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伴有持续性双主动脉弓和前脑脊膜膨出的小儿神经肠囊肿的治疗:病例说明

Management of a pediatric neuroenteric cyst with persistent double dorsal aortae and anterior meningocele: illustrative case.

作者信息

Kim Faith S, Hurtado Christopher, Nelson Jennifer S, Meckmongkol Teerin, Scherer Andrea G, Salazar Sofia E, Westmoreland Tamarah

机构信息

College of Medicine, University of Central Florida, Orlando, Florida.

Department of Surgery, Florida State University College of Medicine, Tallahassee, Florida.

出版信息

J Neurosurg Case Lessons. 2025 Jun 16;9(24). doi: 10.3171/CASE24894.

Abstract

BACKGROUND

Neuroenteric cysts are exceedingly rare congenital anomalies. Although the exact pathogenesis is incompletely understood, neuroenteric cysts can arise when foregut duplication cysts extend into the spinal canal. This process is likely related to failure of endodermal cell separation from ectodermal counterparts during week 3 of development, and symptoms depend on location and degree of encroachment on surrounding structures. Complete resection remains the treatment of choice with the lowest incidence of recurrence, but the optimal surgical approach is debated. Associated congenital anomalies can add surgical complexity.

OBSERVATIONS

The authors present the case of a 7-week-old infant diagnosed with a large mediastinal extradural neuroenteric cyst, along with multiple other congenital anomalies. A combined thoracic and laparoscopic abdominal approach was required for complete resection.

LESSONS

This case illustrates the importance of multispecialty collaboration, advanced imaging for preoperative planning, and thoughtful timing of intervention. A combined thoracoscopic/thoracotomy and laparoscopic approach, while surgically demanding, was safe and provided excellent visibility for complete resection of the large neuroenteric cyst. https://thejns.org/doi/10.3171/CASE24894.

摘要

背景

神经肠囊肿是极其罕见的先天性异常。尽管确切的发病机制尚未完全明了,但当前肠重复囊肿延伸至椎管时可形成神经肠囊肿。这一过程可能与发育第3周时内胚层细胞与外胚层细胞分离失败有关,症状取决于囊肿位置及对周围结构的侵犯程度。完整切除仍是复发率最低的首选治疗方法,但最佳手术入路仍存在争议。相关先天性异常会增加手术复杂性。

观察

作者报告了1例7周大婴儿,诊断为巨大纵隔硬膜外神经肠囊肿,同时伴有多种其他先天性异常。需采用胸腔镜联合腹腔镜腹部入路进行完整切除。

经验教训

该病例说明了多学科协作、术前规划的先进影像学检查以及精心安排干预时机的重要性。胸腔镜/开胸术与腹腔镜联合入路虽然对手术要求较高,但安全可行,能为完整切除巨大神经肠囊肿提供良好视野。https://thejns.org/doi/10.3171/CASE24894

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8bea/12171098/457b7bab642e/CASE24894_figure_1.jpg

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