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Photoreceptor outer segments in aqueous humor: key to understanding a new syndrome.

Retinal detachment with oral dialyses and tears of the nonpigmented epithelium of the ciliary body sometimes accompanies aqueous cells and high intraocular pressure with its marked fluctuation. The aqueous cells consist predominantly of photoreceptor outer segments which obstruct aqueous outflow at the trabecular meshwork and induce high intraocular pressure as ghost cells do in ghost cell glaucoma. A new clinical entity with the combination of these signs, namely, rhegmatogenous retinal detachment with "photoreceptor outer segment glaucoma" is designated as Schwartz-Matsuo syndrome in this report. The detection of photoreceptor outer segments in the anterior chamber indicates that a communication between subretinal space and aqueous humor has been established by some mechanism. The new syndrome should be included in differential diagnoses of iritis and glaucoma.

Aqueous Humor↗

Aicardi syndrome: natural history and possible predictors of severity.

Aicardi syndrome is defined by the clinical triad of infantile spasms, agenesis of the corpus callosum, and pathognomonic chorioretinal lacunae. Almost all patients are girls with severe cognitive and physical handicaps, and epilepsy. Fourteen patients with Aicardi syndrome, seen at The Hospital for Sick Children, Toronto, Ontario, Canada, between 1975 and 1992, were reviewed to document the natural history of the disease and obtain life-table estimates of survival. The relationship between 28 neurologic features present in infancy and clinical outcome, as measured by mobility and cognitive function also was examined. Life-table analysis indicated that the estimated survival rate was 76% at 6 years of age and 40% at 15 years of age. Three of the 14 girls (21%) could walk or crawl and 4 (29%) had some language ability. None of the 28 neurologic features was predictive of ultimate clinical outcome. This information should be discussed with parents of children with Aicardi syndrome.

Abnormalities, Multiple↗

Anatomic and visual outcomes after indocyanine green-assisted peeling of the retinal internal limiting membrane in idiopathic macular hole surgery.

PURPOSE: To report anatomic and visual outcomes after vitrectomy and adjunctive retinal internal limiting membrane (ILM) peeling with and without intravitreal indocyanine green for idiopathic macular hole repair. DESIGN: Retrospective comparative study of consecutive case series. METHODS: Three consecutive groups of idiopathic macular hole cases underwent modifications of surgical technique. Group I (48 eyes of 47 patients) underwent a standard vitrectomy, fluid/gas exchange, and 1 week's face-down positioning, group II (21 eyes of 21 patients) an adjunctive ILM peeling without use of indocyanine green, and group III (28 eyes of 28 patients) an adjunctive peeling of ILM stained with intravitreal application of 0.1 to 0.2 ml of 0.5% indocyanine green dye. RESULTS: Three groups of patients had comparable clinical characteristics as to age, gender, estimated duration of macular hole, preoperative visual acuity, and follow-up time. The rate of macular hole closure after a single surgery, as determined by optical coherence topography was 85.4% in group I, 85.7% in group II, and 100% in group III. Groups I and II showed a statistically significant visual improvement, but group III did not show significant visual acuity improvement as the mean logarithm of the minimal angle of resolution visual acuity was from 0.767 (20/120) preoperatively to 0.691 (20/100) postoperatively (P =.342). Eight cases in group III developed within a few postoperative months of optic disk pallor and irreversible peripheral visual field loss, predominantly affecting the nasal field. CONCLUSIONS: Intravitreal indocyanine green-assisted ILM peeling improves anatomic success in macular hole surgery, but it may potentially lead to unfavorable visual acuity outcome and peripheral visual field loss.

Adult↗

The role of patient age and intraocular gas use in cataract progression after vitrectomy for macular holes and epiretinal membranes.

PURPOSE: To evaluate the rate of increase in nuclear sclerosis and posterior subcapsular cataracts in eyes as a function of patient age and use of intravitreal gas at the time of vitrectomy. DESIGN: Observational case series. METHODS: Nuclear sclerosis and posterior subcapsular cataracts were evaluated as a function of patient age and use of intravitreal gas at vitrectomy. SETTING: A clinical practice. STUDY POPULATION: The study population consisted of 301 consecutive eyes. OBSERVATIONAL PROCEDURE: Nuclear sclerotic cataracts and posterior subcapsular cataracts were graded on a scale from 0 to 4.0 before and after vitrectomy. MAIN OUTCOME MEASURE: Linear regression analysis was performed to measure and compare the rate of change in cataract score over time based on patient age and use of intraocular gas. RESULTS: Nuclear sclerotic cataracts showed minimal increase in patients younger than 50 years of age after vitrectomy (0.13 grades/year). Nuclear sclerotic cataracts increased at a rate of 0.7 to 0.9 grades/year (mean, 0.812) in patients aged 50 to 60 years, 60 to 70 years, 70 to 80 years, and 80+ years, even though the baseline nuclear sclerosis scores were progressively greater for each decade. The increase in nuclear sclerotic cataracts in patients younger than 50 years of age was significantly less (P <.001) than in patients aged 50 years or older. Eyes with intraocular gas use had a higher rate of nuclear sclerosis progression (0.8 grades/year) compared with eyes without intraocular gas bubbles (0.5 grades/year; P <.001). Posterior subcapsular cataract scores showed minimal or no increases in all groups. CONCLUSIONS: Patients older than 50 years of age have a similar rate of increase in nuclear sclerotic cataracts, independent of age. The rate is approximately sixfold greater than in patients younger than 50 years of age. Intravitreal gas bubbles are associated with a nuclear sclerosis increase of approximately 60% compared with eyes without use of a gas bubble.

Adolescent↗

Treatment of an avulsed retinal vessel with infrared diode laser photocoagulation.

PURPOSE: To report the use of the infrared diode laser with long-duration pulses to successfully treat a patient with recurrent vitreous hemorrhages due to an avulsed retinal arteriole. DESIGN: Interventional case report. METHODS: A 70-year-old man presented with repeated, visually significant vitreous hemorrhages due to an avulsed retinal arteriole. Infrared diode laser photocoagulation with long-duration, large, overlapping spots was performed on either side of the avulsed segment. RESULTS: Resolution of the hemorrhage occurred within 1 month, without further episodes of bleeding. Two-year follow-up with fluorescein angiography showed complete cessation of blood flow through the treated segment. CONCLUSIONS: Infrared diode laser photocoagulation with long-duration pulses may be an effective, minimally invasive treatment of the avulsed retinal vessel syndrome.

Aged↗

Autofluorescence as a novel approach to diagnosing macular holes.

PURPOSE: To report the capability of autofluorescence as a novel approach to diagnosing macular holes. DESIGN: Observational case report. METHODS: In an institutional study, autofluorescence photos and optical coherence tomography scans of two patients who had surgery for macular holes were reviewed. RESULTS: Macular hole cases had a strong subfoveal autofluorescence signal, which disappeared after surgical closure of the macular hole. CONCLUSIONS: Autofluorescence is a novel approach to demonstrating a macular hole. This technique can also be used to confirm postoperative outcome after macular hole repair. A lack of autofluorescence corresponds to macular hole closure.

Aged↗

Pathogenesis of macular holes and therapeutic implications.

PURPOSE: To review the literature and identify consistencies and inconsistencies in existing theories of pathogenesis and to consider some of their possible therapeutic implications. DESIGN: Presentation of clinical case material with a synthesis of ideas on macular holes. METHODS: The literature of macular hole surgery is reviewed pertinent to pathogenic theories. Clinical examples of evolving macular holes shown on ocular coherence tomography are presented to illustrate issues. RESULTS: The history of pathogenesis and macular holes is interesting in that, in many ways pathogenic theory has come full cycle. Initially, anteroposterior traction was thought to cause direct formation of a macular hole. Subsequently, degenerative and then tangential tractional etiologies were proposed. Current imaging studies have greatly advanced our understanding of anatomic features of full-thickness holes and early full-thickness hole conditions. These are most consistent with a focal anteroposterior traction mechanism, but some inconsistences in clinical cases suggest a role for degeneration of the inner retinal layers. CONCLUSIONS: Degeneration of the inner retinal layers at the central fovea may predispose the eye to macular hole formation. What may otherwise be incidental tractional forces appear to initiate the hole. These tractional elements are oriented perpendicularly to the retinal surface, rather than tangentially. Further observations, especially with sequential observations from ocular coherence tomography, may yield further insights into the pathogenesis of macular holes as well as implications regarding the best repair techniques.

Humans↗

Optical coherence tomographic findings of dissociated optic nerve fiber layer appearance.

PURPOSE: To examine the features of optical coherence tomographic (OCT) findings of dissociated optic nerve fiber layer (DONFL) appearance. DESIGN: Case-control study. METHODS: Twenty-four eyes undergoing vitrectomy for idiopathic epiretinal membrane or macular hole were examined postoperatively using OCT. RESULTS: Thirteen of the 24 eyes exhibited the DONFL appearance. In these 13 eyes, the OCT images indicated dimples in the retinal nerve fiber layer (RNFL) corresponding to each stria. The RNFL thickness (mean +/- SD) at the dimple site was 38.1 +/- 9.3 microm. The depth of each dimple was 28.6 +/- 8.0 microm. The depths of all the dimples were limited to the RNFL thickness. In the other 11 eyes without the DONFL appearance, OCT images showed no distinct dimples. CONCLUSIONS: The DONFL appearance is caused by dimples the depths of which are limited to the RNFL thickness.

Case-Control Studies↗

Irregularity of photoreceptor layer after successful macular hole surgery prevents visual acuity improvement.

PURPOSE: To investigate the association between a photoreceptor irregularity and visual acuity (VA) after macular hole (MH) surgery. DESIGN: Prospective observational case series. METHODS: Twenty-four eyes with an idiopathic MH that resolved after vitreous surgery were examined by new optical coherence tomography (OCT 3000). The images were divided into regular photoreceptor, detected as a straight line above the retinal pigment epithelium reflex, and irregular, which cannot be detected by it. RESULTS: Regular images were observed in 12 eyes (80%) and irregular images in 3 eyes (20%) with good VA (>or=0.7). Regular images were seen in 3 eyes (33%) and irregular images in 6 eyes (67%) with poor VA (<0.7). The percentage of regular images in the group with good VA was significantly higher than that in the group with poor VA (P <.05). CONCLUSION: A photoreceptor irregularity after MH surgery may prevent VA improvement.

Aged↗

Determination of the incidence and clinical characteristics of subsequent retinal tears following treatment of the acute posterior vitreous detachment-related initial retinal tears.

PURPOSE: To determine the incidence of subsequent retinal tears (SRT) in patients who were previously treated for acute posterior vitreous detachment (PVD)-related retinal tears. DESIGN: Retrospective chart review. METHODS: A retrospective chart review of 155 eyes of 137 consecutive patients treated for acute PVD-related retinal tears was performed. RESULTS: During a median follow-up of 13 months (range 3 to 157 months) after treatment, SRT developed in 19 (12.2%) of 155 treated eyes. Most of the SRT occurred during the first 6 months (12 of 19 eyes) after initial treatment. Subsequent vetinal tears developed within the first 12 months in 15 of 19 treated eyes, however only six of 15 of these eyes were symptomatic. Of those patients who developed SRT after 12 months, all had new visual symptoms. CONCLUSION: Patients who present with acute PVD-related retinal tears are at a low but significant risk for developing SRT. A significant number of patients treated for PVD-associated retinal tears present within 1 year with SRT without symptoms.

Acute Disease↗

Optical coherence tomography of bilateral posterior microphthalmos with papillomacular fold and novel features of retinoschisis and dialysis.

PURPOSE: To report a case of retinoschisis and dialysis associated with bilateral posterior microphthalmos and papillomacular fold. DESIGN: Observational case series. METHODS: Complete ophthalmologic examination of three of five siblings presenting with bilateral posterior microphthalmos and papillomacular fold. Optical coherence tomography (OCT) data are presented to confirm the abnormal anatomy. RESULTS: All subjects have bilateral elevated horizontal papillomacular retinal fold with cystoid macular edema and shallow subretinal fluid. Optical coherence tomography was consistent with our examinations. One subject, a 13-year-old Hispanic, initially presented with retinoschisis in the superotemporal quadrant of the left retina that developed 9 years later into a retinal dialysis without subretinal fluid. The right eye of this same patient developed retinoschisis in the far superotemporal retinal periphery during 9 years of observation. CONCLUSION: Retinoschisis and dialysis may occur in patients with posterior microphthalmos with papillomacular fold. Optical coherence tomography may be helpful in assessing these patients.

Adolescent↗