safety, efficacy, clinical and radiographical results at 24-month follow-up (N = 6 patients) undergoing hip revision surgery with severe acetabular bone defects (Paprosky 2C-3A-3B) using a combination of a novel phase-pure betatricalciumphosphate - collagen 3D matrix with allograft bone chips. Method. Prospective follow-up of 6 consecutive patients, who underwent revision surgery of the acetabular component in presence of massive bone defects between April 2018 and July 2019. Indications for revision included mechanical loosening in 4 cases and history of hip infection in 2 cases. Acetabular deficiencies were evaluated radiographically and CT and classified according to the Paprosky classification. Initial diagnosis of the patients included osteoarthritis (N = 4), a traumatic fracture and a congenital hip dislocation. 5 patients underwent first revision surgery, 1 patient underwent a second revision surgery. Results. All patients were followed-up radiographically with a mean of 25,8 months. No complications were observed direct postoperatively. HHS improved significantly from 23.9 preoperatively to 81.5 at the last follow-up. 5 patients achieved a defined good result, and one patient achieved a fair result. No periprosthetic joint infection, no dislocations, no deep vein thrombosis, no vessel damage, and no complaint about limbs length discrepancy could be observed. Postoperative dysmetria was found to be + 0.2cm (0cm/+1.0cm) compared to the preoperative dysmetria of − 2.4 cm (+0.3cm/−5.7cm). Conclusions. Although used in severe acetabular bone defects, the novel phase-pure betatricalciumphosphate - collagen 3D matrixshowed complete resorption and replacement by newly formed bone, leading to a full implant integration at 24 months follow-up and thus represents a promising method with excellent bone regeneration capacities for complex cases, where synthetic bone grafting material is used in addition to autografts

Introduction. Orthopaedic rehabilitation of adolescences and young adults with high dislocation of the femur is rather challenging. The role of palliative salvage procedures is controversial enough in the cohort of patients. Materials and Methods. Treatment outcomes of 10 patients with congenital hip dislocation were analyzed. Mean age at intervention was 17,8 years (15–22). The grade of dislocation were assessed according to Eftekhar: type C − 2, type D − 8. The mean baseline shortening was 4.7±0.36 cm. All subjects underwent PSO with the Ilizarov method. Another osteotomy for lengthening and realignment was produced at the boundary of the upper and middle third of the femur. The mean time in the Ilizarov frame was 5.3 months. Results. The mean follow-up was 2.6±.1 years (range, 15 to 32 years). Limb shortening of 1 cm to 1. 1. /. 2. cm was observed in four cases. Functional outcomes according to d'Aubigne-Postel were: Pain 4,4±0,15 points. ROM − 4,1±0,3 points. Walking ability − 4,5±0,2 points. Two cases had good results (15–17 points), and seven patients had fair outcomes (12–14 points). A poor result (7 points) was recorded in one female patient 28 years after PSO followed by THA. Conclusions. Hip reconstruction with the Ilizarov method can be used in specific clinical situations as an alternative salvage procedure to delay THR in young patients with high dislocation of the femur

As our understanding of hip function and disease improves, it is evident that the acetabular fossa has received little attention, despite it comprising over half of the acetabulum’s surface area and showing the first signs of degeneration. The fossa’s function is expected to be more than augmenting static stability with the ligamentum teres and being a templating landmark in arthroplasty. Indeed, the fossa, which is almost mature at 16 weeks of intrauterine development, plays a key role in hip development, enabling its nutrition through vascularization and synovial fluid, as well as the influx of chondrogenic stem/progenitor cells that build articular cartilage. The pulvinar, a fibrofatty tissue in the fossa, has the same developmental origin as the synovium and articular cartilage and is a biologically active area. Its unique anatomy allows for homogeneous distribution of the axial loads into the joint. It is composed of intra-articular adipose tissue (IAAT), which has adipocytes, fibroblasts, leucocytes, and abundant mast cells, which participate in the inflammatory cascade after an insult to the joint. Hence, the fossa and pulvinar should be considered in decision-making and surgical outcomes in hip preservation surgery, not only for their size, shape, and extent, but also for their biological capacity as a source of cytokines, immune cells, and chondrogenic stem cells.

Cite this article: Bone Joint Res 2020;9(12):857–869.

Aims

The purpose of this study was to analyze the incidence of the different ultrasound phenotypes of developmental dysplasia of the hip (DDH), and to determine their subsequent course.

Total hip joint replacement (THJR) for high riding congenital hip dislocation (CDH) is often performed in young patients, and presents unique problems with acetabular cup placement and leg length inequality. A database and the NZ Joint Registry were used to identify 76 hips in 57 patients with a diagnosis of CDH who underwent THJR in the Wellington region between 1994 and 2015. Records and radiographs of 46 hips in 36 patients classified pre-operatively as Crowe II, III or IV were reviewed. Surgical technique used a direct lateral approach, the uncemented acetabular component was located in the anatomic hip center and a primary femoral stem was used in all but one hip. Whether a step-cut sub-trochanteric femoral osteotomy was performed depended on degree of correction, tension on the sciatic nerve, and restoration of leg length. For the 36 patients classified as Crowe II or higher, the average age at operation was 44 years (26 – 66), female:male ratio was 4.5:1 and follow-up averaged 10 years (2 – 22.3). Of the 15 hips classified as Crowe IV, 10 required a step-cut sub-trochanteric femoral osteotomy to shorten the femur, but 5 were lengthened without undo tension on the sciatic nerve. Nine Crowe IV hips received a conventional proximally coated tapered primary femoral component. Oxford hip scores for 76% of patients was excellent (> 41/48), and 24% had good scores (34 – 41). All femoral osteotomies healed. Five hips have been revised, one at 2 years for femoral loosening, one at 5 years for dislocation, two at 12 years for liner exchanges, and one at 21 years for femoral loosening. THJR using primary prostheses for CDH can provide durable long-term results

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% vs 20% in hips with no AVN; p < 0.001). A higher rate of AVN was identified when surgery was performed in children aged < 12 months, and when hips were immobilised in ≥ 60°of abduction post-operatively. Multivariate analysis showed that younger age at operation, need for further surgery and post-operative hip abduction of ≥ 60° increased the risk of the development of clinically significant AVN.

Cite this article: Bone Joint J 2014;96-B:279–86.

In this retrospective study we evaluated the proficiency of shelf autograft in the restoration of bone stock as part of primary total hip replacement (THR) for hip dysplasia, and in the results of revision arthroplasty after failure of the primary arthroplasty. Of 146 dysplastic hips treated by THR and a shelf graft, 43 were revised at an average of 156 months, 34 of which were suitable for this study (seven hips were excluded because of insufficient bone-stock data and two hips were excluded because allograft was used in the primary THR). The acetabular bone stock of the hips was assessed during revision surgery. The mean implant–bone contact was 58% (50% to 70%) at primary THR and 78% (40% to 100%) at the time of the revision, which was a significant improvement (p < 0.001). At primary THR all hips had had a segmental acetabular defect > 30%, whereas only five (15%) had significant segmental bone defects requiring structural support at the time of revision. In 15 hips (44%) no bone graft or metal augments were used during revision.

A total of 30 hips were eligible for the survival study. At a mean follow-up of 103 months (27 to 228), two aseptic and two septic failures had occurred. Kaplan-Meier survival analysis of the revision procedures demonstrated a ten-year survival rate of 93.3% (95% confidence interval (CI) 78 to 107) with clinical or radiological failure as the endpoint. The mean Oxford hip score was 38.7 (26 to 46) for non-revised cases at final follow-up.

Our results indicate that the use of shelf autografts during THR for dysplastic hips restores bone stock, contributing to the favourable survival of the revision arthroplasty should the primary procedure fail.

Cite this article: Bone Joint J 2013;95-B:777–81.

Purpose: Total hip arthroplasty in high riding congenital dislocation of the hip is a challenging procedure. In order to position the cup in the true acetabulum, femoral shortening osteotomy is often needed. The purpose of our study was to evaluate the results of two different methods of femoral shortening osteotomy. Method: Thirty-one total hip arthroplasties were performed in 29 cases with high congenital hip dislocation. The acetabular cups were placed at true acetabulum and femoral shortening osteotomies of the femur were performed at proximal (14 hips, group 1) or distal femur (17 hips, group 2). After a mean follow up of 4.2 years, all 31 hips were evaluated with Harris Hip Scores and X-rays. Technical difficulties and complications were also reported. Results: The mean increase in Harris Hip Score was 51 in group one and 52 in group two. There was one peroneal nerve palsy and one early dislocation in group 1, while there was no such perioperative complications in group 2. One acetabular cup and femoral stem were revised in group 1. Non-union happened in two cases of group 2. Special shape (cylindrical, non-tapered and longer than standard) femoral stems were needed for most proximal osteotomy patients. Conclusion: Hip arthroplasty, with insertion of cup at true acetabulum and femoral shortening osteotomy in patients with high congenital dislocation, can produce good results. Either proximal or distal femoral shortening osteotomy could have advantages and disadvantages. Proximal shortening osteotomy is a more challenging procedure, may need special stem design, and could compromise stem fixation

Objective: The presentation of mid-term results of porous tantalum TMT cup in congenital high hip dislocation. Materials and Methods: Between November 1997 and December 2000, we performed 27 total hip replacements in 22 women patiens suffering from high congenital hip dislocation according Xartofilakidis classification. The acetabular component was implanted at the true acetabular bed with restoration of the centre of hip rotation. Clinical and radiological observation took place in regular intervals for an average time of 10.2 years (8.5–12 years). Results: The average Harris Hip Score improved from 48.3 preoperatively (range 15–65) to 89.5 at the latest follow-up (56–100). Oxford hip score declined from 49.5 preoperatively to 21.2 at the first year and to 15.2 at five years examination. The absolute acetabular component’s migration was evaluated by EBRA method in the first 2 years and was at average 0.85 mm at the first year and 1.05 mm at the second year. An incident of gross initial migration was observed. No acetabular revision was performed and there was no case of mechanical loosening. Conclusion: The acetabular TMT component is highly adhesive and porous with a modulus of elasticity close to subchondral bone. It promotes initial stability, induces bone penetration and integration and offers a more “physiologig” load transfer. It also offers adequate polyethylene thickness, even in the smallest sizes, due to its manufacturing. The recent results from its use in high hip dislocation are excellent and justify the further study of longevity and probably the superiority of this material

The adult congenital hip dislocations and dysplasias have been previously classified by Eftekhar, Crowe et al., Hartofilakidis et al., Kerboul et al. and Mendes et al. The most conventient and widely used one is the Hartofilakidis and Crowe classification. Three different types of congenital hip disease in adults have been distinguished by Hartofilakidis and et al. based upon the position of the femoral head relative to the acetabulum: dysplasia; low dislocation; and high dislocation. All these classification systems are only radiological and does not highlight the operative technique in detail and the complications that we can observe perioperatively. Our classification system is also a radiological classification system but more useful for predicting the difficulty of the operative procedure and selecting the right operative method. In our classification system; at type I; dysplasia and less than 25% subluxations, we divided type I in to three subgroups, at type Ia, only dysplastic acetabulums, at type Ib, with elephant’s trunk type osteophyte formation and at type Ic, curtain type osteophytic formations, we included dysplasia and less than 25% subluxations in the same group because of operative technique similarities. At type 2; subluxations between 25% and 75%, we divided type II in two subgroups according to the angle between the inner margin of the teardrop and superior border of the acetabulum, at type IIa, the angle is less than 60°, at type IIb, the angle is greater than 60°, it’s important to show femoral allogreft usage requirement, at type 3; subluxations greater than 75%, at this type there will be no need of femoral allogreft usage but extra-small reamer usage for forming a suitable acetabular bed. At type 4; luxations greater than 100%, we also divided type IV in to two subgroups accordind to the distance between superior margin of true acetabulum and trochanter major line, at type IVa, < 2.5 cm, at type IVb, > 2.5 cm. It’s also important to make the decission of shortening. To form this classification three observers with different levels of training independently classified 412 dysplastic hips (operated between1995 and 2005) on 380 standard anteriorposterior pelvis radiographs, retrospectively according to the criteria defined by us. To assess intraobserver reliability, the measurement was repeated 3 months later. Statistical analysis was performed by calculating the weighted kappa correlation coefficient. System showed good inter- and intraob-server reliability for use in daily practice. Eventually, we determined a significant correlation between the aplied surgical procedures and classification. As a conclusion, we believe that our classification system of osteoarthritis secondary to developmental dysplasia of the hip in adult patients guides the surgical procedure more effectively than the other classification systems

Between November 1997 and December 2000 we performed 27 total hip replacements in 22 patients with high congenital dislocation of the hip using porous tantalum monoblock acetabular components implanted in the true acetabular bed. Clinical and radiological evaluation was performed at regular intervals for a mean of 10.2 years (8.5 to 12). The mean Harris Hip Score improved from 48.3 (15 to 65) pre-operatively to 89.5 (56 to 100) at the final follow-up. The mean Oxford Hip Score was 49.5 (35 to 59) pre-operatively and decreased to 21.2 (12 to 48) at one year and 15.2 (10 to 28) at final follow-up. Migration of the acetabular component was assessed with the EBRA software system. There was a mean migration of 0.68 mm (0.49 to 0.8) in the first year and a mean 0.89 mm (0.6 to 0.98) in the second year, after excluding one initial excessive migration. No revision was necessary for any reason, no acetabular component became loose, and no radiolucent lines were observed at the final follow-up.

The porous tantalum monoblock acetabular component is an implant offering adequate initial stability in conjunction with a modulus of elasticity and porosity close to that of cancellous bone. It favours bone ingrowth, leading to good mid-term results.

A clinical and radiographic study was conducted on 97 total hip arthroplasties (79 patients) performed for congenital hip dislocation using three-dimensional custom cementless stem. The mean age was 48 years (17 to 72). The mean follow up was 123 months (83 to 182). According to Crowe, there were 37 class 1, 28 class 2, 13 class 3 and 19 class 4. The average lengthening was 25 mm (5 to 58 mm), the mean femoral anteversion 38.6° (2° to 86°) and the correction in the prosthetic neck −23.6° (71° to 13°). The average Harris hip score improved from 58 to 93 points. Six hips (6.2%) required a revision. The survival rate was 97.7% ± 0.3% at 13 years. Custom cementless stem allows anatomical reconstruction and good functional results in a young and active population with disturbed anatomy, while avoiding a femoral osteotomy

Introduction: The Acetabular Reinforcement Ring (ARR) (ME Müller®) was introduced in 1977 as successor of the Endler ring, especially to allow total hip replacement (THR) in patients with congenital hip dislocation, serious dysplasia and revision of loose cups. The Polyethylen (PE)-inlay can be freely oriented in the fixed ARR. It is the first implant with screws having angular stability as the screw-heads are blocked when cementing the inlay in the ring. Patients and Methods: Out of 2251 primary THR performed between 1984 and 2005 at our hospital the ARR was used in 399 hips (363 patients). The indication for a THR with ARR was mainly a deficient acetabular bone stock as judged by the operating surgeon. 51 % of the patients had osteoarthritis, 22% dysplasia, 7% fractures, 6,5% osteonecrosis, 5% protrusion acetabuli, 4% rheumatoid arthritis and 4,5% other diagnosis. The mean age at operation was 66.4 years, 64% were females. Intraoperatively, the ARR was placed with its medial border adjacent to the tear drop figure. The weight bearing area was fixed with 3–4 cancellous screws in the acetabular bone stock. Regular clinical and radiological follow-up 3months, 1, 2, 5, 10,15 and 20 years was planned. The radiographs were assessed for osteolysis, radiolucent lines and screw breakage after 15 years. Survival for revision due to aseptic loosening was calculated by Kaplan Meier. Results: The overall survival regarding aseptic loosening of the cup was 100% after 10 years, 97,2% after 15 and 93,2% after 20 years. Of the 96 patients (104 rings) operated at least 15 years ago, one was lost for follow-up. Three rings were revised due to aseptic loosening (11, 12, 16 years after implantation) and one was removed due to infection (1,8 years after implantation). 54 patients (with 56 rings) died before they reached the 15 years control. None of them was revised for aseptic loosening of the ring. 37 patients (43 hips) remained for 15-years control, six of them refused to come and were contacted by questionnaire or phone. Two cups were radiologically loose with broken screws but not symptomatic. The mean HHS after 15 years was 80.1. Conclusion: The ARR shows a very good long term survival even in patients with difficult acetabular situations such as deficient bone stock. The design allows an application in a variety of positions. Even at a teaching hospital good long term results could be reached with this implant

Purpose of the study: Implantation of a total hip arthroplasty (THA) for major misalignment is a difficult procedure and few results have been published. In the 1950s to 1970s, supra-trochanteric osteotomy was proposed for sequelar osteoarthritis of congenital hip dislocation. Subsequent degradation 20 to 30 years later can lead to neo-osteoarthritis of the joint with an effect on hip alignment and overall balance between the knee and the spine. We present a prospective consecutive series of 60 THA performed from 1991 to 2003on hips with Milch and Schanz osteotomies. Material and methods: The objective was to reconstruct an anatomic hip joint by femoral re-alignment de-osteotomy, inferior displacement of the hip joint to enable insertion of an implant with a correctly position center of rotation and normal muscle lever arms. The technique was novel because of the direct approach to the subtrochanteric angle. The step by step procedure enabled insertion of the prosthesis without trochanterotomy. Overall recovery was long, often 12 to 18 months. There were 47 patients 60 hips) with at least 18 months follow-up. None of the patients were lost to follow-up. Results: Results were available for 54 hips (three deaths, six hips). Mean follow-up was eight years. Outcome was good (patient satisfaction, normal x-ray) for 77%. Twelve hips presented poor clinical and radiological results due to loosening and mobilization of the femoral implant with or without nonunion of the deosteotomy. Ten hips were revised at mean five years via a femoral access for insertion of a press-fit distally locked prosthesis with graft of the nonunion (with acetabular replacement in one hip). The outcome was good at last follow-up for nine of these hips. One repeated revision gave satisfactory results. Discussion: The only factors of risk of failure were related to femoral re-alignment and absence of trochanterotomy. A lesser risk of nonunion was related to the technique used for osteotomy, osteosynthesis and grafting. The use of a non-cemented implant with a solid primary stability and in certain cases a custom-made implant can be discussed for selected patients

Purpose of the study: the Pavlik harness has been used for the treatment of congenital hip dislocation since it was designed by Arnold Pavlik in 1950. There remains however a certain debate concerning the best moment to start treatment and its duration. We advocate early use of the Pavlic harness for a short period. Material and methods: Forty-five hips (34 infants) were treated. The diagnosis of dislocation was clinical. The Barlow and Ortolani maneuvers were used to search for clinical instability classed as «positive dislocation test» or «negative test but presence of piston movement». Different classifications of positive tests were used to search for an association with increasing severity of hip instability. Static and dynamic ultrasound was then used to confirm the diagnosis of hip dislocation. A Pavlik harness was installed immediately after diagnosis of congenital hip dislocation, on the day of birth if possible, according to the precepts proposed by the inventor. Results: Among the 43 hips analyzed I the present series, reduction and stabilization was successfully achieved with the Pavlic harness in 40 used as early as possible for a short a period as possible. This 95.6% success rate (2 failures, 0 complications) was achieved within 3 o 8 weeks. Discussion: Our results are comparable with other series reporting early use of the Mubarak method. The duration of treatment was shorter with our therapeutic method. We did not attempt to treat the dysplasia, spontaneous regression was monitored radiographically. Conclusion: We consider congenital hip displasia to be a therapeutic emergency. Treatment should be undertaken as soon as the dislocating intrauterine constraints cease. Early use of the Pavlik harness on easily dislocated or dislocated reducible hips has given excellent results. The shorter treatment duration does not lead to any recurrence as long as clinical stability with formal radiographic confirmation at treatment end

Purpose of the study: The natural history of congenital hip dysplasia with weight-bearing usually progresses towards degenerative joint disease. The anatomic type of the dislocation, whether treated or not, was well classified by Crowe who described four types.

Material and methods: Since 1989, we have used an ABG hydroxyapatite (HA) coated prosthesis for the treatment of congenital hip dysplasia. The hemispheric acetabular implant is coated with hydroxyapatite and the femoral implant, which is inserted in an anatomic position with anteversion, antetorsion and anteflexion, has a HA-coated stem. Forty-three Crowe type 3 or 4 hips (high position) were treated with this technique:

implantation of the cup in the paleoacetabulum;

Results: Cup implantation in the paleoacetabulum was achieved in all patients except two. A screwed autograft was inserted in 75% and remained stable over time for the larger grafts but tended to resorb for smaller grafts. For femoral anteverions, an ABG implant was used in 34 cases: 21 ABG1 stems, 11 ABF2 stems, and one ABG revision stem. The ABG stem enabled satisfactory anatomic restoration in 20 hips but with postoperative stiffness. For 14 hips, due to the important femoral anteversion related to the dysplasia, a reversed ABG-HA implant was used: eight left implants for right hips and six right implants for left hips. This «reversed» curvature gained 24° in the femoral anteversion plane. The outcome was excellent in these 14 cases, particularly with a clear improvement in postoperative external rotation. At close to 15 years follow-up we have had no case of femoral loosening, nor of femoral shaft osetolysis, with this type of implant.

Discussion: Certain authors propose using a custom-made implant for sequelar congenital hip dysplasia, but we prefer the proposed technique which provides very satisfactory results and limits the need for custom-made material.

A clinical and radiological study was conducted on 97 total hip replacements performed for congenital hip dislocation in 79 patients between 1989 and 1998 using a three-dimensional custom-made cementless stem. The mean age at operation was 48 years (17 to 72) and the mean follow-up was for 123 months (83 to 182). According to the Crowe classification, there were 37 class I, 28 class II, 13 class III and 19 class IV hips. The mean leg lengthening was 25 mm (5 to 58), the mean pre-operative femoral anteversion was 38.6° (2° to 86°) and the mean correction in the prosthetic neck was −23.6° (−71° to 13°). The mean Harris hip score improved from 58 (15 to 84) to 93 (40 to 100) points. A revision was required in six hips (6.2%). The overall survival rate was 89.5% (95% confidence interval 89.2 to 89.8) at 13 years when two hips were at risk. This custom-made cementless femoral component, which can be accommodated in the abnormal proximal femur and will correct the anteversion and frontal offset, provided good results without recourse to proximal femoral corrective osteotomy

Purpose: To analyse middle-term outcomes of treatment of patients with congenital luxation of hip using modified overhead traction.

Material and Methods: During the years 1991–2001, a total of 116 patients (138 hip joints) were treated. The group comprised 99 girls and 17 boys with the average age 4.7 months (ranging from 1.5–11 months). Patients were divided into two subgroups: patients who have been treated at our department from the determination of the diagnosis and those referred to our department from other facilities after unsuccessful conservative therapy. Only those patients were evaluated in whom the traction therapy was completed at least 2 years ago, the mean follow-up period was 4.5 years (2–10). After the initial preparatory horizontal traction, we moved to a 4-week overhead regimen wherein we increased the abduction by 10 degrees every five days. After completing the dystraction, every hip joint was examined using arthrography and where the reposition was possible, the therapy continued with the fixation in plaster spike for 6 weeks. Pavlik harnes were used for the final phase of the treatment.

Results: In the group of primarily treated patients, reposition was successful in 78 out of 91 hip joints (85.7%). In another 4 hip joints (4.4%), reluxation occurred after an average period of 3.5 weeks after the removal of the spike. Nine hip joints (9.9%), 7 of type IV and 2 of type IIIB, were non-repositionable. Much worse results were achieved in the group of patients who received previous treatment. Only 12 hip joints (25.5%) were maintained permanently repositionable but neither of them was of type IV. Reluxation within two weeks after the removal of the fixation occurred in another 5 hip joints (10.6%). A total of 30 hip joints (63.9%) could not be reposed due to arthrographic findings of reposition obstacles. All these patients were admitted for treatment after the 6th month of age. We have observed no case of avascular head necrosis in the group of 90 patients who received conservative treatment.

Conclusion: Traction therapy is a safe and mild method of treatment for congenital luxation of the hip joint. The rate of success of the therapy depends on the sonographic findings and age of patients at the beginning of therapy. Considerably worse results are achieved in the group of patients who have already received unsuccessful inadequate treatment.

Purpose: The prognosis of congenital hip displacement basically depends on the time of diagnosis and treatment. Recognising high-risk hips early remains a number one priority. We conducted a prospective study over a 20-year period from 1992 to 2002 to analyse the epidemiology of congenital hip displacement. Material and methods: The series included 1056 children with congenital hip disease (1491 hips). Epidemiological data, ultrasound and x-ray findings were recorded over 20 years. The objective was to identify risk factors and evaluate the impact of prevention measures. Results: The sex ratio showed female predominance, 6:1. The left hip was involved 1.8 times more often, with 41% bilateral involvement. Risk factors were major: family history (31%), breech presentation (25%), postural syndrome (12%); or minor: primiparity (54.4%), birth weight > 4 kg (9.2%). One or more major risk factors were found in 60.5% of the children and 30% had at least one minor risk factor. No risk factor was found in 40%. Screening efficacy improved with a rate of diagnosis before 4 months of 59% in 1983 and 96% in 2002. The number of hips discovered after the age of one year was 15% in 1983 and 6% in 2002. Discussion: The severity of the hip displacement is not influenced by risk factors nor bilateral involvement. Screening has enabled earlier diagnosis with a 37% increase in the rate of identification before the age of four months. This has been made possible by a systematic examination at birth and ultrasonography introduced in 1989. Conclusion: A regional map showing the paediatrician : maternity : general practitioner distributions is an important tool for screening campaigns. Despite adequate screening 40% of these children do not have any risk factor. Repeated examinations, communication and information exchange between healthcare professionals are the keys to success

Beighton and Kozlowski, in 1980, first defined this disorder, in Afrikaners, the syndrome , evident at birth, is constantly manifested by dwarfism, ligamentous hyperlaxity, congenital scoliosis, and multiple dislocations(hip dislocation, radial head dislocation, scoliosis, spatulate thumbs, and generalized ligamentous hyperlaxity, children are of normal intelligence potential. We report A Tunisian family, in which the proband , and her parents family have the presumptive diagnosis of Beighton dysplasia(spondyloepimetaphyseal dysplasia), the proband manifested the full clinical criteria of the disorder, whereas the mother, and other family members are partially manifesting the disorder, but dysplastic hips is the common feature in most of the family members examined(from both paternal and maternal side), up to four family subjects are variably affected, ranging between congenital hip dislocation in two subjects and post adulthood dysplastic arthrosis. The striking clinical findings evolved from the study is the accompaniment of diverse skeletal abnormalities rather than the hip dysplasia, the mother is with adolescent type kyphosis, and two other family subjects are with short stature and scoliosis, fractures were encountered in three family subjects, this was secondary to osteoporosis, which in fact a general clinical feature in all the family subjects. The mode of inheritance of the disorder is compatible with autosomal recessive trait. The purpose of this study is to indicate the importance of the precise recognition of the underlying etiologies in children presented with congenital dysplastic hips, as a matter of fact this is our main strategy in the department