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Sacral agenesis: a clinical evaluation of its management, heredity, and associated anomalies.

In the child born with sacral agenesis, the management of arthrogrypotic-like deformities, spinal and multisystem abnormalities poses several problems to the orthopedist. A clinical evaluation was undertaken on 17 patients and available parents in an effort to define better the heredity and proper management of this disorder. Flexion contractures of the hips and knees adversely affect ambulation potential and are rather resistant to standard treatment. The presence of protective sensation and proprioception, though, warrants aggressive attempts at correction, Subtrochanteric amputations are not necessary to achieve a functional ambulator. Spinal-pelvic instability does not preclude functional ambulation. In fact, there may be some benefit in maintaining motion in this region. Abolishment of spinal-pelvic motion by surgical fusion in the presence of hip contractures could impair sitting ability and the ability to walk upright. Congenital vertebral anomalies in the remainder of the spine, including the neck, occurred in 12 patients. Four patients had idiopathic-like curves. Only one patient had a normal spin above the level of agenesis. From an analysis of families, there was no obvious genetic association. Finally, the high frequency of multisystem associated congential abnormalities will necessitate a team approach to these patients.

Adolescent↗

[Evaluation of treatment of the hip in arthrogryposis multiplex congenita].

In a retrospective review of 77 patients with arthrogryposis multiplex congenita, 21 showed involvement of the hip (28%). The authors studied 18 cases with hip dislocation (11 bilateral and seven unilateral) and three with hip contracture. There were 12 female and nine male patients ranging their age from one month to 14 years. The follow-up in this series lasted from two to 23 years. The treatment was conservative or surgical. The contractures were treated by manipulation and abduction orthosis in one patient and surgically by multiple hip tenotomy in two. The dislocation was treated by abduction orthosis (two cases), closed reduction (two cases) and open reduction (one case) in the 11 cases with bilateral involvement; for the seven unilateral dislocations the treatment was: closed reduction and abduction orthosis (one case), closed reduction with abductor tenotomy and hip spica cast (four cases) and open reduction followed by hip spica and abduction orthosis (one case); one case remained without treatment of the hip involvement because of severity of deformities in knee and foot. Ten patients of this groups were not treated because they were confined to wheelchairs. The overall result was good in six patients, regular in four and poor in two in the dislocation group; for the three cases of hip contracture the result of treatment was good. In conclusion the authors recommend such treatment for the independent ambulatory patients and emphasize the necessity of treatment of patients with unilateral hip involvement.

Adolescent↗

[Rotation-abduction contracture of the hip joint].

The paper presents the rotation-abduction contracture of the hip, a problem of growing magnitude in recent years. The anatomy of the hip joint, the perils related to muscle physiology, the symptoms of the disease, the etiology and means of surgical treatment according to Fernandez de Valderrama and Esteve de Miguel are discussed. The results of surgical treatment of 12 patients at the Orthopedic Department of Wroclaw. The presented procedure has been used in our center since 1993, and the results encourage further use of this technique.

Adolescent↗

Hip dysplasia associated with abduction contracture of the contralateral hip.

Eighteen children with unilateral dysplasia of the hip were found to have asymmetrical gluteal folds and an apparent limb-length inequality secondary to pelvic obliquity caused by an abduction contracture of the contralateral hip. The dysplastic hip was on the left in all but one patient. As a result of the pelvic obliquity, the femoral head on the high side of the pelvis was directed superolaterally, stretching the relaxed capsule still farther. The femoral head was then not concentrically reduced, and the pressure it caused on the superior border of the labrum contributed to the development of the acetabular dysplasia. The eighteen dysplastic hips were treated with abduction splinting and stretching exercises of the contralateral hip to decrease the abduction contracture. All of the dysplastic hips returned to a normal roentgenographic appearance with this treatment. Acetabular dysplasia without dislocation of the hip is not always appreciated by clinical examination, but the asymmetry of the gluteal folds and the apparent limb-length inequality that were seen in all of the children in this series were obvious clinical signs. These findings must be differentiated from the anterior thigh-fold asymmetry that is frequently seen without underlying pathology.

Braces↗

Abduction contracture of the hip in children.

Nine cases of abduction contracture of the hip in children from contracture of the gluteus maximus muscle are reported. Division of the aponeurosis glutens maximus over the greater trochanter always permitted full adduction.

Adolescent↗

Common rotational variations in children.

Most rotational variations in young children, such as in-toeing, out-toeing, and torticollis, are benign and resolve spontaneously. Understanding the normal variations in otherwise healthy children is vital to identifying true structural abnormalities that require intervention. A deliberate assessment of the rotational profile is necessary when evaluating children who in-toe or out-toe. In-toeing is usually attributable to metatarsus adductus in the infant, internal tibial torsion in the toddler, and femoral anteversion in children younger than 10 years. Out-toeing patterns largely result from external rotation hip contracture, external tibial torsion, and external femoral torsion. Although congenital muscular torticollis is the most common explanation for the atypical head posture in children, more serious disorders, including osseous malformations, inflammation, and neurogenic disorders, should be excluded.

Child↗

Treatment of extension contracture of the hip in cerebral palsy.

Twenty patients with cerebral palsy had a total of 35 extension contractures of the hip, resulting from tightness of the gluteus maximus or hamstring muscles, with associated quadricepts muscle spasticity. Associated deformities included anteriorly dislocated hips, patella alta, lumbar lordosis, thoracic kyphosis and calcaneus feet. Active and passive exercises, surgical release of contractures and reduction of anteriorly dislocated hips improved function. Hip flexor or adductor tenotomies must be considered cautiously for patients with spastic hip extensor muscles, because severe extension constricture may develop after either procedure.

Adolescent↗

[Diagnosis and therapy of hip dysplasia in the older child].

Diagnosis of congenital hip dysplasia is based on normal values of the acetabulum, but also criteria such as deficiency of the acetabular rim and an increased distance from the tear drop figure. Instability of the hip joint is seen in arthography. Acetabuloplasty is the way to treat it. Unstable joints will deteriorate, stable ones are frequently improving spontaneously. The technique of open reduction from an inguinal approach medial of the psoas muscle is described. There are many advantages. Simultaneous shortening osteotomy and acetabuloplasty to permit immediate movements out of the hip spica are recommended to avoid stiffening and contractures. Hip dysplasia is corrected by a lateral acetabuloplasty that levers down the acetabular roof in the triradiate cartilage. After consolidation of the cartilage triple pelvic osteotomy for acetabular rotation is the method to correct dysplasia in adolescents and adults. The closer the osteotomies are performed to the hip joint, the better rotation is possible. Our technique has proved successful now in 12 years.

Acetabulum↗