Proptosis in a 6-Month-Old Boy
REPORT OF A CASE
A 6-month-old boy had progressive protrusion of the right eye since birth. He had a proptosis measuring 34 mm with a pigmented subconjunctival mass in the upper fornices and cutaneous pigmentation on the upper eyelid (Figure 1). Multiple pigmented cutaneous lesions were seen throughout the body, ranging from 1 to 10 mm (Figure 2). A computed tomographic scan showed a large lesion infiltrating the right eyeball and posterior orbit, causing scalloped expansion of the orbit (Figure 3). An exenteration was performed (Figure 4).
Figure 1. Clinical photograph showing proptosis of the right globe and cutaneous pigmentation on the upper eyelid.
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Figure 2. Multiple congenital melanocytic nevi seen throughout the body.
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Figure 3. Axial computed tomographic scan revealing a large lesion in the orbit, infiltrating the globe.
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Figure 4. Gross picture of the sectioned eye showing a deeply pigmented tumor filling up the globe and extending to the orbit superoposteriorly. Arrow indicates the anterior part of the eye (note the eyelashes).
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Please e-mail your diagnosis to ophthquiz{at}ama-assn.org. You must include your full name, mailing address, and institutional affiliation in the initial e-mail to be eligible to enter the quiz. The first correct respondent will be recognized in the print journal and on our Web site and will also be able to choose one of the following books published by AMA Press: Clinical Eye Atlas, Clinical Retina, or Users’ Guides to the Medical Literature. Because of the volume of responses we are able to respond to the first person with the correct answer only.
For a complete presentation of this case and an in-depth discussion of the entity, please see next month's edition of Archives of Ophthalmology.
Congratulations to the winner of our May quiz, Christos Christakopoulos, MD, FEBO, Hilleroed Eye Department, Glostrup University Hospital, Copenhagen, Denmark.
The correct answer to our May challenge was complex choristoma.
For a complete discussion of this case, see the Letters: Research Letters section in the June Archives (Tan JCH, Lucarelli MJ, Albert DM, Gentry LR. Complex choristoma masquerading as a dacryocystocele. Arch Ophthalmol. 2009;127[6]:823-826).
Next month's quiz will be available on July 13, 2009, at 3 pm Central time.