Korean J Radiol.  2005 Mar;6(1):52-54. 10.3348/kjr.2005.6.1.52.

Bilateral Transverse (Bowdler) Fibular Spurs with Hypophosphatasia in an Adolescent Girl

Affiliations
  • 1Fatih University Faculty of Medicine Department of Orthopaedics, Ankara, TURKEY. ahmetkaradag@gmail.com

Abstract

Hypophosphatasia is a clinically heterogeneous inheritable disorder characterized by defective bone mineralization and the deficiency of serum and tissue liver/bone/kidney alkaline phosphatase activities. Due to the mineralization defect of the bones, various skeletal findings can be radiologically observed in hypophosphatasia. Bowing and Bowdler spurs of long bones are the characteristic findings. The Bowdler spurs reported on in the previous pertinent literature were observed in the perinatal aged patients and these lesions have rarely involved adolescents. We herein report on a 14-year-old girl with fibular Bowdler spurs.

Keyword

Hypophosphatasia; Adolescent; Fibular Bowdler spurs

MeSH Terms

Adolescent
Bone Diseases/pathology/radiography
Diagnosis, Differential
Female
Fibula/pathology/*radiography
Humans
Hypophosphatasia/pathology/*radiography

Figure

  • Fig. 1 A 14-year-old girl with hypophosphatasia. A. The general appearance of the patient with marked growth retardation and shortness in her right lower extremity. B. The skin overlying the Bowdler spurs shows fibrotic dimpling and brown discoloration with bilateral pes planovalgus deformity of the feet. C. Bilateral fibular Bowdler spurs. D. An irregularity of the articular surfaces of both the femoral head and the acetabulums with narrowing of the joint space, and the diffuse osteoporosis and radiolucent areas in all the bones are prominent.


Reference

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3. Rathbun J. Hypophosphatasia, a new developmental anomaly. Am J Dis Child. 1948. 75:822–827.
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5. Whyte MP, Vrabel LA, Schwartz TD. Alkaline phosphatase deficiency in cultured skin fibroblasts from patients with hypophosphatasia: comparison of the infantile, childhood, and adult forms. J Clin Endocrinol Metab. 1983. 57:831–837.
6. Kozlowski K, Sutcliffe J, Barylak A, Harrington G, Kemperdick H, Nolte K, et al. Hypophosphatasia. Review of 24 cases. Pediatr Radiol. 1976. 5:103–117.
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