Intraorbital Expansion of an Intradiploic Frontal Epidermoid Cyst: A Case Report and Short Review of the Literature

Abstract

Patient: Male, 58-year-old Final Diagnosis: Epidermoid cyst Symptoms: Exophthalmos Medication:— Clinical Procedure: — Specialty: Neurosurgery Objective: Rare disease Background: Calvarial epidermoid cysts (EC) are encased remnants of ectoderm at the third week of gestation. There are also reports which consider them sequelae of head trauma. They are benign lesions. As they develop, they exert a mass effect to adjacent anatomical structures. Case Report: We report the case of a 58-year-old male patient with left-sided exophthalmos. His radiologic examinations depicted an oval cystic lesion (7×5×5.5 cm) arising from the left frontal bone and abutting the ipsilateral orbital roof. Our patient underwent a total extirpation of the lesion through a frontal craniectomy. Cranioplasty was then performed with a Porex ® graft. The pearl-hued lamellae of the lesion macroscopically resembled keratin tissue. Histopathological findings supported the diagnosis of an epidermoid cyst. Postoperatively, our patient had no neurologic deficits and a computed tomography scan showed no residual effects. Conclusions: Large calvarial EC with intraorbital expansion in adults are rare clinical entities. Gross total resection with the infiltrated bone and cranioplasty is the treatment of choice, which also establishes the diagnosis.

Tension Pneumocephalus With Acute Cerebellar Symptoms Due to an Intradiploic Epidermoid Cyst Eroding the Mastoid Bone

“…In our patient, the lesion eroded the mastoid bone resulting in a connection between the mastoid air cells and the intracranial cavity which forms a pathway between the intracranial cavity and the eustachian tube. This growth pattern, erosion of the internal table, and thinning of the external table are typically seen in IDEC cases [ 3 ].…”

“…Clinical manifestations include local deformities (subcutaneous lump), tenderness, headache, and neurologic symptoms (seizure, focal neurologic deficit, meningitis) [ 5 , 6 ]. Some cases have been reported with intra-orbital expansion causing exophthalmos or diplopia [ 3 ]. IDECs also present rarely with intracranial hypertension, either primarily due to a significant mass effect or secondary due to venous occlusion [ 12 ].…”

“…The treatment consists of surgical resection including the complete cystic wall to prevent recurrence [ 2 , 3 ]. A consensus regarding the extent of resection of adjacent bone structures is lacking [ 3 ].…”

“…In rare cases, a penetrating trauma might be the underlying etiology by introducing epithelial cells in the diploic space. This results in a sequestration of ectodermal remnants within the intradiploic space [ 2 , 3 ]. The most frequent location are the occipital, the frontal, and the parietal bone [ 1 , 4 ].…”

Intradiploic Epidermoid Cyst of the Posterior Fossa – Case Report and Review of the Literature

“…7 Despite its size, total removal of the lesion along with its capsule leads to definite treatment with very low morbidity and mortality. 2,[8][9][10][11][12] Conservative resection can also be considered given the slow growing nature of this tumor, especially when its localization near crucial neurovascular structures poses a threat. Excision of the eroded bone and cranioplasty, as performed in our case, may be necessary.…”