Lemos Samuel Sequeira, Sá Maria Inês, Casimiro Carlos Daniel, Guerreiro Carla, Faria Claudia
Department of Neurosurgery, Hospital Santa Maria, Lisboa Portugal.
Department of Neurological Imaging, Hospital Santa Maria, Lisboa Portugal.
Surg Neurol Int. 2024 Jun 7;15:195. doi: 10.25259/SNI_111_2024. eCollection 2024.
Neurenteric cysts are uncommon, benign endoderm-derived lesions that result from aberrant embryologic development of the notochord. They are typically located in the intradural extramedullary spinal cord and rarely located intracranially. Contrary to spinal-located cysts, intracranial cysts are rarer in the pediatric population. Clinically, they may present with symptoms of mass effect, or they can be incidentally discovered.
A 10-year-old healthy female child presented with recurrent headaches. The physical and neurological examination was unremarkable. Brain magnetic resonance imaging (MRI) showed a well-demarcated lesion anterior to the pontomedullary junction with striking T1 and T2/T2 fluid-attenuated inversion recovery high-signal intensity and a small rounded nodule within of low signal on T1, T2, and T2*. On initial conservative strategy with serial brain MRI, there was a progressive enlargement of the lesion with significant mass effect on the brainstem. The patient underwent a right retrosigmoid craniotomy, and the cyst wall was fenestrated and drained. Part of the cyst wall and the solid nodule were adherent to the brainstem and basilar artery and were not removed. The histologic findings were consistent with the diagnosis of a benign endodermal cyst. The postoperative period was uneventful.
We report a successful surgical treatment of this rare congenital cyst located in the ventral brainstem. We present pre-and post-operative imaging findings, intraoperative microscopic images of the procedure, and a brief review of relevant clinical literature on the topic.
神经肠囊肿是罕见的良性内胚层源性病变，由脊索异常胚胎发育所致。它们通常位于硬脊膜内脊髓外，很少位于颅内。与脊髓囊肿不同，颅内囊肿在儿童人群中更为罕见。临床上，它们可能表现为占位效应症状，也可能偶然被发现。
一名10岁健康女童反复头痛。体格检查和神经系统检查均无异常。脑部磁共振成像（MRI）显示脑桥延髓交界处前方有一个边界清晰的病变，在T1加权像和T2加权像/液体衰减反转恢复序列上呈显著高信号，内部有一个小圆形结节在T1、T2和T2*加权像上呈低信号。最初采用连续脑部MRI的保守策略，病变逐渐增大，对脑干产生明显占位效应。患者接受了右乙状窦后开颅手术，囊肿壁被开窗引流。部分囊肿壁和实性结节与脑干和基底动脉粘连，未予切除。组织学检查结果与良性内胚层囊肿的诊断一致。术后恢复顺利。
我们报告了对位于腹侧脑干的这种罕见先天性囊肿的成功手术治疗。我们展示了术前和术后的影像学表现、手术过程中的术中显微镜图像，并对该主题的相关临床文献进行了简要回顾。
