Table 1.

Reported cases of aneurysms in patients with ARPKD (n = 9)

Study	Country	Gender	Age, yrs	Intra- or extracranial	Artery involved	Multiple or single	Size (widest diameter)	Genetic testing	Rupture	Intervention	Outcome
Gately et al., 2020 (current study)	Australia	F	29	Intracranial	R middle cerebral	Multiple	2 mm	Compound heterozygote PKHD1 variants [p.(Thr36Met) and p.(Ser1156Leu)]	No	No	Asymptomatic
					R middle cerebral		2 mm
					R middle cerebral		1.5 mm
					L internal carotid		4.3 mm
					L internal carotid		2.9 mm
					Basilar apex		2.3 mm
					L superior cerebellar		3.3 mm
					R superior cerebellar		2 mm
Helal et al.,S15 2019	Tunisia	M	29	Intracranial	Bilateral (not specified)	Multiple	9.1 mm	NS	Yes	Endovascular	Died
Perez et al.,S3 2018	U.S.	M	10	Intracranial	L anterior communicating	Single	NS	Compound heterozygote PKHD1 variants (NS)	Yes	Extraventricular drain, coiling, craniectomy	Died
Elchediak et al.,8 2017	U.S.	M	36	Extracranial	Aorta	Multiple	3.1 cm	NS	Yes	No	Complete recovery
					Common iliac bilaterally		1.9 cm
					Celiac		1.2 cm
Elchediak et al.,8 2017	U.S.	F	17	Extracranial	Gastric Splenic	Multiple	NS	NS	Yes	Endoscopy, gastrectomy	Died
Chalhoub et al.,S16 2013	Lebanon	M	21	Intracranial	R anterior choroid	Single	5.7 mm	NS	Yes	Clipping	Complete recovery
Lilova and Petkov,9 2001	Bulgaria	F	2	Intracranial	Middle cerebral Posterior cerebral	Multiple	NS	NS	No	No	Asymptomatic
Neumann et al.,S17 1999	Germany	F	31	Intracranial	R middle cerebral	Multiple	12 mm	NS	No	Clipping	Asymptomatic
					L anterior communicating		8 mm
					L posterior communicating		4 mm
					L anterior choroid		3 mm
De Blasi et al.,S2 1997	France	M	12	Intracranial	L basilar tip	Multiple	5.8 mm	NS	Yes	Coiling and clipping	Complete recovery
					L sylvian artery		4 mm

ARPKD, autosomal recessive polycystic kidney disease; F, female; L, left; M, male; NS, not specified; R, right; US, United States.