Document Detail

Osteotomy and intramedullary nailing for the correction of progressive deformity in vitamin D-resistant hypophosphataemic rickets.
MedLine Citation:
PMID:  8382289     Owner:  NLM     Status:  MEDLINE    
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.
K S Eyres; J Brown; D L Douglas
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of the Royal College of Surgeons of Edinburgh     Volume:  38     ISSN:  0035-8835     ISO Abbreviation:  J R Coll Surg Edinb     Publication Date:  1993 Feb 
Date Detail:
Created Date:  1993-03-22     Completed Date:  1993-03-22     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7503110     Medline TA:  J R Coll Surg Edinb     Country:  SCOTLAND    
Other Details:
Languages:  eng     Pagination:  50-4     Citation Subset:  IM    
Department of Orthopaedics, Royal Hallamshire Hospital, Sheffield, UK.
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MeSH Terms
Bone Nails*
Femur / surgery*
Hypophosphatemia, Familial / surgery*
Middle Aged
Phosphates / blood*
Tibia / surgery*
Treatment Outcome
Reg. No./Substance:

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