Case Reports Intracranial Extension of an Orbital Epidermoid Cyst Jordan M. Burnham, M.D., and Kyle Lewis, M.D. Abstract: Epidermoid and dermoid cysts represent the most common cystic lesions of the orbit and commonly arise from bony sutures or the intradiplpoic space of orbital bones. Massive intracranial extension of an epidermoid cyst arising from the intradiploic space of an orbital bone is very rarely seen. We present a case of a 55-year-old male who was incidentally found to have massive intracranial extension of an intradiploic epidermoid cyst of the superolateral orbital bone with minimal symptoms. The cyst was completely excised via a pterional craniotomy and lateral orbitotomy by neurosurgery and oculoplastic surgery teams. The patient suffered no complications and is doing very well.

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ermoid and epidermoid tumors of the orbit are benign developmental choristomas that result from the entrapment of ectodermal rests often within developing bone or suture lines. Superficial or exophytic dermoid cysts usually present early in childhood as a firm mass at the frontozygomatic suture, while in contrast, deep or complex dermoid cysts may have a more insidious onset and present much later in adulthood.1 Intradiploic epidermoid cysts also present insidiously usually later in adulthood as they commonly arise from cranial bones and may be asymptomatic for many years. Pressure excavation from the slow growth of the cyst or bony erosion from inflammation is frequently seen. However, massive intracranial extension from a lesion of the orbital bones is extremely rare.2–6 This case report is compliant with HIPPA guidelines.

CASE We present a 55-year-old male who presented to the emergency room in March of 2012 after a bicycle accident. A computed tomography of the head revealed an incidental large 8 × 6 cm right orbital and frontal mass with erosion of the superolateral orbit and frontal bone. Upon questioning, the patient admitted to 2 years of waxing and waning right temporal headaches and proptosis of the right eye that had been slowly increasing in severity but not enough to prompt him to seek medical evaluation. Further investigation with magnetic resonance imaging confirmed the 8.8 cm extra-axial cystic mass with 1.3 cm midline shift. The mass displayed hypointensity on T1 and hyperintensity on T2 with restricted diffusion consistent with an epidermoid cyst. There was significant bony destruction of the superolateral orbit and scalloping of the cranial bones localizing its point of origin to the intradiploic space of the superolateral orbital wall. There was noticeable calcification along the medial boundary suggesting chronicity (Fig. 1). An excisional biopsy was carried out successfully via a pterional craniotomy and superolateral orbitotomy by neurosurgical and oculoplastic teams. Intraoperatively, the lesion was

noted to be encapsulated and densely adherent to the underlying dura. There was severe destruction and thinning of the superolateral orbital rim and roof and the cyst lining was most adherent in this area requiring polishing with a diamond burr. The tumor was casseous with yellow-tan keratin-like substance throughout. Histopathologic examination revealed numerous sheets of anucleate squames and keratin debris with a lack of dermal appendages. The lateral wall was reconstructed using a medpor sheet fixated to the cranial bone flap and the orbital rim was replated with 2 titanium C-plates. The patient did well postoperatively and experienced no complications.

DISCUSSION Dermoid and epidermoid cysts are among the most common space occupying lesions of the orbit and typically arise 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 dermoids have been seen, this patient’s lesion appears to have arisen within the intradiploic space of the zygomatic process of the frontal bone. Intradiploic epidermoid cysts are among the more common lesions of the developing skull and typically involve the frontal and temporal bones.9 In 1991, Eijpe described the clinical characteristics of 4 patients with intradiploic epidermoid cysts arising from the orbital bones.6 Proptosis and diplopia were among the most common presenting symptoms. Headache, as in our case is the most common presenting symptom in lesions of the cranial cavity. There are only a handful of cases in the literature describing 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 of an elderly patient who presented with a hemorrhagic epidermoid cyst arising from the intradiploic space of the posterolateral orbit with hemorrhage into the frontal lobe. Blanco4 reported a case of an intradiploic epidermoid arising from the lateral orbital wall with extension into the anterior cranial fossa. For large symptomatic cysts, surgical excision is the mainstay of treatment and in the rare cases where there is significant intracranial extension, a combined approach with neurosurgery is required. Surgery should include complete surgical extirpation with microsurgical stripping of the cyst lining from the dura. The orbital walls and orbital roof will frequently be destroyed by the tumor expansion and will need to be reconstructed at the time of surgery to prevent pulsatile enophthalmos.3 Intradiploic epidermoid cysts of the orbital bones are rare. This case illustrates a potential outcome of these lesions with massive intracranial extension. It is remarkable that this patient had very few symptoms. These lesions should be included in the differential of lesions of the orbital bones and surgery should be considered in all cases of dermoid and epidermoid cysts of the orbit.

REFERENCES Department of Ophthalmology, University of Mississippi Medical Center, Jackson, Mississippi, U.S.A. Accepted for publication August 19, 2014. The authors have no financial or conflicts of interest to disclose. This case report is compliant with IRB and HIPPA policies. Address correspondence and reprint requests to Jordan M. Burnham, M.D., Department of Ophthalmology, University of Mississippi Medical Center, Jackson, MS, U.S.A. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000327

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1. Rao AA, Naheedy JH, Chen JY, Robbins SL, Ramkumar HL. A clinical update and radiologic review of pediatric orbital and ocular tumors. J Oncol 2013;2013:975908. 2. Ormond D, Omeis I, Abrahams J. Uncommon presentation of an intradiploic orbital epidermoid tumor: case report. Oral Maxillofac Surg 2011; 15:165–7. 3. Gabibov GA, Sokolova ON, Cherekaev VA, et al. Dermoid cysts of the orbit spreading into the cranial cavity. Zh Vopr Neirokhir Im NN Burdenko 1989;Sep–Oct:49–51.

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J. M. Burnham and K. Lewis

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. 4. Blanco G, Esteban R, Galarreta D, et al. Orbital intradiploic giant epidermoid cyst. Arch Ophthalmol 2001;119:771–3. 5. Srivastava U, Dakwale V, Jain A, Singhal M. Orbital dermoid cyst with intracranial extension. Indian J Ophthalmol 2004;52:244. 6. Eijpe AA, Koornneef L, Verbeeten B, Jr, et al. Intradiploic epidermoid cysts of the bony orbit. Ophthalmology 1991;98:1737–43.

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7. Shields JA, Shields CL. Orbital cysts of childhood-classification, clinical features, and management. Surv Ophthalmol 2004;49:281–99. 8. Rootman J. Diseases of the Orbit: A Multidisciplinary Approach. 2nd ed. Philadelphia, PA: Lippincott and Williams; 2003:417–30. 9. Arana E, Latorre FF, Revert A, et al. Intradiploic epidermoid cysts. Neuroradiology 1996;38:306–11.

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Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.

Intracranial Extension of an Orbital Epidermoid Cyst.

Epidermoid and dermoid cysts represent the most common cystic lesions of the orbit and commonly arise from bony sutures or the intradiplpoic space of ...
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