Division of Pediatric Neurosurgery, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 East Chicago Avenue, Chicago, IL, 60611, USA.
Department of Neurological Surgery, Northwestern University Feinberg School of Medicine, Chicago, USA.
Childs Nerv Syst. 2023 Jan;39(1):127-139. doi: 10.1007/s00381-022-05719-w. Epub 2022 Nov 9.
Controversy remains regarding surgical managements of sylvian fissure arachnoid cyst (SFAC). This review presents our experience in the microsurgical fenestration of pediatric patients with SFAC to define surgical indication, and risks and benefits with special emphasis on postoperative subdural fluid collection (SDFC) and cyst size reduction.
Thirty-four children with SFAC who underwent microsurgical cyst fenestration at a single institution over a 10-year period were retrospectively reviewed for their clinical presentation, neuroimaging findings, and postsurgical course. The SFACs were classified by a novel grading system based on the degree of arachnoid cyst extension from the sylvian fissure to the insular cistern shown on MR images: grade 0 - little or no prominence of sylvian fissure, grade I - SFAC confined to the sylvian fissure, grade II - SFAC partially extending to the insular cistern, grade III - SFAC extending to the entire insular cistern.
There were 26 males and 8 females. SFAC was present in the left side in 24. Twelve patients presented with cyst rupturing to the subdural space. Cyst grading did not show significant difference compared with rupture status (p > 0.9). All patients underwent microsurgical cyst fenestration. Postoperative SDFC is common but often resolved overtime in two-thirds of the cases with the mean average of 6 months. However, 3 patients had symptomatic postoperative SDFC and needed reoperation shortly after the first operation. Microsurgical cyst fenestrations for SFAC effectively resolved the presenting symptoms and often showed restorations of intracranial structures on follow-up imaging. Cyst resolution or reduction greater than 75% was noted in 61.8% of the patients postoperatively which was noted in a half of the SFAC of children even with age of 11 years or older. During the follow-up, no cyst recurrence or SDFC was noted. Patients with greater surgical reduction of cyst size tended to occur in younger children, and those with lower MR grade.
Our results showed a high reduction rate of SFAC and brain re-expansion after microsurgical fenestration together with symptomatic improvements regardless the patient's age. Considering the developing CNS during childhood, reductions of a large space-occupying lesion followed by restorations of the structural integrity of the developing brain are very desirable. However, a multi-center cooperative prospective longitudinal study on long-term comparative data of those treated and untreated of neuro-psychological outcome and cyst rupture incidence is needed.
关于外侧裂蛛网膜囊肿(SFAC)的手术治疗仍存在争议。本研究介绍了我们在单中心对儿童 SFAC 进行显微开窗术的经验,以明确手术适应证、风险和获益,特别强调术后硬膜下积液(SDFC)和囊肿体积缩小的问题。
回顾性分析了 10 年间在单中心接受显微囊肿开窗术的 34 例 SFAC 患儿的临床资料、神经影像学表现和术后病程。根据 MRI 上蛛网膜囊肿从外侧裂向岛叶脑池延伸的程度,将 SFAC 分为新的分级系统:0 级-外侧裂无明显突出;1 级-SFAC 局限于外侧裂;2 级-SFAC 部分延伸至岛叶脑池;3 级-SFAC 延伸至整个岛叶脑池。
男 26 例,女 8 例。左侧 24 例。12 例患者囊肿破裂至硬膜下腔。囊肿分级与破裂状态无显著差异(p>0.9)。所有患者均行显微囊肿开窗术。术后 SDFC 很常见,但三分之二的患者在平均 6 个月后自行缓解。然而,3 例患者有症状性术后 SDFC,在第一次手术后不久需要再次手术。显微囊肿开窗术治疗 SFAC 可有效缓解症状,并常能在随访影像学上显示颅内结构的恢复。术后 61.8%的患者囊肿完全或大部分缩小(>75%),即使在 11 岁及以上的儿童中,也有一半的 SFAC 有这种情况。随访期间无囊肿复发或 SDFC 发生。囊肿体积缩小较大的患者往往年龄较小,MR 分级较低。
我们的研究结果表明,显微开窗术后 SFAC 及脑扩张的缩小率较高,症状改善明显,与患者年龄无关。考虑到儿童中枢神经系统的发育,对大占位病变进行手术切除,随后恢复发育中脑的结构完整性是非常理想的。然而,需要开展多中心合作前瞻性纵向研究,以获得长期比较数据,评估治疗和未治疗患者的神经心理学结局和囊肿破裂发生率。