Ian R. Gorovoy

Donor tissue characteristics in preparation of DMEK grafts.

Purpose: The aim of the study was to determine donor tissue characteristics that contribute to longer peeling times or tissue tears during peeling during Descemet membrane endothelial keratoplasty (DMEK) graft preparation. Methods: The data analyzed included donor age, gender, postmortem tissue age, contralateral eye data, peel time, and peel complications of 116 consecutive DMEK donor tissues, which were prepared by a single surgeon with the submerged cornea using backgrounds away (SCUBA) technique. Results: The mean peel time was 10.1 ± 3.3 minutes (range, 6–24 minutes). Tears were noted in 7 cases (6%) and occurred more commonly with longer peel times. Three of the torn grafts had either peripheral tears or small, noncentral buttonholes or horseshoe tears and were successfully transplanted, for a transplant rate of 97%. However, the host stroma in these cases took longer to clear postoperatively. The donor gender and postmortem day of peeling did not have any statistical significance for the peeling time or tear rate (P > 0.05). Interestingly, tissue from donors older than 65 years had slightly faster peel times, but this difference did not reach statistical significance (⩽65 = 11.0 ± 4 minutes; >65 = 9.3 ± 3.1 minutes; P = 0.07) and had equal rates of graft tears (6%). Most importantly, however, there was a strong correlation between peel times for the 2 eyes from the same donor (r = 0.78, P = 0.03), and the risk of a tear during peeling in the second eye was much greater if the first graft also had a tear during preparation (odds ratio = 7.2, P = 0.02). Conclusions: Exclusion of the contralateral eye tissue when there was difficulty in graft peeling for the first eye may prove to be a unique quality of DMEK donor tissue.

Fungal rhinosinusitis and imaging modalities

This report provides an overview of fungal rhinosinusitis with a particular focus on acute fulminant invasive fungal sinusitis (AFIFS). Imaging modalities and findings that aid in diagnosis and surgical planning are reviewed with a pathophysiologic focus. In addition, the differential diagnosis based on imaging suggestive of AFIFS is considered.

Descemet stripping automated endothelial keratoplasty for spontaneous descemet membrane detachment in a patient with osteogenesis imperfecta.

Purpose: To report the first use of Descemet stripping automated endothelial keratoplasty (DSAEK) for spontaneous Descemet membrane detachment in a patient with osteogenesis imperfecta (OI), keratoconus, and acute bullous keratopathy. Methods: A case report of a 25-year-old man with OI and symptomatic unilateral bullous keratopathy secondary to spontaneous Descemet membrane detachment is described. The patient presented with acute loss of vision in his right eye and was noted on slit-lamp examination to have a totally detached and taut Descemet membrane, spanning the anterior chamber. This diagnosis was confirmed with an anterior segment OCT (Visante; Carl Zeiss Meditec, Dublin, CA). Results: After an attempt to reattach the Descemet membrane with air bubbling was unsuccessful, the patient underwent successful DSAEK. Seven months postoperatively, his best spectacle–corrected visual acuity was 20/50 compared with his other eye, which was 20/40. There were some central folds in the donor noted immediately postoperatively that have persisted. Conclusions: To the best of our knowledge, this case is the first documented spontaneous Descemet membrane detachment in a patient with OI and probable keratoconus treated successfully with DSAEK. Unlike in classical keratoconus hydrops, medical management is unlikely to resolve a total Descemet membrane detachment. This unusual Descemet membrane detachment was tightly stretched across the anterior chamber without any obvious tear, making simple air or gas bubble apposition impossible. Attempts to tear the Descemet membrane and then bubble led to significant folds. DSAEK was successful in removing the detached Descemet membrane and replacing it with the donor tissue.

Spontaneous Regression of a Massive Sporadic Chiasmal Optic Pathway Glioma

Optic pathway gliomas have highly variable and unpredictable natural histories, including spontaneous regression, making management decisions difficult and controversial. We report a case of substantial spontaneous regression of an optic pathway glioma involving the chiasm in a 15-month-old boy. Our case is particularly striking given the massive size at presentation with chiasmal, bilateral optic tract, and midbrain involvement, along with clinical ophthalmologic signs for which the majority of practitioners may have recommended treatment on presentation.

Loss of contrast enhancement of the inferior rectus muscle on magnetic resonance imaging in acute fulminant invasive fungal sinusitis

Importance: This report describes a new magnetic resonance finding in acute fulminant invasive fungal sinusitis that may aid in the diagnosis of orbital involvement. Observations: The charts of two patients who presented with diabetic ketoacidosis and suspected acute fulminant invasive fungal sinusitis were retrospectively examined. Medical and surgical records, imaging studies, and histopathology specimens were reviewed. The two patients, a 58-year old female and 54-year-old male each had a contrast-enhanced magnetic resonance of the paranasal sinuses and orbits. Acute fulminant invasive fungal sinusitis with suspected orbital involvement was associated with focal loss of contrast enhancement of the inferior rectus muscle and adjacent orbital tissues. Fungal involvement of the inferior rectus muscle was confirmed on pathologic evaluation in both patients after exenteration. Conclusions and relevance: The finding of loss of contrast enhancement of an extraocular muscle on magnetic resonance may increase diagnostic confidence of orbital involvement in the setting of acute fulminant invasive fungal sinusitis and help guide surgical planning.

Chronic keratitis with intrastromal epithelioid histiocytes: a new finding in Muckle-Wells syndrome.

Purpose: To report a new corneal finding in Muckle–Wells syndrome (MWS) and its subsequent treatment. Methods: The medical chart of a 45-year-old woman with a history of MWS was reviewed. She presented with progressive bilateral stromal edema and scarring that had been worsening over the past year. The left eye, which had more severe stromal scarring and a best spectacle-corrected visual acuity (BSCVA) of hand motion, had a history of cataract removal with lens implantation, argon laser trabeculoplasty, and trabeculectomy 2 years previously for open-angle glaucoma. The right eye had also undergone argon laser trabeculoplasty and trabeculectomy and had a BSCVA of 20/100. Results: The patient underwent penetrating keratoplasty in the more severely affected left eye. Nine months postoperatively the patient had a BSCVA of 20/40 in the eye that had undergone transplantation. The pathologic specimen demonstrated a chronic keratitis with focal calcification of Bowman layer, localized Descemet membrane breaks, marked endothelial loss, and pronounced intrastromal epithelioid histiocytes without evidence of amyloidosis. Conclusions: To the best of our knowledge, this case highlights a new association with MWS. Epithelioid histiocytes have been demonstrated in other parts of the body in patients with MWS including the skin and joints, which is sometimes accompanied by amyloidosis.

Cystoid macular edema in retinitis pigmentosa patients without associated macular thickening.

Abstract Purpose: To describe the occurrence of cystoid macular edema (CME) in the setting of central foveal thickness (CFT) under 250 μm as measured by optical coherence tomography (OCT) in patients with retinitis pigmentosa (RP). Methods: Stratus OCT was used to measure CFT in a total of 90 eyes from 46 patients with RP. Cross-sectional OCT images were also evaluated for CME, which was defined as cystoid changes in the macula seen on at least two linear scans. Results: CME was identified in 13 of the 46 patients or in 22 of 90 eyes by OCT. In eyes with macular edema, CFT ranged from 224 to 718 μm (mean = 339 ± 137 μm). In eyes without macular edema, CFT ranged from 99 to 273 μm (mean = 184 ± 40 μm). Bilateral CME occurred in 9 of 13 patients (69%). CFT was considered “normal” in 7 of the 22 eyes (32%) with CME. Two patients had bilateral CME with normal CFTs, under 250 μm. Conclusion: We demonstrate the occurrence of CME in RP patients without associated thickening, which has not been described. This concept likely is applicable to other diseases with retinal thinning.

A potential life-saving diagnosis—recognizing Turcot syndrome

A previously healthy 9-year-old girl presented with ataxia, headaches, and nausea of 1 months duration. Magnetic resonance imaging demonstrated a large posterior fossa mass. Posterior segment examination revealed pigmented ocular fundus lesions (POFLs), which included cometoid dark lesions with depigmented tails and smaller, dark midperipheral lesions. The patient underwent resection for a medulloblastoma. Because of these specific retinal lesions in combination with her medullobastoma, a diagnosis of Turcot syndrome was made and subsequently confirmed by genetic testing. Turcot syndrome is one of the familial adenomatous polyposis (FAP) syndromes. This diagnosis may be life-saving because 100% of FAP patients develop colon cancer that can be cured only early with timely colectomy.

Advances in ophthalmic monitoring for hydroxychloroquine toxicity.

A59-year-old woman with a history of sarcoidosis on hydroxychloroquine 200 mg twice daily for 15 years presented for routine hydroxychloroquine ophthalmologic monitoring. She denied visual complaints with normal best corrected visual acuity of 20/20 and normal central automated visual fields in both eyes. However, fundus autofluorescence revealed a classic bilateral bull’s-eye maculopathy (Fig. 1). A control autofluorescence is also shown for comparison (Fig. 2). Given these findings, the patient discontinued her hydroxychloroquine to prevent further retinal damage. Currently, the American Academy of Ophthalmology and the American College of Rheumatology recommend that patients on hydroxychloroquine undergo baseline and annual screening beginning after 5 years of treatment, unless high risk factors are present. Screening consists of 10-2 automated visual fields, which tests the visual function of the macula as this is the central region at risk for hydroxychloroquine toxicity, in addition to spectral domain optical coherence tomography (SD-OCT), multifocal electroretinogram (mfERG), or fundus autofluorescence. Spectral domain optical coherence tomography and fundus autofluorescence are photographic methods that demonstrate cross-sectional images of the retina and a topographic map of the distribution of lipofuscin and other pigments in the retina, respectively. Multifocal electroretinogram measures the bioelectric potential of the different parts of the retina when stimulated by light. The approximate expense and time for these tests are listed in Table 1. Toxicity in the first 5 years of hydroxychloroquine use is exceedingly rare unless patients have risk factors, which include high daily dosage, renal or liver disease, older than 60 years, or previous retinal disease. Newer techniques such as autofluorescence better detect toxicity than dilated fundus examinations, which is critical because visible findings occur late and may progress even after drug cessation. Of these additional options, further work is needed to identify the ideal modality. Fundus autofluorescence may prove to be superior because it is easier to interpret the image compared with SDOCT, where subtle loss of the photoreceptor inner segment/ outer segment of the perifoveal retina is observed in hydroxychloroquine toxicity. In addition, the technology is more widespread and faster to use compared with mfERG. Although hydroxychloroquine toxicity is likely rare, recent work suggests it may be underrecognized. Symptoms include decreased night vision, decreased central vision, loss of color discrimination, metamorphopsia, and photopsias. A recent study of 4000 patients found a prevalence of 6.8 per 1000 patients. Daily doses of greater than 6.5 mg/kg (ideal body weight) place patients at increased risk, but lower daily doses for many years FIGURE 1. Bulls’-eye maculopathy (arrow) visible on fundus autofluorescence. Right eye.

Fundus Autofluorescence in Hydroxychloroquine Toxicity

ing populations: implication of the Halometer DG test for assessment of intraocular light scatter. Appl Ergon. 2009;40(3):545-553. 22. Insurance Institute for Highway Safety (IIHS), Highway Loss Data Institute. Fatality facts 2010: older people. IIHS website. http://www.iihs.org/research/fatality .aspx?topicName=Olderpeopley 2007. 26. Rein DB, Zhang P, Wirth KE, et al. The economic burden of major adult visual disorders in the United States. Arch Ophthalmol. 2006;124(12):1754-1760. 27. Frick KD, Gower EW, Kempen JH, Wolff JL. Economic impact of visual impairment and blindness in the United States. Arch Ophthalmol. 2007;125(4):544550. 28. Wood JM. Aging, driving and vision. Clin Exp Optom. 2002;85(4):214-220. 29. Haymes SA, Leblanc RP, Nicolela MT, Chiasson LA, Chauhan BC. Risk of falls and motor vehicle collisions in glaucoma. Invest Ophthalmol Vis Sci. 2007; 48(3):1149-1155.