Tona Clarissa, Nosadini Margherita, Pelizza Maria F, Pin Jacopo N, Baggio Laura, Boniver Clementina, Gabrieli Joseph D, Causin Francesco, Toldo Irene, Sartori Stefano
Paediatric Neurology and Neurophysiology Unit, Department of Women's and Children's Health, University Hospital of Padua, Padova, Italy.
Neuroradiology Unit, University Hospital of Padua, Padova, Italy.
Neuropediatrics. 2020 Dec;51(6):389-396. doi: 10.1055/s-0040-1710338. Epub 2020 May 5.
Cardiac disorders are the second leading cause of pediatric arterial ischemic stroke (AIS). Limited literature is available on pediatric AIS caused by cardiac myxoma, a rare tumor in childhood.
We describe a new case of pediatric AIS due to a previously unknown atrial myxoma and we conduct a literature review on children with AIS due to cardiac myxoma.
We identified 41 published pediatric cases of AIS and cardiac myxoma, including ours (56% males, median age at AIS was 11 years [range: 3-18]). AIS presentation was most frequently with hemiparesis/hemiplegia (89%). Multiple brain ischemic lesions were detected in 69% of patients, and arteriopathy in 91%. Seven patients underwent mechanical thrombectomy. At AIS presentation, 73% of children had one or more of the following clinical symptoms/signs suggesting a possible underlying cardiac myxoma: Carney's complex, cardiac auscultation abnormalities, extraneurological symptoms/signs, such as skin signs (12, 38, and 65%, respectively). Cardiac myxoma was diagnosed within 72 hours in 68% of cases. Death occurred in 11%, and 40% had persistent neurological deficits.
Neurological presentation of AIS due to cardiac myxoma is similar to that of AIS with other etiologies, although clues suggesting a possible underlying cardiac myxoma can be detected in most cases. A timely diagnosis of cardiac myxoma in patients with AIS may favor prompt identification of candidates for endovascular therapy. Therefore, we suggest that in otherwise-healthy children presenting with AIS, transthoracic echocardiography should be performed early after stroke presentation.
心脏疾病是儿童动脉缺血性卒中（AIS）的第二大主要病因。关于儿童心脏黏液瘤（一种儿童罕见肿瘤）所致AIS的文献有限。
我们描述了一例因先前未知的心房黏液瘤导致儿童AIS的新病例，并对心脏黏液瘤所致儿童AIS的文献进行了综述。
我们确定了41例已发表的儿童AIS合并心脏黏液瘤的病例，包括我们的病例（男性占56%，AIS发病的中位年龄为11岁[范围：3 - 18岁]）。AIS最常见的表现是偏瘫/半身不遂（89%）。69%的患者检测到多个脑缺血性病变，91%的患者检测到动脉病变。7例患者接受了机械取栓术。在AIS发病时，73%的儿童有以下一种或多种临床症状/体征，提示可能存在潜在的心脏黏液瘤：卡尼综合征、心脏听诊异常、神经外症状/体征，如皮肤体征（分别为12%、38%和65%）。68%的病例在72小时内确诊心脏黏液瘤。11%的患者死亡，40%的患者有持续性神经功能缺损。
心脏黏液瘤所致AIS的神经学表现与其他病因所致AIS相似，尽管在大多数情况下可以检测到提示可能存在潜在心脏黏液瘤的线索。对AIS患者及时诊断心脏黏液瘤可能有助于迅速确定血管内治疗的候选者。因此，我们建议，对于出现AIS的其他方面健康的儿童，应在卒中发作后尽早进行经胸超声心动图检查。
