Persistent anterior tunica vasculosa lentis in multisystemic smooth muscle dysfunction syndrome: A case report.

RATIONALE: Multisystemic smooth muscle dysfunction syndrome (MSMDS) is a genetic disease that affects multiple organs. The report here concerns a patient with MSMDS, who is known so far as the youngest among all the reported patients. In addition to the typical manifestations, we observed previously unreported ocular abnormalities, including persistent anterior tunica vasculosa lentis (TVL) and early-onset retinal arteriolar tortuosity, by the fluorescein angiography (FA). PATIENT CONCERNS: The patient was admitted to the neonatal intensive care unit immediately after birth for a diagnosis of urinary system dysplasia during fetal life. After a thorough examination, the patient was found with patent ductus arteriosus, pulmonary hypertension, cerebrovascular disease, hypotonic bladder, intestinal malrotation, and congenital mydriasis. The FA of the eyes undertaken in her 6-week demonstrated perfused vasculature in the persistent anterior TVL and prominent retinal arteriolar tortuosity. The whole exome sequencing revealed a de novo heterozygous ACTA2 gene missense mutation p.R179H. DIAGNOSES: The patient was diagnosed with MSMDS. INTERVENTIONS: Follow-up observation. OUTCOMES: At the 3-month follow-up, no change of the ocular disease was observed. LESSONS: The persistent anterior TVL in this case implies that ACTA2 p.R179H mutation affects not only the smooth muscle cells but also the pericytes, and further affects the TVL regression. The prominent retinal arteriolar tortuosity in this 6-week-old infant indicates that the retinal arteriolar tortuosity can present early in MSMDS.

Uveitis and deficient lens capsules: Effect of glued intraocular lens on the visual outcome and the reactivation of inflammation.

Purpose: To evaluate the visual outcome and complication profile after glued intraocular lens (IOL) in post uveitic eyes. Methods: Patients with history of uveitis who had glued IOL with 3 months antecedent quiet anterior chamber (AC) were included in this prospective observational case series. Visual acuity, slit-lamp examination, fundus evaluation, optical coherence tomography, intraocular pressure, specular count and AC inflammation were analyzed before and after glued IOL procedure. Glued IOL eyes were also compared with their fellow normal capsular bag IOL. Results: Overall 17 eyes (50.7 ± 16.1 years) were analyzed. It included 41.8%, 23.5%, and 35.29% anterior, posterior, and pan uveitis, respectively. The etiologies were tuberculosis (23.53%), toxoplasmosis (11.77%), Fuch's heterochromic cyclitis (5.88%), HLA B27 (11.77%), psoriatic arthritis (5.88%), Rheumatoid arthritis (5.8%), sarcoidosis (11.77%), herpetic kerato-uveitis (5.88%), and idiopathic (17.65%). Cataractous subluxated lens (35.3%), aphakia (23.5%), decentered IOL (23.5%) and intraoperative capsular rupture (17.6%) were the surgical indications. A significant improvement in the mean uncorrected and best corrected visual acuity (P < 0.001) was recorded. The complications were IOL pigment dispersion (47%), macular edema (41%), and epiretinal membrane (24%). There was significant rise in AC reaction on day 1 (P < 0.001) and normal AC was attained by 88.2% eyes at 6 months. AC inflammation reactivation was noted in 11.7% of eyes. Though inflammatory reactivation was similar to the normal IOL, macular edema was higher in glued IOL. Conclusion: Glued IOL can cause inflammation in uveitis eyes which can be managed medically with minimal complications.

Optic shrinkage and retraction in opacified hydrophilic acrylic intraocular lens: an anterior segment optical CT-based observation.

Intraocular lens (IOL) opacification is a rare phenomenon noted with hydrophilic acrylic IOLs. We report a case of advanced IOL opacification appreciated on anterior segment optical CT (ASOCT)as a shrunken biconcave optic retracted away from the posterior capsule (PC), unlike the other eye which had a clear biconvex IOL of similar material abutting the PC. After IOL exchange, the affected eye was noted to have more folds and Elschnig's pearls on the PC when compared with the other eye. Our case points towards rare IOL changes seen in advanced cases of opacification, their association with posterior capsular changes and the aid of ASOCT as a non-invasive tool in diagnosing them correctly.

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.

Coexisting Phacoanaphylaxis and Choroidal Melanoma: An Unusual Intraocular Inflammation.

PURPOSE: To describe an unusual case of panuveitis in coexisting choroidal melanoma and phacoanaphylaxis. METHODS: Clinical records of the patient with intraocular melanoma and pathologic findings of the affected eye were reviewed. Composition of inflammatory infiltrates involving the tumor and lens was evaluated immunohistochemically. RESULTS: Histologically, tumor showed a choroidal mixed cell type malignant melanoma with infiltration of inflammatory cells. The globe also showed typical features of phacoanaphylaxis, characterized by disruptions of the lens capsule with zonal granulomatous inflammation. The inflammatory infiltration revealed the predominant presence of both cytotoxic T cells and histiocytes in phacoanaphylaxis and intra-tumoral inflammation. CONCLUSIONS: The current clinico-pathologic case report suggests that a long standing intraocular melanoma can present with phacoanaphylaxis. The immunophenotype of the inflammatory infiltrates at both sites, lens and the tumor, is similar, cytotoxic T cells and histiocytes, favoring a common immune inflammatory process directed at the tumor and the lens.

Clinical spectrum and surgical outcomes in spherophakia: a prospective interventional study.

PurposeTo study the varied clinical presentations of patients with spherophakia, their management using surgical methods, and the clinical outcomes.Patients and methodsA prospective interventional study of 13 patients of spherophakia who presented to us from January 2014 and were followed up over the course of their treatment, and the data were documented for analysis.ResultsIn all, 26 eyes of 13 patients were reviewed and the median age of presentation was 12±12.05 years. All patients had a bilateral presentation with 22 eyes having lenticular myopia with a mean refractive error of -11.5±12.945 DS. Ten eyes presented with glaucoma of which six had raised intraocular pressure (IOP) >21 mm Hg. A total of 23 eyes underwent lens extraction for dislocation/subluxation. Lens extraction helped lower overall IOP. Refractive rehabilitation was done with ACIOL, posterior chamber intraocular lens (PCIOL) with capsular tension ring, and scleral-fixated intraocular lens (SFIOL) in respective cases with ACIOLs being the most commonly used option.ConclusionsSpherophakia is a rare condition, which exhibits a varying degree of lenticular myopia, glaucoma, and subluxation of the crystalline lens. Lensectomy with proper rehabilitation using ACIOL, PCIOL, or SFIOL is a method of managing subluxation and unacceptable myopia. Lensectomy may also be a viable option of controlling glaucoma alongside medications and glaucoma surgery for the management of glaucoma in such cases.

Angle closure caused by a plateau-like iris associated with an enlarged Soemmering's ring: a case report.

BACKGROUND: This study evaluated the anterior ocular segment in a pseudophakic eye with angle closure due to a plateau-like iris associated with Soemmering's ring, using ultrasound biomicroscopy (UBM) and anterior segment optical coherence tomography (AS-OCT). CASE PRESENTATION: A 60-year-old woman was referred from a local clinic due to sudden-onset ocular pain and uncontrolled intraocular pressure (IOP) in the left eye, which was 56 mmHg after treatment with latanoprost, timolol, and dorzolamide eye drops. Fourteen years earlier, she developed acute primary angle closure. At that time, because the IOP remained elevated after a peripheral iridectomy, cataract extraction combined with goniosynechialysis was added. After the IOP decreased to within the normal range, a secondary intraocular lens was implanted outside the bag. On this admission, UBM and AS-OCT images showed angle closure caused by the combination of a plateau-like iris and contact between the mydriatic pupillary margin and enlarged Soemmering's ring. After adding 2 % pilocarpine four times a day, the mydriasis resolved slightly, and the IOP decreased to the normal range between 8 and 18 mmHg. AS-OCT images showed re-opening of the angle structure after treatment with 2 % pilocarpine. CONCLUSION: The intraocular pressure and angle structure in eyes with a plateau iris after cataract extraction should be followed carefully.

Comparison of Cataract Surgery Alone Versus Cataract Surgery Combined With Trabeculectomy in the Management of Phacomorphic Glaucoma.

PURPOSE: To compare the outcomes of cataract surgery alone versus combined cataract surgery with trabeculectomy in eyes with phacomorphic glaucoma. MATERIALS AND METHODS: In a retrospective comparative case series, 126 eyes of phacomorphic glaucoma presenting within 4 weeks of onset between 1993 and 2012, with at least 6 months of postoperative follow-up were included. Group 1 included 63 eyes with cataract surgery only and group 2 included 63 eyes with combined cataract surgery and trabeculectomy. Primary outcome measure was intraocular pressure (IOP) ≤21 mm Hg (without antiglaucoma medication) 6 months after surgery. RESULTS: The median (interquartile range) preoperative IOP in group 1 was 36 mm Hg (30 to 50 mm Hg) and group 2 was 40 mm Hg (32 to 48 mm Hg) (P=0.34). The median duration of phacomorphic attack was 7 days (3 to 12 d) in group 1 and 7 days (3 to 15 d) in group 2 (P=0.39). The median duration of postoperative follow-up was 23 months in group 1 and 30 months in group 2 (P=0.89). Six months after surgery, 97% of the eyes in group 1 and 100% in group 2 achieved IOP≤21 mm Hg without antiglaucoma medications with a median postoperative IOP of 12 mm Hg in both the groups (P=0.09). The median Log MAR visual acuity was significantly better in group 1 compared with group 2 (0.2 vs. 0.3, P<0.001). The percentage of eyes that achieved visual acuity better than 20/40 at 3 months was 62% in group 1 versus 19% in group 2 and at 6 months it was 75% in group 1 versus 38% in group 2 (P<0.001). CONCLUSIONS: In our cohort of phacomorphic glaucoma presenting within 4 weeks of onset, cataract surgery and combined surgery resulted in similar IOP control at 6 months. However, cataract surgery alone resulted in better and faster visual recovery.

Bilateral congenital cataract with suspected lens-induced granulomatous uveitis.

A healthy newborn baby girl presented with congenital bilateral cataract. Within a few days of presenting she also developed bilateral granulomatous uveitis, a condition generally linked in newborns to congenital infections, most frequently TORCHES syndrome (toxoplasmosis, rubella, cytomegalic inclusion disease, herpesvirus, including Epstein-Barr, syphilis). Extensive investigation did not reveal any underlying etiologic mechanism. Treatment with topical and systemic steroids did not improve the uveitis. However, bilateral lens extraction resulted in a quick resolution of the uveitis.

Surgical outcomes after intraocular lens implantation for posterior lenticonus-related cataract according to preoperative lens status.

PURPOSE: To address the surgical outcomes of pediatric patients with cataracts associated with posterior lenticonus who required cataract extraction and intraocular lens (IOL) implantation according to preoperative lens status. SETTING: Department of Ophthalmology, Seoul National University Children's Hospital, Seoul, South Korea. DESIGN: Comparative case series. METHODS: Patients who had cataract extraction and IOL implantation for posterior lenticonus were divided into 2 groups according to the preoperative lens status. Clinical features and visual outcomes in both groups were comparatively analyzed. RESULTS: Forty-seven eyes of 43 patients were studied. Thirty-five eyes had lens opacities localized to the posterior pole, and 12 eyes presented with total opacity of the lens. Preexisting posterior capsule defect was identified intraoperatively in 11 eyes with total lens opacity. Eyes with preexisting posterior capsule defects more frequently required ciliary sulcus fixation of the IOL (P=.01). The mean follow-up after cataract extraction was 66.9 months ± 35.9 (SD). The mean final corrected distance visual acuity of patients with total opacity (0.37 ± 0.57 logMAR) was better than that of patients with posterior polar opacity (0.56 ± 0.50 logMAR), with borderline significance (P=.05). CONCLUSIONS: A preexisting posterior capsule defect, found most often in eyes that presented with total lens opacity, could be an obstacle to capsular bag fixation of the IOL. Posterior lenticonus patients with total lens opacity had marginally significantly better visual outcomes than patients with posterior polar opacity. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

Dramatic regression of persistent tunica vasculosa lentis associated with retinopathy of prematurity following treatment with intravitreal bevacizumab.

The authors describe a preterm infant who developed advanced retinopathy of prematurity bilaterally with a prominent tunica vasculosa lentis. Treatment with intravitreal bevacizumab resulted in regression of the tunica vasculosa lentis and posterior manifestations of the retinopathy of prematurity. RetCam imaging (Clarity Medical Systems, Pleasanton, CA) of the anterior segment was used to document the dramatic tunica vasculosa lentis resolution.

In-the-bag decentration of a hydrophilic radially asymmetric multifocal intraocular lens secondary to capsule contraction.

We report a case of in-the-bag decentration and tilt of a hydrophilic rotationally asymmetric multifocal intraocular lens (IOL) of the M Plus type secondary to capsule contraction. After uneventful surgery and follow-up for 3 months, progressive decentering and tilting of the IOL secondary to capsule contraction and capsulorhexis phimosis was noticed. A surgical procedure was necessary to restore correct centration of the IOL. The uncorrected distance and near visual acuities were restored to logMAR 0. Hydrophilic multifocal IOLs of this specific design may be sensitive to postoperative decentration. Capsular tension rings may alleviate the problem in a secondary repair procedure. The softness of the C-loop haptics of this IOL type may also play a role in the decentration.

Angle-closure glaucoma secondary to inflammatory capsular block syndrome following routine cataract surgery.

A 78-year-old man had uneventful cataract surgery with implantation of a 1-piece plate-haptic intraocular lens in the capsular bag. Seven weeks later, he presented as an emergency with intense fibrinous uveitis and increased intraocular pressure (IOP). Examination revealed an inflammatory capsular block syndrome (CBS) causing fibrinous anterior uveitis and secondary angle-closure glaucoma. The glaucoma resolved and the patient's vision improved following neodymium:YAG laser posterior capsulotomy. Inflammatory CBS should be considered in pseudophakic patients presenting with fibrinous anterior uveitis, increased IOP, and secondary angle closure.