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A case with Pyle type metaphyseal dysplasia: clinical, radiological and histological evaluation.

Pyle type metaphyseal dysplasia is a rare autosomal recessive disease that is primarily affect metaphyses. Here we present a case with Pyle type metaphyseal dysplasia. The characteristic features of the case were metapyhseal broadening with undertubulation and Erlenmeyer flask sign at distal femoral and proximal tibial metaphyses. There were also platyspondyly with biconcave lens appearance of the vertebral bodies, congenital hip dislocation and normal cranium. Bone histopathology showed decreased number of osteoclasts. To the best of our knowledge, this is the first reported case of Pyle type metaphyseal dysplasia from Turkey.

Abnormalities, Multiple↗

Pavlik harness pathology.

Several complications following Pavlik harness splintage for congenital dislocated hips have been described. Two cases are reported where prolonged Pavlik splintage without concentric hip reduction caused posterior acetabular deficiency. Open reductions were required in both cases to correct deformity.

Arthrography↗

[Salter's innominate osteotomy. 20 years later...].

This is a retrospective review of nineteen innominate osteotomies with an average follow-up of 20, 6 years. These osteotomies were done mainly for treatment of late diagnosed congenital hip dislocation, or occasionally for persistent subluxation after orthopaedic treatment. The mean age at diagnosis was 2 years 2 months, at operation 3 years 5 months. We performed eight combined open reduction and pelvic osteotomies, eight associated pelvic and femoral varisation derotation osteotomies and only 3 isolated pelvic osteotomies. At follow-up the Charnley hip's score is 5.7 for pain; 5.4 for gait and performance, 5.6 for mobility. We found 15 hips without any significant pain and only 4 hips with mechanical pain. The Xray evaluation shows an average CE angle at 28.5 degrees, AC angle at 16 degrees and anterior coverage on "faux profil de Lequesne" at 40 degrees; 4 hips show arthritic changes, 4 others had important deformation of the head of the femur, but without diminution of the height of articular space. 6 hips (1/3) in 4 patients must be reoperated in adolescence because insufficiency of coverage of the head of the femur: 3 because technical errors and 3 because osteonecrosis with progressive cervicocephalic valgus. This historical serie with a very important follow-up, demonstrate that between 1/3 and 1/2 of cases have some kind of problems mainly radiological. The quality of results depends on the respect of the conditions and technique described by Salter, early diagnosis and absence of osteonecrosis eventually caused by prealable orthopaedic treatment.

Activities of Daily Living↗

Avascular necrosis in congenital hip dysplasia: the effect of treatment.

Fifty-one patients known to have developed avascular necrosis during treatment for congenital hip dislocations were studied. Thirty-nine patients were followed for a mean of 23 years, 7 months. They were classified as to the severity of involvement, the subsequent treatment provided, and their final result. The greater the involvement, the worse the outcome. Residual subluxation of hips resulted in a poor prognosis. Intervention with open reduction did not result in success. Varus femoral osteotomies and acetabuloplasties met with limited success. Total hip replacements have not resulted in long-term success.

Adolescent↗

The orthopedic evaluation of the neonate.

A method for the orthopedic evaluation of a child during the neonatal period of life has been discussed. Emphasis has been placed on conditions affecting the lower extremities such as spinal anomalies, congenitally dislocated hip, and congenital foot disorders. Early detection and medical intervention can play an important role in lower extremity function.

Extremities↗

Precise identification of radiographic acetabular landmarks.

Accurate radiographic demonstration of pelvic anatomical landmarks is predicated on proper positioning of the patient. Interpretation of acetabular abnormality especially may be difficult when to the artifact created by pelvic asymmetrical positioning there is added true femoral head displacement. A radiograph of hip joint abnormalities in a child with congenital hip dislocation where positional distortion was present served to illustrate the problem. To clarify the direction and magnitude of acetabluar alterations with specific pelvic positions, radiographs and photographs were taken of anatomic models outlined by suitably placed lead markers. Clues to inappropriate pelvic positioning are loss of symmetry in the widths of the iliac alae, sizes of the obturator foramina, and positions of the "tear drop" figures.

Acetabulum↗

Chiari osteotomy in the treatment of congenital dislocation and subluxation of the hip.

Twenty-nine Chiari osteotomies (eleven in children and eighteen in adolescents and adults) were critically reviewed after a minimum follow-up of two years and an average follow-up of 4.6 years. In four patients with complete dislocation and seven with moderate or severe subluxation, operations performed between the ages of four and fifteen years yielded five excellent or good results, three fair results, and three poor results. In the eighteen adolescent and adult patients, all of whom had pain, there were eleven good, two fair, and five poor results, the poor results being due to technical errors for the most part. The identified technical pitfalls were too low an osteotomy and too much displacement of the osteotomized fragments.

Adolescent↗

Chiari osteotomy.

Chiari osteotomy as the therapeutic approach in congenital hip dislocation has a special value in the adolescent patient with acetabular dysplasia and femoral head subluxation. The advantage is in the preservation of as much as possible of the modeling relationship of femoral head and acetabulum involved in hip joint motion while creating an effective lateral femoral head cover. Experience with Chiari osteotomy is described in a small group of adolescents (average age, 14 years) whose clinical problem is associated with limp, increasing fatigability and recurrent pain, with discussion of the complications contributing to significant morbidity, the technique of performance and the relative advantages and disadvantages of the procedure.

Adolescent↗

An innovative model of system-linked community care: home-based traction as an alternative to institutional treatment.

Home-based traction is an alternative treatment to conventional hospital-based traction for children with orthopedic conditions such as congenital dislocated hip and Legg-Perthes disease. The application of a proposed theoretical model is used to describe the process of the home-based traction innovation. Creativity and innovation occasions a reconceptualization of stresses. The reconceptualization of stress, plus the operational support of coordinated in-home services, can help families and nurses in the community and in the hospital develop, implement, and evaluate this kind of innovative care. In applying the home-based traction professionals empower and legitimize parents as resourceful co-providers of care. The legitimizing of the alternative in-home service affords parents a more optimistic appraisal of their situation as manageable. Parents are free to develop a coordinated response to their overall circumstance, including the treatment of their one child's illness. The evaluation of this home-based traction example found the alternative to be acceptable, safe, effective in accomplishing treatment goals, and less expensive than conventional hospital-based traction.

Adult↗

Congenital hip dysplasia in the light of early and very early diagnosis.

Ortolani summarizes 40 years of experience with about 8,000 children with congenital hip dysplasia, treated from birth or at a very early age. All newborns should be screened and examined for congenital hip dysplasia and this exam should be repeated at one month and again in 3 months. The most reliable sign for the diagnosis of congenital hip dysplasia at birth is the "click" sign. The etiology of congenital hip dysplasia is in a localized fault in embryonic development. The treatment started just as early as the condition was suspected. Surgical treatment is recommended for all complete congenital hip dislocations not amenable to treatment by closed methods.

Gestational Age↗

[Why and how to examine the newborns' hips?].

The authors insist on the importance of a careful clinical examination of newborn's hips, and on the necessity of an early diagnosis of congenital hip dislocation, in order to optimize the probability of therapeutic success. They don't consider hip dislocations resulting from neuromuscular diseases or from complex malformations (e.g. arthrogryposis) included in a wider context with very different prognosis and treatment.

Decision Trees↗

[Salter innominate osteotomy for the treatment of congenital dislocation of the hip].

Thirty-one children (43 hips) were reviewed after having sustained a Salter innominate osteotomy. The average follow up was 10 years. The indication for the osteotomy was a persisting dysplasia after conservative treatment of congenital dislocation of the hip. The X rays study allowed to describe the importance of the remodeling of the femoral head. The overall results were satisfactory in 60 per cent of the cases. The age of the patient at the time of surgery was important: the results being much better in children under 5 years of age. The failures were mostly related to technical faults. However some failures could not be predicted.

Child↗