1 - Serviço de Medicina Física e de Reabilitação, Centro Hospitalar de Lisboa Ocidental, Lisboa
2 - Serviço de Medicina Física e de Reabilitação, Área de Pediatria, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
- Reunião internacional - VIRTUAL ISPRM 2021 Congress “Furthering Rehabilitation in a New World”; Poster;
Resumo:
Background: Osteogenesis Imperfecta (OI) is a connective tissue disorder due to abnormal production of type I collagen. The most common type of OI is type I (Sillence Classification, 1979), which is considered less severe. Type II is a rare form of OI, most often resulting in in-utero or childbirth death. Type III and IV are less severe forms of OI than type II.
Goals: To characterize patients with OI evaluated in a multidisciplinary “fragile bone” consultation (MFBC) of a pediatric hospital.
Methods: Retrospective analysis of the population assessed in a MFBC with OI. Informatic files were reviewed until October of 2020. Basic descriptive data of the patients were registered: date of birth, age, sex, OI type (Sillence and genetic classification), bone deformity characterization, bone fracture characterization, bone resorption inhibition treatment (BRIT) characterization. Correlative statistics regarding the number of bone fractures per OI type and per age group, with or without BRIT, were performed.
Results: N=36 patients were accessed. Mean age 13.2 years. OI Sillence classification: Type I=41.7%, type III=30.6%, type IV=16.7%. Type III was the most common OI type with bone deformities. Scoliosis was identified more often in type III OI. Type I OI patients most often had bone deformities in the lower limbs. Bone fractures were frequent in type III OI and less frequent in type I OI. Peak of incidence of bone fractures in 0-5 and 10-15 years. Type III OI patients required a longer duration of BRIT. There was a reduction of fractures during and after BRIT, mainly in OI type III and IV.
Conclusions: Bone fractures appear to follow the same pattern as in the general population. OI type I seems to have an even distribution of bone fractures in all group ages. In OI type III and IV, there was a great reduction of the number of fractures between 5 years and the end of adolescence. PRM intervention with orthosis and possibly physical modalities may play a role in preventing the development of bone deformities in these patients.
Palavras Chave: bone fracture, Osteogenesis imperfecta, pediatric orthopedics rehabilitation;