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

Area of Science:

• Neurology
• Pediatrics
• Vascular Medicine

Background:

• Moyamoya syndrome (MMS) is a rare cerebrovascular disorder characterized by intracranial artery stenosis and collateral formation.
• Pediatric MMS often presents with ischemic strokes, potentially exacerbated by coexisting conditions like Thalassemia Major.
• Thalassemia Major can negatively impact vascular health, increasing risks in pediatric patients with cerebrovascular conditions.

Purpose of the Study:

• To report a rare pediatric case of Moyamoya syndrome presenting with alternating hemiparesis.
• To emphasize the diagnostic role of advanced neuroimaging in pediatric Moyamoya syndrome.
• To highlight the challenges in accessing definitive surgical care due to socio-economic factors.

Main Methods:

• A case report of a 3-year-old male with Thalassemia Major presenting with stroke-like symptoms.
• Diagnostic workup included neurological examination, laboratory tests, MRI Brain with contrast, and TOF-MRA.
• Treatment involved aspirin and supportive care, with surgical revascularization (EDAS) recommended but deferred.

Main Results:

• The patient exhibited acute neurological deficits, including hemiparesis and altered consciousness, consistent with ischemic stroke.
• Neuroimaging confirmed Moyamoya disease (Suzuki stage 3) with significant internal carotid artery stenosis.
• Initial treatment with aspirin led to partial neurological improvement within 5 days.

Conclusions:

• Alternating hemiparesis can be a rare presenting symptom of pediatric Moyamoya syndrome.
• MRI/MRA are crucial for diagnosing Moyamoya disease in children.
• Socio-economic factors significantly influence the accessibility of timely and definitive surgical interventions for Moyamoya syndrome.