Left Wunderlich syndrome from a ruptured angiomyolipoma in a single functioning kidney, eight years after haemorrhage-induced contralateral nephrectomy in tuberous sclerosis complex: a case report

Abstract Background Wunderlich syndrome (WS) is a rare, potentially life-threatening clinical entity characterised by acute spontaneous renal haemorrhage into the perirenal space. Rupture of a renal angiomyolipoma (AML) associated with tuberous sclerosis complex (TSC) is a recognised cause. WS occurring in a single functioning kidney is exceptionally rare; reports of WS arising in a single or functionally solitary kidney remain scarce, and its occurrence on a background of TSC after a previous contralateral nephrectomy is exceptional. Case presentation We report a 39-year-old man with TSC and a right nephrectomy eight years earlier for a ruptured renal AML, who presented with acute left flank pain, anaemia and haemodynamic instability. Contrast-enhanced computed tomography (CT) of the abdomen and pelvis demonstrated a large perirenal haematoma arising from a ruptured left renal AML with an associated pseudoaneurysm, together with multiple hepatic AMLs and a lesion at the pancreatic tail consistent with an AML. Facial angiofibromas and calcified subependymal nodules on cranial CT supported the diagnosis of TSC. After resuscitation, emergency selective transarterial embolisation was performed, after which haemodynamics and renal function stabilised. Conclusions WS in a single functioning kidney on a background of TSC is a rare presentation in which multimodality imaging is decisive for recognising the source of haemorrhage and guiding a nephron-sparing approach. Awareness of this entity and its characteristic imaging features is essential for timely diagnosis and preservation of renal function. Clinical trial number Not applicable.