Michelle Ganat1, Gautham Upadrasta1, Kush Sharma1, Oleg Otlivanchik1
1Montefiore Medical Center
Background:
Neonatal Marfan Syndrome is more rare with a more severe course and poorer prognosis than classical Marfan Syndrome. Infants are born with clinical features including arachnodactyly, elbow and knee joint contracture, camptodactyly, ectopia lentis among others, and the diagnosis is further supported by a positive fibrillin-1 gene mutation. Neurological complications include acute ischemic stroke. We present a unique case of acute ischemic stroke in a patient with Neonatal Marfan Syndrome, treated with mechanical thrombectomy.
Results:
A 6-year-old boy with history of neonatal Marfan Syndrome, mitral regurgitation with valve repair and aortic root repair, presented to the ED after a fall and progressive lethargy at home. The patient was admitted for syncope work up with cardiac work up revealing globally decreased cardiac function and multiple intracardiac thrombi. He was admitted to the PICU for cardiogenic shock and started on a heparin drip. The patient developed a right MCA syndrome, leading to diagnosis of R MCA M1 branch occlusion that was swiftly treated with a mechanical thrombectomy. MRI brain revealed bilateral MCA infarcts and numerous chronic microhemorrhages, which were deemed to be sequelae of Marfan’s syndrome. He was extubated, started on heparin drip, and cardiac care was continued. Genetics team arranged for follow-up appointment.
Conclusions:
This case demonstrates the use of mechanical thrombectomy as a successful treatment option for ischemic stroke in a patient with Neonatal Marfan Syndrome. Despite the complex nature of the case, including the risks of undergoing anesthesia in a patient with multiple cardiac comorbidities and chronic cortical and subcortical microhemorrhages, the patient was able to undergo a successful mechanical thrombectomy to improve long-term neurological outcomes. This case highlights that mechanical thrombectomy can potentially be a viable option to treat similar patients who present with neurological complications such as cardioembolic stroke from cardiac comorbidities associated with Marfan Syndrome.
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