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First Author: F.Chait Díaz SPAIN
Co Author(s): J. Keller R. Torres M. Latasiewicz J. Torras Sanvicens M. Sainz de la Maza
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To describe amyloidosis as a cause of severe neurotrophic keratitis which may lead to corneal perforation
Ophthalmology service of a tertiary hospital in Spain
Review of the case notes of two patients diagnosed of systemic amyloidosis which had severe neurotrophic keratitis. We analyzed the clinical presentation, management and evolution of each case.
A 36-year-old man with familial amyloidotic polyneuropathy (FAP) presented to our service with a week"s history of left eye redness and discharge that had not improved with topical tobramycin. Biomicroscopic examination showed a 3 x 4 mm neurotrophic ulcer with deep anterior chamber (AC). We performed a tarsoconjunctival flap and amniotic membrane implant. A 68-year-old man diagnosed of immunoglobulin light chain amyloidosis (AL) presented to our emergency service with a 6 day"s history of decreased visual acuity in his left eye. Biomicroscopic examination showed a 3x3mm perforated neurotrophic ulcer and a shallow AC. This patient was treated with a therapeutic contact lens, topical moxifloxacin and autologous serum (50% solution) due to his poor general condition that did not allow surgical intervention. Both patients had a favourable evolution with closure of the epithelial defect and reformation of the anterior chamber. There was only modest improvement in visual function.
Ocular involvement in systemic amyloidosis is a known occurrence, however, corneal neurotrophic ulceration is a very rare complication. There are a few published reports of neurotrophic ulceration in PAF and only one in AL. We believe this one to be the only case of corneal perforation associated with neurotrophic ulcer in AL reported to date. Neurotrophic ulcers result from the dysfunction of the trigeminal nerve that lead to corneal hypoesthesia or anaesthesia. Given the scarce symptomatology of neurotrophic ulcers, patients tend to present late with advanced corneal damage. Other possible causes of this disorder are herpes infection, neoplasia, diabetes or leprosy. We recommend that amyloidosis patients are questioned about ocular symptoms and promplty referred to the ophthalmologist when required. Amyloidosis should be taken into account in the differential diagnosis of neurotrophic keratitis.