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Samantha Fordyce ICO Pediatrics/Binocular Vision Resident American Academy of Optometry Resident’s Day Submission Neovascular Glaucoma with Prominent Iris Bombe Status-Post Funnel Retinal Detachment Abstract – This case represents chronic iris neovascularization status-post funnel retinal detachment in a 22-year-old male, resulting in angle closure secondary to posterior synchiae and prominent iris bombe. Evisceration recommended preventing future phthisis bulbi. I. Case History 22yo African American Male CC: red eye, discomfort, photophobia, and occasional headaches x 1-2 months OD POHx: CRET since birth, EOM surgery as a child, degenerative myopia OD, retinal detachment longstanding 4 years prior, (-) trauma PMHx: No medical history, (-) Diabetes, (-) Hypertension, (-) automimmune disorders Medications: None Patient reports vision has been reduced OD for >1 year. II. Pertinent findings Initial Exam (7/30/15): o VA sc OD: Light Perception, NIPH OS: 20/40 PH:20/25 o EOMs: Restricted abduction OD, FROM OS o Slit Lamp Findings: 1+ injection OD Diffuse corneal edema with superficial punctate keratitis OD Angle: Grade 0, closed 360 degrees AC: possible cell/flare Iris: Significant NVI, Posterior synechiae 360 degrees, Prominent iris bombe Lens: opacified nuclear sclerosis, no red reflex present o IOP: OD: 26 OS: 18 o DFE: OD: not visible, B-scan performed OS: within normal limits, (-) vitritis, (-) neovascularization, temporal ONH pallor. o B-scan, Anterior segment OCT, and slit lamp photos taken Follow-up Exam (7/31/15): o VA sc OD: Light Perception, NIPH OS: 20/40, PH: 20/25 o EOMs: restricted abduction OD, FROM OS o Slit Lamp Findings: 1+ injection OD o Retinal o o o o o o Diffuse corneal edema with superficial punctate keratitis OD Angle: Grade 0, closed 360 degrees AC: 1+ cell/flare Iris: Significant NVI, Posterior synechiae 360 degrees, Prominent iris bombe Lens: opacified nuclear sclerosis, no red reflex present IOP: OD: 17 OS: 18 Specialist Appointment (Patient feeling discomfort again): VA sc: OD: NLP OS: 20/40, PH: 20/25 EOMs: restricted abduction OD, FROM OS Slit Lamp Findings: Mild chemosis, 1+ Injection OD Mild diffuse edema Angle: Grade 0, closed 360 degrees, iris bombe, fibroblastic membrane in angle AC: possible flare Iris: Significant NVI, Posterior synechiae 360 degrees, Prominent iris bombe Lens: yellowing IOP: OD: 34 OS: 17 No view of fundus B-scan analysis: funnel retinal detachment with possible vitreous hemorrhage OD III. Differential diagnosis Primary: Neovascular Glaucoma Others: Chronic Angle Closure Glaucoma, Acute Angle Closure Glaucoma, Inflammatory Glaucoma, Uveitis/Panuveitis IV. Diagnosis and discussion Neovascular Glaucoma o Retinal ischemia causes the release of VEGF, causing the growth of new, leaky blood vessels. These leaky blood vessels travel from the retina forward through the pupil, onto the iris and into the angle. o Due to the neovascularization of the angle, fibroblastic membranes form and block the trabecular meshwork. o Most common causes: diabetic retinopathy, central retinal vein occlusion, and branch retinal vein occlusion. o Other possible causes: ocular ischemic syndrome, tumors, chronic inflammation, chronic retinal detachment, and radiation therapy. o Signs: Possible mild anterior chamber cells and flare due to leaky blood vessels (neovascularization) Conjunctival Injection Corneal Edema with acute IOP increase Hyphema o o o o Unique o o o o o o Eversion of pupillary margin The most important is to identify the cause of neovascularization and start treating immediately. Even with IOP control, studies have shown 3-48% will still lose light perception. Stages of Neovascular Glaucoma: Stage 1: NVI present, no angle closure, no elevated IOP Stage 2: NVA, elevated pressure, decreased vision Treatment Options: Anti-VEGF injections, usually Lucentis or Avastin, or PRP to help blood vessels retract and stop growing. IOP lowering drops or orals Trabeculectomy or glaucoma drainage implant Topical steroid to decrease inflammation In severe cases (vision is already lost) cyclodestructive procedure is an option Evisceration/Enucleation Features: Dense cataract OD did not allow for a posterior pole view, therefore the conclusion of the cause of NVI is unknown. Due to the patient’s eye history and B-scan performed at initial exam, it is likely due to a chronic funnel retinal detachment more than 4 years ago. NVI can cause mild anterior chamber cells and flare. Due to longstanding and prominent NVI, cell/flare eventually caused 360 posterior synechiae and resulted in significant iris bombe and angle closure. At initial exam, same-day Laser Peripheral Iridotomy (LPI) was considered, but due to the amount of NVI and prominence of blood vessels, it could not be performed due to risk of causing hyphema. The same was decided for breaking the posterior synechiae, it likely would have resulted in a hemorrhage/hyphema. Also, an LPI is contraindicated because it is uncertain what is going on behind the iris, there could be a hemorrhage, the retinal detachment or choroid could be pushing on the iris, etc. To maintain patient comfort, IOP lowering drops were prescribed and the patient was monitored until referral appointment to analyze the cause of NVI and treatment plan could be devised. At retinal specialist appointment, IOP had increased to 34. To ensure the patient did not have a systemic autoimmune disorder such as sarcoidosis causing inflammation and NVI, OS was dilated and examined. No signs were found. Patient was not in significant pain or discomfort and vision had been decreased to count fingers from retinal detachment 4 years prior, so the main treatment plan was to maintain patient comfort until referral appointment. At retinal specialist appointment, patient was NLP. Retinal specialist recommended evisceration to prevent phthisis bulbi. If patient had visual potential or was light perception, would consider anti-VEGF injection, glaucoma shunt implant, and retinal surgery. V. Treatment, management Most important in this case was patient comfort. o IOP lowering drops: Combigan TID OD o Refer to retinal specialist for evaluation of NVI o Retinal o o o Follow-up: 1 day for IOP check IOP decreased to 17, patient felt more comfortable and in less pain. Slit lamp findings did not change. Continue Combigan BID OD until retinal specialist appointment in 1 week. Specialist Appointment: Combigan BID OD, Cyclopentolate BID OD, and Pred Forte BID OD to help improve symptoms Recommend evisceration OD, referral to oculoplastic surgeon to prevent further progression of phthisis bulbi and improve cosmesis Monitor OS annually VI. Conclusion Pathogenesis: Retinal ischemia causes the release of vascular endothelial growth factor (VEGF), causing the growth of new, leaky blood vessels. These leaky blood vessels travel from the retina forward through the pupil, onto the iris and into the angle. The blood vessels as well as the fibroblastic membranes form and block the trabecular meshwork. Most importantly, identify the cause of neovascularization and start treating immediately, such as anti-VEGF injections or PRP. Even with IOP control, 3-48% will still lose light perception. If no light perception or no visual potential of the eye, evisceration/enucleation recommended preventing phthisis bulbi and improving cosmesis. VII. References Every, S. Molteno A. Bevin, T. Herbison, P. “Long-term Results of Molteno Implant Insertion in Cases of Neovascular Glaucoma.” Archives of Ophthalmology. 2006; 124 (3): 355-360. Huang, A. “Preserving Vision in Neovascular Glaucoma: To effectively manage this disease, be sure to address the underlying causes, not just the elevated pressure.” Review of Ophthalmolgy. 2015 Jan; 56-58. Gerstenblith, A. Rabinowitz, M. The Will’s Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. Sixth Edition. City: Lippincott Williams & Wilkins, 2012. 230-232. Print. Mermoud, A. Salmon, JF. Alexander, P. Straker, C. Murray, AD. “Molteno tube implantation for neovascular glaucoma. Long-term results and factors influencing the outcome.” Ophthalmology. 1993 Jun;100(6)897-902.