CONGENITAL OPTIC PIT WITH CENTRAL SEROUS RETINOPATHY

A.Shahnawaz1, Deepak Mishra2, M.A.Akbar1, P.Bhushan3

1Upgraded Dept. of Ophthalmology, DMCH, Darbhanga, Bihar A B ijcrr 2Regional Institute of Ophthalmology, Indira Gandhi Institute of Medical Vol 04 issue 02 Sciences, Patna Category: Case Report 3Dept. Of Ophthalmology, I.M.S.,B.H.U.,Varanasi, U.P. Received on:03/11/11 Revised on:15/11/11 E-mail of Corresponding Author: [email protected] Accepted on:26/11/11

ABSTRACT Title: Congenital Optic Pit with Central Serous Retinopathy Purpose. To document association of pit and Central Serous Retinopathy Method. Case report/Research article. Results The patient presented with progressive defective vision of 8 months duration in her left eye with best corrected vision of 6/60 not improving further with glasses or pinhole. Ocular examination was within normal limits except for abnormal fundus findings. Fundus examination revealed the presence of congenital optic disc pit with a serous macular elevation in the left. Conclusions. Central Serous Retinopathy should also be considered as an association of congenital Optic disc pit ______

Key Words. Optic disc pit, Central Serous these patients have a greater propensity to Retinopathy, progressive defective vision develop normal tension glaucoma, although the arcuate defects may be INTRODUCTION caused by the optic pit itself rather than Optic pits are congenital excavations of the by glaucomatous damage. head that may be associated A 27 year old female patient presented to with other abnormalities of the optic nerve our OPD with progressive defective vision and peripapillary . Optic pits occur in of 8 months duration in her left eye. Ocular about one in 10,000 people, with no gender examination revealed a best corrected predilection, and are usually sporadic. vision of 6/60 not improving further with Optic pits are usually incidental findings on glasses or pinhole. Ocular examination was fundus examination and remain within normal limits except for abnormal asymptomatic unless complicated by fundus findings. Fundus examination macular lesions such as edema, schisms or revealed the presence of congenital optic serous detachment. disc pit with a serous macular elevation in A patient with macular involvement the left eye. (Fig 1) generally presents with of Laser photocoagulation was used to worse than 20/70 in the affected eye and 80 produce several rows of laser burns percent of these eyes loose visual acuity to between the area of the serous retinal 20/200 or worse. It has been suggested that detachment and the optic disc. The

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objective was to achieve a very light white serous detachments of the retina, with laser burn with little collateral damage to associated full-thickness or laminar retinal the nerve fiber layer. This presumably holes, retinal pigment epithelium mottling creates a wall of scar tissue to block the and general cystic changes. The retinal passage of fluid from the optic pit to the detachments are usually confined between inner retinal schisis cavity and subretinal the superior and inferior vascular arcades space Vitreous surgery and internal and are contiguous with the optic disc, tamponade: Combinations of posterior sometimes through a visible isthmus of , photocoagulation and gas subretinal fluid. The elevated retina tamponade was suggested and patient was contains cystic cavities in the outer plexi- referred to higher center for treating optic form layer. pit–associated maculopathy. Successful Optical coherence tomography - OCT of macular reattachment and improved central an optic pit usually shows a schisis like vision can be achieved using vitrectomy separation between the inner and outer with induction of PVD and gas tamponade. retina and a larger . Visual field testing. Optic pits may be associated with visual field changes, which can be due to one or both of the following mechanisms: _ An optic pit, especially if large, may displace nerve fibers to produce an arcuate or may lead to an Fig.1 Fig.2 enlarged blind spot. _ Associated serous macular detachment may manifest as metamorphopsia or blurred vision, and visual fields may demonstrate central scotoma. However, unlike degenerative or reticular , there is no absolute Fig.3 Fig.4 scotoma in optic pit maculopathy. Fluorescein angiography: Fluorescein angiography is usually unremarkable in cases of optic pit. There is no dye accumulation in the area of the serous

Fig.5 detachment, although there may be late hyperfluorescence of the optic pit. It has DISCUSSION been suggested that vitreopapillary traction Congenital pits of the optic nerve head vary in this area may cause leakage from optic in size, shape, depth and location. They disc blood vessels. appear as small, hypopigmented, grayish, Electrophysiological testing: An oval or round excavated depressions in the electroretinogram (ERG) may show poorly optic nerve head. They are usually about defined and low-amplitude waveforms, 500 μm in size and may be bilateral in 10 consistent with schisis and serous to 15 percent of cases. Optic pits are most detachment. Preoperative evaluation of commonly located on the temporal side of macular function is important for the optic disc, but they may be situated predicting the likelihood of central vision centrally or anywhere along the margin of recovery after successful macular the optic disc. Optic pits along the rim of reattachment. Patients with a poor ERG the optic disc are most likely to lead to response are less likely to experience visual 33 International Journal of Current Research and Review www.ijcrr.com Vol. 04 issue 02 January 2012

acuity improvement even after anatomical source of the fluid. Brown and colleagues reattachment. suggested that there may be a connection Differential Diagnosis: between the vitreous and the submacular A dilated biomicroscopic fundus fluid, based on the findings in their canine examination is essential for differentiating model of optic pit1. Using India ink they optic pits from the following conditions: found a direct communication between the _ Optic disc anomalies such as choroidal vitreous, the optic pit and the subretinal and sclera crescent. space in three collie dogs with congenital _ Tilted disc syndrome. optic pits. _ Circumpapillary staphyloma. Two-layer separation. Serous macular _ Hypoplastic disc. detachment associated with optic pit was _ Glaucomatous optic neuropathy. (Any thought to be due to direct communication change in the appearance of the optic pit between the optic pit and the subretinal over time suggests that the lesion may be space, facilitating fluid accumulation under an acquired notch of the neuroretinal rim the macula. However, Lincoff and secondary to glaucomatous damage.) colleagues suggested that the primary _ Central serous retinopathy and subretinal communication from the optic pit may be neovascular membranes. (These conditions to the retina2. Fluid may move into the are alternative considerations for serous retina, causing a schisislike separation of macular detachment). the inner and outer layers, with the Pathophysiology neurosensory serous retinal detachment Congenital optic pits result from an occurring secondary to this schisis. Recent imperfect closure of the superior edge of OCT findings confirm this separation. the embryonic fissure. They are Vitreous traction. Vitreous traction appears asymptomatic unless complicated by to be an important factor in the secondary macular changes. They typically pathogenesis of optic pit– related macular lead to a two-layered maculopathy detachment. Traction, vitreomacular or consisting of a primary inner retinal layer vitreopapillary, may permit entry of fluid schisis and a secondary outer layer into the retina through the optic pit. detachment. Although the exact mechanism by which optic pits cause macular Management detachment is not known, various theories Patients with asymptomatic optic pits need about the source of fluid and the macular regular monitoring for the onset of any changes have been proposed, including: macular involvement. The management of Subretinal fluid. It has not been optic pits with associated macular established conclusively whether the involvement is not well defined; various subretinal fluid originates from the vitreous treatment modalities have been tried with cavity, from the subarachnoid space or variable success. Less invasive treatments from leakage from the retinal vessels like laser photocoagulation should be tried around the optic disc. Studies involving initially, followed by a combination of intrathecal fluorescein injections and vitrectomy, complete posterior vitreous histological tissue analysis have failed to detachment (PVD) induction and internal provide any evidence of the optic pit acting gas tamponade if as a conduit between the subarachnoid and symptoms persist .When the optic pit is subretinal spaces. The lack of dye leakage asymptomatic, the patient should be from retinal vessels makes it unlikely that advised about the importance of regular the retinal vasculature is the comprehensive eye exams, including

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dilated retinal evaluations and threshold limiting membrane peeling in addition to visual fields. Patients should be educated removing the posterior vitreous for about the use of home visual acuity relieving all tractional components4 . assessment and Amsler grid testing to monitor for the onset of maculopathy. They CONCLUSION should be made aware of the signs and Maculopathy caused by optic pits has an symptoms (e.g., blurred vision and overall poor prognosis, and long-term metamorphopsia) of macular studies involving large groups of these complications. patients are lacking. Given that the exact Laser photocoagulation. This is used to pathophysiology is still a matter of debate, produce one or several rows of laser burns management should be tailored to the between the area of the serous retinal visual disability and macular changes of the detachment and the optic disc. The specific patient. objective is to achieve a very light white laser burn with little collateral damage to ACKNOWLEDGEMENT the nerve fiber layer. This presumably Authors acknowledge the immense help creates a wall of scar tissue to block the received from the scholars whose articles passage of fluid from the optic pit to the are cited and included in references of this inner retinal schisis cavity and subretinal manuscript and also of the patient for his space (although the scarring may also co-operation and consent involve peripapillary retinal tissue). While studies have reported successful resolution REFERENCES of the serous detachment in eyes that have 1. Brown GC, Shields JA, Patty BE, been treated with photocoagulation, this Goldberg RE. Congenital pits of the does not always translate into improved optic nerve head.I.Experimental studies final visual outcome. in collie dogs. Arch Ophthalmol. 1979 Macular buckling. Macular buckling has Jul;97(7):1341-4. been reported as a treatment option for 2. Lincoff H, Lopez R, Kreissig I, serous detachment associated with optic Yannuzzi L, Cox M, Burton T. pit. Scleral buckling converts the posterior Retinoschisis associated with optic hyaloid traction from an inward to an nerve pits. Arch Ophthalmol. 1988 outward vector, promoting reattachment of Jan;106(1):61-7 the macula. 3. Hirakata A, Hida T, Ogasawara A, Vitreal surgery and internal tamponade. Iizuka N. Multilayered retinoschisis Combinations of posterior vitrectomy, associated with optic disc pit. Jpn J photocoagulation and gas tamponade are Ophthalmol. 2005 Sep-Oct;49(5):414-6 used for treating optic pit–associated 4. Gandorfer A, Kampik A. [Role of maculopathy. Successful macular vitreoretinal interface in the reattachment and improved central vision pathogenesis and therapy of macular can be achieved using vitrectomy with disease associated with optic pits]. induction of PVD and gas tamponade3 .A Ophthalmologe.2000,Apr;97(4):276-9. complete PVD helps relieve vitreous traction. Indeed, spontaneous macular reattachment has been observed in eyes undergoing posterior vitreous separation. Gandorfer and Kampik advocate internal

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