内镜下经鼻经斜坡入路治疗儿童脑桥海绵状血管畸形

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中文摘要

背景与目的： 脑干海绵状血管畸形（CMs）因其位于重要功能区而具有高出血风险和发病率。尽管现代显微外科入路强调安全进入区，但内镜下经鼻经斜坡入路（EETA）已成为一种用于腹侧脑桥病变的微创中线通道，可能减少对脑干的牵拉。然而，儿童经验仍然有限。我们评估了EETA治疗儿童脑桥CMs的安全性、可行性和早期结局，并描述了该人群特有的解剖和技术考虑。
方法： 我们进行了一项单机构回顾性病例系列研究，纳入2011年至2025年间治疗的6例儿童患者（年龄5个月至17岁）。所有患者均因腹侧脑桥CMs出现症状性出血和/或局灶性神经功能缺损。术前评估包括MRI、计算机断层扫描和脑积水评估。手术规划强调儿童限制因素，包括有限的鼻腔通道和发育中的颅底解剖，并采用多层重建技术以尽量减少脑脊液（CSF）漏风险。常规使用术后CSF分流（腰大池引流或脑室外引流）。结局包括切除范围、并发症、CSF漏、感染、神经功能恢复和吞咽功能。
结果： 所有患者均实现全切除。5例神经功能改善，包括颅神经病和偏瘫恢复；1例持续外展神经麻痹。发生1例CSF漏伴脑膜炎，经脑室外引流置入和皮瓣翻修后缓解，无后遗症。未观察到其他漏。2例患者需要临时胃造瘘管，但所有患者均恢复吞咽功能。无动脉损伤、新的永久性缺损或死亡。最年轻患者（5个月）在适当规划和专用器械下证明了婴儿期的可行性。
结论： 对于经过选择的儿童腹侧脑桥CMs，EETA是一种安全且可行的通道，当根据儿童解剖结构进行调整并仔细选择患者时，可获得良好的早期结局。

英文摘要

BACKGROUND AND OBJECTIVES: Brainstem cavernous malformations (CMs) carry high hemorrhage risk and morbidity due to their eloquent location. Although modern microsurgical approaches emphasize safe entry zones, endoscopic endonasal transclival approaches (EETA) have emerged as a less invasive midline corridor for ventral pontine lesions, potentially reducing brainstem manipulation. However, pediatric experience remains limited. We evaluated the safety, feasibility, and early outcomes of EETA for pontine CMs in children and characterized anatomic and technical considerations unique to this population.
METHODS: We performed a single-institution retrospective case series of 6 pediatric patients (ages 5 months-17 years) treated between 2011 and 2025. All presented with symptomatic hemorrhage and/or focal neurological deficits from ventral pontine CMs. Preoperative evaluation included MRI, computed tomography, and hydrocephalus assessment. Operative planning emphasized pediatric constraints, including limited nasal corridors and developing skull base anatomy, with multilayer reconstruction techniques to minimize cerebrospinal fluid (CSF) leak risk. Routine postoperative CSF diversion (lumbar drain or external ventricular drain) was used. Outcomes included extent of resection, complications, CSF leak, infection, neurological recovery, and swallow function.
RESULTS: Gross total resection was achieved in all patients. Five demonstrated neurological improvement, including recovery of cranial neuropathies and hemiparesis; one had persistent abducens palsy. One CSF leak occurred with meningitis and resolved after external ventricular drain placement and flap revision without sequelae. No other leaks were observed. Two patients required temporary gastrostomy tubes, but all regained swallowing function. There were no arterial injuries, new permanent deficits, or mortality. The youngest patient (5 months) demonstrated feasibility in infancy with appropriate planning and specialized instrumentation.
CONCLUSION: EETA is a safe and feasible corridor for selected pediatric ventral pontine CMs, with favorable early outcomes when tailored to pediatric anatomy and careful patient selection.