Eyres K S, Brown J, Douglas D L
Department of Orthopaedics, Royal Hallamshire Hospital, Sheffield, UK.
J R Coll Surg Edinb. 1993 Feb;38(1):50-4.
We have reviewed the results of surgical treatment of vitamin D-resistant hypophosphataemic rickets (VDRR) and describe a technique of corrective osteotomy and intramedullary nailing. From 1978 to 1986, epiphysiodesis (n = 4) and osteotomy (n = 8) was performed in 6 children (mean age 13, range 10-16 years) for the correction of progressive lower limb deformity. Realignment and internal fixation of a pathological fracture of the femur was performed in an adult (aged 24). Epiphysiodesis resulted in recurrent deformity in all patients and reapplication of staples for loosening was required in three. Corrective osteotomies were secured with staples (n = 3), plates (n = 4), or plaster alone (n = 1) and were complicated by non-union in one patient, and recurrent deformity in two patients. Double-plating of the femoral fracture resulted in union but recurrent deformity. Compliance to treatment with phosphate and vitamin D was variable. In order to manage progressive recurrent deformity, we have performed corrective osteotomy and closed intramedullary nailing of the tibia (n = 2) and femur (n = 3) in 4 skeletally mature patients (mean age 31). All osteotomies united and no complications were encountered. Deformity has been corrected in all cases and all patients are satisfied with the outcome at least 2 years after surgery. We conclude that rigid methods of fixation spanning the whole length of the bone are required to maintain limb alignment in skeletally mature patients with VDRR. Since the quality of bone in VDRR is variable, experience with intramedullary techniques is essential. We stress the importance of appropriate medical therapy throughout the treatment of these patients.
我们回顾了抗维生素D性低磷血症佝偻病（VDRR）的手术治疗结果，并描述了一种矫正截骨术和髓内钉固定技术。1978年至1986年期间，对6名儿童（平均年龄13岁，范围10 - 16岁）进行了骨骺固定术（n = 4）和截骨术（n = 8），以矫正下肢进行性畸形。对一名24岁的成年人进行了股骨病理性骨折的复位和内固定。骨骺固定术导致所有患者出现复发性畸形，三名患者因固定钉松动需要重新应用。矫正截骨术采用固定钉（n = 3）、钢板（n = 4）或单纯石膏（n = 1）固定，一名患者出现骨不连，两名患者出现复发性畸形。股骨骨折采用双钢板固定实现了骨愈合，但出现了复发性畸形。患者对磷酸盐和维生素D治疗的依从性各不相同。为了处理进行性复发性畸形，我们对4名骨骼成熟的患者（平均年龄31岁）进行了胫骨（n = 2）和股骨（n = 3）的矫正截骨术和闭合髓内钉固定术。所有截骨均愈合，未出现并发症。所有病例的畸形均得到矫正，所有患者在术后至少2年对结果感到满意。我们得出结论，对于骨骼成熟的VDRR患者，需要采用跨越整个骨骼长度的刚性固定方法来维持肢体对线。由于VDRR患者的骨质各不相同，髓内技术的经验至关重要。我们强调在这些患者的整个治疗过程中进行适当药物治疗的重要性。
