Posters
Posterior polymorphous corneal dystrophy: a case series of three sibling patients with different manifestations
Poster Details
First Author: D.Aragon Roca SPAIN
Co Author(s): D. Aragón-Roca N. Sevilla X. Garrell-Salat F. Trejo-Velasco S. Banderas J. Rigo
Abstract Details
Purpose:
To describe the clinical features of three siblings diagnosed with posterior polymorphous corneal dystrophy (PPCD), a rare disorder which can be associated with several ocular manifestations.
Setting:
Glaucoma Unit and Cornea and Ocular Surface Unit. Vall d´Hebron Hospital, Barcelona, Spain.
Methods:
A case series of three sibling patients with PPCD is presented. A 41-year-old male came into the emergency room complaining of episodes of intermittent ocular pain and blurred vision in his right eye. On the slit lamp examination, he presented corectopia, microcystic corneal edema, abnormal endothelium, elevated intraocular pressure (55/20 mmHg) and bilateral broad peripheral anterior synechiae in gonioscopy. Specular microscopy revealed peripheral endothelial abnormality and broad endothelial bands bilaterally. We studied his family members. His 37-year-old sister presented endothelial vesicles and his 34-year-old brother presented posterior corneal snail tracks and undetermined structures in gonioscopy. Both had abnormal specular microscopies.
Results:
The index patient had unilateral elevated intraocular pressure which could not be controlled with topical treatment. Therefore, a trabeculectomy is planned. His siblings have a potential risk of developing stromal edema and elevated intraocular pressure, amongst others. Consequently, they do follow-up visits.
Conclusions:
We present a case series of three sibling patients with PPCD. Whereas most patients remain asymptomatic, follow-up is required because they can develop stromal edema, elevated intraocular pressure and both angle-closure and open-angle glaucoma, which can require eventually endothelial keratoplasties and filtering surgeries, respectively.
Financial Disclosure:
None
