成骨不全症患者的假性髋臼前突畸形。

Pseudo-Protrusio Acetabular Deformity in Osteogenesis Imperfecta Patients.

机构信息

Department of Orthopaedic Surgery and Institute for Rare Diseases, Korea University Guro Hospital.

Division of Pediatric Orthopaedics, Seoul National University Children's Hospital, Seoul.

出版信息

J Pediatr Orthop. 2021 Mar 1;41(3):e285-e290. doi: 10.1097/BPO.0000000000001739.

DOI:10.1097/BPO.0000000000001739

PMID:33534367

Abstract

BACKGROUND

Osteogenesis imperfecta (OI) can develop a protrusio acetabuli deformity. However, the authors observed a pseudo-protrusio-type acetabular deformity (PPAD) on 3-dimensional computed tomography (3D-CT). Hence, we systematically reviewed 3D-CT and pelvis radiographs of OI patients and report the incidence and patterns of acetabular deformity in OI patients and the associated radiographic signs.

METHODS

The study included 590 hips of 295 OI patients, who were older than 5 years, and did not have a pelvic fracture. The incidence of a deformed acetabulum (center-edge angle >40 degrees) and its correlation with disease severity were investigated. In 40 hips for which 3D-CT was available, 3-dimensional morphology of the acetabular deformity was analyzed to delineate PPAD. On plain radiographs, PPAD-related signs were determined, focusing on the contour of ilioischial line, iliopectineal line, acetabular line, and their relationship. These radiographic signs were also evaluated in the remaining hips with deformed acetabula that did not have 3D-CT.

RESULTS

One hundred twenty-three hips of 590 hips (21%) showed deformed acetabula. The incidence of deformed acetabula was significantly associated with disease severity (P<0.001). Three-dimensional analysis showed that 10 hips had protrusio acetabuli, whereas 17 had PPAD, which showed that the hemipelvis was crumpled, the acetabular roof was rotated upwardly and medially, and the hip center migrated superiorly, uncovering the anterior femoral head. Among the PPAD-related signs, superomedial bulging of the iliopectineal line was the most predictive radiographic sign (73% sensitivity and 100% specificity). This sign was also observed in almost one third of deformed acetabula of those investigated only with plain radiographs.

CONCLUSIONS

This study showed that acetabular deformity is common in OI patients and is associated with disease severity. A substantial number of hips showed PPAD, which may not cause femoroacetabular impingement but result in anterior uncovering of the hip joint. Superomedial bulging of the iliopectineal line suggests this pattern of acetabular deformity.

LEVEL OF EVIDENCE

Lever IV-prognostic studies.

摘要

背景

成骨不全症（OI）可发展为髋臼前突畸形。然而，作者在三维 CT（3D-CT）上观察到一种假性前突型髋臼畸形（PPAD）。因此，我们系统地回顾了 OI 患者的 3D-CT 和骨盆 X 线片，并报告了 OI 患者髋臼畸形的发生率和类型以及相关的影像学征象。

方法

该研究纳入了年龄大于 5 岁且无骨盆骨折的 295 例 OI 患者的 590 髋。研究了髋臼畸形（中心边缘角>40 度）的发生率及其与疾病严重程度的相关性。在 40 髋有 3D-CT 的情况下，分析髋臼畸形的三维形态以描绘出 PPAD。在骨盆平片上，确定了与 PPAD 相关的征象，重点关注坐骨耻骨线、坐骨髂耻线、髋臼线及其关系的轮廓。在其余没有 3D-CT 的髋臼畸形髋中也评估了这些影像学征象。

结果

590 髋中有 123 髋（21%）显示髋臼畸形。髋臼畸形的发生率与疾病严重程度显著相关（P<0.001）。三维分析显示，10 髋有髋臼前突，17 髋有 PPAD，表现为半骨盆皱缩，髋臼顶向上、向内旋转，髋关节中心向上迁移，显露股骨头前侧。在与 PPAD 相关的征象中，坐骨髂耻线的中上侧隆起是最具预测性的影像学征象（73%的敏感性和 100%的特异性）。该征象在仅行骨盆平片检查的髋臼畸形中也观察到近三分之一。