Sudden death due to subarachnoid haemorrhage in an infant with autosomal dominant polycystic kidney disease

Intracranial aneurysm rupture is the most serious and potentially lethal extra-renal manifestation of autosomal dominant polycystic kidney disease (ADPKD). Almost all cases of ruptured intracranial aneurysm occur in adult patients with a median age of rupture of 40 years. We report the occurrence of sudden death in a newborn infant born to a mother with typical ADPKD in the first week of life. Post-mortem examination revealed the cause of death to be subarachnoid haemorrhage with focal glomerular and tubular cysts detected in the kidney. This is the earliest reported case of intracranial aneurysm rupture in ADPKD and should raise awareness of this rare but lethal complication in younger patients.