Fibrodysplasia ossificans progressiva in children (review and clinical analysis of 5 case reports)

Abstract

Ossificans progressive fibrodysplasia (OPF) presents a rare genetically caused osteopenia manifested by calcification and ossification of muscles, tendons, ligaments, fascia, and subcutaneous tissues due to aggressive fibroblasts proliferation. The disease is usually associated with congenital skeletal abnormalities: clinodactyly of the first toe, ankylosis of the first interphalangeal joints, and flexion contracture of the first finger. Surgical attempts of ossifications excision are unsuccessful and worsen the situation. 5 patients with OPF are presented. A course of the disease, clinical pattern, and surgery outcomes are disclosed. Both orthopedic manifestations such as congenital hand and foot abnormalities being a key to early clinical evaluation and secondary changes are described in detail. Also, postoperative complications are presented. Surgeonsare recommended to be on guard against this disease when planning the operations for heterotopic ossifications as well as for relating hand and foot abnormalities.

Keywords

• прогрессирующая оссифицирующая фибродисплазия
• пороки развития кистей и стоп
• врожденная клинодактилия
• fibrodysplasia ossificans progressiva
• congenital feet and hands deformities
• toe clinodactyly