Outcome of combined cataract surgery and iris prosthesis implantation in iris melanoma patients previously treated with iridocyclectomy: A national retrospective non-comparative case series.

PURPOSE: To examine complications, visual outcomes, photic patient-reported symptoms, corneal morphology, IOL tilt, and intraocular pressure after implantation of an intraocular lens (IOL) and iris prosthesis (IP) following iridocyclectomy. METHODS: Patients with previous iridocyclectomy treated with an IOL and IP at the Copenhagen University Hospital Rigshospitalet between 2007 and 2018 were included in this national retrospective non-comparative case series. The assessment encompassed BCVA, PRO questionnaire, corneal topography, and anterior segment OCT. RESULTS: 45 patients were included. Eight of 45 patients were previously treated with ruthenium-106 brachytherapy in conjunction with iridocyclectomy. Six of 45 patients developed endothelial dysfunction four of whom had received ruthenium-106 brachytherapy. Five of 45 patients had subluxation of the IOL/IP complex due to incomplete zonula apparatus. BCVA improved for all patients after lens surgery. 26 patients participated in the invited follow-up examination. 19 of 26 (73%) reported none or mild photic symptoms after IP instalment. Five (19%) reported ongoing severe photic symptoms. The corneal astigmatism significantly increased after iridocyclectomy but did not change after lens surgery. CONCLUSIONS: Implantation of an IOL and IP is a safe procedure, alleviating photic symptoms in most patients. It comes with higher risk of complications due to a more demanding procedure and larger surgical traumas from previous treatments. Ruthenium-106 brachytherapy increases the complication risk. Corneal astigmatism is induced by iridocyclectomy but does not change after lens surgery.

Isolated Ocular Sarcoidosis Mimicking Ring Melanoma.

We report the case of a 25-year-old female, who presented with a large, pale tumor of her ciliary body that extended to >180° of the anterior chamber angle. All preoperative examinations including ultrasound biomicroscopy, scleral transillumination, and MRI indicated melanoma. A thorough systemic work-up was negative. A diagnosis of ring melanoma was suspected, and the patient was scheduled for enucleation. However, perioperative frozen section indicated granulomatous inflammation. The enucleation was cancelled, and a subtenon injection of triamcinolone was administered, which resulted in the disappearance of the tumor. Together, the findings meet the criteria for the diagnosis of isolated ocular sarcoidosis. This case demonstrates that a sarcoid granuloma can mimic all clinical features of a ring melanoma. Therefore, a biopsy should be done before destructive surgery is carried out.

Comparison of refractive predictability and endothelial cell loss in femtosecond laser-assisted cataract surgery and conventional phaco surgery: prospective randomised trial with 6 months of follow-up.

OBJECTIVE: Femtosecond laser-assisted cataract surgery (FLACS) decreases the use of energy and provides a more precise capsulorhexis compared with conventional phaco surgery (CPS). The purpose of this study was to examine if the lower energy use in FLACS caused less endothelial cell loss compared with CPS and if there was a difference in refractive predictability between CPS and FLACS. METHODS AND ANALYSIS: This was a randomised controlled study of 96 patients with a 6-month follow-up comparing one eye surgery by FLACS and the contralateral eye operated by CPS (divide and conquer technique). Both eyes had intraocular aspheric lenses implanted. Uncorrected distance visual acuity, corrected distance visual acuity (CDVA), central corneal endothelial cell count and hexagonality (non-contact endothelial cell microscope) were assessed preoperatively at 40 and at 180 days postoperatively. RESULTS: The mean phaco energies were 6.55 (95% CI 5.43 to 7.66) and 9.77 (95% CI 8.55 to 10.95) U/S (p<0.0001) by FLACS and CPS, respectively. At day 40, the mean endothelial cell loss (ECL) was 344 cells/mm2 (95% CI 245 to 443) by FLACS (12.89%) and 497 cells/mm2 (95% CI 380 to 614) by CPS (18.19%) (p=0.027). At day 180, ECL was 362 cells/mm2 (95% CI 275 to 450) in FLACS (13.56%) and 465 cells/mm2 (95% CI 377 to 554) in CPS (17.03%) (p=0.036).The mean absolute difference from the attempted refraction was 0.43 (95% CI 0.36 to 0.51) dioptres (D) at day 40 and 0.46 D (95% CI 0.39 to 0.53) at day 180 by FLACS compared with 0.43 D (95% CI 0.36 to 0.51) at day 40 (p=0.95) and 0.46 D (95% CI 0.37 to 0.52) at day 180 (p=0.91) with CPS. CONCLUSION: ECL was significantly lower in FLACS compared with CPS at both day 40 and day 180. ECL was correlated to the energy used. We found no difference in refractive predictability or CDVA between the groups.

Orbital precursor B-lymphoblastic lymphoma involving the extraocular muscles in a 56-year-old male and a review of the literature.

The aim of the present study was to describe a rare case of orbital precursor B-lymphoblastic lymphoma (B-LBL) in an adult. A 56-year-old male in complete remission of a gastric precursor B-LBL was referred to our orbital clinic due to rapid development of left-sided painless periorbital swelling, diplopia, and proptosis. Complete ophthalmoplegia was observed. Notably, magnetic resonance imaging showed swelling of the medial and inferior rectus muscles in the left orbit and biopsies were performed. Following histological diagnosis of precursor B-LBL, the patient was treated with radiotherapy (2Gy × 20) and chemotherapy according to the NOPHO ALL 2008 protocol. The disease progressed and the patient succumbed after 5 months. Histomorphologically, a lymphoblastic infiltrate was observed within the skeletal muscle tissue. The tumor cells were small and immature, and stained strongly for cluster of differentiating (CD)10, CD79a, paired box 5 and B cell lymphoma-2. The Ki-67 proliferative index was 90%. Multiplex ligation-dependent probe amplification and array comparative genomic hybridization detected whole chromosomal gain of X and 12, and both hemizygous and homozygous deletion on 9p comprising cyclin dependent kinase inhibitor 2A/B. Furthermore, array comparative genomic hybridization detected copy number imbalances consisting of focal or smaller deletions on chromosomes 1, 9, 10, 11 and 20. The final diagnosis was precursor B-LBL relapse in the extraocular muscles. Orbital precursor B-LBL is extremely rare in adults, and the diagnosis may be challenging to make. It is recommended to obtain material for cytogenetic and molecular analyses.

Outcome after repair of concurrent upper and lower canalicular lacerations.

PURPOSE: To investigate the functional and cosmetic results after primary surgical repair of bicanalicular lacerations. METHODS: We identified patients diagnosed with bicanalicular lacerations at the Eye Clinic of Rigshospitalet, Copenhagen from 2000 through 2009. Patient charts were reviewed and a telephone interview was conducted according to a standard questionnaire, regarding tearing and cosmesis. Symptoms (no epiphora, epiphora only when outdoors, epiphora when indoors, or epiphora with discharge) were compared to the healthy eye and scarring was graded from 0 to 10 by the patient (none to very disturbing). RESULTS: Fifteen patients had bicanalicular lacerations. Canalicular silicone stents were used in six different ways: two monostents to the lacrimal sac (n = 1), one monostent to the nose in the upper canaliculus (n = 2), two monostents to the nose (n = 2), bicanalicular stent to the nose (n = 7), bicanalicular anular stent (n = 2), bicanalicular stent and dacryocystorhinostomy (n = 1). The surgery was carried out by 6 different surgeons. Eleven patients could be followed up. In these, epiphora was reported after primary surgery by 7 patients, and scarring was graded to more than 1 in 4 patients. However, it is noteworthy that 3 of 4 patients operated with monostents had no symptoms; the fourth patient reported having epiphora indoors. Furthermore, the four patients operated with monostents graded their scaring to be 0 (n = 3) or 1 (n = 1) only. CONCLUSION: Sequelae are common after bicanalicular lacerations. The use of one or two monostents to the nose appears to be a reasonable alternative to the traditional use of one bicanalicular stent.

Effect of glial cell line-derived neurotrophic factor on retinal function after experimental branch retinal vein occlusion.

PURPOSE: The objective of the study was to investigate the effect of glial cell line-derived neurotrophic factor (GDNF) on the multifocal electroretinogram (mfERG) following an induced branch retinal vein occlusion (BRVO) in pigs. METHODS: Electrophysiological examination of the retina was performed in 20 pigs with standard and four-frame mfERG 4 weeks after induced BRVO and intravitreal injection of GDNF or vehicle. BRVO was induced by intraocular diathermia of the superior retinal vein. Inner retinal function was measured by analysis of the four-frame mfERG (iN1) and outer retinal function with standard mfERG (P1). RESULTS: In GDNF-treated BRVO eyes, P1 and iN1 amplitudes (P = 0.51 and 0.78) or implicit times (P = 0.08 and 0.99) did not differ from those in healthy fellow eyes. After vehicle injection, P1 and iN1 amplitudes of BRVO eyes were significantly lower than in the healthy fellow eye (P = 0.022 and 0.013). The log ratios of mfERG amplitudes between experimental and healthy fellow eyes were calculated (BRVO/healthy). GDNF improved the ratios of the four-frame mfERG (1.29 [0.88-1.88]) compared with vehicle (0.32 [0.21-0.50], P < 0.001). Equally, GDNF improved the ratios of the standard mfERG; GDNF (0.75 [0.51-1.10]) and vehicle (0.42 [0.27-0.63], P = 0.048). CONCLUSIONS: GDNF appears neuroprotective on retinal electrophysiological function after BRVO. The efficacy and safety of GDNF remain to be investigated in primate eyes.

Toxicity profiles of subretinal indocyanine green, Brilliant Blue G, and triamcinolone acetonide: a comparative study.

BACKGROUND: This study introduces a novel porcine model to examine the histopathological and electrophysiological consequences of retinotoxicity exerted by dyes commonly used for internal limiting membrane (ILM) staining. METHODS: Indocyanine green (ICG) 0.5 mg/ml, Brilliant Blue G (BBG) 0.25 mg/ml and triamcinolone acetonide (TA) 13 mg/ml was injected subretinally in 12 vitrectomized pig eyes. At 6 weeks, retinas were examined by multifocal electroretinography (mfERG), ophthalmoscopy, fluorescein angiograpy, histopathology, and apoptosis assay. RESULTS: mfERG responses were significantly lower in ICG-injected eyes than in healthy fellow eyes (p = 0.039). The ratio between injected eyes and healthy fellow eyes was lower in the ICG group than in the BBG (p = 0.009) and TA group (p = 0.025). No difference between BBG and TA existed. All retinas were reattached, and fluorescein angiographies showed a window defect corresponding to the injected areas but no blood-retina barrier break-down. Histopathology confirmed damage to the outer retina after ICG, but not after BBG and TA. No apoptosis was found at 6 weeks. CONCLUSIONS: Subretinal ICG induces histological and functional damage to the retina, suggesting that ICG should be used with caution in macular hole surgery, where subretinal migration can occur. In contrast, BBG and TA appear safe after subretinal injection.

The effect of subretinal viscoelastics on the porcine retinal function.

The functional consequence of long-term retinal detachment in the porcine model is examined by multifocal electroretinography (mfERG). Retinal detachment (RD) in humans leaves permanent visual impairment, despite anatomical successful reattachment surgery. To improve treatment, adjuvant pharmaceutical therapy is needed, and can only be tested in a suitable animal model. The porcine model is promising and the mfERG is well validated in this model. RD was induced in 18 pigs by vitrectomy and healon injection of various concentrations. Preoperatively and 6 weeks postoperatively eight animals were examined by mfERG. The major component P1 was analyzed statistically. Indirect ophthalmoscopy and bilateral color fundus photography (FP) were performed. Selected animals underwent high-resolution optical coherence tomography (OCT). Examination by ophthalmoscopy and FP showed that the RDs remained detached for the 6 weeks of follow-up. The P1 amplitude of the mfERG did not differ significantly between the detached areas, the surrounding attached areas, and the healthy eye (p = 0.25). Similarly, P1 implicit time did not differ between the areas (p = 0.85). The lack of functional consequences of long-term RD makes the porcine model unsuitable for examining adjuvant pharmaceutical RD treatment. Future studies should focus on foveated primates.

Electrophysiological consequences of experimental branch retinal vein occlusion in pigs and the effect of dorzolamide.

PURPOSE: To study the electrophysiological consequences of experimental branch retinal vein occlusion (BRVO) in pigs and the effect of dorzolamide. METHODS: BRVO was induced in 16 pigs by diathermia. At 4 weeks animals were examined with multifocal electroretinography (mfERG) before and after dorzolamide or vehicle. The direct component P1 (outer retina) and indirect component iN1 (inner retina) were analyzed. Ophthalmoscopy, fundus photography, and fluorescence angiography were performed. RESULTS: BRVO eyes displayed signs of retinal damage and ischemia on ophthalmoscopy, fundus photography, and fluorescence angiography. mfERGs were affected by surgery; amplitude ratios (BRVO/healthy) were less than one (P1 = 0.30 [0.20-0.45]; iN1 = 0.35 [0.23-0.54]), and implicit time ratios were above one (1.04 [1.03-1.06] and 1.03 [1.02-1.05)]. In healthy eyes, iN1 amplitudes after treatment normalized to baseline (after/before) were lower in dorzolamide-treated animals than in the vehicle group (P = 0.05). After dorzolamide iN1 amplitude ratios (BRVO/healthy) were significantly higher than after vehicle (P = 0.01) and were not significantly different from one (0.97 [0.74-1.26]), indicating that the iN1 amplitudes in BRVO eyes were not different from those in healthy eyes after dorzolamide. CONCLUSIONS: BRVO in pig eyes examined by mfERG is a promising model for testing new treatment strategies in retinal ischemia. The local effects of BRVO are detectable on the mfERG and can be altered by dorzolamide. The decreased iN1 amplitudes caused by dorzolamide in healthy eyes were not seen in BRVO eyes possibly because of an increase in preretinal oxygen tension and improved function of the ischemic retina counteracting the effect of inner retinal acidification.

Fuel utilization in subjects with carnitine palmitoyltransferase 2 gene mutations.

Patients with the myopathic form of carnitine palmitoyltransferase II (CPT II) deficiency typically experience muscle pain, cramps, and myoglobinuria during prolonged exercise. It has been suggested that carriers of CPT2 gene mutations also may have milder clinical symptoms, but fatty acid oxidation (FAO) has never been investigated in vivo in this group. We studied fuel utilization by indirect calorimetry and stable isotope methodology in four patients with CPT II deficiency, three subjects who carried one CPT2 gene mutation, and five healthy control subjects. Cycle exercise at a constant workload of 50% of maximal oxygen uptake capacity was used to facilitate FAO. We found that in vivo oxidation of long-chain fatty acids was normal at rest but severely impaired during prolonged, low-intensity exercise in patients with CPT II deficiency, and that two of the single CPT2 gene mutation carriers, who displayed symptoms of CPT II deficiency, had an FAO comparable with the patients. These results indicate that residual CPT II activity is sufficient to maintain long-chain FAO at rest in CPT II deficiency but not to increase FAO during exercise. The findings also suggest that single CPT2 gene mutations may exert a dominant-negative effect on the tetrameric CPT II protein.

Effect of diet on exercise tolerance in carnitine palmitoyltransferase II deficiency.

It is generally believed that a diet high in carbohydrate improves exercise tolerance in patients with carnitine palmitoyltransferase II (CPT II) deficiency, but it has never been systematically investigated. The authors investigated the effect of a high- vs low-carbohydrate diet on exercise tolerance in four patients with CPT II, who cycled at a constant workload of 50% of VO2max. Exercise tolerance, assessed by exercise duration and perceived exertion, improved on the carbohydrate-rich diet.