Small Incision Lenticule Extraction in Myopic Eyes With Corectopia After Intraocular Lens Implantation.

ABSTRACT: We present a case of ametropia corrected by small incision lenticule extraction (SMILE) in a corectopia eye after cataract surgery. The patient's optical zone, nomogram, and centration were carefully considered. The intended corrections were -6.60 - 1.25 × 175 OD and -6.85 - 0.50 × 10 OS. M-sized and S-sized cones were recorded in the right and left eye, respectively. The diameter of the right eye optical zone was 7.00 mm, whereas that of the left eye was 6.50 mm. The corneal vertex was determined using the film mark method as the scanning center. Three months after surgery, the uncorrected distance visual acuities were 20/20 OD and 20/13 OS. The effective optical zone was 6.5 and 5.5 mm in the right and left eyes, respectively. It is feasible to correct ametropia with SMILE in patients with corectopia after intraocular lens implantation. The large optical zone will compensate for the visual disturbance.

Automated Pupillometry as an Adjunct to Clinical Examination in Patients With Acute Vision Loss.

BACKGROUND: Despite automated pupillometry's (AP) improved detection of relative afferent pupillary defects (RAPDs) compared with the Swinging Flashlight Test (SFT), AP remains uncommon in clinical practice. This study examined barriers to routine use of AP in evaluation of acute vision loss. METHODS: (1) Ophthalmologists and optometrists' perceptions of AP were captured via electronic survey. (2) Ophthalmologists were presented with clinical vignettes to assess their use of AP in clinical decision-making. (3) Patients presenting with decreased vision to an ophthalmology urgent care clinic underwent manual SFT and AP screening to evaluate ophthalmologists' perceptions of the device. RESULTS: Surveys indicated that clinicians were "neutral" to "somewhat likely" to use AP. In clinical vignettes, more physicians proceeded with workup for optic nerve pathology when presented with an RAPD by AP than SFT (77% vs 26%, P = 0.003). When SFT and AP results were discordant, more physicians proceeded with workup for optic nerve disease when AP was positive and SFT was negative than vice versa (61% vs 18%, P = 0.008). In the clinical study of 21 patients, 50% of RAPDs detected by AP were not detected by SFT, although ophthalmologists rated AP's usefulness as only "neutral" to "somewhat useful." CONCLUSION: Clinicians value pupillary examination and trust AP over SFT; however, widespread adoption and perceived value of AP may depend on its impact on clinical outcomes. Within a comprehensive diagnostic device, AP may be an important tool, but is not necessary to screen for optic nerve disease or evaluate acute vision loss.

The Aravind Pseudoexfoliation Study: Surgical and First-Year Postoperative Results in Eyes without Phacodonesis and Nonmiotic Pupils.

PURPOSE: To compare intraoperative complication rates, 1-year visual outcomes, and postoperative complication rates over the first postoperative year in eyes with and without pseudoexfoliation undergoing cataract surgery. DESIGN: Prospective, comparative, interventional study. PARTICIPANTS: Nine hundred thirty eyes with cataract and uncomplicated pseudoexfoliation (without phacodonesis, clinically shallow anterior chambers, or pupil size <4 mm) and 476 controls with cataract but without pseudoexfoliation recruited from 4 centers of the Aravind Eye Care System in Southern India. The 2 groups were randomized separately to receive either a single-piece acrylic intraocular lens (IOL; SA60AT; Alcon Laboratories, Fort Worth, TX) or a 3-piece acrylic IOL (MA60AS; Alcon Laboratories). The pseudoexfoliation group also was randomized to receive or not receive a capsular tension ring. METHODS: All eyes underwent phacoemulsification with IOL implantation and were followed up at 1 day, 1 month, 3 months, and 1 year after surgery. MAIN OUTCOME MEASURES: Association of pseudoexfoliation status with intraoperative complication rates, 1-year best-corrected visual acuity, and any other complications. RESULTS: Mean ages were 63.0±6.9 years and 57.9±7.3 years in the pseudoexfoliation and control groups, respectively (P < 0.001). Pseudoexfoliation patients were more likely to be men (P = 0.014), to have a nuclear opalescence grade of more than 4 (P = 0.001), and to have a pupil size of less than 6 mm (P < 0.001) when compared with controls. Intraoperative complication rates were 2.9% and 1.9% in the pseudoexfoliation and control groups, respectively (P = 0.29). One-year postoperative best-corrected visual acuity was comparable (P = 0.09). Complication rates at 1 year were 2.7% and 2.5% in the pseudoexfoliation and control groups, respectively (P = 0.82). Average endothelial cell loss was 14.7% in the pseudoexfoliation group and 12.7% in the control group at 1 year (P = 0.066) when adjusting for age and nuclear opacity. CONCLUSIONS: Pseudoexfoliation eyes without shallow anterior chamber, small pupils, or apparent zonulopathy may represent eyes with lower risks of complications. Despite smaller pupils and denser cataracts, pseudoexfoliation eyes without clinically apparent preoperative zonulopathy were not at a higher risk of intraoperative or postoperative complications or worse visual outcomes after cataract surgery.

Pathogenicity of a Human Laminin ß2 Mutation Revealed in Models of Alport Syndrome.

Pierson syndrome is a congenital nephrotic syndrome with eye and neurologic defects caused by mutations in laminin ß2 (LAMB2), a major component of the glomerular basement membrane (GBM). Pathogenic missense mutations in human LAMB2 cluster in or near the laminin amino-terminal (LN) domain, a domain required for extracellular polymerization of laminin trimers and basement membrane scaffolding. Here, we investigated an LN domain missense mutation, LAMB2-S80R, which was discovered in a patient with Pierson syndrome and unusually late onset of proteinuria. Biochemical data indicated that this mutation impairs laminin polymerization, which we hypothesized to be the cause of the patient's nephrotic syndrome. Testing this hypothesis in genetically altered mice showed that the corresponding amino acid change (LAMB2-S83R) alone is not pathogenic. However, expression of LAMB2-S83R significantly increased the rate of progression to kidney failure in a Col4a3-/- mouse model of autosomal recessive Alport syndrome and increased proteinuria in Col4a5+/- females that exhibit a mild form of X-linked Alport syndrome due to mosaic deposition of collagen α3α4α5(IV) in the GBM. Collectively, these data show the pathogenicity of LAMB2-S80R and provide the first evidence of genetic modification of Alport phenotypes by variation in another GBM component. This finding could help explain the wide range of Alport syndrome onset and severity observed in patients with Alport syndrome, even for family members who share the same COL4 mutation. Our results also show the complexities of using model organisms to investigate genetic variants suspected of being pathogenic in humans.

Laminin-521 Protein Therapy for Glomerular Basement Membrane and Podocyte Abnormalities in a Model of Pierson Syndrome.

Background Laminin α5ß2γ1 (LM-521) is a major component of the GBM. Mutations in LAMB2 that prevent LM-521 synthesis and/or secretion cause Pierson syndrome, a rare congenital nephrotic syndrome with diffuse mesangial sclerosis and ocular and neurologic defects. Because the GBM is uniquely accessible to plasma, which permeates endothelial cell fenestrae, we hypothesized that intravenous delivery of LM-521 could replace the missing LM-521 in the GBM of Lamb2 mutant mice and restore glomerular permselectivity.Methods We injected human LM-521 (hLM-521), a macromolecule of approximately 800 kD, into the retro-orbital sinus of Lamb2-/- pups daily. Deposition of hLM-521 into the GBM was investigated by fluorescence microscopy. We assayed the effects of hLM-521 on glomerular permselectivity by urinalysis and the effects on podocytes by desmin immunostaining and ultrastructural analysis of podocyte architecture.Results Injected hLM-521 rapidly and stably accumulated in the GBM of all glomeruli. Super-resolution imaging showed that hLM-521 accumulated in the correct orientation in the GBM, primarily on the endothelial aspect. Treatment with hLM-521 greatly reduced the expression of the podocyte injury marker desmin and attenuated the foot process effacement observed in untreated pups. Moreover, treatment with hLM-521 delayed the onset of proteinuria but did not prevent nephrotic syndrome, perhaps due to its absence from the podocyte aspect of the GBM.Conclusions These studies show that GBM composition and function can be altered in vivovia vascular delivery of even very large proteins, which may advance therapeutic options for patients with abnormal GBM composition, whether genetic or acquired.

Is overactive bladder a nervous or bladder disorder? Autonomic imaging in patients with overactive bladder via dynamic pupillometry.

PURPOSE: To evaluate the changes in dynamic pupillometry in patients with idiopathic overactive bladder (OAB). METHODS: The study included 40 female patients with idiopathic OAB and 40 healthy female volunteers as a control group. Demographic and clinical data were recorded. Dynamic pupillometric parameters were measured with a commercially available unit (MonPack One, Metrovision, France) at baseline and on the 30th day of treatment with an antimuscarinic treatment (drug-agent) (solifenacin 5 mg daily). Initial, minimum, maximum and mean pupil diameters, the latency and duration of contraction and dilatation of the pupil, the amplitude of contraction and dilatation velocity were automatically measured and compared between the groups. RESULTS: There were no significant differences between two groups with respect to age and body mass index (p = 0.288, 0.755, respectively). The measurements of initial, minimum and mean pupil diameters were significantly lower in patients with OAB compared to healthy controls (p = 0.007, 0.002, 0.001, respectively). OAB patients had significantly longer latency of pupil dilatation, latency of pupil contraction and shorter duration of pupil contraction than control group (p = 0.028, 0.029, 0.021, respectively). After the antimuscarinic treatment, latency of pupil contraction, latency of pupil dilatation and duration of pupil contraction shortened significantly (all p < 0.001). Pupil dilatation velocity increased significantly during the treatment (p < 0.001). CONCLUSIONS: The dynamic pupillometric findings in this study imply impaired autonomic dysfunction, mostly the increased parasympathetic action, in OAB patients and the modulatory effects of antimuscarinic treatment.

Hidden mechanisms beyond the pupillary block in acute angle closure: ultrasound biomicroscopic study.

BACKGROUND: To evaluate the mechanisms of acute angle closure (AAC) other than the pupillary block using ultrasound biomicroscopy. DESIGN: Retrospective chart review. PARTICIPANTS: All patients who were diagnosed with AAC in Ramathibodi Hospital, Bangkok, Thailand, between June 2011 and February 2015 were enrolled. METHODS: Seventy-two patients who were diagnosed with AAC underwent a detailed ocular examination. The diagnosed mechanism of AAC was confirmed by UBM and ocular biometry. MAIN OUTCOME MEASURES: Primary mechanism responsible for acute angle closure. RESULTS: In 72 patients, the mean age was 62.33 ± 10.4 years, 18 (25%) patients were male and 54 (75%) patients were female. The primary mechanism of AAC was iridolenticular wrapping (crowded-angle (CR) plus anterior lens subluxation (LS)) in 49 eyes (68.1%), pupillary block (PB) in 17 (23.6%) eyes, and plateau iris (PL) in 6 (8.3%) eyes. Thirty (41.7%) out of 72 eyes without previous iridotomy before UBM examination were analyzed. The most common primary mechanism in this group was iridolenticular wrapping (20 eyes, 66.7%). There were seven (23.3%) eyes that had a pupillary block, and only three (10.0%) eyes had plateau iris syndrome as the primary mechanism of AAC. There was a single mechanism in 14 (46.7%) eyes, and there were combined mechanisms in 16 (53.3%) eyes. CONCLUSIONS: The most common mechanism contributing to AAC development in this Thai population was iridolenticular wrapping. From this study, we suggest that iridolenticular wrapping was the most common hidden mechanism beyond pupillary block among Thai patients.

Mechanism and management of angle closure in uveitis.

PURPOSE OF REVIEW: To summarize the multiple mechanisms responsible for angle closure in uveitis and to outline the management principles and treatment modalities. RECENT FINDINGS: Angle closure in uveitis is a heterogeneous disease with multiple mechanisms. Recent advances in anterior segment imaging have provided insights into the mechanisms of angle closure in uveitis. Uveitic eyes with angle closure from pupil block require surgical iridectomy with mobilization of the peripheral iris and viscogoniosynechiolysis of both posterior synechiae and peripheral anterior synechiae. Systemic conditions associated with uveitis can result in anterior displacement of the iris-lens diaphragm, and present as acute angle closure. Pupil block is not the predominant mechanism in these eyes, and management is primarily medical. Data are limited on the optimal treatment of angle closure in uveitis, and the role of glaucoma filtration surgery, cataract extraction, minimally invasive glaucoma surgery and newer modalities of cycloablation require evaluation. SUMMARY: The management of angle closure in uveitis should adhere to the principles of managing both uveitic glaucoma and angle closure. Identification of the mechanism of angle closure in uveitic eyes may enable treatment to be targeted at the responsible mechanism.

Congenital Oculomotor Nerve Paresis With Isolated Cyclic Pupillary Spasms.

Cyclic oculomotor nerve paresis is a rare and usually congenital disorder. It is characterized by unilateral third nerve paresis with periodic spasms causing eyelid elevation, miosis, and contraction of 1 or more of the extraocular muscles innervated by the third nerve. We report a 20-month-old girl who presented initially with a congenital partial right third nerve paresis without ptosis. She subsequently developed isolated cyclic spasms of the pupil followed several months later by permanent partial ptosis.

Congenital fixed dilated pupils due to ACTA2- multisystemic smooth muscle dysfunction syndrome.

Congenital fixed dilated pupils (congenital mydriasis) is characterized by hypoplasia or aplasia of the iris muscles, with absence of iris between the collarette and pupillary border, creating a scalloped pupillary margin. This condition has been reported in a multisystemic smooth muscle cell dysfunction syndrome, combined with congenital patent ductus arteriosus, cerebrovascular disease (Moya-moya-like), coronary artery disease, thoracic aorta aneurysm, and dysfunction of smooth muscle cells in organs throughout the body. All affected individuals carry a p.R179H heterozygous mutation in the ACTA2 gene. We add to the ophthalmologic involvement with 3 more patients. Congenital fixed dilated pupils is a rare condition and should alert ophthalmologists to the possibility of the coexistence of systemic life-threatening disorders.