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This 8-year-old girl presented with a history that on two occasions the right eye "popped out" between the lids. Description by the family indicated that the lids were actually behind the equator. The mother said she "pushed" on the eye and it "popped back" behind the lids. The child is in school, but the work is difficult for her. She has two siblings who are normal in appearance and no other blood relative looks like the patient. Visual acuity is 20/20 in each eye and refractive error is +0.50 in each eye. The media are clear and the fundus is normal in each eye.

1.	Based on the appearance of the child including features such as: exophthalmos, hypertelorism, short upper lip, hypoplasia of the maxilla with relative prognathism, and beaked nose, the most likely diagnosis is:
	d -- Crouzon syndrome This child has the typical appearance of Crouzon syndrome.  Since there are no other members of the family affected, this probably represents a new mutation.  The condition is passed on genetically as an autosomal dominant with a wide range of expression.  Apert syndrome is acroencephalosyndactyly.  Hermann-Opitz syndrome is craniostenosis, brachydctyly of the hands, and absent toes. Carpenter syndrome is acroencephalosyndactyly.  Weiss syndrome is craniostenosis and foot anomalies.
2.	This condition is caused by:
	c -- autosomal recessive inheritance This condition is caused by premature closure of the coronal suture and sometimes also by the lambdiodal suture.  This premature suture closure can be treated surgically.  It is becoming more common for these patients to be followed in a multidisciplinary clinic that brings in a variety of specialists so that the various anomalies can be approached in a coordinated manner.
3.	These patients exhibit the following:
	e -- all of the above All of the characteristics listed are seen in patients with Crouzon syndrome.  The prolapsed globe should be gently reposited.  The patient should avoid any known behavior that would contribute to this prolapse.  Probably due to optic nerve stretching, even without prolapse, optic nerve damage can be present.