Asymptomatic intracranial aneurysms in beta-thalassemia: a three-year follow-up report

Abstract

Background: No information is currently available regarding the natural history of asymptomatic intracranial aneurysms in beta-thalassemia, raising several concerns about their proper management. Methods: We performed a prospective longitudinal three-year-long MR-angiography study on nine beta-thalassemia patients (mean-age 40.3 ± 7.5, six females, 8 transfusion dependent) harboring ten asymptomatic intracranial aneurysms. In addition, we analyzed the clinical files of all adult beta-thalassemia patients (160 patients including those followed with MR-angiography, 121 transfusion dependent) referring to our Centers between 2014 and 2019 searching for history of subarachnoid hemorrhage or history of symptomatic intracranial aneurysms. Results: At the end of the three-year-long follow-up, no patient showed any change in the size and shape of the aneurysms, none presented new intracranial aneurysms or artery stenoses, none showed new brain vascular-like parenchymal lesions or enlargement of the preexisting ones. Besides, in our database of all adult beta-thalassemia patients, no one had history of subarachnoid hemorrhage or history of symptomatic intracranial aneurysms. Conclusions: Incidental asymptomatic intracranial aneurysms do not seem to be associated, in beta-thalassemia, with an increased risk of complications (enlargement or rupture) at least in the short term period, helping to optimize human and economic resources and patient compliance during their complex long-lasting management.

Cerebral venous sinus thrombosis and aneurysm in a patient with double heterozygous beta-thalassemia major

“…[11] A three-year MRI and MRA report recorded nine patients with beta-thalassemia harboring asymptomatic intracranial aneurysms. [12] The results showed that aneurysms did not change in size or shape. However, it is worth noting that all the patients recorded in the study had relatively small aneurysms at baseline (2-5 mm) with a very low PHASES aneurysm risk score.…”

“…However, a controlled study showed that there was no difference in the incidence of aneurysms or size between beta-thalassemia and control [11] . A three-year MRI and MRA report recorded nine patients with beta-thalassemia harboring asymptomatic intracranial aneurysms [12] . The results showed that aneurysms did not change in size or shape.…”

Brain perfusion changes in beta-thalassemia

“…White matter microvascular impairment is also questionable, at least among patients treated according to the current guidelines [ 5 , 6 ]. Intracranial vasculature (arteries and venous sinuses) does not appear to be significantly involved as increased rate or evolution of intracranial stenosis, venous thrombosis, and aneurysms were not confirmed in recent literature [ 5 , 7 ]. Recently, a regionally specific vulnerability of the white matter was shown in a large sample of anemias, including beta-thalassemia [ 8 ].…”