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Answers: 2009 Series : December 22, 2009
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To see views enlarged, click on the individual pictures...

This newborn presented as shown above with a huge mass behind the right lower lid. In the left orbit no discrete globe could be seen. The large mass in the right lower lid transilluminated with a red glow suggesting the cyst was filled with clear fluid. The pregnancy and delivery were uneventful. There was no family history of similar occurrences and no consanguinity.

1. A likely diagnosis is:

d -- microphthalmos or anophthalmos with cyst

This is an example of microphthalmos or anophthalmos with cyst.  If it is microphthalmos, it represents an extreme example of  this condition which can occur in a spectrum -- from just a small colobomatous cyst composed of tissue from the eye wall associated with an otherwise fairly normal eye to a huge cyst that is much larger than an eye would be.  In general, the larger the cyst, the smaller the eye.  This begs the question, where does one draw the line between microphthalmos and anophthalmos?

2. The origin of this type of lesion can be described best as follows:

-- (a) and (d)

This condition can occur as an isolated evenet or in association with a variety of intracrnial or systemic anomalies.  When this type of condition is encountered, a careful workup should be undertaken including imaging of the brain and general physical examination and additional imaging as indicated.  It is also an extreme example.

3. This condition would be managed with:

c -- excision of the cyst

This condition would indicate the need for careful excision to lessen the problems caused by an already bad situation.  The parents are faced with the daunting challenge of a child with no sight and essentially no eyes.  This protruding mass which has a good chance of getting even larger just adds more misery to the situation and is best removed.

This case was presented with a probable diagnosis of hemangioma.  This is unlikely because of the size/timing of this large mass and the strong likelihood of the diagnosis of microphthalmos with cyst.  If it were a hemangioma, excision would be challenging but could be done.  However, the chance of this being a hemangioma is so slight that speculation is not fruitful.