Prevalence And Pathophysiology
- Rare - 0.5 – 4.6 % of all Aneurysms
- Genetic Structural Defects increase predisposition (Lasjaunias)
- Collagen Vascular Disorders (AD PKD, Coarctation of Aorta, COL4A Mutation, Fibro-Muscular Dysplasia)
- PHACES Syndrome (Posterior Fossa Malformation, Haemangioma, Arterial Anomalies, Cardiac abnormality, Coarctation of Aorta, Eye Abnormalities, Sternal Abnormalities)
- Traumatic Aneurysms (more common in children, 20% of all aneurysms - Cavernous Carotid, Peri-Callosal)
- Infectious/Mycotic Aneurysms (more common in children) - Distal location, Multifocal (e.g. Bacterial endocarditis, Fungal infection, HIV/AIDS)
- Haemodynamic - Congenital Vascular Absence, Hypertension with or without Coarctation of Aorta, AVMs
Morphology
- Fusiform & Dissecting > Saccular
- Higher rate of Multiplicity (especially if secondary to Infection/Vasculopathy)
- Posterior Fossa location (twice frequency of Adults)
- Giant Aneurysms (commoner in Children)
- Terminal ICA (commonest in Children; ~25%)
- Fusiform highly associated with underlying abnormality (NF1, MOPD Type II, Ehlers-Danlos, Osteogenesis Imperfecta)
Presentation and Recurrence
- Mass effect > Haemorrhage
- Haemorrhage (Twice as common in Saccular vs Fusiform)
- Vasospasm is LESS likely in Children (53% rate on Angiography without VS 70% Radiological in Adult SAH)
- Recurrence Rates Higher (2.6% vs 0.5%)
- Growth Rates Higher (7.8% vs 1.5%)
- Possibly increased association with vasculopathies
- Possibly increased haemodynamic stress on already dysplastic vessels
Rupture & Haemorrhage Risk
- Best available literature on guidance is in Adults
- ISUIA, PHASES
- Counselling difficult in specialist Paediatric pathologies (e.g. Infectious, Vasculopathy related, Traumatic)
Management
- Rare condition in paediatric practice
- Multidisciplinary Decision Making Crucial (Endovascular VS Microsurgical)
- Joint Operating / Decision Making with Adult Neurovascular Surgical colleagues is crucial
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