通过右小胸廓切开术全切除幼年卡尼复合体左心房肌瘤:病例报告。

Kazumasa Matsunaga, Shigeru Ikenaga
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引用次数: 0

摘要

背景:卡尼综合征是一种以皮肤色素沉着、内分泌紊乱和肌瘤为特征的罕见综合征。它尤其以表现出侵袭性心脏肌瘤的倾向而闻名。在此,我们介绍一例被诊断为卡尼综合征的青少年女性患者,她接受了右侧小胸廓切开术:病例介绍:一名 13 岁女孩突然出现左侧偏瘫和构音障碍。磁共振成像显示多处弥散受限。超声心动图检查发现左心房有肿瘤,根据病史和体格检查结果,怀疑与卡尼综合征有关。手术通过右侧小胸廓切开术进行,最大程度地降低了栓塞风险,并确保了良好的外观效果。左心房壁缺损用自体心包修复。术后 3 年,随访超声心动图显示肿瘤未复发,心功能正常:结论:由于卡尼复合体的侵袭性及其高复发率,持续的随访至关重要。右外侧小胸廓切开术具有避免再次切除胸骨和最大程度减少粘连剥离的优势,因此是该病例的最佳选择。
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Total resection via right mini-thoracotomy for left atrial myxoma in juvenile Carney complex: a case report.

Background: Carney complex is a rare syndrome characterized by skin pigmentation, endocrine disorders, and myxomas. It is particularly notorious for its tendency to exhibit aggressive cardiac myxomas. Herein, we present a case of a juvenile female patient diagnosed with Carney complex who underwent a right lateral mini-thoracotomy.

Case presentation: A 13-year-old girl presented with sudden-onset left hemiplegia and dysarthria. Magnetic resonance imaging revealed multiple areas of restricted diffusion. Echocardiography identified a tumor in the left atrium, suspected to be related to Carney complex based on her medical history and physical examination findings. Surgery was performed via right lateral mini-thoracotomy, which minimized the risk of embolism and ensured a cosmetically favorable outcome. The left atrial wall defect was repaired with autologous pericardium. At 3 years postoperatively, follow-up echocardiography indicated no tumor recurrence and normal cardiac function.

Conclusions: Ongoing follow-ups are essential due to the aggressive nature of the Carney complex and its high recurrence rates. Right lateral mini-thoracotomy offers the advantage of avoiding re-sternotomy and minimizing adhesion dissection, making it the optimal choice for this case.

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