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Year : 2020  |  Volume : 68  |  Issue : 5  |  Page : 904-906

Posterior keratoconus in a patient with familial exudative vitreoretinopathy

Dr Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi, India

Date of Submission06-Oct-2019
Date of Acceptance21-Nov-2019
Date of Web Publication20-Apr-2020

Correspondence Address:
Dr. Pulak Agarwal
Dr Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, Ansari Nagar, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1790_19

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Keywords: Avascular retina, familial exudative vitreoretinopathy, keratoconus, retinal detachment

How to cite this article:
Agarwal P, Kumar V, Azad S, Dhull C. Posterior keratoconus in a patient with familial exudative vitreoretinopathy. Indian J Ophthalmol 2020;68:904-6

How to cite this URL:
Agarwal P, Kumar V, Azad S, Dhull C. Posterior keratoconus in a patient with familial exudative vitreoretinopathy. Indian J Ophthalmol [serial online] 2020 [cited 2020 May 26];68:904-6. Available from: http://www.ijo.in/text.asp?2020/68/5/904/282945

Posterior keratoconus is a rare nonprogressive noninflammatory condition unlike anterior keratoconus. It can be congenital or acquired, sporadic or familial, unilateral or bilateral. Bilateral sporadic posterior keratoconus is an extremely rare entity (as bilateral cases are predominantly familial). It is associated with many systemic and ocular features. Various posterior segment manifestations are described in association with posterior keratoconus. We describe a case of FEVR in the setting of bilateral sporadic posterior keratoconus. An occurrence was not reported previously.

A 17-year-old male presented with gradual painless diminution of vision OU for 2 years and nystagmus since birth. There was no history of trauma or ocular surgery. Family history was noncontributory.

Best-corrected visual acuity (BCVA) was 20/600 in OD and 20/1200 in OS. Deep-set eyeballs with horizontal pendular nystagmus of low frequency and low amplitude were noted. Slit-lamp examination revealed OU localized depression of posterior corneal curvature measuring 4 mm in diameter suggestive of posterior keratoconus [Figure 1]a and [Figure 1]d, which was confirmed on anterior segment OCT [Figure 1]b and [Figure 1]e. Typical oil droplet reflex was noted on retro-illumination [Figure 1]c and [Figure 1]f. Rest of the anterior segment was normal in OU.
Figure 1: (a-c) slit-lamp photograph of OD showing posterior keratoconus, anterior segment OCT of OD showing posterior keratoconus, clinical photograph in retroillumination. (d-f) slit-lamp photograph of OS showing posterior keratoconus, anterior segment OCT of OS showing posterior keratoconus, clinical photograph in retroillumination

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Dilated fundus examination of OU revealed small, tilted optic disc with gliotic changes on its surface. Old rhegmatogenous retinal detachment was seen along with multiple subretinal bands [Figure 2]a and [Figure 2]b in bilateral eyes. Tortuous retinal vessels with supernumerary branches ending short of equator were noted, leaving peripheral retina avascular. These findings were confirmed on UWFA [Figure 2]c and [Figure 2]d. In addition, peripheral lattice degeneration in temporal retina with multiple holes was observed OU. Based on clinical and diagnostic evidences, patient was diagnosed to be a case of both eye posterior keratoconus with associated FEVR and RD.
Figure 2: (a and b) wide-field clinical photograph of fundus OD showing attached retina with temporal buckle indent with lattice with hole with temporal avascular retina, FFA showing temporal avascular retina. (c and d) wide-field clinical photograph of fundus of OS showing retinal detachment with subretinal band with temporal avascular retina, FFA showing temporal avascular retina

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After informed consent, the patient underwent scleral buckling surgery in OU sequentially. One month postoperatively, BCVA improved to 20/120 in OD and 20/200 in OS with retina attached. The patient was advised regular follow-up.

  Discussion Top

Posterior keratoconus is developmental in most cases and is due to anterior chamber cleavage anomaly.[1] Acquired causes, on the other hand, are rare and mostly post trauma.[2] There is constellation of posterior segment features quoted in the literature that can occur along with posterior keratoconus with undetermined genetic linkage.[3],[4],[5],[6],[7],[8] Our patient had a unique association of bilateral sporadic posterior keratoconus with FEVR, which to our knowledge never reported earlier. Atypical presentation of FEVR with associated retinal detachment and subtle posterior keratoconus makes our case a diagnostic challenge.

To conclude, FEVR can occur in the setting of posterior keratoconus.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Rao SK, Padmanabhan P. Posterior keratoconus. An expanded classification scheme based on corneal topography. Ophthalmology 1998;105:1205-12.  Back to cited text no. 1
Bareja U, Vajpayee RB. Posterior keratoconus due to iron nail injury--a case report. Indian J Ophthalmol 1991;39:30.  Back to cited text no. 2
[PUBMED]  [Full text]  
Silas MR, Hilkert SM, Reidy JJ, Farooq AV. Posterior keratoconus. Br J Ophthalmol 2018;102:863-7.  Back to cited text no. 3
Rejdak R, Nowomiejska K, Haszcz D, Jünemann AG. Bilateral circumscribed posterior keratoconus: Visualization by ultrasound biomicroscopy and slit-scanning topography analysis. J Ophthalmol 2012;2012:587075. doi: 10.1155/2012/587075.  Back to cited text no. 4
Streeten BW, Karpik AG, Spitzer KH. Posterior keratoconus associated with systemic abnormalities. Arch Ophthalmol 1983;101:616-22.  Back to cited text no. 5
Lonsberry BB, Boyce P. Circumscribed posterior keratoconus: Case report. J Am Optom Assoc 1999;70:773-6.  Back to cited text no. 6
Wolter JR, Haney WP. Histopathology of keratoconus posticus circumscriptus. Arch Ophthalmol 1963;69:357-62.  Back to cited text no. 7
Gupta R, Jinagal J, Khurana S, Gupta PC, Ram J. A rare case of bilateral circumscribed posterior keratoconus in microcornea associated with unilateral irido-fundal coloboma. J Clin Exp Ophthalmol 2016;7:6.  Back to cited text no. 8


  [Figure 1], [Figure 2]


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