26. Kongress des Medizinisch-Wissenschaftlichen Beirates der Deutschen Gesellschaft für Muskelkranke (DGM) e.V.

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Fat embolism in Duchenne Muscular Dystrophy – report on a novel case and systematic analysis of published patients

Abstract-Text (inkl. Referenzen) Introduction Fat embolism (FE) in Duchenne muscular dystrophy (DMD) has been described after traumatic fractures of either femur, pelvis, or tibia, similar to FE in the healthy population. Methods We report a non-ambulatory 13-year-old boy with DMD who developed severe acute respiratory distress and coma after elective soft tissue surgery. The clinical picture was suggestive for FE. We performed a systematic literature review for publications on FE in DMD. Together with radiological and clinical data from our case, we analysed the information on age, steroid treatment, ambulation, evidence and localisation of fractures and outcome of available reports. Results Post-surgery radiological evidence of recent bilateral femoral fractures and osteopenia were found as possible cause of cerebral and pulmonal FE in our patient. FE was demonstrated with MRI showing multiple T2-hyperintense and spot-like SWI-lesions. Likely, the fractures occurred during the multi-level tendon-lengthening. After five months, the patient remained in a state of minimal consciousness. Ten publications were identified with case reports of 22 patients with DMD and FE. Most common causes were falls resulting in femoral fractures. Median age at FE was 14 years. The majority had radiological evidence of osteopenia. Summary We want to raise awareness that FE with severe residual impairment can occur in adolescents with DMD from traumatic bone fractures and also after elective surgery.

Ebene 6 Wandelgang Nord: Multiorgan-Manifestationen

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