Surgical Treatment Combined with NSAIDs in Fibrodysplasia Ossificans Progressiva

Abstract

Fibrodysplasia ossificans progressiva (FOP) is a rare and disabling genetic disorder characterized by congenital malformation of the great toes and by progressive heterotopic ossification. There is no effective treatment. Conservative management is unsuccessful, and operation result in failure because new ectopic bone forms at the operative site. We report a 10-year-old boy with FOP who underwent surgical management combined with non-steroidal anti-inflammatory drugs (NSAIDs).Keywords: ectopic ossification, fibrodysplasia ossificans progressiva, chest wall Ann Thorac Cardiovasc Surg 2012; 18: 61-63 doi: 10.5761/atcs.cr.11.01677 larged. It was hard, 6-cm sized, and palpated over the right pectoralis muscle. He complained of a progressively decreased range of motion in his right shoulder due to pain (45° of forward elevation, and 45° of abduction). Physical examination revealed typical congenital malformations of the great toes (Fig. 1). His parents did not show any similar abnormalities during their physical examinations. No history of local trauma was reported. A chest roentgenogram demonstrated an abnormal calcification. A computed tomography scan showed a 6-cm sized abnormal heterotopic ossification between the pectoralis major muscle and pectoralis minor muscle (Fig. 2). The diagnosis of FOP was made based on the typical features including congenital malformations of great toes and progressive heterotopic ossification. Surgical excision of heterotopic ossification from the right chest was performed to improve range of motion for supportive therapy. The intubation was difficult owing to stiffness of the neck during the preoperative induction. The heterotopic bone located in the anterior aspect of his chest was excised as much as possible via anterior approach. We incised pectoralis major muscle and length of incision was about 5cm. During the operation, meticulously cautious efforts were made to avoid surgical trauma. An operation site incision and blunt muscular dissection was applied as little as possible. Convalescence was uneventful. On the second postoperative day, the patient started active physiotherapy. Naproxen (500 mg, once daily, orally) was administered for two weeks

Feasibility of bisphosphonate therapy in an Indian pediatric patient of fibrodysplasia ossificans progressiva

“…[3] Diagnosis of FOP depends on the clinical and radiological recognition of the characteristic congenital malformations of the great toes and the progressive ossification of soft tissues. [4]…”

“…The bone develops independently of the normal skeleton and forms discrete skeletal elements that can fuse with normal skeletal bone. [4] The patient presented with severe musculoskeletal deformities but no congenital malformation.…”

Fibrodysplasia ossificans progressiva: Review and research activities in Japan

“…The management of joint contractures and trunk deformities due to HO is extremely difficult. Surgery is contraindicated because this almost inevitably causes additional HO and worsens the condition, with only rare exceptions . The safety of physical therapy has not been established but a passive range of motion exercises should be avoided because they may hurt muscles and evoke flare‐ups.…”

Fibro Dysplasia Ossificans Progressiva

“…8 Probably, it is for the reason why it is also named Stoneman's disease. 9 I congratulate the authors for a magnificent, detailed and very well-structured report with perfect inspection, physical examination and radiological shares, especially for this rare case report. Best Regards.…”