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Pupillary-block glaucoma associated with childhood cystinosis.

Cystinosis is a rare autosomal recessive metabolic disorder that results in the widespread accumulation of cystine crystals in ocular tissues as well as in bone marrow, liver, spleen, lymph nodes, and kidneys. We treated a case of pupillary-block glaucoma in a 19-year-old woman caused by cystine accumulation in the iris stroma. Trabeculectomy and iridectomy relieved the pupillary block and decreased the intraocular pressure. Histologic examination disclosed the presence of crystals in the conjunctival and iris stroma and in the iris pigment epithelium. Crystals were also found within conjunctival mast cell granules, confirming the lysosomal nature of cystinosis.

Acute Disease↗

Neodymium-YAG laser therapy to the anterior hyaloid in aphakic malignant (ciliovitreal block) glaucoma.

In three aphakic eyes and two eyes with intraocular lens implants (five patients, four women and one man, ranging in age from 65 to 76 years) persistent shallow or flat anterior chamber was observed despite multiple patent laser or surgical iridectomies. In each case direct application of the neodymium-YAG laser (3 to 11 mJ) to the anterior hyaloid face resulted in immediate deepening of the anterior chamber. This deepening was sustained in four of the cases. These results confirm previous observations suggesting that the anterior hyaloid can be the locus of abnormality in aphakic malignant (ciliovitreal block) glaucoma. Neodymium-YAG "hyaloidotomy" may be better than penetrating surgery in such cases.

Aged↗

Edward J. Curran and the concept of relative pupillary block.

The historical development of the concept of relative pupillary block and its role in the pathogenesis of angle-closure glaucoma is reviewed. Prior to 1920, the mechanism of action of iridectomy in glaucoma was not understood, its indications unclear, and its technique unsatisfactory. Edward J. Curran's original statement of this concept, and its cure by peripheral iridotomy, were outstanding contributions to the treatment of glaucoma. The controversial responses of other ophthalmologists are related.

Australia↗

The glaucomas in aphakia and pseudophakia.

Intraocular pressure may become temporarily or permanently elevated at various intervals following cataract surgery. There are several mechanisms by which glaucoma develops as a complication of the cataract extraction itself. The presence of a pseudophakos may or may not contribute to the pathogenesis. Important diagnostic clues include the anterior chamber depth, the presence or absence of an iridectomy, gonioscopic findings, and the appearance of the optic nerve head. Life-long medical treatment is frequently justified, as alternative laser or surgical modalities may not be successful. The exact causes for high failure of filtration surgery in aphakic eyes is not clearly understood; scarring of the conjunctiva, the vitreous, and altered characteristics of the aqueous humor have all been incriminated. Current research to improve surgical success includes the development of effective artificial drainage implants or the use of pharmacologic modulators of wound healing, which promote filtration by preventing scar formation.

Aphakia↗

Climatic droplet keratopathy.

Climatic droplet keratopathy (CDK) is a degenerative condition characterized by the accumulation of translucent material in the superficial corneal stroma within the interpalpebral strip, beginning peripherally and spreading centrally. Progressive accumulation in later life can lead to significant visual disability, and people leading an outdoor life are particularly at risk. The translucent corneal deposits are composed of protein, but although they share some of the staining characteristics of degenerated protein, such as that found in pingueculae, their exact histochemical nature remains uncertain. The corneal deposits are thought to be derived from plasma proteins, which diffuse into the normal cornea, and may be photochemically degraded by excessive exposure to ultra-violet light (UV). The degraded protein material may then be deposited in the superficial stroma. UV light is now widely accepted to be the main etiological factor in the pathogenesis of CDK. Sector iridectomy, corneal epithelial debridement, lamellar keratoplasty, and penetrating keratoplasty have all been employed in the treatment of visually incapacitating CDK.

Blood Proteins↗

Enhancement of S-antigen and its mRNA in the irides of uveitic patients.

S-antigen (S-Ag) and its mRNA were analysed by immunohistochemistry and in situ hybridization in 32 iridectomy specimens from 29 uveitic patients and 10 non-uveitic patients. S-Ag was detected in one iris and its mRNA was detected in 12 uveitic patients. Neither S-Ag nor its mRNA was found in the controls (P < 0.003). Ten of the 12 patients who had detectable S-Ag mRNA, while only four of the 17 patients who did not, had received corticosteroids for more than 3 years (P = 0.006). We also demonstrated S-Ag and its mRNA in bovine iris by immunoprecipitation and polymerase chain reaction. These results indicate that S-Ag and its mRNA accumulate in the irides of some uveitic patients. This accumulation may be the result of local immunoregulatory factors and an effect of corticosteroid treatment, and may modulate ocular inflammation.

Adrenal Cortex Hormones↗

Primary viscocanalostomy for juvenile open-angle glaucoma.

PURPOSE: To evaluate the efficacy and safety of primary viscocanalostomy for medically uncontrolled juvenile open-angle glaucoma (JOAG). DESIGN: Prospective, noncomparative, interventional case-series study. METHODS: The study included 20 eyes of 20 consecutive patients with medically uncontrolled JOAG who were treated by viscocanalostomy at one institution. No surgical or laser procedure preceded viscocanalostomy. Surgical outcome was defined as an overall success by the following criteria: no visual field deterioration, no optic-neuropathy progression, postoperative intraocular pressure IOP < or =20 mm Hg, and IOP reduction > or =30% compared with baseline values with or without medication. When medications were not required, success was defined as complete. Cases that did not fulfill the aforementioned criteria and cases in which a surgical revision or further goniopuncture was performed were defined as a failure. RESULTS: Gender distribution was similar. Fourteen eyes belonged to the white race; five eyes belonged to the black race, and one eye belonged to Arab ethnicity. Mean age (+/-SD) at operation was 33.77 +/- 6.16 years, with the mean preoperative IOP (+/-SD) at 22.9 +/- 4.77 mm Hg. Thirty-six months after operation, 16 cases (80%) were considered an overall success. In 11 cases (55%), success was complete. Four cases (20%) were considered failures. No serious complications were documented either during or after operation. In two cases (10%), we documented a spontaneously reabsorbed microhyphema. Trabeculo-Descemet-membrane microperforation occurred in two cases (10%). In two other cases (10%), Trabeculo-Descemet-membrane perforation occurred and was accompanied by iris prolapse that needed peripheral iridectomy. CONCLUSION: Primary viscocanalostomy can efficiently and safely reduce intraocular pressure in cases of medically uncontrolled JOAG and provide a rational alternative to conventional surgical modalities.

Adolescent↗

The relationship between refractive and biometric changes during Edinger-Westphal stimulated accommodation in rhesus monkeys.

Experiments were undertaken to understand the relationship between dynamic accommodative refractive and biometric (lens thickness (LT), anterior chamber depth (ACD) and anterior segment length (ASL=ACD+LT)) changes during Edinger-Westphal stimulated accommodation in rhesus monkeys. Experiments were conducted on three rhesus monkeys (aged 11.5, 4.75 and 4.75 years) which had undergone prior, bilateral, complete iridectomies and implantation of a stimulating electrode in the Edinger-Westphal (EW) nucleus. Accommodative refractive responses were first measured dynamically with video-based infrared photorefraction and then ocular biometric responses were measured dynamically with continuous ultrasound biometry (CUB) during EW stimulation. The same stimulus amplitudes were used for the refractive and biometric measurements to allow them to be compared. Main sequence relationships (ratio of peak velocity to amplitude) were calculated. Dynamic accommodative refractive changes are linearly correlated with the biometric changes and accommodative biometric changes in ACD, ASL and LT show systematic linear correlations with increasing accommodative amplitudes. The relationships are relatively similar for the eyes of the different monkeys. Dynamic analysis showed that main sequence relationships for both biometry and refraction are linear. Although accommodative refractive changes in the eye occur primarily due to changes in lens surface curvature, the refractive changes are well correlated with A-scan measured accommodative biometric changes. Accommodative changes in ACD, LT and ASL are all well correlated over the full extent of the accommodative response.

Accommodation, Ocular↗

Anterior chamber intraocular lens (ACIOL) placement after pars plana lensectomy in pediatric Marfan syndrome.

INTRODUCTION: The management of subluxed crystalline lenses in patients with Marfan syndrome is challenging and may require lensectomy. Options for postoperative optical correction include aphakic spectacles, aphakic contact lens, posterior chamber IOLs sutured to the ciliary sulcus, and open-loop flexible anterior chamber IOLs (ACIOL). We recently began placing ACIOLs in all patients with Marfan Syndrome who were younger than 18 years of age and required lensectomy. METHODS: We used a retrospective chart review design for this study. RESULTS: Eight eyes of 5 patients were treated with pars plana vitrectomy, pars plana lensectomy, and primary ACIOL placement. Follow-up ranged from 9 to 16 months, with a mean of 12.7 months. Ages ranged from 5 to 17 years with a mean of 9 years. Three eyes were functionally aphakic at the time of surgery. Of the 5 eyes with a portion of the lens remaining in the visual axis, none maintained accommodative function by near visual acuity testing. Best-corrected distance visual acuity preoperatively ranged from 20/50 to 20/400, with an average of 20/80 (0.65 logMAR). Postoperative acuity ranged from 20/20 to 20/50, with an average acuity of 20/32 (0.20 logMAR). One patient had an iatrogenic sector iridectomy. No patients experienced corneal decompensation, increased intraocular pressure, persistent inflammation, IOL displacement, or explantation during the follow-up period. CONCLUSIONS: Although long-term follow-up data are unavailable, our cases of primary ACIOL placement in children with Marfan syndrome indicate that this therapy should be considered.

Adolescent↗

Angle-supported phakic intraocular lenses in hyperopia.

PURPOSE: To evaluate the efficacy and safety of angle-supported phakic intraocular lenses (PIOLs) in hyperopia. SETTING: Private practice, Siena, Italy. METHODS: A prospective noncomparative single-surgeon interventional case series comprised 42 consecutive hyperopic eyes of 22 patients having implantation of a type PIOL (Morcher) through a sclero-corneal 5.5 mm x 3.0 mm tunnel along the steepest meridian, with surgical iridectomy. The mean preoperative defocus equivalent (DEQ) was 7.30 diopters (D) +/- 1.89 (SD). The mean spherical equivalent (SE) was 6.61 +/- 1.47 D (range 4.0 to 10.5 D), and the mean refractive astigmatism was 1.51 +/- 1.33 D (range 0 to 5 D). The mean age was 29.9 +/- 6.0 years (range 22 to 42 years). Thirteen patients (59%) were men. RESULTS: Postoperative mean SE was 0.38 +/- 0.52 D (range 0 to 2 D); mean DEQ 0.93 +/- 0.98 D; mean astigmatism 0.95 +/- 1.17 D; mean surgically induced astigmatism (vector analysis) 0.67 +/- 0.58 D (95% confidence interval, 0.49-0.85); 38 eyes (90%) were within +/-2 D of DEQ, 34 eyes (81%) within +/-1 D, and 24 eyes (57%) within +/-0.5 D. Safety index was 1.05; efficacy index 0.85. Complications were night halos 10%; pupil ovalization 7%; pupillary block reversed by neodymium:YAG laser 7%; monocular anterior subcapsular opacity 5%; monocular iridocyclitis 5%; and intraocular pressure rise due to topical steroids 2%. Endothelial cell loss at 12 month was 6% (range +2.5% to -11%). CONCLUSION: High hyperopia was corrected safely and predictably by an angle-supported PIOL with limited complications.

Adult↗

Iris-cyst-simulating luxated lens.

A 2-month-old child was referred to our clinic for evaluation and management of a luxated lens. Under general anesthesia, an irregularly shaped iris was observed with the presence of a large iris cyst in the anterior chamber. Sector iridectomy with excision of the cyst was performed. Histopathologic examination confirmed the diagnosis of a stromal cyst of the iris. At the 3-year follow-up, there was no evidence of recurrence of the iris cyst or of a secondary cataract. This case report provides evidence that although a primary cyst of the iris stroma is uncommon, it should be considered in the differential diagnosis for an apparent lens luxation.

Cysts↗

Vitrectomy-phacoemulsification-vitrectomy for the management of aqueous misdirection syndromes in phakic eyes.

OBJECTIVE: To describe vitrectomy-phacoemulsification-vitrectomy, a sequential 3-step surgical approach, in the management of malignant glaucoma/aqueous misdirection syndromes in phakic eyes. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Five eyes (4 angle-closure glaucoma and 1 open-angle glaucoma) of 5 patients with mean age of 66 years (range, 56-78). Four patients presented with aqueous misdirection syndrome and 1 patient presented for cataract extraction, having previously had malignant glaucoma in the fellow eye after phacoemulsification surgery. INTERVENTION: The operation performed had three steps: vitrectomy, phacoemulsification, and vitrectomy. Step 1: Preliminary vitrectomy involved limited core vitrectomy to "debulk" the vitreous and soften the eye. Step 2: Phacoemulsification was performed in a standard manner. Step 3: Residual vitrectomy, zonulohyaloidectomy and peripheral iridectomy (if not already present) were performed to create a free communication between the posterior and anterior segments. MAIN OUTCOME MEASURES: Intraocular pressure, visual acuity, biomicroscopic anterior chamber depth, and complications. RESULTS: The time interval between the onset of malignant glaucoma and surgery ranged from 2 weeks to 3 months. All 4 patients with aqueous misdirection syndrome had relief of the aqueous misdirection postoperatively with anterior chamber deepening. Intraocular pressures on day 1 ranged from 6 to 28 mmHg (mean 15.6, mmHg), and at the last visit ranged from 8 to 30 mmHg (mean, 20.4 mmHg). One eye developed an early choroidal serosanguinous effusion requiring drainage. CONCLUSIONS: The vitrectomy-phacoemulsification-vitrectomy approach was effective in this pilot series in the management of aqueous misdirection syndromes and malignant glaucoma in phakic eyes.

Aged↗

Anterior segment tumors: current concepts and innovations.

The most common anterior segment tumors are primary neuroepithelial cysts, uveal melanomas, metastatic tumors, and benign tumors. In the majority of cases, the diagnosis can be made utilizing a careful clinical history and ocular examination. Ultrasound examination (low and high frequency) has become an indispensable tool used to determine tumor extension and involvement of the surrounding structures. In particular, high-frequency ultrasound has been used to uncover iris pigment epithelial cysts, to allow for the diagnosis of small ciliary body melanomas, and to measure tumors for plaque radiation planning. Whereas fluorescein angiography and computerized tomography have come to play a limited role, fine-needle aspiration biopsy has been found to be quite helpful in selected cases. Once the diagnosis is established, treatment decisions depend on the tumors' location, size, local extension, patterns of growth, and secondary complications. Most anterior segment tumors can be observed for growth prior to treatment. Other options include local resection (iridectomy, lamellar sclerouvectomy, or eye-wall resection) and radiation (ophthalmic plaque or external beam). Enucleation is typically employed if these eye- and vision-sparing treatments are not possible and for uncontrollable secondary glaucoma. This review examines the unique role of high-frequency ultrasonography for the diagnosis and treatment of anterior segment tumors as well as an overview of clinical practice.

Anterior Eye Segment↗

Phakic posterior chamber intraocular lens for the correction of anisometropia and treatment of amblyopia.

PURPOSE: To assess the potential visual benefits of posterior chamber phakic intraocular lens implants in eyes of children with anisometropic amblyopia. METHODS: In a prospective study, three girls 9, 14, and 18 years old with high anisometropia and deep amblyopia were included in this study. The phakic posterior chamber intraocular lens (ICL; STAAR Surgical AG, Nidau, Switzerland) was used to correct the anisometropia. This intraocular lens was inserted in the anterior chamber through a 3.0-mm temporal clear cornea incision and manipulated into the posterior chamber using an iris manipulator. A peripheral iridectomy was performed using the Ocutome Probe (Storz; Premiere, St. Louis, Missouri). Local therapy with corticosteroids and antibiotics were prescribed for 2 weeks, and patients were followed regularly for a period of 6 to 9 months. RESULTS: In the three amblyopic eyes of the three patients, the preoperative best-corrected visual acuity of 6/30, 6/60, and 6/30 improved, to 6/7.5 (20/25), 6/30 (20/100), and 6/15 (20/50), respectively, 6 months after the surgery. Binocular functions with development of fusional abilities and stereopsis were observed in two of these patients after the intraocular lens implantation. In the third patient, the fusional abilities developed only after surgical correction of the exotropia. The intraocular pressure remained within normal limits, and there was no significant change in the corneal endothelial cell count during the period of follow-up. No major intraoperative or postoperative complications were observed, except for a temporary pigment dispersion. CONCLUSIONS: Implantation of phakic posterior chamber intraocular lenses may be beneficial for the treatment of amblyopia in children with anisometropia. Although additional cases and long-term follow-up observations are necessary, it appears that amblyopia may be overcome by the use of posterior chamber phakic intraocular lens implants, even in eyes of children beyond the age generally considered to be responsive to anti-amblyopic treatment.

Adolescent↗

Gastric signet ring cell adenocarcinoma metastatic to the iris.

PURPOSE: To report a case of gastric signet ring cell adenocarcinoma metastatic to the iris. METHODS: Case report. A 38-year-old man with a history of gastric signet ring cell adenocarcinoma presented with a white lacework-like mass on the iris of the left eye. RESULTS: Systemic medical evaluation demonstrated no other metastasis. Because of increased intraocular pressure, trabeculectomy and peripheral iridectomy were performed. Histology of the iris specimen demonstrated metastasis of gastric signet ring cell adenocarcinoma to the iris. CONCLUSION: Gastric signet ring cell carcinoma can metastasize to the iris. Ophthalmologists should include this possibility in the differential diagnosis when encountering a patient with a history of gastric adenocarcinoma and an iris mass.

Adult↗

Pupil block glaucoma in phakic and pseudophakic patients after vitrectomy with silicone oil injection.

PURPOSE: To describe pupil block glaucoma in phakic and pseudophakic patients after vitrectomy with silicone oil injection. DESIGN: Interventional case series. METHODS: Cases were collected from January 1997 to July 2000 from three tertiary referral centers. RESULTS: Seven phakic patients (seven eyes) and one pseudophakic patient (one eye) presented 1 to 90 days after vitrectomy and silicone oil injection with intraocular pressures of 36 to 70 mm Hg. Five patients had an observed or potential weakness of the iris-lens diaphragm. Treatment with Nd:YAG-laser peripheral iridotomy or inferior iridectomy provided a temporary reduction in intraocular pressure for some patients, but all eventually required removal of silicone oil. CONCLUSION: Pupil block glaucoma after silicone oil injection is well recognized in aphakic patients, but ophthalmologists should be aware that it can occur in phakic and pseudophakic patients, particularly in complicated cases and patients with a weakness of the iris-lens diaphragm.

Adult↗

Posttraumatic exogenous Nocardia endophthalmitis.

PURPOSE: To report a case of posttraumatic exogenous Nocardia endophthalmitis. DESIGN: Interventional case report. METHOD: A 46-year-old man presented with counting fingers vision, severe eye pain, hypopyon, and an iris mass consistent with endophthalmitis following a penetrating injury to the left eye. RESULTS: Despite oral fluconazole and repeated intravitreal injections of vancomycin, gentamicin, and amphotericin B, the eye remained painful with a persistent hypopyon and recurrent iris masses. Cultures of the vitreous and iris masses remained negative for organisms. Despite vitrectomy and sector iridectomy of the iris mass with repeat injections of intravitreal antibiotics, the patient continued to have severe pain, poor vision, and developed new iris masses. Enucleation cultures confirmed Nocardia asteroides. CONCLUSIONS: Posttraumatic exogenous Nocardia endophthalmitis is extremely rare, and its clinical signs can mimic a fungal infection.

Anterior Eye Segment↗

Gonioscopic evaluation of haptic position in transsclerally sutured posterior chamber lenses.

PURPOSE: To determine the haptic position in transsclerally sutured posterior chamber lenses. METHODS: Fifty eyes with transsclerally sutured posterior chamber lenses were evaluated gonioscopically to determine the superior haptic position using previous peripheral iridectomies. RESULTS: The superior haptic of the transsclerally sutured posterior chamber lens was visualized in 31 (62%) of the eyes. In 24 (77%) of the 31 eyes, the haptic was within the ciliary sulcus, and in seven (23%), it was located posterior to the sulcus. A fibrotic membrane was detected around the sutured haptic in 20 (83%) of the 24 lenses located within the sulcus, whereas no fibrosis was seen surrounding the seven haptics outside the sulcus (P < .0001). CONCLUSIONS: Proper positioning of the haptics may be correlated with the development of fibrosis surrounding the haptic and therefore may be an important factor in the long-term stability of transsclerally sutured posterior chamber lenses.

Gonioscopy↗