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Photos courtesy of: Jean Shein, M.D. Photos taken by: Joann Givens, CRA, OCT-C.
Used with permission. Not to be reproduced.

A six-year-old girl was presented for examination with a history of having a “white bump” at the “edge” of her left eye. Vision was 20/20 in each eye and motility was normal. Refraction was + 0.50 sphere in each eye and the fundus was normal.

1.	The most likely diagnosis is:
	c -- dermoid This lesion is typical for a limbal dermoid, and is located in the inferior temporal location where these limbal lesions usually occur. Dermoid cysts can also present attached to the orbital rim as a rounded or domed lesion that is fixed to the periostium. When anterior to the orbital septum they can be removed completely with relative ease. If the cyst extends into the orbit behind the orbital septum, excision can be more difficult. When excising these orbital dermoids, especially those extending behind the septum, care must be exercised to avoid spilling the contents into the orbit because of intense inflammatory reaction that is likely to occur. Retinoblastoma extending outside the eye would be associated with destruction of the eye. A retained foreign body would have more reaction, have a recognized onset, and a possible history of trauma. A phlyctenule occurs in conjunctivitis or keratitis and is the result of a response from an antigen, usually M. tuberculosis or S. aureus.
2.	A lesion like this is most likely to be associated with:
	a -- Goldenhar syndrome Limbal dermoids are frequently associated with Goldenhar syndrome. These patients also have pretragal skin tags and vertebral abnormalities. If affected patients also have hemifacial microsomia (smaller jaw and face on one side) the syndrome is called Goldenhar-Gorlin. Alport’s is a congenital renal disease that also exhibits changes in the lens, cornea, and retina. Aniridia patients can develop a vascular pannus of the retina. Hallermann-Streiff syndrome is characterized by a thin pointed nose, small chin, veined skin, wispy hair, small stature, and normal intelligence.
3.	Which of the following is (are) true?
	d -- (a) and (b) A dermoid is a choristoma which is defined as normal tissue in an abnormal location. They can contain hair, skin and the glands associated with skin especially sebaceous glands, fibrous tissue, and blood. If large they can distort the cornea causing astigmatism. These tumors can be dealt with surgically by simply shaving them off flush with the cornea or slightly below the corneal surface. However, the deeper cornea is always at least somewhat cloudy. Dermoids can extend deep into the cornea. In some cases, when the dissection is carried out deep in the cornea, a lamellar corneal graft can be placed. Even if relatively clear immediately after surgery, these grafts tend to become cloudy.