Aneurysmal subarachnoid hemorrhage and Moya Moya disease

Case contributed by Dr Rajalakshmi Ramesh


29 year-old, previously well female, presenting following an unwitnessed and unconscious collapse. GCS 4 (E1V1M2) with a fixed and dilated left pupil.

Patient Data

Age: 37
Gender: Female

Large intraventricular hemorrhage evident with blood evident in all ventricles, greatest in the right lateral ventricle and third ventricles. There is associated marked hydrocephalus and signs of raised intracranial pressure with effacement of the cortical sulci and basal cisterns. There are probable small amounts of subarachnoid blood evident.

Abnormality is seen in relation to the A1 segments of the anterior cerebral arteries, M1 segments of the middle cerebral arteries and distal internal carotid arteries. These vessels are of narrow caliber and there is irregular luminal enhancement, particularly involving the A1 segments bilaterally. There are mildly prominent collateral vessels. A definite focal aneurysm is not identified. The differential diagnosis for this appearance includes vasospasm, secondary to the large amount of hemorrhage. Other causes would include an obstructive vasculopathy (moya-moya) and vasculitis. Whilst not identified on this study, an aneurysm is not excluded.

Diffuse and severe narrowings are demonstrate at bilateral anterior cerebral and middle cerebral arteries and their branches. Multiple anastomoses are demonstrated between the posterior and anterior and middle cerebral arteries via leptomeningeal arteries. Similarly, anastomoses are demonstrated between the left external and left internal carotid vessels. A small, 1.2 x 1.3 cm aneurysm is demonstrated at the distal right anterior choroidal artery. This correlates with the distribution of blood demonstrated on the CT Brain. Multiple small vessels are demonstrated at bilateral lenticulo-striate territories. No definite evidence of further aneurysms. Bilateral foetal posterior communication arteries identified. Moderate fibromuscular dysplasia noted at the left internal carotid artery.

Conclusion: Changes are consistent with moya-moya disease and right distal anterior choroidal artery aneurysm.

The patient was therefore diagnosed with a WFNS grade V, Fischer grade 4 subarachnoid hemorrhage, secondary to a ruptured right anterior choroidal artery aneurysm and multiple cerebral blood vessel abnormalities consistent with Moya Moya disease. After discussion with neurosurgery, it was decided that given the inaccessibility of the aneurysm by surgery, it was not unreasonable to perform right anterior choroidal artery occlusion by coiling. Multiple attempts were made at coiling of the right anterior choroidal artery distal aneurysm (supplied by the inferior branch of the anterior choroidal artery). However, due to technical difficulties, the procedure was subsequently aborted. The patient failed to make any significant functional nor neurological recovery, and was palliated in due course. 

Case Discussion

This case illustrates a catastrophic subarachnoid bleed secondary to a ruptured right anterior choroidal artery aneurysm, that was not amenable to surgical nor interventional treatment. Narrow caliber vessel and luminal enhancement abnormalities were identified (on CT angiography) in the A1 segments of the anterior cerebral arteries, M1 segments of the middle cerebral arteries and distal internal carotid arteries. These vessel abnormalities were later confirmed with cerebral angiography to be consistent with Moya Moya disease. 



Case courtesy of Associated Professor Pramit Phal

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Case information

rID: 34306
Published: 15th Feb 2015
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included