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A 61-year-old man with cystoid macular edema and chorioretinal folds after cataract surgery
Digital Journal of Ophthalmology 2017
Volume 23, Number 3
August 29, 2017
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Andrew Lee, MD | Department of Ophthalmology and Visual Neuroscience, University of Minnesota
Sandra R. Montezuma, MD | Department of Ophthalmology and Visual Neuroscience, University of Minnesota
Diagnosis and Discussion
In our patient, the finding of chorioretinal folds prompted further evaluation of the differential diagnosis. His cataract surgeries were performed outside the hospital; hence in our initial evaluation we had no preoperative records. The patient had CME but did not report a history of diabetic retinopathy or DME. The patient’s high hyperopia was discovered after operative and preoperative records became available. Ancillary testing revealed a short axial length and an increased retinal-choroidal-scleral thickness (Figure 4), resulting in a diagnosis of nanophthalmos.

Nanophthalmos is a rare condition; a preliminary diagnosis can be made with a measurement of axial length <21 mm.(6) Presentation is normally bilateral, and patients can have slight enophthalmos and narrow palpebral fissures.(7) Other characteristics include severe hyperopia, increased retinal-choroidal-scleral thickness, a crowded anterior chamber, and chorioretinal folds.(5,6,8,9) The crowded anterior chamber of nanophthalmos is of particular concern, because it predisposes the patient to acute angle-closure glaucoma.(10) Additional complications arise from a thick scleral collagen, which can result in choroidal effusion, retinal detachment, and cystoid macular edema.(11)

Most cases of nanophthalmos are spontaneous, although familial types exist. Cases of autosomal recessive (AR) and dominant (AD) nanophthalmos have been attributed to mutations on chromosome 11.(7) AR nanophthalmos has been reported to involve the MFRP (membrane frizzled-related protein) gene. The MFRP protein is expressed in the retinal pigment epithelium and ciliary body and is important to later stages of ocular development.(6,7) It is thought that mutations in MFRP result in decreased ocular growth, leading to crowding and thickening of the retina and choroid, abnormal foveal development, and chorioretinal folds.(6,7,8,12) A gene TMEM98 (transmembrane protein 98) on chromosome 17 has also recently been implicated in a form of AD nanophthalmos.(13,14) Nanophthalmos can additionally be present in less common conditions such as oculo-dento-digital and Kenney Caffey syndromes.(7)

Nanophthalmos patients are at increased risk for cataract surgery complications. (2,9,15). Two recent studies have reported the complication rate of cataract surgery in nanophthalmos patients: Steijns et al reported a 27.9% complication rate (n = 43), whereas Day et al reported 15.5% complication rate (n = 103).(2,15) Surgical complications in nanophthalmos include choroidal effusion, zonular dehiscence, uveitis, malignant glaucoma, and cystoid macular edema.(2,9,15,16) It has been suggested that retinal-choroidal-scleral thickness be measured preoperatively in hyperopic eyes at risk of glaucoma to diagnose properly nanophthalmos and to allow more careful planning of surgery.(9) Preventative measures to be considered prior to cataract surgery include laser iridotomy, iridoplasty, trabeculectomy, and creation of scleral windows.(16) The use of intravenous mannitol to maintain pressure during surgery might also be considered.
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