Bulat Evgeny, Maranho Daniel A, Kalish Leslie A, Millis Michael B, Kim Young-Jo, Novais Eduardo N
1Department of Orthopedic Surgery (E.B., D.A.M., M.B.M., Y.-J.K., and E.N.N.) and Clinical Research Center (L.A.K.), Boston Children's Hospital, Boston, Massachusetts 2Ribeirao Preto Medical School, University of Sao Paulo, Sao Paulo, Brazil.
J Bone Joint Surg Am. 2017 Oct 18;99(20):1760-1768. doi: 10.2106/JBJS.17.00341.
The etiology of hip instability in Down syndrome is not completely understood. We investigated the morphology of the acetabulum and femur in patients with Down syndrome and compared measurements of the hips with those of matched controls.
Computed tomography (CT) images of the pelvis of 42 patients with Down syndrome and hip symptoms were compared with those of 42 age and sex-matched subjects without Down syndrome or history of hip disease who had undergone CT for abdominal pain. Each of the cohorts had 23 male and 19 female subjects. The mean age (and standard deviation) in each cohort was 11.3 ± 5.3 years. The lateral center-edge angle (LCEA), acetabular inclination angle (IA), acetabular depth-width ratio (ADR), acetabular version, and anterior and posterior acetabular sector angles (AASA and PASA) were compared. The neck-shaft angle and femoral version were measured in the patients with Down syndrome only. The hips of the patients with Down syndrome were further categorized as stable (n = 21) or unstable (n = 63) for secondary analysis.
The hips in the Down syndrome group had a smaller LCEA (mean, 10.8° ± 12.6° compared with 25.6° ± 4.6°; p < 0.0001), a larger IA (mean, 17.4° ± 10.3° compared with 10.9° ± 4.8°; p < 0.0001), a lower ADR (mean, 231.9 ± 56.2 compared with 306.8 ± 31.0; p < 0.0001), a more retroverted acetabulum (mean acetabular version as measured at the level of the centers of the femoral heads [AVC], 7.8° ± 5.1° compared with 14.0° ± 4.5°; p < 0.0001), a smaller AASA (mean, 55.0° ± 9.9° compared with 59.7° ± 7.8°; p = 0.005), and a smaller PASA (mean, 67.1° ± 10.4° compared with 85.2° ± 6.8°; p < 0.0001). Within the Down syndrome cohort, the unstable hips showed greater femoral anteversion (mean, 32.7° ± 14.6° compared with 23.6° ± 10.6°; p = 0.002) and worse global acetabular insufficiency compared with the stable hips. No differences between the unstable and stable hips were found with respect to acetabular version (mean AVC, 7.8° ± 5.5° compared with 7.6° ± 3.8°; p = 0.93) and the neck-shaft angle (mean, 133.7° ± 6.7° compared with 133.2° ± 6.4°; p = 0.81).
Patients with Down syndrome and hip-related symptoms had more retroverted and shallower acetabula with globally reduced coverage of the femoral head compared with age and sex-matched subjects. Hip instability among those with Down syndrome was associated with worse global acetabular insufficiency and increased femoral anteversion, but not with more severe acetabular retroversion. No difference in the mean femoral neck-shaft angle was observed between the stable and unstable hips in the Down syndrome cohort.
Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.
唐氏综合征患者髋关节不稳定的病因尚未完全明确。我们研究了唐氏综合征患者髋臼和股骨的形态，并将髋关节的测量结果与匹配的对照组进行比较。
将42例有髋关节症状的唐氏综合征患者的骨盆计算机断层扫描（CT）图像与42例年龄和性别匹配、无唐氏综合征或髋关节疾病史且因腹痛接受CT检查的受试者的图像进行比较。每个队列中有23名男性和19名女性受试者。每个队列的平均年龄（及标准差）为11.3±5.3岁。比较了外侧中心边缘角（LCEA）、髋臼倾斜角（IA）、髋臼深度-宽度比（ADR）、髋臼旋转角度以及髋臼前、后扇形角（AASA和PASA）。仅对唐氏综合征患者测量颈干角和股骨旋转角度。将唐氏综合征患者的髋关节进一步分为稳定组（n = 21）和不稳定组（n = 63）进行二次分析。
唐氏综合征组的髋关节LCEA较小（平均为10.8°±12.6°，而对照组为25.6°±4.6°；p < 0.0001），IA较大（平均为17.4°±10.3°，而对照组为10.9°±4.8°；p < 0.0001），ADR较低（平均为231.9±56.2，而对照组为306.8±31.0；p < 0.0001），髋臼更后倾（在股骨头中心水平测量的平均髋臼旋转角度[AVC]为7.8°±5.1°，而对照组为14.0°±4.5°；p < 0.0001），AASA较小（平均为55.0°±9.9°，而对照组为59.7°±7.8°；p = 0.005），PASA较小（平均为67.1°±10.4°，而对照组为85.2°±6.8°；p < 0.0001）。在唐氏综合征队列中，与稳定髋关节相比，不稳定髋关节的股骨前倾角更大（平均为32.7°±14.6°，而稳定组为23.6°±10.6°；p = 0.002），整体髋臼发育不良更严重。在髋臼旋转角度（平均AVC，7.8°±5.5°与7.6°±3.8°；p = 0.93）和颈干角（平均为133.7°±6.7°与133.2°±6.4°；p = 0.81）方面，不稳定髋关节和稳定髋关节之间未发现差异。
与年龄和性别匹配的受试者相比，有髋关节相关症状的唐氏综合征患者髋臼更后倾、更浅，股骨头的整体覆盖范围减小。唐氏综合征患者的髋关节不稳定与更严重的整体髋臼发育不良和股骨前倾角增加有关，但与髋臼后倾程度加重无关。在唐氏综合征队列中，稳定髋关节和不稳定髋关节的平均股骨颈干角未观察到差异。
预后水平III。有关证据水平的完整描述，请参阅作者须知。
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