Intradural intramedullary epidermoid cyst in a 17-year-old male: An exceptionally rare case report and review of the literature

Int J Surg Case Rep. 2024 Mar:116:109331. doi: 10.1016/j.ijscr.2024.109331. Epub 2024 Feb 2.

Abstract

Introduction: Spinal epidermoid tumors are exceptionally rare, comprising less than 1 % of all spinal tumors. True intramedullary epidermoid cysts (IECs) are even more infrequent, constituting only 0.8 % of all spinal epidermoid tumors, with a notable cranial preference. Due to their gradual growth, the clinical presentation varies widely based on factors such as size, location, and the patient's age.

Case presentation: A 17-year-old male patient was admitted after experiencing an eight-month-long gradual weakening of both lower limbs. Initially, he experienced paresthesia in the right lower extremities, which progressed to gait disturbances, impacting balance and coordination. Clinical examination indicated bilateral lower limb weakness, reduced vibration sense, and proprioception with a positive clonus sign and extensor plantar responses. Magnetic resonance imaging (MRI) revealed an intramedullary lesion at the T3-T4 level, appearing hypointense on T1-weighted and hyperintense on T2-weighted images. Subsequently, the patient underwent laminectomy of the T2-T5 vertebrae and microsurgical resection of the intramedullary lesion. Histopathological analysis confirmed the diagnosis of an epidermoid cyst. Following two months of physiotherapy, there was progressive improvement in the lower limb coordination and mobility.

Clinical discussion: Progressive neurological deficits emphasize the importance of comprehensive neurological evaluation. Diagnosis involves clinical manifestations, imaging, and histopathological examination. Patients often exhibit gradual motor weakness, sensory alterations, and varying degrees of pain. Advanced neuroimaging such as MRI aids diagnosis. Surgical resection is the primary treatment with potential complications.

Conclusion: A multidisciplinary approach is imperative for timely diagnosis and patient management, ensuring favorable outcomes while minimizing complications.

Keywords: Case report; Intradural; Intramedullary epidermoid cyst; Spinal epidermoid tumor; Spine.

Publication types

  • Case Reports