Surgical strategies for adult patients with hypophosphatemic rickets
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 46 years,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.
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