Artikel
The characteristics of cerebral aneurysms in young patients with tuberous sclerosis
Merkmale zerebraler Aneurysmen von jungen Patienten mit tuberöser Sklerose
Suche in Medline nach
Autoren
Veröffentlicht: | 8. Mai 2019 |
---|
Gliederung
Text
Objective: Tuberous sclerosis complex (TSC) is a rare multisystem genetic disease. Arterial wall developmental disorders in TSC such as aneurysms have been well described for extracranial vasculature. The characteristics of intracranial aneurysms (IA) in TSC have not been addressed in the literature yet. This study aimed to perform a systematic review to identify and assess the distinct characteristics of IA in patients with TSC.
Methods: We searched PubMed, Scopus and Web of Science for publications describing cases with TSC and IA reported before August 7th, 2018. Moreover, we report two cases of IA in TSC patients treated at our institution.
Results: Thirty-three TSC patients with a total of 42 IA were included in this review. Three individuals presented with subarachnoid hemorrhage. IA were large or giant in 57.1% and fusiform in 45.2% of the cases. Most of the IA originated from the ICA (61.9%, n=26). There was a higher prevalence of pediatric cases (66.7%) and male patients (63.6%, 21 of 32 individuals with known sex) in the collected series.
Conclusion: TSC patients with IA are characterized with a higher proportion of large/giant and fusiform IA in young patients, so that a rapid aneurysmal growth was suspected. Furthermore, there is a distinct location pattern of IA and an inverse sex ratio in comparison to the healthy population. Large population-based patient registers are required for a better understanding of epidemiology and pathophysiology of IA formation in TSC.