|Ahead of print
Left atrial myxoma presenting with recurrent, bilateral thalamic infarction in a child
Sasikumar Sheetal1, Reji Thomas1, Sajan Z Ahmad2
1 Department of Neurology, Pushpagiri Institute of Medical Sciences and Research, Thiruvalla, Kerala, India
2 Department of Cardiology, Pushpagiri Institute of Medical Sciences and Research, Thiruvalla, Kerala, India
|Date of Submission||03-Dec-2020|
|Date of Decision||27-Mar-2021|
|Date of Acceptance||28-Mar-2021|
|Date of Web Publication||07-Jan-2022|
“16 F&G,” The Edge, Skyline Apartment, Thirumoolapuram, 689115, Kerala.
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Stroke in children is a rare entity, with long-term consequences. Cardiac causes constitute a significant majority in pediatric stroke; however, atrial myxomas as a cause of pediatric stroke are rare. Neurological manifestations may be the presenting feature of atrial myxomas, even before the appearance of constitutional symptoms. The most common regions reported to be affected are basal ganglia, temporal, and parietal regions. Thalamus is less commonly affected. We hereby report the case of a 13-year-old girl, who presented with acute onset of left hemiparesis, whose magnetic resonance imaging of the brain revealed acute infarction of the right thalamus with multiple old infarcts in bilateral thalami, and transthoracic echocardiography showed a left atrial myxoma.
Keywords: Bilateral, left atrial myxoma, pediatric stroke, recurrent, thalamic infarcts
| Introduction|| |
Stroke in children is relatively rare, with high morbidity and long-term consequences. The most common cause of pediatric stroke is reported to be cardiac, the prevalence ranging from 10% to 31%. The common cardiac causes accounting for ischemic stroke in children are cyanotic and complex congenital heart disease, rheumatic heart disease, prosthetic valves, infective endocarditis, cardiomyopathies, and post cardiac repair or catheterization., However, ischemic stroke due to atrial myxoma in children younger than 18 years of age is uncommon. In studies on strokes resulting from atrial myxomas, the common sites of infarction reported are basal ganglia, cerebellum, and parietal and temporal regions. Thalamic infarcts are uncommon and have been reported to be seen in around 9%. We hereby report the case of a 13-year-old girl, who presented with acute onset left hemiparesis and was diagnosed to have left atrial myxoma on echocardiography. Her magnetic resonance imaging (MRI) of the brain revealed acute infarction of the right thalamus with multiple old infarcts of varying ages, in bilateral thalami.
| Case History|| |
A 13-year-old girl was attending her mid-term examination at school, when she felt a sudden weakness of her left upper and lower limb. She called out to her teacher, who noticed a deviation of her angle of mouth to the right side. She was supported to get up from her seat and required assistance to walk. She denied any history of headache, fever, or vomiting. She did not complain of any visual blurring, double vision, or dysarthria. Her medical history was unremarkable and she did not have any similar episodes in the past.
On examination, she had a pulse rate of 86/minute, regular in rhythm, and blood pressure of 120/80 mmHg. Cranial nerve examination was remarkable for a left upper motor neuron type of facial palsy. Motor system examination revealed grade 3/5 power in the left upper and lower limbs, with brisk deep tendon reflexes in the left upper and lower limbs and left extensor plantar response. The sensory system examination revealed impaired touch and pin prick sensation over the left side of face and left upper and lower limbs. There were no cerebellar signs. Examination of other systems was unremarkable. MRI brain with MR angiography (MRA) was done which showed hyperintensity involving the right thalamus on diffusion-weighted images [Figure 1]A and [Figure B] with corresponding areas showing low ADC values, suggestive of acute infarct [[Figure 1]C. Hypointensity was also noted in bilateral thalamus on diffusion images [Figure 1]A, orange arrows]. On T2-weighted images, hyperintense lesions were noted in bilateral thalamus, with one lesion in the right thalamus showing FLAIR hypointensity, indicating a cystic change [Figure 2]. MRA showed normal caliber of the cerebral vessels. Hence, she was noted to have recurrent, bilateral thalamic infarctions of varying ages. Her hemogram and blood biochemistry were normal. Vasculitic work-up and hypercoagulability screen were negative. A transthoracic echocardiography showed a large, mobile mass, with irregular surface in the left atrium, partially obstructing the mitral valve orifice, suggestive of a left atrial myxoma [Figure 2]. She underwent emergency surgical resection of the mass, during the second week of hospitalization. At the end of 1 month, she regained grade 5/5 power in her left upper and lower limbs.
|Figure 1: A and B. DWI showing hyperintensity involving the right thalamus (green arrows) and hypointensity involving the bilateral thalamus (orange arrows). C. ADC showing hypointensity, corresponding to the hyperintensity on DWI (green arrow) and another hyperintense lesion in the right thalamus. D. T2 images showing multiple hyperintense lesions involving bilateral thalamus. E. FLAIR sequence showing hyperintensities in the bilateral thalamus, with another hypointense lesion in the right thalamus. F. Normal MRA|
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|Figure 2: Transthoracic echocardiography showing a large, mobile mass, with irregular surface in the left atrium, partially obstructing the mitral valve orifice, suggestive of a left atrial myxoma|
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| Discussion|| |
The incidence of stroke in children ranges from 1.2 to 13 cases per 100,000 children under 18 years of age, as per previous reports. The morbidity caused by stroke in children is paramount, with significant social implications. In contrast to adults, where around 80% of strokes are ischemic in origin, in children only about a 50% are ischemic. The clinical presentation can vary. The most common presenting symptom is hemiplegia; others include cranial nerve palsies, language abnormalities, seizures, and headache.
Cardiac disease accounts for nearly one-third of the cases of pediatric stroke, and these include cyanotic and complex congenital heart disease, rheumatic heart disease, prosthetic valves, infective endocarditis, cardiomyopathies, and atrial myxomas. Around 30% of the children with cardiac disease and stroke have their stroke in the peri-procedural period related to cardiac surgery or other cardiac interventions. Causes other than cardiac include hypercoagulable states, vasculitis, arterio-venous malformations, sickle cell disease, infections, and malignancies. Atrial myxomas can cause embolism to the brain, and a neurological deficit may often be the first presenting symptom of an atrial myxoma in 20–45%. Other presentations include constitutional symptoms such as fatigue, malaise, fever, anorexia, and weight loss. Our patient had no constitutional symptoms, and her presenting symptom was acute onset of left hemiparesis. In the study by Yuan and Humuruola on 82 patients with atrial myxomas, the most common site of cerebral embolism was the middle cerebral artery and the most common infarct regions were the basal ganglia, cerebellum, and parietal and temporal regions. Thalamic infarcts were uncommon and have been reported to be seen in around 9% in their study. In our patient, acute infarct was noted in the right thalamus; however, multiple old infarcts were noted in bilateral thalami. Our case is unique in that multiple infarcts were noted, in varying ages, involving the thalamus, a less frequently reported site for infarction. It was remarkable that all the infarcts were confined to the thalami and other territories were spared. Recurrent cerebral infarctions have been reported with atrial myxomas., Hence, emergent surgical resection of the myxoma is advocated to prevent further cerebrovascular events. Our patient underwent surgical resection of the myxoma in the second week of hospitalization.
| Conclusion|| |
Though rare, atrial myxoma should be considered in the differential diagnosis of children presenting with stroke, especially if there is a history of recurrent cerebral infarcts. It should be kept in mind that cerebral infarcts can occur before the onset of constitutional symptoms in myxomas. Urgent surgical removal of myxomas should be considered to prevent recurrent cerebral infarctions.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]