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Abstract：
Spondyloepiphyseal dysplasia (SED) is rare genetic condition which leads to skeletal and joint deformities that can predispose patients to degenerative joint disease. There are limited reports on the results of total hip arthroplasty (THA) in this patient population. The purpose of this study is to review clinical and radiographic outcomes of THA performed in patients with SED at one institution.
Among 43,917 patients undergoing primary THA from 1970 to 2015, we identified 50 THAs performed in 29 patients with SED; 21 patients underwent bilateral THA (none simultaneous). There were 16 females and 13 males; mean age, body mass index, and height were 39 years, 28.7 kg/m2, and 145 cm, respectively. All patients were able to ambulate prior to the THA. Mean follow-up was 11 years (range 2-38).
Mean implant survival for primary THA in SED patients at the 5, 10, and 20-year time points was 96%, 85%, and 55%, respectively. Thirteen patients required revision THA, most commonly for polyethylene wear (n = 6) and aseptic loosening (n = 5), and 4 additional patients underwent nonrevision reoperations. Prior to surgery, 90% of patients had severe or moderate pain, which was reduced to 8% of patients postoperatively (P < .001). Mean Harris Hip Score improved from 47 to 87 (P < .001). Prior to surgery, 64% of patients required gait aids, which reduced to 34% postoperatively (P < .001).
THA provided significant pain reduction and improvement in function, with a majority of patients ambulating independently following the procedure. There was a high incidence of complications following THA in patients with SED, most commonly secondary to polyethylene wear and osteolysis from conventional polyethylene and historical implants.
Level IV, Therapy.
Among 43,917 patients undergoing primary THA from 1970 to 2015, we identified 50 THAs performed in 29 patients with SED; 21 patients underwent bilateral THA (none simultaneous). There were 16 females and 13 males; mean age, body mass index, and height were 39 years, 28.7 kg/m2, and 145 cm, respectively. All patients were able to ambulate prior to the THA. Mean follow-up was 11 years (range 2-38).
Mean implant survival for primary THA in SED patients at the 5, 10, and 20-year time points was 96%, 85%, and 55%, respectively. Thirteen patients required revision THA, most commonly for polyethylene wear (n = 6) and aseptic loosening (n = 5), and 4 additional patients underwent nonrevision reoperations. Prior to surgery, 90% of patients had severe or moderate pain, which was reduced to 8% of patients postoperatively (P < .001). Mean Harris Hip Score improved from 47 to 87 (P < .001). Prior to surgery, 64% of patients required gait aids, which reduced to 34% postoperatively (P < .001).
THA provided significant pain reduction and improvement in function, with a majority of patients ambulating independently following the procedure. There was a high incidence of complications following THA in patients with SED, most commonly secondary to polyethylene wear and osteolysis from conventional polyethylene and historical implants.
Level IV, Therapy.
摘要：
脊椎骨phy发育不良（SED）是一种罕见的遗传性疾病，可导致骨骼和关节畸形，使患者容易患上退行性关节病。关于该患者人群的全髋关节置换术（THA）结果的报道有限。这项研究的目的是回顾在一个机构的SED患者中进行的THA的临床和影像学结果。
在1970年至2015年接受原发性THA的43,917例患者中，我们确定了29例SED患者中的50例THA；21例患者接受了双侧THA（无同时）。有16名女性和13名男性；平均年龄，身体质量指数,身高39岁,28.7千克/平方米，145厘米，分别。所有患者都能够在THA之前走动。平均随访时间为11年（范围2-38）。
在5、10和20年时间点，SED患者原发性THA的平均植入物存活率为96%，85%,55%,分别。13名患者需要翻修THA，最常见的是聚乙烯磨损(n=6)和无菌性松动(n=5)，另外4例患者接受了非修正性再次手术.手术前,90%的患者有重度或中度疼痛，术后患者减少到8%(P<.001)。平均Harris髋关节评分从47分提高到87分(P<.001)。手术前,64%的患者需要步态辅助，术后降至34%(P<.001)。
THA显著减轻疼痛，改善功能，大多数患者在手术后独立行走。在SED患者中，THA后并发症的发生率很高，最常见的继发性聚乙烯磨损和骨溶解从传统的聚乙烯和历史植入物。
IV级,治疗。
在1970年至2015年接受原发性THA的43,917例患者中，我们确定了29例SED患者中的50例THA；21例患者接受了双侧THA（无同时）。有16名女性和13名男性；平均年龄，身体质量指数,身高39岁,28.7千克/平方米，145厘米，分别。所有患者都能够在THA之前走动。平均随访时间为11年（范围2-38）。
在5、10和20年时间点，SED患者原发性THA的平均植入物存活率为96%，85%,55%,分别。13名患者需要翻修THA，最常见的是聚乙烯磨损(n=6)和无菌性松动(n=5)，另外4例患者接受了非修正性再次手术.手术前,90%的患者有重度或中度疼痛，术后患者减少到8%(P<.001)。平均Harris髋关节评分从47分提高到87分(P<.001)。手术前,64%的患者需要步态辅助，术后降至34%(P<.001)。
THA显著减轻疼痛，改善功能，大多数患者在手术后独立行走。在SED患者中，THA后并发症的发生率很高，最常见的继发性聚乙烯磨损和骨溶解从传统的聚乙烯和历史植入物。
IV级,治疗。
