J Neurosurg Pediatr. 2026 Mar 13:1-8. doi: 10.3171/2025.11.PEDS25460. Online ahead of print.
ABSTRACT
OBJECTIVE: Moyamoya arteriopathy and middle aortic syndrome (MAS) are each rare, often progressive vascular diseases; their comorbid intersection increases their complexity because of the independent and contradictory demands on blood pressure. The management of pediatric patients with these comorbid conditions requires multidisciplinary consultation given the high risk involved. In the current study, the authors aim to describe the clinical and surgical history of pediatric patients with comorbid moyamoya arteriopathy and MAS and describe a multidisciplinary approach to care for these patients.
METHODS: This study is a retrospective review of the clinical and radiological records of patients at Boston Children’s Hospital who were treated with cerebral revascularization surgery for comorbid moyamoya arteriopathy and MAS from January 2004 to January 2024. Analysis of collected perioperative, surgical, inpatient, and follow-up data was conducted using R version 4.4.2 (R Foundation for Statistical Computing).
RESULTS: Eleven patients, 5 male (45.5%), were included in the study. At presentation, 9 patients (81.8%) had symptoms from brain ischemia. Nine patients were on antihypertensive medications (median 2, IQR 1-2). All patients underwent surgery for moyamoya arteriopathy, and 6 patients (54.5%) also underwent surgical treatment for MAS. After moyamoya surgery, 4 patients (36.4%) required an intensified blood pressure regimen to achieve the same goal, whereas 3 patients (27.3%) had a reduced need for blood pressure medications. Four (66.7%) of the 6 patients who underwent MAS surgical treatment did so after moyamoya treatment. Perioperative stroke occurred after renal stent angioplasty before moyamoya surgery but also after aortic bypass following moyamoya surgery.
CONCLUSIONS: Managing comorbid moyamoya arteriopathy and MAS is challenging, as patients have a higher risk of ischemic perioperative complications. These patients may have hypertension secondary to their aortic disease, and treating the aortic disease may correct the hypertension but with possible new relative hypotension and relative cerebral hypoperfusion. Consequently, moyamoya revascularization prior to aortic repair may be a strategy to reduce the risk of stroke.
PMID:41825070 | DOI:10.3171/2025.11.PEDS25460
