Congenital oculomotor nerve synkinesis associated with fetal retinoid syndrome.
BACKGROUND
Isotretinoin (RA), used for the treatment of cystic acne, is a powerful teratogen, causing craniofacial dysmorphisms and neural tube defects. We present two patients with RA embryopathy and oculomotor nerve synkinesis.


METHODS
Retrospective review of patient records.


RESULTS
Two patients presented with third nerve synkinesis and fetal RA exposure. Both had marked elevation of the upper eyelids on adduction such that the lid fissures alternately opened and closed on gaze from side to side. Both patients showed typical dysmorphisms of RA embryopathy. The first patient had complete agenesis of the cerebellar vermix and died at 2 years. The second patient had restricted extraocular muscles in one eye and was exotropic and hypotropic.


CONCLUSIONS
Both patients demonstrated simultaneous innervation of the medial rectus and levator palpebrae muscles causing coincident lid elevation in adduction. This evidence of oculomotor nerve synkinesis is consistent with animal studies showing abnormalities in the formation of cranial nerve ganglia following fetal RA exposure.


CONCLUSIONS
RA is a powerful teratogen. These patients provide additional clinical evidence of its influence on neural migration during early development.
Morrison
David G
DG
The Children's Hospital of Alabama, Department of Ophthalmology, University of Alabama at Birmingham, Birmingham, AL 35205, USA.
Elsas
Frederick J
FJ
Descartes
Maria
M
D000152:Acne Vulgaris / Q000188:drug therapy; D000287:Administration, Topical; D000293:Adolescent; D000328:Adult; D002531:Cerebellum / Q000473:pathology; D003337:Corpus Callosum / Q000473:pathology; D005133:Eye Movements / Q000502:physiology; D005143:Eyelids / Q000294:innervation; D017809:Fatal Outcome; D005260:Female; D005315:Fetal Diseases / Q000139:chemically induced* / Q000175:diagnosis; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D015474:Isotretinoin / Q000008:administration & dosage / Q000009:adverse effects; D008279:Magnetic Resonance Imaging; D018811:Maternal Exposure / Q000009:adverse effects; D009801:Oculomotor Muscles / Q000294:innervation / Q000503:physiopathology / Q000601:surgery; D009802:Oculomotor Nerve / Q000187:drug effects / Q000196:embryology; D015840:Oculomotor Nerve Diseases / Q000150:complications / Q000151:congenital*; D011247:Pregnancy; D046608:Synkinesis / Q000150:complications / Q000151:congenital* / Q000601:surgery
D015474:Isotretinoin