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Cerebellar arteriovenous malformations in children
P D Griffiths1, S Blaser, D Armstrong
1University of Sheffield, Royal Hallamshire Hospital, UK.
Insights
Cerebellar arteriovenous malformations (AVM) in children often present as ruptured lesions causing hemorrhage, leading to poor outcomes. Associated vascular pathologies like aneurysms and venous obstruction are common, impacting survival rates.
Area of Science:
- Pediatric Neurology
- Vascular Neurosurgery
- Neuroradiology
Background:
- Cerebellar arteriovenous malformations (AVM) in children are associated with poor outcomes, even with advanced medical interventions.
- Hemorrhage from ruptured cerebellar AVMs presents a significant clinical challenge in pediatric populations.
Purpose of the Study:
- To review the clinical presentation, imaging characteristics, and outcomes of pediatric patients diagnosed with cerebellar AVMs.
- To identify factors contributing to the poor prognosis in children with cerebellar AVMs.
Main Methods:
- Retrospective review of 18 pediatric patients with cerebellar AVMs.
- Analysis of clinical data, including presentation, neuroimaging (CT, angiography, MRI), and patient outcomes.
- Correlation of AVM characteristics and associated vascular pathologies with clinical severity and prognosis.
Main Results:
- 17 out of 18 children presented with ruptured cerebellar AVMs, leading to intracranial hemorrhage.
- High mortality rate observed, with 6 of 17 children dying within 7 days of symptom onset.
- Associated vascular pathologies, including intranidal and venous aneurysms and venous outflow obstruction, were found in 10 of 14 patients undergoing angiography.
- Acute hematoma size was a reliable predictor of clinical presentation severity and patient outcome.
Conclusions:
- Cerebellar AVMs in children frequently present as ruptured lesions with significant morbidity and mortality.
- The presence of associated vascular pathologies may contribute to the severity and poor outcomes observed in these pediatric cases.
- Prompt diagnosis and management, considering associated vascular findings and hematoma size, are crucial for improving outcomes in pediatric cerebellar AVMs.
Abstract:
We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM). This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques. All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the study had MRI. Of the 18 children, 17 presented with a ruptured AVM producing intracranial haemorrhage. The remaining child presented with temporal lobe epilepsy and was shown to have temporal, vermian and cerebellar hemisphere AVM. This child had other stigmata of Osler-Weber-Rendu syndrome. Three other children had pre-existing abnormalities of possible relevance. One had a vascular malformation of the cheek and mandible, one a documented chromosomal abnormality and another a midline cleft upper lip and palate. Six of the 17 children with a ruptured cerebellar AVM died within 7 days of the ictus. Vascular pathology other than an AVM was found in 10 of the 14 children with a ruptured cerebellar AVM who had angiography: 4 intranidal aneurysms, 5 venous aneurysms and 2 cases of venous outflow obstruction (one child having both an aneurysm and obstruction). The severity of clinical presentation was directly related to the size of the acute haematoma, which was a reasonable predictor of outcome.