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Deformity Correction in Children with Hereditary Hypophosphatemic Rickets

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Clinical Orthopaedics and Related Research

Abstract

X-linked hereditary hypophosphatemic rickets can induce various multiplanar deformities of the lower limb. We evaluated our ability to correct these deformities and assessed complications and recurrence rates in 10 children (eight girls and a pair of twin boys) followed from early childhood to skeletal maturity. We performed 37 corrective operations in 10 children. Depending on the patient’s age, external fixation was used in 53 segments: Kirschner wires in 18, DynaFix® in three, the Taylor Spatial Frame® device in 13, and the Ilizarov device in 19. Internal fixation with intramedullary nailing was performed in 12. After bone consolidation, we radiographically determined the mechanical axis at an average distance of 0.5 cm medial to the center of the knee. The average mechanical lateral distal femoral angle was 85° (range, 83°–92°) and the average mechanical medial proximal tibial angle was 91° (range, 85°–92°). Deviation of the mechanical axis and knee orientation lines was increased at the followups conducted during a period of 5 to 12 months. Additional followups revealed a recurrence rate of 90% after the first corrective procedure and 60% after a second procedure.

Level of Evidence: Level IV, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.

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Authors and Affiliations

  1. Department of Pediatric Orthopedics, Orthopedic Hospital Vienna-Speising, Speisinger Strasse 109, 1130, Vienna, Austria

    Gert Petje MD, Roland Meizer MD, Christof Radler MD, Nicolas Aigner MD & Franz Grill PhD, MD

Authors
  1. Gert Petje MD
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  2. Roland Meizer MD
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  3. Christof Radler MD
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  4. Nicolas Aigner MD
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  5. Franz Grill PhD, MD
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Correspondence to Gert Petje MD.

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Each author certifies that he has no commercial concerns (eg, consultancies, stock ownership, equity interest, patent/licensing arrangements, etc) that might pose a conflict of interest in connection with the submitted article.

Each author certifies that his institution has approved the human protocol for this investigation, that all investigations were conducted in conformity with ethical principles of research, and that informed consent was obtained.

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Petje, G., Meizer, R., Radler, C. et al. Deformity Correction in Children with Hereditary Hypophosphatemic Rickets. Clin Orthop Relat Res 466, 3078–3085 (2008). https://doi.org/10.1007/s11999-008-0547-2

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  • DOI: https://doi.org/10.1007/s11999-008-0547-2

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