成人低磷性佝偻病的手术治疗策略探讨

Surgical strategies for adult patients with hypophosphatemic rickets

目的:探讨成人低磷性佝偻病患者的手术治疗方案、临床疗效及并发症。方法:回顾性分析2015年12月至2022年5月收治的25例(48侧)低磷性佝偻病患者的临床资料,女22例,男3例;年龄16~46岁,平均(27.1±7.3)岁;体重指数21.9~38.7 kg/m2,平均(28.5±4.2)kg/m2。下肢内翻的43侧作为内翻组,下肢外翻的5侧作为外翻组。测量手术前后的髋膝踝角(HKA)、关节线倾斜角、股骨远端外侧角(LDFA)、胫骨近端内侧角(MPTA)、胫骨远端外侧角(LDTA)、股骨远端后侧角(PDFA)、胫骨近端后侧角(PPTA)和胫骨远端前侧角(ADTA)等影像学指标,记录术前及术后的膝关节、踝关节活动度及下肢功能评分(LEFS),采用Likert满意度量表进行患者满意度评价。结果:25例患者获得12~81个月随访,平均随访时间(35.6±18.7)个月。有41侧下肢使用外固定器治疗,外固定器佩戴时间为3.6~12.9个月,中位数(四分位数)为6.8(5.2,7.8)个月,其余7侧下肢单纯使用内固定治疗。内翻组术后HKA、LDFA、MPTA、LDTA、PDFA与术前比较,差异均有统计学意义(P均<0.05),术后均值均在正常值范围;外翻组术后HKA、LDFA与术前比较差异均有统计学意义(P均<0.05),LDFA和MPTA术后均值略高于正常值。膝关节和踝关节活动度由术前131.3°±7.7°降至术后124.9°±11.2°(t=5.432,P<0.001);踝关节活动度由术前53.1°±6.1°降至术后49.8°±7.9°(t=3.441,P=0.001)。LEFS由术前(60.7±2.4)分升高至术后(70.3±1.3)分(t=-27.219,P<0.001)。Likert满意度结果非常满意21人,满意3人,一般1人。结论:成人低磷性佝偻病的下肢畸形可以通过合理的手术治疗得到安全有效的矫正,其中结合股骨急性矫正和胫骨逐步矫正的方法可获得满意的临床结果。

Objective:This study aimed to explore surgical management,clinical outcomes,and incidence of complications in adult patients with hypophosphatemic rickets.Methods:A retrospective analysis was conducted on the clinical data of 25 patients(48 limbs)with hypophosphatemic rickets treated from December 2015 to May 2022.The cohort included 22 females and 3 males,aged 16 to 46years,with an average age of(27.1±7.3) years;the body mass index ranged from 21.9 to 38.7 kg/m2,with a mean of 28.5±4.2 kg/m2.Limbs were divided into two groups based on deformity:43 with varus deformities(varus group) and 5 with valgus deformities(valgus group).Radiological parameters,including hip-knee-ankle angle(HKA),knee joint line inclination angle,lateral distal femoral angle(LDFA),medial proximal tibial angle(MPTA),lateral distal tibial angle(LDTA),posterior distal femoral angle(PDFA),posterior proximal tibial angle(PPTA) and anterior distal tibial angle(ADTA),were measured pre-and post-operatively.Additionally,the range of motion(ROM) of the knee and ankle joints and lower extremity functional scale(LEFS) were recorded before and after surgery.Patient satisfaction was evaluated using the Likert scale.Results:Twenty-five patients were followed up for 12 to 81 months,with an average follow-up period of(35.6±18.7) months.Forty-one limbs were treated with external fixators,worn for 3.6 to 12.9 months,with a median and interquartile range of 6.8(5.2,7.8) months.The remaining 7 limbs were treated with internal fixation only.In the varus group,postoperative HKA,LDFA,MPTA,LDTA,and PDFA showed statistically significant improvements compared to preoperative values(all P<0.05),with postoperative means within the normal range.In the valgus group,postoperative HKA and LDFA also showed statistically significant improvements compared to preoperative values(both P<0.05),although postoperative LDFA and MPTA values were slightly above the normal range.The ROM of the knee joint decreased from 131.3° ±7.7° preoperatively to 124.9° ±11.2°postoperatively(t=5.432,P<0.001),and the ankle ROM decreased from 53.1°±6.1° preoperatively to 49.8°±7.9° postoperatively(t=3.441,P=0.001).The LEFS score increased from(60.7±2.4) preoperatively to(70.3±1.3) postoperatively(t=-27.219,P<0.001).The Likert scale results showed that 21 patients were very satisfied,3 were satisfied,and 1 was neutral.Conclusions:Surgical correction of lower limb deformities in adult patients with hypophosphatemic rickets is both safe and effective.A strategy combination of acute femoral correction and gradual tibial correction is associated with favorable clinical outcomes.