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Transcript
Case History: • A 32 yo WM presents with dull aches OU associated with high intraocular pressure OD • Medical History: Atrial septal defect • Ocular History: Congenital retinal disinsertion syndrome and cataract OD • No systemic medications • Ocular medication: unknown glaucoma drops non-compliant to dosing frequency Pertinent Findings: BCVA: OD LP and OS 20/400 with nystagmus Pupils: 1+APD OD with anisocoria and unequal size in bright and dim illumination Exophthalmometry: 15/16 mm OD/OS • Biomicroscopy: OD: Iris rubeosis with PAS, dense opacified lens material gravitated in posterior chamber, narrow Van Herick angles • Goldmann: 52/16 mmHg • Gonioscopy: OD: open to schwalbe’s superior/inferior and anterior TM temporal/nasal OS: open to posterior TM superior/inferior/temporal and anterior TM nasal. • Posterior Segment: OD: Obstructed abnormal disc appearance with retinal vascular stalk off optic nerve accompanied by hyperplasia and peripheral chorioretinal atrophy. OS: Pale nerve, significant vascular ischemia, hyperplasia 360, peripheral chorioretinal atrophy, pigmented hole and atrophic hole inferior temporally. Differential Diagnosis: • Congenital retinal disinsertion syndrome with narrow angle glaucoma OD and gyrate atrophy OU • Persistent hyperplastic primary vitreous with narrow angle glaucoma OD and gyrate atrophy OU • Retinitis Pigmentosa OU with narrow angle glaucoma OD and gyrate atrophy OU Diagnosis and Discussion: •Congenital retinal disinsertion syndrome is caused by the failure of the invaginating anterior optic cup to contact the posterior layer of the retina resulting in a fluid filled space. • Ocular findings relate to an enucleated eye from a two-month old infant with glaucoma found to contain two developmental anomalies which have opposite effects on ocular size: congenital nonattachment of the retina and dysplasia of the chamber angle (Foos et al) •Rubeosis irides has been described as concomitant of retinal detachment and a similar, but still unknown, mechanism may have been operative in this case of congenital retinal disinsertion syndrome. (Foos et al) • Two groups have been identified for the above diagnosis. Our patient has the best fit for group two. It includes healthy children with a unilateral detachment often associated with microphthalmos and cataracts (Boniuk and Hittner) Ocular Management: •Treatment: Simbrinza OD TID with follow-up schedule in 4 days for IOP check. Advised that use of drops are prescribed to keep eye pain free. Follow patient for routine IOP check •OCT, fundus photography, B-scan and referral to low vision. Conclusion: Although correction of an underdeveloped eye is not possible, proper referral for use of low vision devices and rehabilitation can help maximize daily activities. References: Foos RY, Kiechler RJ, Allen RA. Congenital nonattachment of the retina. Am J Ophthalmol 1968:65:208, 210 Boniuk M, Hittner HM. Congenital retinal disinsertion syndrome. Trans Am Acad Ophthalmol Otolaryngol 1975;79:833