ABSTRACT
Tuberous sclerosis complex is a genetic disease that can affect multiple organs, commonly the renal, central nervous, cardiac and respiratory systems. Here, we present the peripartum management of a woman with a new diagnosis of tuberous sclerosis complex associated with massive renal angiomyolipomas. There was significant risk of catastrophic haemorrhage from the angiomyolipomas during pregnancy. This created a challenging scenario for the anaesthetists, obstetricians, urologists and interventional radiologists involved in their care, necessitating collaborative working, numerous investigations and honest patient-centred discussions. Evidence for the management of tuberous sclerosis and angiomyolipomas in pregnancy does not appear to have been previously reported. After much consideration, arterial embolisation of the most vascular area of the angiomyolipoma was completed at 28 weeks gestation under remifentanil sedation. An elective caesarean section under combined spinal epidural anaesthesia was completed at 37 + 3 weeks gestation. Central nervous system involvement must always be considered; benign tumours within the brain and spinal cord may contraindicate neuraxial techniques. Early multidisciplinary involvement is essential due to the diverse and complex nature of tuberous sclerosis. Individuals differ in their presentation and disease severity; therefore, they must be assessed on an individual basis with management tailored accordingly.
