Extended follow-up of unruptured intracranial aneurysms detected by presymptomatic screening in patients with autosomal dominant polycystic kidney disease

Abstract

Background and objectives: Autosomal dominant polycystic kidney disease (ADPKD) patients have an increased risk for intracranial aneurysms (IAs). The importance of screening for unruptured IAs (UIAs) depends on their risks for growth and rupture.

Design, setting, participants, & measurements: ADPKD patients with UIAs found by presymptomatic screening with magnetic resonance angiography (MRA) during 1989 to 2009 were followed initially at 6 months and annually, and less frequently after demonstration of stability.

Results: Forty-five saccular aneurysms were detected in 38 patients from 36 families. Most were small (median diameter 3.5 mm) and in the anterior circulation (84%). Median age at diagnosis was 49 years. During cumulative imaging follow-up of 243 years, one de novo UIA was detected and increased in size from 2 to 4.4 mm over 144 months and two UIAs grew from 4.5 to 5.9 mm and 4.7 to 6.2 mm after 69 and 184 months, respectively. Seven patients did not have imaging follow-up. No change was detected in the remaining 28 patients. During cumulative clinical follow-up of 316 years, no aneurysm ruptured. Five patients died from unrelated causes and two were lost to follow-up after 8 and 120 months. Three patients underwent surgical clipping.

Conclusions: Most UIAs detected by presymptomatic screening in ADPKD patients are small and in the anterior circulation. Growth and rupture risks are not higher than those of UIAs in the general population. These data support very selective screening for UIAs in ADPKD patients, and widespread screening is not indicated.

Figure 1.

Twenty-nine-year-old man (M32**) found to harbor a 3-mm right MCA trifurcation aneurysm in 1995. The patient returned in 1997 for a follow-up MRA that demonstrated stability of the right MCA trifurcation aneurysm but development of a de novo 2-mm mirror left MCA trifurcation aneurysm (arrowhead). Subsequent imaging showed enlargement of the left MCA trifurcation aneurysm to a size of 4.4 mm in 2009 (arrow).

Figure 2.

Forty-five-year-old man (P34) was found to have a 4.5-mm AcoA aneurysm identified on screening MRA in 1998 (arrowhead). Subsequent MRA demonstrated enlargement to 5.9 mm in 2004 (arrow).

Figure 3.

Forty-five-year-old woman (M72) with a family history of SAH was found to have a 4.7-mm left superior cerebellar artery aneurysm during screening in 1995. Subsequent imaging demonstrated slight enlargement to a maximal size of 6.2 mm in 2010.

Figure 4.

Serial MRAs of a 2.0-mm right superior cerebellar aneurysm (arrow) in a 45-year-old woman (M80). No change was seen in aneurysm size or morphology between 1991 and 2009. Before 2000, MRAs were performed using a magnetic field strength of 1.5 T; subsequent studies were performed at 3.0 T.

Figure 5.

A series of patients with stable aneurysms (arrows). (A) A 59-year-old woman (P9) before a major surgery in 1991 was found to have a 4.0-mm right carotid siphon aneurysm that was unchanged during 17 years of follow-up. (B) A 45-year-old woman (M69) with a family history of SAH screened with MRA was found to have a 2.0-mm basilar tip aneurysm that remained unchanged between 1991 and 2008. (C) A 49-year-old woman (M32*) with a family history of IA who underwent screening before a kidney transplant was found to have a 6.0-mm right carotid siphon aneurysm in 1992. Subsequent follow-up has not shown a significant change in the size of the aneurysm.