Related Experiment Video
Updated: Apr 23, 2026
Author Spotlight: Assessing Ischemic Stroke Damage Through Middle Cerebral Artery Occlusion Model
Published on: August 11, 2023
Stroke after adenotonsillectomy in patients with undiagnosed moyamoya syndrome
Annie K Ahn1, Adam Honeybrook2, Lori C Jordan3
1Department of Otolaryngology, Vanderbilt University Medical Center, Nashville, Tennessee.
Importance:
Moyamoya syndrome is a rare, occlusive cerebrovascular arteriopathy with significant risk for stroke. Populations that frequently undergo otolaryngologic procedures, including patients with Down syndrome and sickle cell disease, are particularly at risk for moyamoya. The initial presentation of moyamoya syndrome as stroke in the perioperative period of an otolaryngologic procedure has not been reported.
Observations:
A retrospective medical record review assessed the relationship of otolaryngologic operations and the onset of moyamoya symptoms. Moyamoya syndrome was present in 137 patients. Of these, 19 patients underwent otolaryngologic procedures; 3 children had strokes 2 to 4 days after adenotonsillectomy, including 2 children with Down syndrome. Intraoperative carotid artery injury was considered but was proven not to be the cause of stroke. Bilateral moyamoya disease was diagnosed in all 3 patients via vascular imaging studies; all subsequently underwent revascularization procedures.
Conclusions And Relevance:
Clinicians should be aware of an elevated prevalence of moyamoya syndrome in Down syndrome and sickle cell disease populations and should consider moyamoya syndrome in the differential diagnosis of postoperative stroke. Stroke risk is magnified in the perioperative setting related to perioperative dehydration and hypotension. Awareness and screening for cerebral vasculopathy in high-risk populations could prompt measures to decrease the occurrence of postoperative strokes after adenotonsillectomies.
Insights
Moyamoya syndrome, a rare cerebrovascular disease, can present as stroke after otolaryngologic surgery, especially in children with Down syndrome. Early awareness and screening are crucial for high-risk populations to prevent perioperative strokes.
Area of Science:
- Neurology
- Vascular Surgery
- Genetics
Background:
- Moyamoya syndrome is a rare occlusive cerebrovascular arteriopathy associated with a high risk of stroke.
- Patients with Down syndrome and sickle cell disease have an elevated prevalence and are at increased risk.
- The presentation of moyamoya syndrome as stroke in the perioperative period of otolaryngologic procedures is not well-documented.
Observation:
- A retrospective review examined the link between otolaryngologic operations and moyamoya symptoms.
- Three pediatric patients experienced strokes 2-4 days post-adenotonsillectomy, two with Down syndrome.
- Vascular imaging confirmed bilateral moyamoya disease in all three, necessitating revascularization; intraoperative carotid injury was ruled out.
Findings:
- Moyamoya syndrome can manifest as stroke in the perioperative period following otolaryngologic procedures.
- Postoperative strokes occurred in children with moyamoya syndrome, particularly those with Down syndrome, after adenotonsillectomy.
- The identified cases highlight a previously unreported presentation of moyamoya syndrome.
Implications:
- Clinicians must consider moyamoya syndrome in the differential diagnosis of postoperative stroke, especially in high-risk groups.
- Perioperative dehydration and hypotension can exacerbate stroke risk in patients with moyamoya syndrome.
- Screening for cerebral vasculopathy in at-risk populations may reduce postoperative stroke incidence after procedures like adenotonsillectomy.
Related Concept Videos
01:21Aneurysm II: Clinical Manifestations and Diagnostic Studies
01:26Tonsillitis II: Management