Bilateral nanophthalmos and pigmentary retinal dystrophy--an unusual syndrome.
BACKGROUND
To report the clinical picture of the rare association of nanophthalmos and pigmentary retinal dystrophy and its cataract surgery outcome.


METHODS
We report a case of a 60-year-old female who presented with bilateral slowly progressive visual loss.


RESULTS
The patient presented with bilateral light perception visual acuity, exotropia, brunescent cataract hindering fundus examination and hypodontia. Ultrasonography revealed bilateral nanophthalmos. A visual-evoked potential was also performed preoperatively. Cataract surgery with +40D IOL implantation was uneventful. Postoperative fundus examination revealed pigmentary retinal dystrophy, confirmed by electrophysiologic tests. Glycosaminoglycan urinary excretion was normal.


CONCLUSIONS
Congenital bilateral nanophthalmos may rarely be associated with pigmentary retinal dystrophy. We suggest thorough preoperative evaluation in nanophthalmic eyes for the exclusion of significant features concerning visual prognosis.
Proença
Helena
H
Department of Ophthalmology, University of Lisbon, Lisbon, Portugal. helenproenca@hotmail.com
Castanheira-Dinis
A
A
Monteiro-Grillo
M
M
D005074:Evoked Potentials, Visual; D005260:Female; D007839:Functional Laterality; D006801:Humans; D008850:Microphthalmos / Q000150:complications* / Q000000981:diagnostic imaging; D008875:Middle Aged; D012174:Retinitis Pigmentosa / Q000150:complications* / Q000175:diagnosis; D013577:Syndrome; D014463:Ultrasonography; D014792:Visual Acuity