Study to determine if brain aneurysms should be sought in patients with PKRAD

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the progressive development of renal cysts, as well as by external abnormalities in the kidneys, including cerebral aneurysms or weaknesses of the cerebral blood vessels that swell and fill with blood and may subsequently to break up. A new study published in CJASN examined whether patients with PKRAD should be systematically screened for cerebral aneurysms.

For the study, Vincente E. Torres, MD, PhD (Mayo Clinic) and colleagues reviewed the medical records of 812 patients with PKRA evaluated between 1989 and 2017 and subjected to brain imaging tests despite the absence of neurological symptoms.

Among the main discoveries:

• 94 brain aneurysms were diagnosed in 75 out of 812 (9%) patients who underwent screening. None of the aneurysms failed after a mean follow-up of 9 years.
• Sex, age, race, and genetics related to PKRAD were similar in groups with and without aneurysm, but hypertension and smoking history were more common in the aneurysm group.
• 29% of patients with aneurysms compared to 11% of those who did not have had a family history of meningeal hemorrhage, a type of stroke caused by bleeding in the space surrounding the brain.
• Among patients who had aneurysms during initial screening, new cerebral aneurysms were detected in 5 patients during an average follow-up of 8 years and none of the aneurysms failed.
• Of 135 of the 737 patients without cerebral aneurysms detected during the first screening and who were screened for additional screening, 3 patients developed aneurysms after an average follow-up of 7 years and 2 patients had a rupture. cerebral aneurysm. Both patients had significant risk factors for development and rupture of cerebral aneurysm.

The researchers noted that their findings were inconclusive as to whether generalized or selective screening of brain aneurysms was beneficial for PKRAD. A large prospective study would be needed to determine the benefits and costs of such strategies.

Our approach has been to recommend screening in patients with PKRAD who have a family history of aneurysm. We also recommend that patients with PKRAD be screened before major non-emergency surgical procedures (including transplantation), those who practice a high-risk occupation, and those who, having been properly informed about the available data, wish to be screened for their condition. rebadure. We educate our patients on the importance of correcting conditions badociated with the development and / or rupture of aneurysms, particularly smoking and poorly controlled hypertension. The results of our study do not provide a reason to change our current approach. "

Dr. Vincente E. Torres, Mayo Clinic

In an accompanying editorial, Ivana Kuo, PhD (Loyola University School of Medicine) and Arlene Chapman, MD (University of Chicago) indicated that "this report provides additional confirmation information regarding the 39, increased frequency of intracranial aneurysms, traditional features of intracranial aneurysms, ADPKD similar to the general population, and a significant need for more mechanistic studies to determine the central role of ADPKD proteins in the formation of intracranial aneurysms. "