Ka. Mchale et al., PERCUTANEOUS INTRAMEDULLARY FIXATION OF LONG-BONE DEFORMITY IN SEVEREOSTEOGENESIS IMPERFECTA, Clinical orthopaedics and related research, (305), 1994, pp. 242-248
Reported here are seven patients who had severe osteogenesis imperfect
a, and who were extremely fragile, with three having cardiac disease.
Their age ranged between 8 and 35 months. All seven patients had unbra
ceable deformities. Twenty five long bones, including 14 tibiae, 10 fe
mora, and 1 ulna underwent percutaneous intramedullary fixation. Four
patients had all four lower extremity long bones operated on simultane
ously, Followup was 2 to 11 years. There were no neurologic or vascula
r complications, compartment syndromes, growth plate problems, or tran
sfusion requirements. All bones heated. One patient had migration of a
femoral pin into the knee joint. After bracing, all patients were abl
e to sit; five were able to stand and eventually walk. All living pati
ents were able to be sustained until successful definitive long bone f
ixation could be accomplished, approximately 2 years or longer after t
his initial procedure, With this safe, reproducible method, early stab
le fixation can be provided to patients too young and/or too sick for
definitive or extensive open surgery. Future treatment regimens are no
t compromised.