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The effect of osteotomy on bowing and height in children with X-linked hypophosphatemia
M T Rohmiller1, C Tylkowski, V M Kriss
1Shriners Hospital for Children, Lexington, Kentucky, USA.
Insights
Surgery combined with medication improved bowing angles in X-linked hypophosphatemia patients but did not improve height. Obesity at presentation was linked to needing surgery.
Area of Science:
- Pediatric Orthopedics
- Medical Genetics
Background:
- X-linked hypophosphatemia (XLH) is a rare genetic disorder characterized by phosphate wasting, leading to rickets and skeletal deformities.
- Current treatment strategies for XLH involve phosphate and active vitamin D supplementation, but surgical intervention is sometimes necessary for severe deformities.
Purpose of the Study:
- To evaluate the long-term efficacy of medication versus combined medication and surgical treatment for X-linked hypophosphatemia.
- To identify predictors of surgical intervention in pediatric XLH patients.
Main Methods:
- Retrospective analysis of clinical records from 40 XLH patients followed for at least 36 months.
- Patients were categorized into two groups: medication only (Group A) and medication plus surgery (Group B).
- Outcomes assessed included changes in lower limb bowing angles and patient height; predictors for osteotomy were analyzed.
Main Results:
- Group B (medication and surgery) showed significant improvement in bowing angles compared to Group A (medication only).
- No height improvement was observed in Group A, while Group B experienced a significant decrease in height.
- Patient height and initial bowing angles did not predict the need for future osteotomies. Obesity at presentation was a significant predictor for requiring surgery.
Conclusions:
- Combined surgical and medical treatment effectively corrects lower limb deformities in XLH but may lead to decreased height.
- Obesity is a significant risk factor for requiring surgical correction in pediatric XLH patients.
- Further research is needed to optimize treatment strategies balancing deformity correction and linear growth in XLH.
Abstract:
The clinical records of 40 patients with X-linked hypophosphatemia who were treated and followed for at least 36 months were examined retrospectively. The patients were divided into those treated with medication only (group A) and those treated with medication and surgery (group B). At follow-up, significant improvement in bowing angles was noted for group B patients compared with those treated medically. There was, however, no improvement in the height of children in group A, and there was a significant decrease in height among the children treated surgically (group B). Neither height nor bowing angles of femurs or tibias at presentation predicted the need for future osteotomies. However, children who eventually needed surgery were found to be obese at presentation significantly more frequently than children who did not require osteotomy.