1 - Neuroradiology Department, Hospital de Braga, Braga, Portugal.
2 - Neuroradiology Department, Centro Hospitalar Universitário do Porto, Porto, Portugal.
3 - Neurosurgery Department, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa, Portugal. 4Neuroradiology Department, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa, Portugal.
- 45th ESNR Annual Meeting, 14-18 Setembro, Lisboa
- Neuroradiology (2022) 64 (Suppl 1):S1-S165 https://doi.org/10.1007/s00234-022-03012-w
Introduction: Vascular anomalies are localized defects of the vasculature secondary to errors in vascular morphogenesis that can lead to progressive steno-occlusive disease, leading to impaired cerebral blood flow (CBF). Several surgical revascularization techniques have been adopted to restore adequate CBF, being indirect techniques suitable in younger children whose small-caliber vessels make direct anastomosis difficult. The multiple cranial burr hole (MCBH) surgery has been adopted as a sole procedure with excellent clinical results: drilling multiple holes through the skull stimulates anastomotic angiogenesis from branches of the external carotid artery (ECA).
Methods: We present two pediatric cases with cerebral steno-occlusive disease treated with MCBH technique with a special focus on their clinical and associated imaging evolution.
Results: A 7-month-old male presented with myoclonus and jerking movements of the left hemibody and left-sided hemiparesis. Brain MRI showed ischemic lesions involving the right middle and posterior cerebral arteries’ territories. MRA showed marked stenosis of the distal internal carotids arteries (ICAs) and of the top of the basilar trunk, with concomitant marked hypertrophy of the perforating branches, suggesting a moya-moya pattern. Follow-up MRI, seven months later, revealed new recent ischemic lesions on both hemispheres. After DSA characterization, surgical revascularization with MCBH was performed. During a 6-years follow-up there was no new ischemic events, persisting a mild residual left hemiparesis and poor motor coordination. Later control neuroimaging showed hypertrophy of the ECA branches bilaterally, in relation to the neo-vascular recruitment through the multiple burr holes. MRI-perfusion showed no abnormal hypoperfused parenchymal areas. A 9-year-old male was admitted for right-sided hemiparesis. Brain MRI revealed an acute ischemic lesion in the territory of the left medial cerebral artery (MCA) and other bilateral lesions from different timepoints. MRA showed associated cervical and intracranial vascular malformations, later-on better characterized by DSA: bilateral occlusion of the ICAs and vertebral arteries, with CBF mainly supplied through the anterior spinal artery. Four days later he was submitted to MCBH, with good post-surgical clinical evolution. During a 2-years follow-up there was no new ischemic events, and the patient was clinically stable only with a mild right hemiparesis. Later MRIs confirmed multiple vascular anastomoses between branches of the ECA and leptomeningeal vessels through the burr holes, leading to an evident improvement in the intracranial perfusion profile.
Conclusion: Indirect revascularization techniques represent a valuable therapeutic tool to manage vascular steno-oclusive syndromes and neuroimaging is essential for diagnosis, detailed characterization of the cerebrovascular anatomy and post-surgical follow-up.
Keywords: Cerebral angiopathy, moya-moya disease, pediatric acute stroke, indirect revascularization, multiple cranial burr hole.