Cervical spine stenosis in chondrodysplasia punctata.

Chondrodysplasia punctata (CDP) is a rare skeletal dysplasia characterized by stippled epiphyses during infancy. The frequency is probably underdiagnosed because of the large heterogeneity in this group. Many genotypic variations exist. Although cervical instability is commonly seen in many skeletal dysplasias, cervical spine stenosis associated with CDP is very rare. We report a boy with phenotypic features of brachytelephalangic chondrodysplasia punctata (BCDP) who had severe cervical spine stenosis successfully corrected by vertebrectomies of C6 and C7 with a fibular strut graft. We discuss the significance of this association.

Case reports: Atlantooccipital and atlantoaxial traumatic dislocation in a child who survived.

A 4-year-old child with multilevel spinal injury including atlantooccipital and atlantoaxial dislocation, and thoracic spinal fractures was managed nonoperatively with no significant sequelae from the cervical injuries, but persistent paraplegia from the associated thoracic spinal fractures. Although treatment guidelines cannot be established with the result of one case, we propose an algorithm for treatment of such a rare and severe situation.

Ultrasound: a helpful technique in the analysis of congenital vertical talus. A case report.

Congenital vertical talus is a rare condition. In newborns, the diagnosis is evident in severe forms, but it can be difficult to confirm in mild ones. Non-ossified tarsal navicular cannot be visualized on standard roentgenograms until it is ossified. This work demonstrates that ultrasound is helpful in the early diagnosis of congenital vertical talus and in the evaluation of the therapeutic concept and effects.

Simple bone cysts of the proximal humerus complicated with growth arrest.

A simple bone cyst of the proximal humeral metaphysis was found to cause growth disturbance with shortening and deformity in four patients. All had one pathological fracture. Three of them were treated with cortisone injections; the fourth patient, who presented an erosion of the physis, was treated with saline solution irrigation. Four hypotheses about the aetiology of growth arrest in the evolution of this benign lesion, are discussed: iatrogenic lesion of the physis, growth plate involvement by fracture, cortisone injection, and increased cyst pressure leading to erosion and even perforation of the growth plate.

Local autograft bone in the surgical management of adolescent idiopathic scoliosis.

STUDY DESIGN: A retrospective study. OBJECTIVES: The study was performed to determine the efficacy of local autograft bone in idiopathic scoliosis surgery on patients who had had spinal fusion with a Cotrel Dubousset device between 1990 and 1995. SUMMARY OF BACKGROUND DATA: Studies seemed to promote the used of autograft bone (iliac crest, ribs), allograft bone, or bone graft substitutes in adolescent idiopathic surgery. To the authors' knowledge, there have been no previous articles concerning in situ local auto graft bone in idiopathic scoliosis surgery in the literature. MATERIALS AND METHODS: Twenty five patients with idiopathic scoliosis, aged 11 to 18 years, were treated by posterior correction and arthrodesis using Cotrel-Dubousset instrumentation. Posterior spinal fusion was performed using only local bone grafts. The patients were reviewed with a mean postoperative observation time of 6 years (5-9). The results were assessed clinically and radiographically. RESULTS: Preoperative single curves averaged 56 degrees in the frontal plane with an average postoperative curve of 16 degrees. Preoperative double curves in the frontal plane averaged 55 degrees in thoracic curves and 49 degrees in lumbar curves with an average postoperative curve of 18 degrees in thoracic and 13 degrees in lumbar. At the final follow-up, there was no losing in the correction. There was no pseudarthrosis clinically or radiographically. CONCLUSIONS: In adolescent idiopathic scoliosis surgery, local autograft bone allows to avoid the necessity of any over graft.

Long-term outcome in Desbuquois dysplasia: a follow-up in four adult patients.

Desbuquois dysplasia is a rare chondrodysplasia characterized by short stature, joint laxity, and specific radiographic findings. We report the natural history of four patients (three boys and one girl) with Desbuquois dysplasia ages 16-22 years. The mean height in adulthood was 114 cm (-8.5 SD) with progressive deceleration of the growth curve from birth (-4 SD) to adulthood. Obesity was noted consistently and facial abnormalities were still present but less obvious than in childhood. Three of four patients had mental retardation of varying degree. Hyperlaxity was persistent but limited motion of various joints was also noted. Orthopedic complications included coxa vara or valga (3/4), scoliosis (3/4), marked lordosis (3/4), and ambulatory difficulties (3/4). Surgical treatment was necessary for all four patients, involving large joints, spine and hands. Other complications included acute open-angle glaucoma secondary to a congenital malformation of the angle in one case. In addition to consistent radiological findings, elevated greater trochanter, generalized osteoporosis especially of the spine, scoliosis and/or lordosis, wide metaphyses, flat epiphyses, and coxa vara or valga were part of the natural history of the disorder. Our study emphasizes the care of older patients with Desbuquois dysplasia.