Naslov
Intramedullary canal sclerosis - a possible complication of prolonged bisphosphonate therapy in children with osteogenesis imperfecta

Autori
Jeleč, Željko ; Antičević, Darko ; Primorac, Dragan

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
Balancing life with OI / - Stockholm, 2023, 49-50

Skup
Balancing life with OI - Topical meeting on the impact of pain in osteogenesis imperfecta (OI)

Mjesto i datum
Stockholm, Švedska, 09.06.2023. - 10.06.2023

Vrsta sudjelovanja
Predavanje

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
osteogenesis imperfecta, intramedullary sclerosis, bisphosphonate

Sažetak
Objectives The pain is one of the biggest complaints of patients with osteogenesis imperfecta (OI). Successful treatment of the disease leads to satisfactory pain decreasing. Bisphosphonate (BP) intravenous infusions are a cornerstone of the treatment of OI. Prolonged BP therapy in case of significant deformity of the long bone will cause a problem during a surgery – sclerosis of intramedullary (IM) canal. Te main goal of our study was to show the changes in long bone morphology which were connected with prolonged BP administration before the surgery. Methods The inclusion criteria for this study were patients without previous surgery, severe deformation of long bone segment (more then 50 degree of varus or procurvatum) and prolonged treatment with BP before the surgery. All radiographs were reviewed by single reviewer. IM sclerosis was measured on lateral radiographs because severe deformed segments had a specific “rib shape”. Both ends points of sclerotic area were determined, and a whole surface of sclerotic area as well as percentage of sclerotic part was calculated. An angles of varus and procurvatum were measured also Results We found 17 segments (14 femurs and 3 tibias) in 9 patients which had some amount of IM sclerosis. All of included children were treated with intravenous infusions of pamidronate. The percentage of sclerosing part is decreasing till the fifteenth pamidronate infusion, after that we found its increasing. Obstacles during surgery were splitting of bone during drilling ; drill breakage ; “false route” ; prolonged surgical time and increased blood losses. Our suggestions how to avoid surgical difficulties are: precise preoperative planning, precise operative technique, many x-ray controls, gradual and patient reaming, reaming with surgical drill (instead of canullated drill) and excision of the bone block (triangular or trapezoidal shape). Conclusion The correct way of treatment of OI is to start with BP first To prevent long bone IM sclerosis one should decide when to perform surgical long bone deformity correction. According to our results, the threshold for the operative treatment should be 15 cycles of BP infusions. Anticipating and avoiding difficulties and complications of surgical treatment leads to better results and consecutively adequately pain decreasing.

Izvorni jezik
Engleski

POVEZANOST RADA