Introduction
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 are pinched off and form cysts, which are adjacent to the suture line. Approximately 50% of these tumors that involve the head are found in or adjacent to the orbit.
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.
Mortality/Morbidity
- Orbital dermoid cysts almost never cause death.
- Morbidity is usually of a cosmetic nature; more rarely, proptosis and diplopia may result. A traumatically ruptured dermoid may result in dramatic orbital and periocular inflammation.
Age
These tumors are most often noted in young children; however, they may appear or grow at any age.
Clinical
History
- Patients generally complain of a mass, which is visible in the orbital area. Growth of these lesions is generally slow.
- 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
- The most common location is in the superior temporal aspect of the orbit.
- The mass is generally less than 1 cm in diameter, nontender, and oval shaped.
- Little displacement of the globe usually occurs.
- 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, possibly growing or eroding their way into adjacent structures.
- Inflammation
- If the cyst ruptures, either spontaneously or with trauma, a pronounced inflammatory response will occur that may mimic orbital cellulitis.
- The inflammation may be suppressed by corticosteroids, but excision is required to prevent recurrence.
- Neurologic findings
- Rarely, the cyst may press on the optic nerve and create the typical symptoms of optic nerve compression, which typically produces reduced visual acuity, reduced color and brightness perception, and a relative afferent pupillary defect.
- 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 exist.
- Other diagnostic considerations
- Ruptured dermoid cysts may mimic rhabdomyosarcoma.
- Pediatric metastatic cancers
- Orbital cellulitis
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References
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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].
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].
McNab A. Manual of Orbital and Lacrimal Surgery. Butterworth-Heinemann Medical; 1998.
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].
Rootman J. Orbital Surgery: A Conceptual Approach. Raven Press; 1995.
Schick U, Hassler W. Pediatric tumors of the orbit and optic pathway. Pediatr Neurosurg. Mar 2003;38(3):113-21. [Medline].
Shields JA, Shields CL. Orbital cysts of childhood--classification, clinical features, and management. Surv Ophthalmol. May-Jun 2004;49(3):281-99. [Medline].
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].
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].
Further Reading
Keywords
orbital dermoid cysts, epidermoid cysts, dermoid cysts, orbital tumors, choristoma, epidermoid
Overview: Dermoid, Orbital