The charts and radiographs of 79 patients with osteogenesis imperfecta (OI) actively followed at the authors' institution were reviewed to determine the incidence of acetabular protrusion (AP) in OI and to evaluate the clinical consequences of this pelvic deformity. All 79 patients had iliac crest bone biopsy and pelvic radiographs. They were subdivided into OI type 1, 3, and 4, according to the Sillence classification, and type 5, according to Glorieux. AP was measured on pelvic radiographs, using the center-edge angle of Wiberg and the acetabular ilioischial line. Twenty-six patients (33%) had radiologic evidence of AP, with the highest incidence in OI type 3 (69%) and OI type 5 (54%). Only 3 of these 24 patients (13%) had medical problems that could be related to AP: severe constipation and hip ankylosis. The authors conclude that in this series of 79 patients with OI, AP is not uncommon and in rare cases may lead to severe medical complications. Key Words: Acetabular protrusion-Osteogenesis imperfecta.Osteogenesis imperfecta (OI) is a genetically and clinically heterogeneous group of disorders of bone and connective tissue characterized by osteoporosis, fragile bones, multiple fractures, bony deformities, hyperextensible joints, dentinogenesis imperfecta, blue sclerae, and adult-onset hearing loss. In 1979, Sillence et al. (9) proposed a classification that included four types: type 1, a mild form; type 2, the most severe form, causing perinatal death; type 3, a form with a high frequency of fractures, causing severe deformity and short stature; and type 4, in which deformities and short stature are present but to a lesser degree than in type 3. More recently, Glorieux et al.(3) added a type 5 to this classification, characterized by hypertrophic bone callus and ossification of the interosseous membranes in the legs and forearms.Although the radiologic findings of acetabular protrusion (AP) in OI have been well described, the incidence of AP in each type of OI has not been previously reported in the French or English literature to the best of our knowledge. The purpose of this study was to evaluate AP in our own series of OI patients, specifically looking at the incidence of this deformity in each type and at the clinical consequences of this pelvic deformity in OI. METHODSThe charts and radiologic films of all consecutive patients with OI actively followed at our institution were retrospectively reviewed. There were a total of 240 patients, of whom 161 were excluded either because they were not typed yet with iliac crest bone biopsy or because no pelvic radiographs were available. This left 79 patients, which constituted the subjects of this study. These were subdivided into four groups (type 1, 3, and 4 according to the classification of Sillence, plus type 5 according to the Glorieux classification). The lethal form (type 2) was excluded. The OI types, mean age, and sex are shown in Table 1.The AP in all 79 patients was measured (Fig. 1) using the Kohler line, as reported by McCollum and Nunley (8), and t...

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.

A dermal substitute, Integra (Integra Neurosciences Implants SA, Sophia Antipolis, France) artificial skin, was used for wound management on three children with lower limb injuries in our institutions. In one case this biosynthetic material was applied directly to a bony surface (patella). This technique allows an early wound coverage and provides a satisfactory preparation for autograft. It can be a useful adjunct in the treatment of severe tissue defects in child limb injuries.

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