RECONSIDERATIONS REGARDING TIME OF FRACTURE HEALING IN PYCNODYSOSTOSIS

ABSTRACTObjective: To discuss what has been described so far in the literature regarding the time taken for fracture consolidation in pycnodysostosis. Materials and Methods: Thirteen new cases were studied, as available from the medical records and radiographic examinations, thus encompassing a total of 44 fractures in patients evaluated between November 1970 and August 2004 at the Orthopedics Hospital, Goiânia. Field research, simultaneous clinical monitoring for new fractures in two patients and retrospective evaluation of medical records were undertaken. The purpose was to determine the total number of fractures in each patient and to determine which of these were viable for this study. The patient group was composed of three women and two men of mean age 51.4 years. The tibia was the bone most affected, followed by the femur. Fractures for which the follow-up was done at another clinic were excluded. Results: Out of the 12 fractures that were considered fully suitable for the study, nine occurred in femurs (six in the left femur and three in the right femur); one in the right tibia; one in the right clavicle; and one in the left ulna. Among these 12 fractures, eight developed pseudarthrosis after an average of 29.25 months; three consolidated well after an average of 5.83 months; and one evolved with delayed consolidation in just 2 months. Conclusion: In combination with genetic and micromorphological evaluations, further studies are awaited for reconfirmation of the diagnosis of such a rare clinical entity.