Abstract
Purpose: :
To report the first case of a neurotrophic keratopathy associated with a trigeminal trophic syndrome.
Methods: :
A retrospective case study.
Results: :
A seven– year–old girl presented with a two–week history of redness and decreased vision in her left eye. Her prior medical history was significant for resection of posterior fossa ganglioglioma. On examination she was found to have a large epithelial defect in the left cornea. Her corneal sensation was decreased in the left eye due to CN V deficit related to the brain tumor resection. She was diagnosed with a neurotrophic keratopathy and treated with frequent lubrication and patching. The neurotrophic keratopathy improved gradually with treatment. Two months later, she developed non–healing deep ulcerations on the left side of her nose, and on the eyelid near the left medial canthus. The cultures of the lesions were negative. Due to progressive nature of the facial lesions and tissue destruction, she was referred to dermatology, and ultimately diagnosed with a trigeminal trophic syndrome. The syndrome, characterized by trigeminal anesthesia, facial paresthesias, and frequent destruction of ala nasi, has been reported in children infrequently. The facial ulcerations are usually self–inflicted and difficult to eradicate. Behavior modification therapy, including covering the lesions, and hand splint wear at night, resulted in a marked improvement in the appearance of the nasal and eyelid lesions over time in our patient.
Conclusions: :
To our knowledge, this is the first report of a neurotrophic keratopathy associated with the trigeminal trophic syndrome. Ophthalmologists should be aware that neurotrophic keratopathy can be associated with deep facial ulcerations, especially near ala nasi. Early recognition of the trigeminal trophic syndrome by the ophthalmologist is important in order to institute the appropriate therapy and minimize tissue destruction.
Keywords: cornea: clinical science • innervation: sensation • cornea: epithelium