OPTIC DISK COLOBOMA AND CONTRALATERAL OPTIC DISK PIT MACULOPATHY TREATED BY VITRECTOMY IN A PATIENT WITH NOONAN SYNDROME WITH MULTIPLE LENTIGINES.

PURPOSE: To report a case of Noonan syndrome with multiple lentigines with unusual ocular features. METHODS: The authors describe a case of a 7-year-old girl with Noonan syndrome with multiple lentigines and anomalous optic disks. RESULTS: A 7-year-old girl with genetically proven Noonan syndrome with multiple lentigines ( PTPN11 gene mutation) and anomalous optic disks was referred for treatment of persistent macular detachment after 1 year of conservative follow-up. The right eye demonstrated an optic disk coloboma with the best-corrected visual acuity of 20/32, the left eye demonstrated an optic disk pit with serous macular detachment (best-corrected visual acuity 20/50-20/80). Optical coherence tomography demonstrated a neurosensory detachment. Twenty-five gauge pars plana vitrectomy was performed with posterior hyaloid detachment, drainage over disk pit area, and SF6 20% gas tamponade. Surgery resulted in subretinal fluid reduction and improvement of the visual acuity to 20/32. CONCLUSION: A case of Noonan syndrome with multiple lentigines with optic disk coloboma in the right eye and optic disk pit with related maculopathy in the left eye. To the best of the authors' knowledge, this is the first reported case describing the association of Noonan syndrome with multiple lentigines and congenital optic disk anomalies. Optic disk pit maculopathy was managed surgically because of its longstanding nature with the deteriorating visual acuity.

A CASE OF TRAUMATIC SOFT CONTACT LENS DISLOCATION INTO THE VITREOUS SPACE AFTER CORNEAL PERFORATION IN A PHAKIC EYE: A NOVEL INDICATION FOR MEMBRANEBLUE-DUAL.

PURPOSE: To describe a case of traumatic soft contact lens dislocation into the vitreous space after a large-sized corneal perforation with a clout nail, causing aniridia but leaving the crystalline lens intact. METHODS: A case report of a 44-year-old male patient with a corneal perforation. RESULTS: The patient presented with a traumatic corneal perforation after a large clout nail impacted on his left eye. Aniridia and inferior zonulolysis were seen, but the crystalline lens was perfectly clear. Because computed tomography imaging and B-scan ultrasonography showed no signs of intraocular foreign body, a vitrectomy was performed seven days after primary repair to allow for proper wound closure. During vitrectomy, a completely intact soft contact lens was found in the vitreous cavity. The soft contact lens was stained with MembraneBlue-Dual (D.O.R.C., the Netherlands) to enhance visualization and ensure complete removal by the vitreous cutter. CONCLUSION: MembraneBlue-Dual can be used to facilitate complete removal of a clear and otherwise difficult to see corneal contact lens from the vitreous cavity. This case also demonstrates the importance of exploratory vitrectomy in all trauma cases with posterior segment involvement, even when imaging modalities show no signs of intraocular foreign bodies.

Review of Small Gauge Vitrectomy: Progress and Innovations.

Purpose. To summarise the surgical advances and evolution of small gauge vitrectomy and discuss its principles and application in modern vitreoretinal surgery. The advent of microincisional vitrectomy systems (MIVS) has created a paradigm shift away from twenty-gauge vitrectomy systems, which have been the gold standard in the surgical management of vitreoretinal diseases for over thirty years. Advances in biomedical engineering and surgical techniques have overcome the technical hurdles of shifting to smaller gauge instrumentation and sutureless surgery, improving surgical capabilities and expanding the indications for MIVS.

Macular translocation with 360 degree retinotomy for management of age-related macular degeneration with subfoveal choroidal neovascularization.

PURPOSE: The aim of this study is to evaluate the functional outcome in a group of patients treated with full macular translocation (FMT) with 360-degree retinotomy for treatment of age-related macular degeneration (ARMD) with subfoveal choroidal neovascularization. DESIGN: Consecutive interventional case series. METHODS: Fifty consecutive eyes (50 patients) with ARMD and subfoveal neovascularization who underwent a FMT in our department from January 1999 to July 2000 are included in this study. Compensatory muscle surgery, as described by Eckardt and associates, was performed on all the eyes. The median follow-up is 21 months (range, 12 to 36; SD, 5.4). RESULTS: The best-corrected postoperative visual acuity (BCVA) was improved by 2 or more Snellen lines in 33 eyes (66%) and remained stable (+/-1 line) in 14 eyes (28%). Only 3 eyes (6%) experienced a deterioration of the BCVA of 2 or more lines. The final BCVA was 20/50 or better in 32% of the cases; only 8 eyes (16%) had a final BCVA < 20/200. Thirty-four (68%) patients are able to read newspaper print (3.3/10) with normal (+3 diopters to +4 diopters) or increased (+5 diopters to +8 diopters) reading ads. Other patients are able to read with magnifying systems. Complications included proliferative vitreoretinopathy (PVR) in 9 eyes (18%), recurrent choroidal neovascularization in 5 eyes (10%), diplopia in 3 eyes (6%), choroidal hemorrhage in 2 eyes (4%), macular hole in 1 eye, and temporary hypotony in 1 eye. CONCLUSIONS: As 68% of the patients in the study group regained reading vision with reading glasses, FMT can be considered an effective approach in cases of subfoveal choroidal neovascularization. Further investigations are necessary to determine which patients will have the most benefit from this complex therapeutic method.

Proliferative vitreoretinopathy.

Proliferative vitreoretinopathy is a sophisticated disease that complicates vitreoretinal pathologies like retinal detachments. Since its first description in the 1960s, we have learned a lot about this pathological entity; however, despite a large number of promising laboratory and clinical pharmacological research projects, we are still unable to stop proliferation and are certainly not able to prevent this terrible complication. As a result, vitreoretinal surgeons still have to fight membrane formation in complicated and long vitreoretinal procedures. Fortunately, a revolution has taken place in equipment for these procedures. This chapter will examine the pathophysiology, classification, and pharmacotherapy, and describe the strategy and rationale of how to deal with this recurrent condition and to limit and contain its sequelae.

Early effects of intravitreal triamcinolone acetonide on inflammation and proliferation in human choroidal neovascularization.

OBJECTIVE: To evaluate the early effects of triamcinolone acetonide (TA) on inflammation, proliferation, and vascular endothelial growth factor (VEGF) in human choroidal neovascularization (CNV). METHODS: Retrospective review of an interventional case series of 29 patients who underwent macular translocation. Fourteen CNV membranes without previous therapy (control CNV group) and 4 CNV membranes excised 3 days after photodynamic therapy (PDT CNV group) comprised the control groups. Eleven patients were treated with intravitreal TA (TA CNV group; n = 5) or PDT and TA combined (PDT+TA CNV group; n = 6) 3 to 9 days preoperatively. The CNV membranes were stained for cytokeratin 18, CD34, VEGF, intercellular adhesion molecule-1 (ICAM-1), E-selectin, CD68, CD45, Ki-67, and Thy-1. RESULTS: Treatment with TA and PDT+TA resulted in increased immunostaining of ICAM-1 in endothelial cells and the stroma and a higher percentage of Thy-1 expression than controls. The density of macrophages was significantly increased in PDT+TA CNV membranes. Leukocyte density and proliferative activity were lower in TA and PDT+TA CNV membranes. The total VEGF score was significantly increased in TA and PDT+TA CNV membranes compared with the control CNV membranes. Evidence of VEGF in the retinal pigment epithelium of PDT+TA CNV membranes was stronger than in control CNV membranes. CONCLUSIONS: Triamcinolone acetonide has no inhibitory effect on macrophage infiltration or ICAM-1, Thy-1, or VEGF expression in CNV membranes in the early term. The clinical benefits of TA are probably not based on pure antiinflammatory or VEGF-suppressing mechanisms.

Effect of bevacizumab on inflammation and proliferation in human choroidal neovascularization.

OBJECTIVE: To evaluate the effect of bevacizumab (Avastin; Genentech, Inc, South San Francisco, California) on inflammation and proliferation in human choroidal neovascularization (CNV) secondary to age-related macular degeneration. METHODS: Retrospective review of interventional series of 38 patients who underwent choroidal neaovascular membrane (CNVM) extraction. Twenty-four patients received intravitreal bevacizumab 1 to 154 days preoperatively (bevacizumab CNV group). Fourteen patients received no preoperative therapy (control CNV group). The CNVM were stained for cytokeratin 18, CD68, CD45, intercellular adhesion molecule (ICAM)-1, E-selectin, Ki-67, Thy-1, and endostatin. RESULTS: No significant difference was detected in ICAM-1 and E-selectin expression between groups. The density of leukocytes in the bevacizumab CNV group (median, 271.61 cells/mm(2)) was higher than in the control CNV group (median, 116.87 cells/mm(2); P = .07), but without significance. Density of macrophages (median, 4661.95 cells/mm(2)), proliferative activity (median, 160.19 cells/mm(2)), and percentage of Thy-1-expressing vessels (median, 100%) were significantly higher in the bevacizumab CNV group than in the control CNV group (median, 882.66 cells/mm(2), P < .001; median, 34.34 cells/mm(2), P < .001; and median, 80%, P < .001, respectively). Endostatin immunoreactivity was considerably stronger in the retina pigment epithelium (RPE)-Bruch membrane complex (median, 3; range, 2-3; P < .001), and stroma (median, 3; range, 1-3; P < .001) of the bevacizumab CNV group than control CNV group (median, 1.5; range, 0-3 and median, 1; range, 0-3, respectively). CONCLUSIONS: Unexpectedly, CNVM from patients treated by bevacizumab are characterized by significantly high inflammatory and proliferative activity and enhanced endostatin expression. These characteristics need to be considered when protocols for combination therapies are established.