Ocular manifestations of oblique facial clefts.
BACKGROUND
In the Tessier classification, craniofacial clefts are numbered from 0 to 14 and extend along constant axes through the eyebrows, eyelids, maxilla, nostrils, and the lips. We studied a patient with bilateral cleft 10 associated with ocular abnormalities.


METHODS
Clinical report with orbital and cranial computed tomography.


RESULTS
After pregnancy complicated by oligohydramnios, digoxin, and lisinopril exposure, a boy was born with facial and ocular dysmorphism. Examination at age 26 months showed bilateral epibulbar dermoids, covering half the corneal surface, and unilateral morning glory anomaly of the optic nerve. Ductions of the right eye were normal, but the left eye had severely impaired ductions in all directions, left hypotropia, and esotropia. Under anesthesia, the left eye could not be rotated freely in any direction. Bilateral Tessier cleft number 10 was implicated by the presence of colobomata of the middle third of the upper eyelids and eyebrows. As the cleft continued into the hairline, there was marked anterior scalp alopecia. Computed x-ray tomography showed a left middle cranial fossa arachnoid cyst and calcification of the reflected tendon of the superior oblique muscle, trochlea, and underlying sclera, with downward and lateral globe displacement.


CONCLUSIONS
Tessier 10 clefts are very rare and usually associated with encephalocele. Bilateral 10 clefts have not been reported previously. In this case, there was coexisting unilateral morning glory anomaly and arachnoid cyst of the left middle cranial fossa but no encephalocele.


CONCLUSIONS
Bilateral Tessier facial cleft 10 may be associated with alopecia, morning glory anomaly, epibulbar dermoids, arachnoid cyst, and restrictive strabismus.
Ortube
Maria Carolina
MC
Department of Ophthalmology, Jules Stein Eye Institute, David Geffen School of Medicine at UCLA, Los Angeles, California, USA.
Dipple
Katrina
K
Setoguchi
Yoshio
Y
Kawamoto
Henry K
HK
Demer
Joseph L
JL
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging; D000505:Alopecia; D016080:Arachnoid Cysts; D002972:Cleft Palate / Q000000981:diagnostic imaging*; D019465:Craniofacial Abnormalities / Q000000981:diagnostic imaging*; D003884:Dermoid Cyst; D005124:Eye Abnormalities / Q000000981:diagnostic imaging*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D013285:Strabismus; D014057:Tomography, X-Ray Computed