ABSTRACT

Case Report
An 8-year-old girl was referred for evaluation of limp, scoliosis, and limited abduction. The family history was significant concerning the father possessing a similar gait, scoliosis, and coarse facial features. Examination showed a pain-free bilateral gluteus médius lurch. The patient was short in stature (10th percentile) and possessed an odd facial appearance with frontal bossing and increased head circumference (97th percentile). Her teeth were poorly formed and had multiple caries. She had a moderate right thoracic scoliosis and increased lumbar lordosis. The clavicles were not palpable beyond the midportion. Hip flexion contractures of 40° were present bilaterally; minimal hip abduction was possible and range of motion was nontender. The remainder of the extremity examination was unremarkable with the exception of short distal phalanges. Radiographs revealed bilateral coxa vara with a neck-shaft angle of 85° on the right and 75° on the left (Fig 1). The pubic symphysis was absent. Deficiency of the posterior arch was present at several vertebral levels. The lateral portion of both clavicles was not present. Hand films showed an extra physis at the proximal end of the second metacarpal and delayed ossification of the carpáis. Her cranial sutures were unossified.

The patient was diagnosed as having cleidocranial dysostosis, and valgus osteotomy for correction of bilateral coxa vara was recommended. At age 9 years, she underwent a three-part Wagner valgus osteotomy on the right side. Because of the length of the procedure, the correction of the left side was not performed simultaneously. The neck-shaft angle was increased from 75° to 130°. Healing was uneventful, and the Trendelenburg sign was no longer present on the right side postoperatively. The leg was lengthened 2 cm.

Although the procedure was judged to be successful, the patient's mother perceived the child's condition to be worse due to the resulting limb length discrepancy and exaggerated short-leg component of the limp. She would not consent to correction of the left side until the patient was 1 1 years and 10 months of age. The neck-shaft angle on the left side had progressed to 72° and the center-edge (CE) angle was 0° versus 24° on the right side (Fig 2). A two-part valgus osteotomy, secured with a 140° compression screw, was performed. Postoperatively, the limb lengths were equalized, the left Trendelenburg sign was positive after 30 seconds delay, and the patient's gait was much improved. The osteotomy healed satisfactorily, but when seen at age 14 years, the left hip was noted to be subluxated with a CE angle of - 10° (Fig 3). Despite this. she had good function, and when last examined at age 15 years, she had an excellent range of motion bilaterally, negative Trendelenburg signs, and complained only of minimal leg discomfort with exertion. It remains to be seen if this subluxation predisposes to early degenerative changes in the left hip.

Discussion

Coxa vara is an abnormal decrease in the neck-shaft angle of the proximal femur to less than 110°. Multiple etiologies are described, both congenital and acquired. It is frequently present in cleidocranial dysostosis. Developmental coxa vara is the preferred term when the defect is localized to the cervical region of the femur, with widening and vertical orientation of the capital femoral epiphysis. The femoral head is spherical. A pathognomonic radiographic feature is a triangular metaphyseal fragment that is seen medially at the base of an inverted "Y" formed by the physis. The femoral shaft is normal and the acetabulum minimally dysplastic. If the neck-shaft angle is less than 1 10°, the natural history is progression of the varus angulation, gait abnormalities due to shortening of the abductor…