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Scoliosis in association with infantile hypophosphatasia: a case study in two siblings
1Queen's Medical Centre, Nottingham, United Kingdom. arearoon@lycos.co.uk
Insights
Scoliosis in infantile hypophosphatasia presents unique surgical challenges. Despite disease severity, surgical correction is feasible, with outcomes varying unpredictably, highlighting the need for careful postoperative monitoring.
Area of Science:
- Pediatric Orthopedics
- Metabolic Bone Diseases
- Spinal Surgery
Background:
- Infantile hypophosphatasia is a rare metabolic disorder.
- Scoliosis is an uncommon complication of infantile hypophosphatasia.
- Limited data exists on scoliosis progression and surgical management in this condition.
Observation:
- Two siblings with infantile hypophosphatasia presented with rapidly progressing scoliosis.
- Surgical interventions included spinal fusion, stabilization, and corrective osteotomies.
- Outcomes varied, with one sibling achieving spontaneous fusion and the other requiring multiple procedures.
Findings:
- Scoliosis progression and fusion rates were not correlated with disease severity or biochemical markers.
- Surgical correction is possible for these rapidly progressive curves, even with the metabolic challenges of hypophosphatasia.
- Postoperative complications such as hypercalcemia and seizures require vigilant management.
Implications:
- This study provides valuable insights into the surgical management of scoliosis in infantile hypophosphatasia.
- Understanding potential complications is crucial for optimizing patient care.
- Further research may elucidate factors influencing surgical outcomes in this rare condition.
Study Design:
A case study of scoliosis in two siblings with infantile hypophosphatasia and review of literature are presented.
Objectives:
To report the rare occurrence of scoliosis in two siblings with infantile hypophosphatasia and detail problems in the surgical management of scoliosis in this rare disease.
Summary Of Background Data:
To the authors' knowledge there is only one reported case of scoliosis in infantile hypophosphatasia. However, there is no report describing the nature of the curve, its progression, or the surgical management of scoliosis in infantile hypophosphatasia.
Methods:
The first sibling was diagnosed shortly after birth to have scoliosis that progressed rapidly despite brace treatment. At 4 years, he underwent anterior convex arthrodesis and posterior T6-L1 Luque trolley stabilization. Because of further curve progression, he had to undergo corrective anterior and posterior osteotomies, stabilization, and fusion at 11 years of age. The second sibling was diagnosed to have scoliosis at the age of 3 years and underwent anterior thoracoscopic release and posterior Luque trolley stabilization.
Results:
The first sibling with a mild form of infantile hypophosphatasia had to undergo multiple procedures to attain fusion and arrest of curve progression. The second sibling with a severe variety of infantile hypophosphatasia surprisingly attained a spontaneous fusion of the curve following the initial correction and stabilization with no further progression at 9-year follow-up.
Conclusions:
Curve progression and fusion rates following scoliosis correction are not related to the disease severity or biochemical findings. These rapidly progressive curves are amenable to surgical correction despite the unfavorable metabolic nature of the disease. Hypercalcemia and seizures responding to pyridoxine are complications in the postoperative period of which the surgeon should be aware.
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