Peri-operative management of an adult with tetra-amelia undergoing major abdominal surgery

Tetra-amelia is a rare birth defect characterised by the complete absence of all four limbs. Affected infants are often stillborn or die shortly after birth. There is therefore limited experience in the management of this condition in surviving adults, and published guidelines on peri-operative and anaesthetic management do not exist. We present a 34-year-old woman with tetra-amelia who underwent an elective distal pancreatectomy and splenectomy for a mucinous tumour of the body and tail of the pancreas. The anaesthetic challenges highlighted by this case include difficult intravenous access, unachievable blood pressure monitoring, management of fluid balance status peri-operatively, effective drug dose titration and temperature management. In the absence of intra-operative blood pressure monitoring, other modalities were used to monitor haemodynamic and volume status peri-operatively. As drug dosing guidelines do not exist for this condition and drug pharmacodynamics may be unpredictable, we recommend careful dose titration and bespoke multimodal analgesia, which is best achieved in a high dependency setting.