Mosher Amanda M, Ruppert-Gomez Marcella, Albanese John S, Papadakis Joanna E, Lopes Carolina, Proctor Mark R, Fehnel Katie P
Department of Neurosurgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Dana Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts.
J Neurosurg Case Lessons. 2025 Jul 28;10(4). doi: 10.3171/CASE25165.
Neurenteric cysts (NECs) can form anywhere along the neuroaxis.
Thirteen patients with NECs were treated at a single institution: 7 with intraspinal lesions and 6 with cranial lesions. Spinal cysts presented with pain (28.6%), scoliosis (42.9%), gait changes (28.6%), and progressive weakness (14.3%). Infratentorial cysts (n = 4) presented with headache and neck pain; the CN III cyst (n = 1) presented with CN III palsy, headaches, and facial pain; and the convexity NEC (n = 1) presented with seizures. Complete resection was achieved without recurrence for all spinal and infratentorial NECs. Both supratentorial NECs had radiographic recurrence within 1 year of surgery, one of which recurred after gross-total resection. The CN III NEC did not require reintervention with durable improvement in pain, headaches, and CN III function postoperatively. Convexity recurrence was associated with recurrent seizures; complete re-resection was achieved with durable improvement in seizures. No perioperative complications were associated with supratentorial, infratentorial, or sacral NECs. All complications were associated with ventral thoracic NECs and included a CSF leak (n = 1), neurological worsening (n = 1), and symptomatic delayed tethering (n = 2).
NECs can occur anywhere within the neuroaxis. Symptomatic cysts require treatment. The cyst location impacts the surgical approach and can inform discussions regarding the operative risk and anticipated outcomes for patients and their families. https://thejns.org/doi/10.3171/CASE25165.
神经肠囊肿(NECs)可沿神经轴在任何部位形成。
13例神经肠囊肿患者在同一机构接受治疗:7例为脊髓内病变,6例为颅内病变。脊髓囊肿表现为疼痛(28.6%)、脊柱侧弯(42.9%)、步态改变(28.6%)和进行性肌无力(14.3%)。幕下囊肿(n = 4)表现为头痛和颈部疼痛;动眼神经囊肿(n = 1)表现为动眼神经麻痹、头痛和面部疼痛;凸面神经肠囊肿(n = 1)表现为癫痫发作。所有脊髓和幕下神经肠囊肿均实现完全切除且无复发。两个幕上神经肠囊肿在手术后1年内均出现影像学复发,其中1例在次全切除后复发。动眼神经神经肠囊肿术后无需再次干预,疼痛、头痛和动眼神经功能持续改善。凸面复发与癫痫复发相关;再次完全切除后癫痫持续改善。幕上、幕下或骶部神经肠囊肿均未出现围手术期并发症。所有并发症均与胸段腹侧神经肠囊肿相关,包括脑脊液漏(n = 1)、神经功能恶化(n = 1)和症状性延迟脊髓栓系(n = 2)。
神经肠囊肿可发生在神经轴内的任何部位。有症状的囊肿需要治疗。囊肿位置影响手术入路,并可为患者及其家属关于手术风险和预期结果的讨论提供参考。https://thejns.org/doi/10.3171/CASE25165