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Pagets disease - femoral fracture (Significance (Due to poor bone…
Pagets disease - femoral fracture
Pagets - process of unknown etiology that causes excessive reabsorption and subsequent abnormal remodelling, resulting in abnormally thickened bone and paradoxical reduced tensile strength (genetic basis, freq in anglo-saxon
Significance
Due to poor bone modelling and turnover
Fractures more likely
Disorganised fracture healing
Higher risk malunion, non-union
may have deformity
pain common
Increased risk osteosarcoma
Vascular hypertrophy
Greater risk of bleeding
Risk of high output heart failure
Due to increased size and weight of skull
Hearing loss and impaired balance - increased falls risk
Optic neuropathy - increased falls risk
Nuerological symptoms (cerebellar compression, cerebral tonsillar herniation
Hydrocephalus
Vertebral abnormalities
Compression C spine possible
Atlantoaxial instability
Compression of nerve roots
Lumbar spine stenosis
Extra calcium deposition
Calcification of cardiac structure
Arrhythmia, heart block
Greater risk PVD
Hypercalcaemia
Treatment and adverse events
Antimitotic drugs (colchicine) - symptom relief BUT bone marrow suppression
Bisphosphonates - treat bone pain and high output cardiac failure BUT risk of osteomalacea, stress and pathological fracture
Calcitonin - Treats bone pain BUT limited by development of resistance
Mithramycin - treatment of hypercalcaemia and refractory pain BUT highly cytotoxic, N&V, plt dysfx, hepatotox, nephrotox
Anaesthetic
Preop
Hx and Ex
time course of Pagets and complications
therapy and complications
comorbidities (including ? carotid bruits)
Ix
Greater risk bleeding (G+H, FBE, coags)
Potential electrolyte abnorm
ECG
Imaging - consider for intracranial/spinal pathology, changes to airway management
If sig. deformity of thorax consider spirometry
Intraop
Preparation: possible massive bleeding
Airway - may be difficult (care with C-spine)
Positioning - limited by pain and deformity, avoid excessive force (iatrogenic fracture)
Regional - Possible but care not to needle pathological bone, care with excessive bleeding risk
Likely surgically difficult and prolonged case