Neurotrophic Keratopathy In A Patient With Chordoma Tumours. Extreme Corneal Thinning And Impending Perforation Associated With Erlotinib- Tarceva

To present a case of significant Neurotrophic Keratitis that quickly progressed to impending perforation after the initiation of Erlotinib and the management plan that was used.

Corneal Service, 2nd Ophthalmology Department, Aristotle University of Thessaloniki

A 35 year old female patient was referred to our corneal service with impending perforation from her general ophthalmologist. She complained of reduced vision in her left eye for the past 6 days. The patient suffered from chordomas/Chordomata- a slow growing malignant tumour (sarcoma) that are often located at the base of the skull and the spinal chord. She had 2 Neurosurgical procedures in the past with entry via the nasal route and through craniotomy and after her latest surgery she was told that no more surgery could be performed. A month before her clinical visit she was started on Tarceva-Erlotinib, which is  a tyrosine kinase inhibitor (TKI) binding on the tumour's epidermal growth factor receptors (EGFR). Although used for non-small cell lung carcinomas and pancreatic carcinomas, the medication appeared to halt disease progression.

Upon examination the patient had Greatly reduced Corneal sensation L more than the Right eye; Visual Acuity of 1.0 in the Right and CF Left eye and mild RAPD in the Left associated with mild Left Optic atrophy. Few superficial erosions were noted in the Right eye and extreme corneal thining on the left. After discussion with the patient's Oncologist Erlotinib was (at least temporarily) stopped),Corneal swabs taken, punctal plugs were inserted and the patient was started on oral Doxycycline and very copious lubrication during the day and Lanolin based eye ointment at night leading to slow improvement with a VA of 0.2 on the left and healing.

Chordomas/Chordomata are slow growing malignant tumours most commonly found at the base of the skull and the spinal cord. Surgical excision is the treatment of choice that prolongs survival but removal en block is often impossible due to their location. Neurotrophic Cornea is often a result of either the disease itself or associated with surgical excision.

Erlotinib-Tarceva that was shown to halt disease progression in this case probably exacerbated Neurotrophic Keratopathy and led to impending perforation. Close monitoring by an Ophthalmologist-Corneal specialist is recommended in cases of Erlotinib use to screen Neurotrophic keratopasthy progression and dicuss with the treating physician the possibility of regimen change.