Background and Aims Apert’s syndrome is a rare congenital disorder,characterised by premature fusion of the cranial sutures. Abnormalities of the skull, cervical spine, facial and tracheal architecture make airway management challenging1. These and other musculoskeletal abnormalities and comorbidities can greatly increase anaesthesia risks.
Methods A 53-year-old lady with Apert’s syndrome presented for primary hip replacement. She had multiple previous craniofacial, foot and hand operations; several fused cervical vertebrae; and an emergency tracheostomy in the past. She reported difficult vascular access and difficulty breathing through the nose. Her BMI was 35. Due to the high risk of airway and respiratory complications, successful regional anaesthesia was deemed imperative.
Results Ultrasound was used to assist the successful performance of spinal anaesthesia. Low dose propofol by infusion was used as anxiolytic sedation. The two hour procedure was extremely well tolerated.
Conclusions This case highlights the importance of being able to apply regional anaesthesia as a sole technique, and the utility of ultrasound assistance for neuraxial techniques in potentially difficult patients2. This relatively young patient is likely to require future lower limb surgery such as revision hip surgery. The patient’s positive experience, which has made future awake surgery more acceptable, and demonstration to future anesthetists of the technique’s viability, was vital and was achieved.
In such patients, where significant comorbidities render general anaesthesia hazardous, regional anaesthesia techniques may be lifesaving. This will be ever more the case as our population’s burden of comorbidities increases and the epidemic of revision joint replacements is upon us.
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