An unusual presentation of Wunderlich syndrome

[摘要] Spontaneous renal haemorrhage secondary to renal angiomyolipoma, or Wunderlich syndrome, is rare. Renal angiomyolipomata are composed of abnormal vasculature, smooth muscle and adipose tissue. They are more likely to be symptomatic if they are larger than 4 cm, presenting with a history of flank pain, a palpable mass and rarely gross haematuria. Our case illustrates a rare but catastrophic presentation of Wunderlich syndrome. A 29-year-old woman, with no significant past medical history, presented with sudden onset right-sided abdominal pain. The patient appeared pale and revealed localised peritonitis in the right iliac fossa. A diagnosis of acute appendicitis was made and expeditious laparoscopy was performed. At surgery a large right-sided retroperitoneal haematoma extending to the perinephric area was identified. Due to continuing significant haemorrhage an emergency radical nephrectomy was necessary. The patient made an excellent recovery and the histology confirmed large renal angiomyolipoma as the underlying pathology. This is the first case report of Wunderlich syndrome in a previously asymptomatic fit young patient. This novel presentation of Wunderlich syndrome highlights the diagnostic difficulties when faced with the acute abdomen. This report aims to equip clinicians with knowledge and heightened awareness of this rare condition to optimise patient care.

[学科分类] 传染病学

[关键词] Angiomyolipoma;Wunderlich syndrome;retroperitoneal haemorrhage