Surgical outcome in fibrodysplasia ossificans progressiva: A case study
Journal Title: Ortopedia Traumatologia Rehabilitacja - Year 2004, Vol 6, Issue 5
Abstract
Background. Fibrodysplasia ossificans progressiva (FOP), also known as Munchmeyer's Disease, is a rare inherited disease. By 1996 there had been no more than 200 cases described in world literature. The disease is characterized by progressive ectopic ossification and congenital deformity of the hallux. Patients with FOP require full-time nursing care.Case history. This article presents the case of a female patient, initials LP, age 42, who has been suffering from FOP since early childhood. The authors describe the course of the disease, the patient's current clinical status, the results of surgical treatment for a decubitus ulcer on the right thigh, and a review of the literature on this rare disease. At present the patient is almost completely immobilized. The spine is completely stiffened in a slightly kyphotic position. The ribs are not mobile. The decubitus ulcer has been excised. After surgery on the knee, the range of motion in the operated knee joint increased to 20°. The improvement in mobility is stable and has remained unchanged for 16 months after surgery.Conclusions. In this patient with FOP it turned out to be possible to operate on the knee, provided that a delicate surgical technique was used, with the appropriate use of non-steroid analgesic drugs, along with biphosphonians and glycosteroids. The range of motion in the knee was increased by releasing the tendon of the femoral biceps, which improved the patient's quality of life.
Authors and Affiliations
Bogdan Wziętek, Bogusław Frańczuk
Keywords
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