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

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.

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Publication Details

Journal
Clinical Case Reports
Published
2026-02-23
DOI
https://doi.org/10.1002/ccr3.72176
Primary Topic
Moyamoya disease diagnosis and treatment
Type
article
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article

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

Raja Babu Gupta, Anup Katwal, Shristi Gupta, Kshitiz Parajuli et al.
Clinical Case Reports
Moyamoya disease diagnosis and treatment
article

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

Raja Babu Gupta, Anup Katwal, Shristi Gupta, Kshitiz Parajuli, Aron Neupane, Nischal Timsina, Sagar Bhagat, Anand Sah, Abhisek Jha
article en

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.

Clinical Case ReportsVol. 14(3)
Nepal Medical College Teaching Hospital (NP), Bir Hospital (NP)
Openalex Percentile: Top 44%
Moyamoya disease diagnosis and treatment
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