Menezes Arnold H, Dlouhy Brian J
Department of Neurosurgery, University of Iowa Hospitals & Clinics, Stead Family Children's Hospital, 200 Hawkins Drive, 1824 JPP, Iowa City, IA, 52242, USA.
Childs Nerv Syst. 2020 Jul;36(7):1379-1384. doi: 10.1007/s00381-020-04604-8. Epub 2020 Apr 23.
Neurenteric cysts (NEC) are rare benign tumors of the central nervous system. Even more rare are the NEC at the ventral foramen magnum in children that are misdiagnosed. They cause distortion of the vertebrobasilar vascular system and cervicomedullary neural structures and are adherent to the latter. The inferior portion lies ventral to the upper most dentate ligament. This has implications in the surgical approach for complete excision.
We reviewed our series of five children with ventral foramen magnum NEC in the MRI era and analyzed presentation, radiological findings and surgical management. A literature review is also presented.
There were two females and five males. Headache and neurological deficit was present in all five; one child presented with recurrent meningitis. The NEC was present ventral to the cervicomedullary junction in all cases. The posterolateral transcondylar approach was used for complete excision with no recurrences.
Neurenteric cysts at the ventral foramen magnum can be confused with other cystic lesions. Headaches are the most common presentation. The posterolateral transcondylar approach to the ventral cervicomedullary junction provides the most reliable avenue for entire resection.
神经肠囊肿（NEC）是中枢神经系统罕见的良性肿瘤。儿童枕骨大孔腹侧的神经肠囊肿更为罕见，且易被误诊。它们会导致椎基底血管系统和颈髓神经结构变形，并与之粘连。囊肿下部位于最上方齿状韧带的腹侧。这对完整切除的手术入路有影响。
我们回顾了MRI时代的5例儿童枕骨大孔腹侧神经肠囊肿病例，分析了其临床表现、影像学表现及手术治疗情况。并进行了文献综述。
5例患者中，2例为女性，3例为男性。所有5例均有头痛和神经功能缺损；1例患儿表现为复发性脑膜炎。所有病例的神经肠囊肿均位于颈髓交界处腹侧。采用后外侧经髁入路完整切除，无复发。
枕骨大孔腹侧的神经肠囊肿可能与其他囊性病变混淆。头痛是最常见的表现。后外侧经髁入路至颈髓腹侧交界处是完整切除的最可靠途径。
