Neurotrophic keratitis in a patient with disseminated lymphangiomatosis

Jared E Knickelbein^1,2, Susan T Stefko¹, Puwat Charukamnoetkanok¹

¹Department of Ophthalmology, ²Medical Scientist Training Program, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA

Introduction: Neurotrophic keratitis, a degenerative corneal disease caused by trigeminal nerve impairment, has many etiologies and remains very difficult to treat.

Methods: Case report of a 23-year-old male with a right corneal ulcer that failed to improve despite broad-spectrum antimicrobials.

Results: Prior diagnosis of disseminated lymphangiomatosis with a lesion in the right petrous apex effacing Meckel’s (trigeminal) cave in conjunction with a history of nonhealing corneal abrasions suggested a neurotrophic etiology. Drawstring temporary tarsorrhaphy, in addition to antibiotics and autologous serum, lead to successful clearing of the infection and resolution of the corneal ulcer. Visual acuity improved from light perception (LP) at the peak of infection to 20/40 six weeks after treatment.

Conclusions: To our knowledge, we report the first case of neurotrophic keratitis in a patient with disseminated lymphangiomatosis that caused a mass effect in Meckel’s (trigeminal) cave leading to compression of the trigeminal nerve. The antibiotic-resistant corneal ulcer was successfully treated with drawstring tarsorrhaphy, confirming the utility of this therapeutic measure in treating neurotrophic keratitis.

Keywords: neurotrophic keratitis, corneal abrasion, drawstring tarsorrhaphy, disseminated lymphangiomatosis