KHA-CARI Autosomal Dominant Polycystic Kidney Disease Guideline: Management of Intracranial Aneurysms

Autosomal dominant polycystic kidney disease and the heart and brain

“…Magnetic resonance angiography (MRA) with gadolinium enhancement and computed tomographic angiography (CTA) are recommended for screening in ADPKD patients with normal renal function, 67 but time-of-flight MRA without gadolinium is the imaging test of choice because it is noninvasive and poses no risk of nephrotoxicity or contrast allergy. 3,68 Further, gadolinium should be avoided in patients whose eGFR is 30 mL/min/1.73 m 2 or less because of risk of nephrogenic systemic sclerosis and fibrosis.…”

“…3,68 Further, gadolinium should be avoided in patients whose eGFR is 30 mL/min/1.73 m 2 or less because of risk of nephrogenic systemic sclerosis and fibrosis. 67,68 The sensitivity of time-of-flight MRA screening for intracranial aneurysm varies depending on the size of aneurysm; 67% for those less than 3 mm, 79% for those 3 to 5 mm, and 95% for those larger than 5 mm. 69 The sensitivity of CTA screening is 95% for aneurysms larger than 7 mm and 53% for those measuring 2 mm.…”

“…Screening for intracranial aneurysm is recommended at the time of ADPKD diagnosis for all high-risk patients, ie, those who have a family history of intracranial hemorrhage or aneurysm in an affected first-degree relative. 67 It is also recommended for ADPKD patients with a history of sudden-onset severe headache or neurologic symptoms. 67 A third group for whom screening is recommended is AD-PKD patients who have no family history of intracranial aneurysm or hemorrhage but who are at risk of poor outcome if an intracranial aneurysm ruptures (eg, those undergoing major elective surgery, with uncontrolled blood pressure, on anticoagulation, with a history of or current smoking, and airline pilots).…”

“…67 It is also recommended for ADPKD patients with a history of sudden-onset severe headache or neurologic symptoms. 67 A third group for whom screening is recommended is AD-PKD patients who have no family history of intracranial aneurysm or hemorrhage but who are at risk of poor outcome if an intracranial aneurysm ruptures (eg, those undergoing major elective surgery, with uncontrolled blood pressure, on anticoagulation, with a history of or current smoking, and airline pilots). 67 Patients found to have an intracranial aneurysm on screening should be referred to a neurosurgeon and should undergo repeat MRA or CTA imaging every 6 to 24 months.…”

“…67 A third group for whom screening is recommended is AD-PKD patients who have no family history of intracranial aneurysm or hemorrhage but who are at risk of poor outcome if an intracranial aneurysm ruptures (eg, those undergoing major elective surgery, with uncontrolled blood pressure, on anticoagulation, with a history of or current smoking, and airline pilots). 67 Patients found to have an intracranial aneurysm on screening should be referred to a neurosurgeon and should undergo repeat MRA or CTA imaging every 6 to 24 months. 3 High-risk ADPKD patients with normal findings on initial screening should have repeat MRA or CTA screening in 5 to 10 years unless they suffer from sudden-onset severe headache or neurologic symptoms.…”