How Far Can we Go? Managing Moyamoya Syndrome in a 3‐Year‐Old With Thalassemia Major: A Rare Pediatric Case From Nepal

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Authors: Kshitiz Parajuli, Abhisek Jha, Aron Neupane, Shristi Gupta, Nischal Timsina, Sagar Bhagat, Raja Babu Gupta, Anand Sah, Anup Katwal
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Abstract

Moyamoya syndrome (MMS) is a rare progressive cerebrovascular disorder causing stenosis of intracranial arteries and collateral vessel formation. Pediatric presentations often include ischemic strokes, and coexisting Thalassemia Major may worsen vascular outcomes. A 3-year-old male with Thalassemia Major presented with acute right-sided weakness, altered consciousness, and a prior left-sided stroke. Examination revealed facial asymmetry, aphasia, and right-sided hemiparesis. Laboratory tests excluded metabolic, infectious, and cardio-embolic causes. MRI Brain with contrast and TOF-MRA demonstrated chronic right-sided changes, acute left-sided ischemia, and marked narrowing of the supraclinoid and cavernous segments of the left internal carotid artery, confirming Moyamoya disease, Suzuki stage 3. The patient received aspirin (3 mg/kg/day) and supportive care. After 5 days, neurological status improved (GCS 14, right limb power 3/5). Surgical revascularization via EDAS was advised but deferred due to financial constraints. This case highlights alternating hemiparesis as a rare pediatric MMS presentation, the diagnostic utility of MRI/MRA, and the impact of socio-economic limitations on access to definitive surgical care.

Fields of Study & Subjects

Moyamoya disease diagnosis and treatment Advanced MRI Techniques and Applications Vascular Malformations Diagnosis and Treatment

Publication details

Journal / source
Clinical Case Reports
Volume
14
Issue
3
Pages
e72176–e72176
Publication date
23 February 2026
Language
EN