Neurology and Neurosurgery, Universidade Federal de São Paulo, São Paulo, São Paulo, Brazil; Neurosurgery, Hospital Beneficência Portuguesa de São Paulo, São Paulo, São Paulo, Brazil.
Epilepsy Unit, Hospital del Mar, Barcelona, Spain.
World Neurosurg. 2023 Nov;179:177. doi: 10.1016/j.wneu.2023.08.103. Epub 2023 Aug 30.
Adamantinomatous craniopharyngioma (ACP) is a rare sellar region tumor seen in 0.5-2 cases per million persons each year, presenting a bimodal distribution that peaks at 5-15 years in children and 45-60 years in adults. Arising from embryonic remnants of the Rathke pouch epithelium, ACPs are associated with calcifications in 90% of cases and grow cranially toward the floor of the diencephalon. Craniopharyngiomas are benign but locally aggressive tumors, with microsurgery being the best chance of cure. The natural history is to compress the optic apparatus and hypothalamic-pituitary axis as they expand, with a propensity to encase the carotids. Endoscopic transbasal approaches have gained wide acceptance in the management of these tumors. However, open microsurgical approaches via pterional and orbitozygomatic craniotomies afford wider visualization of different corridors that help mitigate the surgical risks. The orbitozygomatic craniotomy allows lesions that extend above the optic chiasm to be safely approached from an inferior-to-superior corridor. The wide exposure of the basal arachnoid cisterns allows protection of the lenticulostriate perforators during resection. We demonstrate a step-by-step orbitozygomatic approach with dissection of the sylvian, carotid, carotid-oculomotor, chiasmatic, and lamina terminalis cisterns that allowed safe resection of a third ventricular ACP. The patient was a male in his 70s, who presented with progressive headaches and visual impairment. Magnetic resonance imaging showed a multicystic suprasellar lesion extending through the third ventricle. The surgery was performed with no complication (Video 1). Postoperative vision stabilized, and magnetic resonance imaging showed complete resection.
颅咽管瘤(ACP)是一种罕见的鞍区肿瘤,每年每百万人中可见 0.5-2 例,呈双峰分布,儿童高峰在 5-15 岁,成人高峰在 45-60 岁。ACP 来源于 Rathke 囊上皮的胚胎残余,90%的病例伴有钙化,向颅前窝方向生长,抵达间脑底部。颅咽管瘤是良性但具有局部侵袭性的肿瘤,显微手术是治愈的最佳机会。其自然病程是随着肿瘤的生长逐渐压迫视器和下丘脑-垂体轴,肿瘤还有包裹颈动脉的倾向。经鼻内镜颅底入路已广泛应用于这些肿瘤的治疗。然而,经翼点和眶颧入路的开放式显微外科手术可提供更广泛的不同入路视野,有助于降低手术风险。眶颧入路可安全地从下至上经视神经交叉上方的通道处理延伸至视交叉上方的病变。广泛显露基底蛛网膜下腔允许在切除过程中保护纹状体动脉穿支。我们展示了一个经眶颧入路的分步解剖,包括外侧裂、颈内动脉、颈内动脉-动眼神经、视交叉和终板池的解剖,从而安全地切除了第三脑室颅咽管瘤。患者为 70 多岁男性,表现为进行性头痛和视力障碍。磁共振成像显示一个多囊性鞍上病变延伸至第三脑室。手术过程顺利,无并发症(视频 1)。术后视力稳定,磁共振成像显示完全切除。