ABSTRACT
OBJECTIVE: 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.
ABSTRACT
OBJETIVO: Discutir o que vem sendo descrito na literatura até então a respeito do tempo de consolidação das fraturas na picnodisostose. MÉTODOS: Treze novos casos foram estudados por questão de disponibilidade de prontuários e exames radiográficos, totalizando 44 fraturas englobando pacientes avaliados no período de novembro de 1970 a agosto de 2004, no Hospital Ortopédico de Goiânia. Pesquisa em campo, acompanhamento clínico simultâneo, por novas fraturas, de duas pacientes e avaliação retrospectiva de prontuários foram feitos, com base em se determinar o número de fraturas totais de cada paciente e quais destas tinham viabilidade para esta pesquisa. O grupo de pacientes compõe-se de três mulheres e dois homens com idade média de 51,4 anos. A tíbia foi o osso mais acometido, seguido pelo fêmur. Foram excluídas as fraturas cujo acompanhamento tenha sido feito em outro serviço. RESULTADOS: Das 12 fraturas consideradas plenas para o estudo, nove aconteceram em fêmures (seis no fêmur E e três no fêmur D; uma em tíbia (D); uma em clavícula (D) e uma em ulna (E)). Dentre as 12 fraturas, oito evoluíram com pseudartrose em um tempo médio de 29,25 meses; três consolidaram bem em uma média de 5,83 meses e um paciente evoluiu com retardo de consolidação em apenas dois meses. CONCLUSÃO: Associadas à pesquisa gênica e estudos micromorfológicos, aguarda-se novos estudos para reconfirmação diagnóstica numa entidade clínica tão rara.
OBJECTIVE: To discuss what has been described so far in the literature regarding the time of consolidation of fractures in Pycnodysostosis. MATERIALS AND METHODS: Thirteen new cases were studied, as a matter of the availability of medical records and radiographic examinations, totaling 44 patients in the period from November 1970 to August 2004 in the Hospital Ortopédico de Goiânia. Field research and simultaneous clinical monitoring for new fractures in two patients, and the retrospective evaluation of medical records were made on the basis of determining the total number of fractures for each patient to determine which of these were viable for this study. The group of patients is composed of three women and two men aged 51.4 years. The tibia was the bone most affected, followed by the femur. Fractures the follow-up for which had been done in another facility were excluded. RESULTS: Of the 12 fractures fully considered for the study, nine occurred in femurs (six in the femur E and three in the femur D; one in the tibia (D); one in the clavicle (D); and one in the ulna (E)). Among the 12 fractures, eight developed pseudoarthrosis at an average of 29.25 months, three consolidated well in an average of 5.83 months, and one patient progressed with delayed consolidation in just 2 months. CONCLUSION: In combination with genetic and micromorphological studies, we await further studies to reconfirm the diagnosis of such a rare clinical entity.
