Pagkou Diamantoula, Del Maestro Mattia, Luzzi Sabino, Morbini Patrizia, Foroglou Nikolaos, Galzio Renato Juan
Department of Neurosurgery, Aristotle University of Thessaloniki, AHEPA University Hospital, Thessaloniki, Greece.
PhD School in Experimental Medicine, Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Pavia.
Surg Neurol Int. 2021 Mar 17;12:100. doi: 10.25259/SNI_578_2020. eCollection 2021.
Epidermoid cysts are benign slow-growing congenital lesions, constituting approximately 1% of all cranial tumors. Most of these lesions are located intradurally, while about 10-25% of them are located within the diploic spaces. Intradiploic epidermoid cysts are usually discovered incidentally and may remain asymptomatic for many years, but in rare instances, they may grow intracranially and produce brain compression. Sometimes, intradiploic epidermoid cysts may occlude the main cranial venous sinuses causing intracranial hypertension.
We present the case of a 24-year-old male harboring a paramedian right occipital intradiploic cyst with erosion of both outer and inner bony tables, which occluded the torcular herophili producing a worsening symptomatology with acute-onset diplopia from right sixth cranial nerve palsy; the patient also presented bilateral papilledema, but only reported mild headache and dizziness. Neuroradiological studies evidentiated a lesion compatible with intradiploic epidermoid cyst with intralesional hemorrhagic component, overlying and almost completely occluding the torcular herophili. Considering the fast worsening of symptomatology and the evidence of intracranial hypertension, the patient was operated on immediately after completion of clinical and radiological assessment. The lesion was radically removed with almost immediate reversal of signs and symptoms. Histopathology confirmed the diagnosis of epidermoid cyst with intralesional hemorrhagic components.
Intradiploic epidermoid cysts may cause intracranial hypertension by occlusion of main cranial venous sinuses; intralesional hemorrhage may act as precipitating factor in occlusion of the torcular herophili, producing rapidly worsening intracranial hypertension, which requires prompt surgical treatment to reverse symptomatology. Radical surgical resection is necessary to avoid recurrence.
表皮样囊肿是良性、生长缓慢的先天性病变，约占所有颅内肿瘤的1%。这些病变大多位于硬膜内，约10%-25%位于板障间隙。板障内表皮样囊肿通常为偶然发现，可能多年无症状，但在罕见情况下，它们可能向颅内生长并压迫脑组织。有时，板障内表皮样囊肿可能阻塞主要的颅内静脉窦，导致颅内高压。
我们报告一例24岁男性患者，其右枕部旁正中板障内囊肿，内外骨板均有侵蚀，阻塞窦汇，导致症状加重，因右侧展神经麻痹急性发作出现复视；患者还出现双侧视乳头水肿，但仅诉有轻度头痛和头晕。神经放射学检查显示病变符合板障内表皮样囊肿，伴有瘤内出血成分，覆盖并几乎完全阻塞窦汇。考虑到症状迅速恶化及颅内高压的证据，患者在完成临床和放射学评估后立即接受手术。病变被彻底切除，体征和症状几乎立即缓解。组织病理学证实为伴有瘤内出血成分的表皮样囊肿。
板障内表皮样囊肿可通过阻塞主要颅内静脉窦导致颅内高压；瘤内出血可能是窦汇阻塞的促发因素，导致颅内高压迅速恶化，这需要及时手术治疗以缓解症状。彻底的手术切除对于避免复发是必要的。
