Wrist Arthrodesis Following Ulnar Bar Excision in Fibrodysplasia Ossificans Progressiva

Abstract

Fibrodysplasia ossificans progressiva is a rare disorder characterized by the progressive development of heterotopic bone in the connective tissues of skeletal muscle, ligaments and tendons. Surgical trauma is one of the most potent stimuli for ossification and surgical treatment is generally considered to be contraindicated in this condition. We report a good functional result in a patient with severe hand disability secondary to an ulna-carpal bar in fibrodysplasia ossificans progressiva.

Developmental Anomalies of the Cervical Spine in Patients With Fibrodysplasia Ossificans Progressiva Are Distinctly Different From Those in Patients With Klippel-Feil Syndrome

“…[1][2][3][4][5][6][7][8][9][10][11] In addition to the great toe malformations seen in approximately 95% of FOP patients, 1,4,6,7,9 -11 joints become ankylosed by heterotopic bone formation, leaving most patients wheelchair-bound by the third decade of life. [1][2][3][4][5][6][7][8][9][10][11] Currently, there is no effective treatment to stop the progression of this genetic disease, 9 -11 although a growing body of evidence suggests that interruption of an overactive BMP pathway may be a promising treat- 21 Recent studies suggest that the causal mutation of this devastating disease may be found within genes of the BMP signaling pathway, which contribute to embryonic skeletal patterning and postnatal heterotopic ossification. [22][23][24][25][26][27][28] All of the lateral cervical spine radiographs of the 70 FOP patients reviewed in this study showed similar vertebral abnormalities consisting of narrow vertebral bodies, enlarged pedicles, and large spinous processes consistent with previous reports.…”

“…Fibrodysplasia ossificans progressiva (FOP) is a rare, autosomal dominant disorder of connective tissue characterized by congenital malformation of the great toes [1][2][3][4][5][6][7][8][9][10][11] and by progressive, disabling heterotopic ossification of soft connective tissue in characteristic anatomic patterns. [1][2][3][4][5][6][7][8][9][10][11] Reproductive fitness is low in patients with FOP due to severe progressive disability.…”

“…[1][2][3][4][5][6][7][8][9][10][11] Reproductive fitness is low in patients with FOP due to severe progressive disability. There are few known familial occurrences with most cases occurring sporadically.…”

When Limb Surgery Has Become the Only Life-Saving Therapy in FOP: A Case Report and Systematic Review of the Literature

“…In these seventeen articles, thirty two procedures were described in twenty patients. Ten procedures involved the upper limbs (12)(13)(14)(15)(16)(17)(18)(19), twenty-two the lower limbs ( Table 1) (12,13,(19)(20)(21)(22)(23)(24)(25)(26)(27)(28).…”

“…All ten surgeries performed on the upper limbs were done to remove either an undiagnosed swelling or mature HO. The reoccurrence of HO was described for eight of the ten procedures (12-15, 18, 26) and the clinical outcome for nine cases (12,14,15,18,19,26). Reoccurrence of HO was observed in all eight procedures, however, in four of the nine procedures for which the clinical outcome was described, a clinical improvement was noted (12,15,19).…”