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Background

Dermoid and epidermoid cysts are examples of choristomas, tumors that originate from aberrant primordial tissue. These tumors contain normal-appearing tissue in an abnormal location. As two suture lines of the skull close during embryonic development, dermal or epidermal elements may be pinched off and form cysts, which are adjacent to the suture line (this is shown in the image below). Approximately 50% of dermoids that involve the head are found in or adjacent to the orbit.

Temporal-zygomatic suture line on the lateral orbital wall. The location of the periosteal attachment of most orbital dermoids.

Pathophysiology
Orbital dermoid cysts may displace structures in the orbit, especially the globe. If the displacement is great, interference with vision by compression of the optic nerve may result or ocular motility may be disturbed, resulting in diplopia.

Epidemiology
Frequency
United States At an ocular oncology center, dermoid tumors were found to comprise 2% of the orbital tumors that came to surgery.[1]

Mortality/Morbidity

Orbital dermoid cysts almost never cause death. Morbidity is usually of a cosmetic nature; occasionally, loss of vision, diplopia, or orbital inflammation may occur.

Race
Dermoid tumors show no racial predilection.

Sex
Dermoid tumors have an equal incidence in males and females.

Age
These tumors are most often noted in young children; however, they may appear or grow at any age.

History

Patients generally complain of a mass, which is visible in the orbital area. Growth of these lesions is generally slow. Occasionally, a history of inflammation will be present. In adults, dermoids may become symptomatic for the first time and grow considerably over a year. Based on this fact, some conclude that these lesions may be dormant for many years or have intermittent growth.

Physical

Children o The most common location is in the superior temporal aspect of the orbit. o The mass is generally less than 1 cm in diameter, nontender, and oval in shape. o Little displacement of the globe usually occurs. o Orbital dermoid cysts are not attached to the skin, which helps differentiate them from sebaceous cysts. The cyst usually is tethered to the periosteum of the bone near suture lines, including the sinuses or intracranial cavity. Adults: The cysts are palpated less easily and have more vague borders. They are more likely to displace the globe and may erode their way into adjacent structures. Inflammation o If the cyst ruptures, either spontaneously or with trauma, an inflammatory response may be seen. This response may be limited to injection of the conjunctiva or may be severe and mimic orbital cellulitis. Occasionally, subconjunctival droplets of fat are seen.[2] Neurologic findings o Rarely, the cyst may press on the optic nerve and produce symptoms of optic nerve compression; reduced visual acuity, color vision and brightness perception, and a relative afferent pupillary defect. o More rarely, the cyst may induce diplopia by physically restricting movement of the globe or by compressing cranial nerves III, IV, or VI.

Causes

No known causes for orbital dermoid exist. Other diagnostic considerations o Ruptured dermoid cysts may mimic rhabdomyosarcoma. o Pediatric metastatic cancers o Orbital cellulitis

Differentials

Exophthalmos Lacrimal Gland Tumors Optic Neuropathy, Compressive Sebaceous Gland Carcinoma Thyroid Ophthalmopathy Tumors, Orbital

Imaging Studies

Radiography: Radiographs often show radiolucent defects where the cyst has eroded into bone. These defects can be large with distinct margins and may show sclerotic changes. CT or MRI studies have largely supplanted plain radiography for evaluating dermoid cysts. o A review of 160 CT studies of orbital dermoids revealed that 65% were lateral and 30% were medial to the globe, only one was entirely behind the globe, 85% had changes in adjacent bone, 73% had a visible wall, 27% had a CT attenuation similar to orbital fat, 14% had calcification, 5% had a fluid level, and 20% had abnormal soft tissue outside the cyst.[3] o On MRI, features include a cystic appearance, internal fat attenuation (T1 hyperintensity), internal calcification, and fluid levels.[4] These features are uncommon in rhabdomyosarcoma. Ultrasound characteristics of dermoid cysts include a smooth contour and variable echogenicity.[5] Color Doppler imaging of dermoid cysts shows no intralesional blood flow, which can help differentiate them from hemangioma and rhabdomyosarcoma.[5]

Histologic Findings
The external layer of the cyst has variable thickness and may be exceedingly thin. The cyst is generally connected to periorbita by fibrovascular tissue. Epidermoid cysts have a lining of epithelial cells, usually stratified, that produce keratin. Dermoid cysts contain blood vessels, fat, collagen, sebaceous glands, and hair follicles. The material in the cyst varies from a tan, oily liquid to a white or yellow substance that resembles cottage cheese or even a relatively solid mass. Often, high cholesterol content is present. The cysts commonly are inflamed and may contain free blood.

Medical Care

No medical care usually is required for an orbital dermoid. Inflammation that results from a ruptured dermoid cyst may be controlled with oral prednisone.

Surgical Care

Dermoid cysts usually are cosmetic problems. The location of the dermoid cyst in the orbit helps determine the appropriate type of orbitotomy. A method for percutaneous

drainage and ablation of orbital dermoid cysts[6] and endoscopic-assisted removal of orbital dermoid cysts has been reported.[7] Inflammation from preoperative or intraoperative rupture of the cyst can be controlled with the use of prednisone. Failure to remove the entire cyst may result in persistent inflammation, a draining sinus, or recurrence of the cyst.

Medication Summary
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Corticosteroids
Class Summary
Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli. View full drug information

Prednisone (Deltasone, Orasone, Meticorten)


The most commonly used oral corticosteroid to control inflammation. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Further Outpatient Care

After surgical excision of an orbital dermoid, infrequent follow-up care is necessary.

Complications

The dermoid cyst may displace the globe, depending on the location of the cyst. Orbital dermoid cysts may cause neurologic complications if they compress the optic nerve or cranial nerves III, IV, or IV. If the cyst ruptures, a marked inflammatory response follows. Operative complications are those common to other orbitotomy procedures. o Damage to the eye or adnexal structures, motility restriction, infection, inflammation, and hemorrhage may occur. o Partial excision of the dermoid cyst may result in persistent inflammation, a draining sinus, or recurrence.

Prognosis

Dermoid cysts generally have a benign prognosis. o If they are excised completely, usually only a minimal scar occurs. o If they are observed rather than excised, slow growth can be expected.

Patient Education

Patients should understand that these tumors are benign. Tell patients that surgery generally is successful, but that serious complications can be associated with any orbitotomy (eg, ptosis, diplopia, blindness, death).

References 1. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. Ophthalmology. May 2004;111(5):997-1008. [Medline]. 2. Jung BY, Kim YD. Orbital dermoid cysts presenting as subconjunctival fat droplets. Ophthal Plast Reconstr Surg. 2008;24(4):327-9. [Medline]. 3. Chawda SJ, Moseley IF. Computed tomography of orbital dermoids: a 20-year review. Clin Radiol. Dec 1999;54(12):821-5. [Medline]. 4. Chung EM, Smirniotopoulos JG, Specht CS, Schroeder JW, Cube R. From the archives of the AFIP: Pediatric orbit tumors and tumorlike lesions: nonosseous lesions of the extraocular orbit. Radiographics. Nov-Dec 2007;27(6):1777-99. [Medline]. [Full Text]. 5. Neudorfer M, Leibovitch I, Stolovitch C, Dray JP, Hermush V, Nagar H, et al. Intraorbital and periorbital tumors in children--value of ultrasound and color Doppler imaging in the differential diagnosis. Am J Ophthalmol. Jun 2004;137(6):1065-72. [Medline]. 6. Golden RP, Shields WE 2nd, Cahill KV, Rogers GL. Percutaneous drainage and ablation of orbital dermoid cysts. J AAPOS. Oct 2007;11(5):438-42. [Medline]. [Full Text]. 7. Prabhakaran VC, Hsuan J, Selva D. Endoscopic-Assisted Removal of Orbital Roof Lesions via a Skin Crease Approach. Skull Base. Sep 2007;17(5):341-5. [Medline]. [Full Text]. 8. McNab A. Manual of Orbital and Lacrimal Surgery. Butterworth-Heinemann Medical; 1998. 9. Rootman J. Orbital Surgery: A Conceptual Approach. Raven Press; 1995. 10. Schick U, Hassler W. Pediatric tumors of the orbit and optic pathway. Pediatr Neurosurg. Mar 2003;38(3):113-21. [Medline]. 11. Shields JA, Shields CL. Orbital cysts of childhood--classification, clinical features, and management. Surv Ophthalmol. May-Jun 2004;49(3):281-99. [Medline]. 12. Sreetharan V, Kangesu L, Sommerlad BC. Atypical congenital dermoids of the face: a 25-year experience. J Plast Reconstr Aesthet Surg. 2007;60(9):1025-9. [Medline]. [Full Text].

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