The abdominal angiographic spectrum of tuberous sclerosis.

Tuberous sclerosis can present with a variety of clinical, roentgenographic and pathological manifestations. Although we are not advocating abdominal angiography in every patient with tuberous sclerosis, the clinical diagnosis may be difficult in certain cases, particularly in the young patient, and abdominal visceral angiography may lead to the correct diagnosis. In two of our patients, infants ten months of age, the diagnosis of tuberous sclerosis was not made until abdominal angiography had been performed. The most common changes are seen in the kidney where angiographic demonstration of hamartomas, arterial aneurysms, and multiple small cysts in the renal parenchyma are noted. Renal microcysts have not been previously described angiographically although they have been noted in pathological specimens and represent an important feature of the disorder spectrum. In addition, vascular hamartomatous lesions may be demonstrated angiographically in other abdominal organs such as the adrenal gland and liver.