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Abstract

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.

Obstructive Constipation in Two Patients With Severe Osteogenesis Imperfecta and Acetabular Protrusio

“…Acetabular protrusio has been reported in 33% to 55% of the patients with OI and in an estimated 70% of the patients specifically with type 3 OI. 5 , 6 Although intuition suggests a correlation between acetabular protrusio and constipation, the limited data available have been inconsistent; Violas et al found that only 8% of patients with acetabular protrusio reported symptoms of constipation, whereas Lee et al demonstrated an increased risk of chronic constipation and recurrent abdominal pain in the presence of acetabular protrusio. 3 , 5 No published studies exist that seek to assess the relationship between pelvic deformity and severity of constipation.…”

Pauwels Osteotomy for Femoral Neck Nonunion in Two Adult Siblings with Osteogenesis Imperfecta

“…The manifestations of this disease in the musculoskeletal system vary 1 2) . The OI hip often has a varus deformity as well as “pincer” type impingement because of the acetabular protrusio 3 4 5) . With the resulting excessive forces on the femoral neck by the impingement, a “stress” fracture of the femoral neck can develop.…”