Neurotrophic keratopathy associated with congenital agenesis of the corpus callosum

2012 
Purpose The corpus callosum (CC), with its central position in the cerebrum, has an essential role in relaying sensory, motor, and cognitive information from homologous brain regions across cerebral hemispheres. Corpus callosum agenesis (CCA) is a rare congenital malformation which is caused by an alteration in embryonic development. Methods We report the case of a 3 year old child who was diagnosed of complete corpus callosum agenesis with a normal karyotype. The neurological findings were spastic tetraparesis with dystonia, several delayed psychomotor and epilepsy. In ophthalmology examination he presented esotropia, bilateral optic nerve hypoplasia, delayed visual evoked potential flash type and altered corneal sensibility in both eyes which is manifested clinically by recurrent corneal ulcers and epithelial disruption associated with stromal edema. Results After Neurotrophic keratopathy suspected, treatment with autologous serum was decided and response to treatment was favorable in both eyes with closure of ulcers and good management of symptoms. CCA has also been described in association with other ocular abnormalities such as optic nerve hypoplasia, retinal detachments, cataracts, morning glory syndrome, iris and optic nerve colobomas, among many others. Conclusion In all patients diagnosed with agenesis of the corpus callosum, the possible alteration of corneal sensitibility is a fact that will be suspected to perform early diagnosis and appropriate treatment and follow-up.
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