Intracranial Extension of an Orbital Epidermoid Cyst

Case Reports

Intracranial Extension of an Orbital noted to be encapsulated and densely adherent to the underlying dura. There was severe destruction and thinning of the supero- Epidermoid Cyst lateral orbital rim and roof and the cyst lining was most adherent Jordan M. Burnham, M.D., and Kyle Lewis, M.D. in this area requiring polishing with a diamond burr. The tumor was casseous with yellow-tan keratin-like substance through- Abstract: Epidermoid and dermoid cysts represent the out. Histopathologic examination revealed numerous sheets most common cystic lesions of the orbit and commonly of anucleate squames and keratin debris with a lack of dermal arise from bony sutures or the intradiplpoic space of orbital appendages. The lateral wall was reconstructed using a medpor bones. Massive intracranial extension of an epidermoid cyst sheet fixated to the cranial bone flap and the orbital rim was arising from the intradiploic space of an orbital bone is very replated with 2 titanium C-plates. The patient did well postop- rarely seen. We present a case of a 55-year-old male who was eratively and experienced no complications. incidentally found to have massive intracranial extension of an intradiploic epidermoid cyst of the superolateral orbital DISCUSSION bone with minimal symptoms. The cyst was completely excised via a pterional craniotomy and lateral orbitotomy by Dermoid and epidermoid cysts are among the most com- neurosurgery and oculoplastic surgery teams. The patient mon space occupying lesions of the orbit and typically arise suffered no complications and is doing very well. within and around the zygomaticofrontal suture. Growth is insidious, and patients often present later in life with diplopia and proptosis.7,8 While cases of intracranial extension of orbital ermoid and epidermoid tumors of the orbit are benign dermoids have been seen, this patient’s lesion appears to have developmental choristomas that result from the entrapment D arisen within the intradiploic space of the zygomatic process of of ectodermal rests often within developing bone or suture lines. the frontal bone. Superficial or exophytic dermoid cysts usually present early in Intradiploic epidermoid cysts are among the more com- childhood as a firm mass at the frontozygomatic suture, while in mon lesions of the developing skull and typically involve the contrast, deep or complex dermoid cysts may have a more insid- frontal and temporal bones.9 In 1991, Eijpe described the clini- ious onset and present much later in adulthood.1 Intradiploic cal characteristics of 4 patients with intradiploic epidermoid epidermoid cysts also present insidiously usually later in adult- cysts arising from the orbital bones.6 Proptosis and diplopia were hood as they commonly arise from cranial bones and may be among the most common presenting symptoms. Headache, as in asymptomatic for many years. Pressure excavation from the our case is the most common presenting symptom in lesions of slow growth of the cyst or bony erosion from inflammation is the cranial cavity. frequently seen. However, massive intracranial extension from a lesion of the orbital bones is extremely rare.2–6 This case report There are only a handful of cases in the literature describ- is compliant with HIPPA guidelines. ing lesions arising from the intradiploic space of an orbital bone with intracranial extension,2,4,6 and none with the massive intracranial extension seen in our case. Ormond2 reported a single CASE case of an elderly patient who presented with a hemorrhagic We present a 55-year-old male who presented to the epidermoid cyst arising from the intradiploic space of the pos- emergency room in March of 2012 after a bicycle accident. terolateral orbit with hemorrhage into the frontal lobe. Blanco4 A computed tomography of the head revealed an incidental reported a case of an intradiploic epidermoid arising from the large 8 × 6 cm right orbital and frontal mass with erosion of lateral orbital wall with extension into the anterior cranial fossa. the superolateral orbit and frontal bone. Upon questioning, the For large symptomatic cysts, surgical excision is the main- patient admitted to 2 years of waxing and waning right tem- stay of treatment and in the rare cases where there is significant poral headaches and proptosis of the right eye that had been intracranial extension, a combined approach with neurosurgery slowly increasing in severity but not enough to prompt him to is required. Surgery should include complete surgical extirpation seek medical evaluation. Further investigation with magnetic with microsurgical stripping of the cyst lining from the dura. The resonance imaging confirmed the 8.8 cm extra-axial cystic mass orbital walls and orbital roof will frequently be destroyed by the with 1.3 cm midline shift. The mass displayed hypointensity on tumor expansion and will need to be reconstructed at the time of T1 and hyperintensity on T2 with restricted diffusion consistent surgery to prevent pulsatile enophthalmos.3 with an epidermoid cyst. There was significant bony destruction Intradiploic epidermoid cysts of the orbital bones are of the superolateral orbit and scalloping of the cranial bones rare. This case illustrates a potential outcome of these lesions localizing its point of origin to the intradiploic space of the with massive intracranial extension. It is remarkable that superolateral orbital wall. There was noticeable calcification this patient had very few symptoms. These lesions should be along the medial boundary suggesting chronicity (Fig. 1). included in the differential of lesions of the orbital bones and An excisional biopsy was carried out successfully via a surgery should be considered in all cases of dermoid and epi- pterional craniotomy and superolateral orbitotomy by neurosur- dermoid cysts of the orbit. gical and oculoplastic teams. Intraoperatively, the lesion was REFERENCES 1. Rao AA, Naheedy JH, Chen JY, Robbins SL, Ramkumar HL. A Department of Ophthalmology, University of Mississippi Medical Center, clinical update and radiologic review of pediatric orbital and ocular Jackson, Mississippi, U.S.A. tumors. J Oncol 2013;2013:975908. Accepted for publication August 19, 2014. The authors have no financial or conflicts of interest to disclose. 2. Ormond D, Omeis I, Abrahams J. Uncommon presentation of an This case report is compliant with IRB and HIPPA policies. intradiploic orbital epidermoid tumor: case report. Oral Maxillofac Address correspondence and reprint requests to Jordan M. Burnham, Surg 2011; 15:165–7. M.D., Department of Ophthalmology, University of Mississippi Medical 3. Gabibov GA, Sokolova ON, Cherekaev VA, et al. Dermoid cysts of Center, Jackson, MS, U.S.A. E-mail: [email protected] the orbit spreading into the cranial cavity. Zh Vopr Neirokhir Im NN DOI: 10.1097/IOP.0000000000000327 Burdenko 1989;Sep–Oct:49–51.

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FIG. 1. A, Axial computed tomography demonstrating destruction of the superolateral bony orbit; B, Sagittal magnetic resonance imaging (MRI) showing intracranial extension; C, T2 MRI showing large right frontal mass with midline shift; and D, Coronal MRI showing superolateral bony destruction from intracranial extension.

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