Ott Stefanie, Lübke Andreas, Mohme Malte, Westphal Manfred
1Department of Neurosurgery, UKE Hamburg Eppendorf, Hamburg, Germany; and.
2Center for Diagnostics, Institute of Pathology with the Sections Molecular Pathology and Cytopathology, UKE Hamburg Eppendorf, Hamburg, Germany.
J Neurosurg Case Lessons. 2023 Dec 18;6(25). doi: 10.3171/CASE23614.
Epidermoid cyst tumors can arise as intradiploic tumors in the frontal skull bones around the fontanel in childhood but are mostly found at the frontal or frontotemporal base of the brain or in the cerebellopontine angle. Therefore, finding a symptomatic intradiploic lesion in the convexity in late adulthood is uncommon. Intradiploic epidermoids can cause complications as they grow, by eroding and perforating their surroundings, and in cases of destruction of the wall of a pneumatized sinus, they can cause pneumocephalus.
In the present case, a female patient presented with a skull lesion that had grown progressively over 64 years, resulting in spontaneous pneumocephalus. Surgery with subsequent cranioplasty was performed. The histological examination confirmed the presence of an intradiploic epidermoid.
This case highlights that complete resection of the lesion with subsequent cranioplasty is recommended before symptoms and reconstructive challenges due to the enormous size of the defect. This case serves as a reminder that intradiploic epidermoids, although uncommon, will expand throughout life and can cause significant complications such as pneumocephalus after decades. Timely surgical interventions after diagnosis are recommended to prevent further complications and to achieve a successful outcome in terms of complete resection and reconstruction.
表皮样囊肿可在儿童期于囟门周围的额颅骨内板层形成肿瘤，但大多见于脑的额部或额颞底部或桥小脑角。因此，在成年后期凸面发现有症状的板障内病变并不常见。板障内表皮样囊肿生长时可因其侵蚀和穿破周围组织而引发并发症，若气房窦壁遭到破坏，可导致气颅。
在本病例中，一名女性患者出现颅骨病变，该病变在64年间逐渐增大，导致自发性气颅。进行了手术及后续颅骨成形术。组织学检查证实存在板障内表皮样囊肿。
该病例强调，鉴于巨大缺损所致的症状和重建难题，建议在出现这些情况之前对病变进行完整切除并随后进行颅骨成形术。该病例提醒我们，板障内表皮样囊肿虽不常见，但会终生生长，数十年后可导致气颅等严重并发症。建议诊断后及时进行手术干预，以防止进一步并发症的发生，并在完整切除和重建方面取得成功结果。
