Long-term follow-up of invasive ocular surface squamous cell carcinoma treated with excision, cryotherapy, and topical mitomycin C.

BACKGROUND: To evaluate the efficacy of surgical excision, cryotherapy and topical Mitomycin C (MMC) for ocular surface squamous cell carcinoma (SCC) with at least 24 months follow-up. METHODS: Seventeen patients with primary and recurrent invasive SCC of ocular surface underwent surgical excision and map biopsy of the margins, and double freeze-thaw cryotherapy of the bed and margins. Topical MMC (0.04%, 4 times daily) was commenced 7-10 days after operation. It was delivered in cycles that consisted of medication 4 times daily for 7 consecutive days followed by 7 consecutive days of no medication. Patients with primary SCC received two cycles (protocol 1) and those with recurrent SCC received three cycles (protocol 2). Patients with intra-ocular and or orbital extension or those with less than 24 months of follow-up were excluded. The frequency of tumor recurrence and complications associated with treatment were measured. RESULTS: Mean age was 70.7 years (SD = 10, range: 48-80). Mean follow-up time was 48 months (SD = 21, range: 24-89). There were 12 patients with primary SCC and five with recurrent SCC. Mean largest diameter was 9.7 mm (3-20). Surgical margins were free in 12 cases. One patient with recurrent SCC who received protocol 2 treatment developed recurrence 9 months after excision, and remained free of recurrence 24 months after second treatment. Complications comprised transient mild punctate corneal epithelial erosion (eight), irritation and conjunctival hyperemia (11), corneal scar (six), and scleral thinning (three). All except one responded well to conservative management. One scleral thinning required scleral patch graft 1 year after treatment. CONCLUSION: The combination of surgical excision, cryotherapy, and post-operative topical mitomycin-C was effective treatment for ocular surface invasive SCC in long-term follow-up.

Foveal Avascular Zone and Vessel Density in Healthy Subjects: An Optical Coherence Tomography Angiography Study.

PURPOSE: To report the normal characteristics and correlations of the foveal microvascular networks using optical coherence tomography angiography (OCTA) in a healthy Iranian population. METHODS: Enface 3x3 OCTA images were obtained using the RTVue Avanti spectral-domain optical coherence tomography with AngioVue software (Optovue, Fremont, CA, USA). Foveal avascular zone (FAZ) area, central foveal point thickness and inner retinal thickness at the foveal center and the vascular density of the superficial retinal capillary plexus (SCP) and deep retinal capillary plexus (DCP) in the fovea were recorded. RESULTS: Seventy normal eyes of 70 subjects (range, 9 to 71 years) were studied. Mean FAZ area was 0.32 ± 0.11 (range, 0.13-0.67) mm2 in SCP and 0.50 ± 0.13 (range, 0.19-0.94) mm2 in DCP. Mean SCP vessel density was 29.6 ± 4.7 (range, 16.3-40.3) % in the fovea. Mean DCP vessel density was 27.0 ± 5.9 (range, 15.0-45.2) % in the fovea. The FAZ area at SCP level was negatively correlated to the central subfield thickness (P < 0.001). The FAZ area at DCP level correlated negatively to the central subfield thickness and was significantly associated to age (both P < 0.001). The foveal SCP vessel density significantly correlated with foveal thickness and the foveal DCP vessel density correlated significantly with central foveal subfield thickness and was inversely related to age (all P < 0.05). CONCLUSION: In this study, central foveal subfield thickness was a major determinant of the FAZ size and foveal vessel density. Age was a determinant for FAZ area and whole image vessel density in DCP.

Gene mutation analysis in Iranian children with nephronophthisis: a two-center study.

INTRODUCTION: Nephronophthisis is of the most commonly inherited ciliopathies that leads to end-stage renal disease in children. The NPHP1 gene is the first identified gene responsible for nephronophthisis and related diseases. This study assessed mutations of the NPHP1 gene in 16 Iranian families with at least one member presenting features of nephronophthisis. MATERIALS AND METHODS: Fifty-seven patients diagnosed with chronic kidney disease or end-stage renal disease were referred to Imam Hossein Children Hospital, in Isfahan, Iran. The gene analysis study was carried on 16 patients and their first-degree relatives (40 DNA samples) suspicious of having nephronophthisis. The NPHP1 deletion analysis was performed for exons 5, 7, and 20 of the NPHP1 gene. RESULTS: The patients' median age was 15 years. The mean and median age of the first presentation was 10.06 ± 2.59 years and 10.5 years, respectively. A homozygous deletion was identified in the NPHP1 gene spanning at least from exon 5 to exon 20 in two families. High-throughput mutation analysis identified a homozygous truncating mutation (c.1504C>T, p.R502*) in the NPHP5 in 5 families. CONCLUSIONS: By combining NPHP1 deletion analysis with multiplex-polymerase-chain-reaction-based high-throughput mutation analysis we could identify the molecular disease-cause in 7 of 15 families from Iran. In 8 families, the molecular disease cause remained unknown.

Intravitreal erythropoietin injection for the treatment of non-arteritic anterior ischaemic optic neuropathy.

AIM: To evaluate the effect of intravitreal injection of erythropoietin for the treatment of non-arteritic anterior ischaemic optic neuropathy (NAION). METHODS: In this prospective interventional case series, 31 eyes of 31 patients with NAION were included. Patients received intravitreal injection of 2000 unit (0.2 cm³) of erythropoietin within 1 month of the onset of the disease. Visual acuity and visual field were recorded before injections and 1 week, 1 month, 3 months and 6 months after the injections. RESULTS: The mean duration of symptoms before injections was 11.2 ± 5.5 days. Six months after injections, visual acuity improved in 27 eyes (87%), and 17 eyes (54.8%) had ≥ 3 lines of visual improvement. The mean preinjection visual acuity was 1.01 ± 0.88 logMAR and 0.58 ± 0.58 logMAR (p<0.001) at last follow-up. Visual acuity improvement occurred in 61.2% of patients within the first month. It followed a biphasic pattern in which there was continuous improvement up to 3 months and then started to deteriorate, although it remained significantly better than baseline until the last follow-up. No patient lost any lines of visual acuity compared with the baseline values. The mean of mean deviations of visual field was -19.6 ± 5.7 dB at baseline and -18.6 ± 6.3 dB (p = 0.6) at last follow-up. CONCLUSIONS: Intravitreal injection of erythropoietin may be safe and effective in the treatment of NAION. The effect may last for a few months and then decline.

Clinical profile of patients with nonarteritic anterior ischemic optic neuropathy presented to a referral center from 2003 to 2008.

BACKGROUND: We conducted this study to report the demographics and clinical profile of patients with nonarteritic anterior ischemic optic neuropathy referred to a referral neuro-ophthalmology center in Iran. METHODS: During a five-year period, 107 patients with nonarteritic anterior ischemic optic neuropathy were studied. A detailed history of previous or current systemic diseases was obtained and a complete ophthalmic evaluation including best corrected visual acuity, color vision testing, and computerized perimetry was performed. RESULTS: Sixty-six men and 41 women with a mean+/-SD age of 52.7+/-10.3 (range: 30 - 80) years were studied. Most (62.2%) of the patients aged more than 50 years. Twenty-two (20.5%) patients had had an episode of nonarteritic anterior ischemic optic neuropathy in the fellow eye. Overall, 51 (47.7%) patients had no evidence of a previous or current systemic disease. Diabetes mellitus and hypertension were reported in 40.1% and 26.1% of the patients, respectively. The best corrected visual acuity was 20/200 or worse in 43 (40.1%) eyes and 20/40 or better in 43 (40.1%) eyes. The best corrected visual acuity was significantly better in nondiabetic patients (0.62+/-0.69 LogMAR) than diabetics (0.96+/-0.84, P=0.03). The visual field analysis of reliable fields (76 eyes) revealed that the mean deviation ranged from -32.6 to -1.3 dB with a mean+/-SD of -19.7+/-8.08 dB. Diffuse defect was the most prevalent defect detected on 36.8% of visual fields of the study eyes followed by inferior altitudinal defect (26.3+/-). In the fellow eyes, without any evidence of prior optic nerve problem, inferior and superior arcuate scotoma were found in 30% and 20% of the eyes, respectively. CONCLUSION: Characteristics of nonarteritic anterior ischemic optic neuropathy patients in a population of Iranian patients were similar to nonarteritic anterior ischemic optic neuropathy as described in previous studies, with the exception of higher proportion of younger patients and a higher propensity for diabetes. Visual field defects were common in clinically normal fellow eyes.

Refractive error and axial length in nonarteritic anterior ischemic optic neuropathy.

PURPOSE: The aim of this study was to evaluate the ocular axial length and refractive error as risk factors for development of nonarteritic anterior ischemic optic neuropathy (NAION). METHODS: This prospective study included 78 patients with NAION and 80 normal volunteers. Axial length and refractive error of NAION eyes were compared with the normal fellow eyes and control eyes. RESULTS: The 2 groups were frequency matched by sex and age. The mean refractive error (in spherical equivalent) was -0.07 +/- 1.60 diopters (D) in NAION eyes, 0.00 +/- 1.6 D in normal fellow eyes (P = 0.7), and -0.04 +/- 1.1 D in control eyes (P = 0.9). No significant difference was found between axial length measurements of NAION eyes (22.86 +/- 0.82 mm) and fellow eyes (22.80 +/- 0.88 mm; P = 0.8) and control eyes (23.1 +/- 0.78 mm; P = 0.1). Most of the NAION eyes (50%) were emmetropic (between +0.5 and -0.5 D). CONCLUSION: Axial length and refractive error may not be risk factors in NAION.