What is the role of neurofibromatosis in the etiology of glaucoma?

Updated: Jun 25, 2020
  • Author: Andrew A Dahl, MD, FACS; Chief Editor: Hampton Roy, Sr, MD  more...
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Answer

Neurofibromatosis is a common autosomal dominant systemic disorder divided into types 1 and 2 whose ocular and systemic findings are well documented. See Neurofibromatosis-1.

Ocular findings include enlargement of the eye and cornea, heterochromia, ectropion uveae, and Lisch nodules.

Glaucoma usually is unilateral and associated with eyelid thickening and contour abnormalities.

Different mechanisms for elevated intraocular pressure have been proposed, as follows: (1) direct infiltration of the angle tissue, (2) secondary angle closure due to ciliary body and choroid thickening, (3) fibrovascular membrane formation over the angle, and (4) anterior chamber dysgenesis.

Extensive medical therapy is tried before the surgical approach, which must be tailored to the cause and often is unsatisfactory. Goniosurgery, filtration surgery with or without cytotoxic agents, and cyclodestructive surgeries have been tried without significant success.


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