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A woman with tuberous sclerosis and acute onset right sided abdominal pain

BMJ 2009; 338 doi: 10.1136/bmj.b987 (Published 24 June 2009)
Cite this as: BMJ 2009;338:b987

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  1. Anu Balan, specialist registrar in radiology,
  2. David Kessel, consultant in vascular radiology
  1. 1Leeds General Infirmary, Leeds LS1 3EX
  1. A Balan balan.anu{at}gmail.com

    A 40 year old woman with a history of tuberous sclerosis presented to the accident and emergency department with severe, acute onset right sided abdominal pain. On clinical examination she looked pale, had poor capillary refill, and was tachycardic and hypotensive. Her abdomen was distended, with bilaterally tender, palpable flank masses. Her haemoglobin measured 8 g/dl. After aggressive resuscitation with intravenous fluids, she was deemed stable enough to undergo computed tomography, as an intra-abdominal source of acute haemorrhage was suspected.

    Figures 1 ⇓ and 2⇓ are computed tomography images of the mid-abdomen and lower abdomen.

    Fig 1 Computed tomogram of the mid-abdomen

    Fig 2 Computed tomogram of the lower abdomen

    Questions

    • 1 What is the likely cause of the masses seen in the figures?

    • 2 What is the cause of the patient’s symptoms in this clinical scenario?

    • 3 What are the management options for the patient?

    Answers

    Short answers

    • 1 Bilateral renal angiomyolipoma is the most likely cause of the masses seen in the figures. The computed tomography scans show that the renal parenchyma is abnormal, with enhancing vessels and low attenuation fat representing vascular and fatty components, respectively, within the angiomyolipoma.

    • 2 The patient’s symptoms are caused by acute haemorrhage. Her physical condition and the imaging findings can be explained by haemorrhage from the vascular component of the angiomyolipoma.

    • 3 Selective renal arterial embolisation is the treatment of choice. Embolisation not only stops further …

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