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Ectopic Cerebrospinal-like Fluid From Retrobulbar Cysts as a Possible Cause of Pediatric Retinal Detachment Associated With Optic Disc Coloboma:  New Implications for Management

Chetan K. Patel, FRCOphth; Simon J. Berg, FRCA; Gerardine Quaghebeur, FRCR; Peter Richards, FRCS; Tengku A. Kamalden, MRCOphth; Rosemary Brennan, FRCOphth; Andrew Elliot, FRCOphth; Namir Kafil-Hussain, FRCOphth; Cathy Williams, FRCOphth; Viki Worthington, BSc, PhD
Arch Ophthalmol. 2012;130(8):1065-1067. doi:10.1001/archophthalmol.2012.516.
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Extract

Retinal detachment in adults with posterior segment coloboma is thought to be rhegmatogenous. Vitrectomy techniques are therefore used therapeutically. There is indirect evidence that subretinal fluid in retinal detachment, associated with optic nerve cavitation, is cerebrospinal fluid (CSF).1 We report 2 pediatric cases, referred for the management of coloboma-related retinal detachment, that provide direct evidence that the fluid is like CSF.

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Figures

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Grahic Jump Location

Figure 1. Retcam images of case 1 showing the left eye at 33 (A), 38 (B), and 41 (C) weeks' gestational age developing subtotal retinal detachment and the right eye (D) with disc and chorioretinal detachment without retinal detachment.

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Grahic Jump Location

Figure 2. Retcam images of case 2 at 4 months of age showing retinal detachment in the right eye (A) and an attached retina in the left eye (B). The left eye developed subtotal retinal detachment 6 months later (C) associated with closure of excavation (compare the black arrows in parts B and C), pigmentary retinopathy (C, white arrow), and herniation of the retina into the excavation (C, green line). Corresponding optical coherence tomography in the left eye (D).

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Grahic Jump Location

Figure 3. Unenhanced axial (A) and coronal (C) computed tomographic scans with Omnipaque-assisted cisternography (B) showing that cerebrospinal fluid failed to track beyond the optic chiasm (B) into the subretinal space of the left eye in case 1. T1-weighted (D) and T2-weighted (E) axial and coronal fat-suppressed (F) magnetic resonance imaging sequences confirm bilateral retinal detachments and retrobulbar cysts intimately related to the entrance of the optic nerve into the eye, similar to the left eye of case 1.

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