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Surgical outcome in fibrodysplasia ossificans progressiva: A case study
Bogdan Wzietek1, Bogusław Frańczuk
1Indywidualna Praktyka Specjalistyczna, Chrzanów.
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 degrees . 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.
Insights
This case study shows that surgical intervention on the knee is possible for patients with Fibrodysplasia Ossificans Progressiva (FOP), a rare genetic disorder. Delicate surgical techniques and specific medications improved the patient's mobility and quality of life.
Area of Science:
- Genetics
- Orthopedics
- Rare Diseases
Background:
- Fibrodysplasia Ossificans Progressiva (FOP), or Munchmeyer's Disease, is a rare inherited disorder characterized by progressive ectopic ossification and congenital hallux deformity.
- FOP leads to severe immobilization and requires extensive nursing care.
- Fewer than 200 cases were documented by 1996, highlighting its rarity.
Purpose of the Study:
- To present a case study of a 42-year-old female patient with FOP.
- To describe the disease progression, clinical status, and surgical outcomes.
- To review literature on FOP management and treatment.
Main Methods:
- Detailed case history of a 42-year-old female patient with FOP.
- Surgical intervention for a decubitus ulcer and subsequent knee surgery.
- Pharmacological management including non-steroid analgesics, biphosphonates, and glycosteroids.
- Literature review on Fibrodysplasia Ossificans Progressiva.
Main Results:
- The patient experienced significant immobilization, including a stiffened spine and immobile ribs.
- Surgical excision of a decubitus ulcer was performed.
- Knee surgery resulted in a stable 20-degree increase in range of motion, maintained for 16 months.
- The release of the femoral biceps tendon contributed to improved knee mobility.
Conclusions:
- Surgical intervention on the knee joint is feasible in FOP patients using delicate techniques.
- A multimodal approach involving specific medications (analgesics, biphosphonates, glycosteroids) is crucial.
- Improved knee mobility through surgical release of the biceps tendon enhanced the patient's quality of life.
