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Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 804-805

Spontaneous acute hydrops with intrastromal clefts in pellucid marginal corneal degeneration and its management

1 Department of Cornea, Anand Eye Institute, Hyderabad, India
2 Department of Glaucoma, Anand Eye Institute, Hyderabad, India
3 Department of Refractive Surgery, Ramesh Lasik and Laser Centre, Hyderabad, Telangana, India

Date of Submission05-Oct-2020
Date of Acceptance11-Mar-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Arjun Srirampur
Department of Cornea, Cataract and Refractive Surgery, Anand Eye Institute, Habsiguda, Hyderabad - 500 007, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_3161_20

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Keywords: Acute hydrops, descemetopexy, pellucid marginal corneal degeneration

How to cite this article:
Srirampur A, Kola P, Mansoori T, Gampa R. Spontaneous acute hydrops with intrastromal clefts in pellucid marginal corneal degeneration and its management. Indian J Ophthalmol Case Rep 2021;1:804-5

How to cite this URL:
Srirampur A, Kola P, Mansoori T, Gampa R. Spontaneous acute hydrops with intrastromal clefts in pellucid marginal corneal degeneration and its management. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 19];1:804-5. Available from: https://www.ijoreports.in/text.asp?2021/1/4/804/327662

A 36-year-old man with PMCD in both eyes presented with sudden diminution of vision in the left eye (LE) for the last 3 days.

On examination, his best-corrected visual acuity was 6/9 in the right eye (RE) and hand movements in LE. Slit-lamp examination of RE showed corneal thinning, adjacent to the inferior corneoscleral limbus. [Figure 1]a LE cornea showed diffuse stromal edema. [Figure 1]b Anterior chamber (AC) showed a detached floating membrane in the AC, suggestive of descemet's membrane detachment (DMD). Topography of the RE showed typical “clab claw” 'appearance suggestive of PMCD.
Figure 1: (a) Slitlamp image of the Right eye showing inferior corneal thinning and ectasia just above the area of corneal thinning. (b) Slitlamp image of the Left eye showing diffuse corneal edema suggestive of acute hydrops. (c) Intraoperative image of the left eye showing complete anterior chamber fill with air bubble, just before making corneal stab incisions. (d) Slitlamp image of the Left eye at 3 months follow up showing clearing of the corneal edema in the visual axis. Note area of the break of descemet's membrane (DM) (solid arrow) and a faint linear opacity at the level of the endothelium suggestive of the folds of the DM after getting attached (dotted arrow)

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Anterior segment optical coherence tomography (AS-OCT) of the RE showed stromal thinning in inferior cornea. AS-OCT of the LE showed increased corneal thickness with multiple intrastromal clefts. [Figure 2]a
Figure 2: (a) Preoperative ASOCT image of the left eye showing dense corneal edema along with numerous intrastromal fluid clefts suggestive of acute corneal hydrops. (b) Postoperative ASOCT image showing resolution of corneal edema with a well attached DM. The solid arrow indicates the break in DM just adjacent to the area of corneal thinning. Dotted arrow indicates the folds in DM corresponding to the slit lamp image

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Patient underwent descemetopexy with the injection of intracameral C3F8 gas [Figure 1]c. After a complete air fill in the AC, four venting, stab wounds with a 45° angulations in relation to the corneal plane were made into the corneal stroma using a 20-gauge microvitreoretinal (MVR) blade. As soon as MVR blade was retracted, fluid egress was noted through the venting incisions. The air was replaced by injecting 0.1 mL of isoexpansile 14% C3F8 gas.

Postoperatively, the patient was started on topical difluprednate 0.05% and moxifloxacin 0.5% one hourly. The patient was advised to maintain supine position for 3 days. The patient was seen regularly and at 3 months the corneal edema resolved with vision of 6/12. [Figure 1]d and [Figure 2]b.

  Discussion Top

Pellucid marginal corneal degeneration (PMCD) is a non-inflammatory, progressive, corneal ectasia, characterized by a peripheral band of inferior corneal thinning.[1] In advanced cases, DMD leads to an acute hydrops.[2] Only few reports mention the use of intracameral air or gas to attach the DMD in acute hydrops in PMCD.[3],[4] For the first time we describe descemetopexy along with corneal venting incisions in a case of PMCD.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

Sridhar MS, Mahesh S, Bansal AK, Nutheti R, Rao GN. Pellucid marginal corneal degeneration. Ophthalmology 2004;111:1102-7.  Back to cited text no. 1
Jeng BH, Aldave AJ, McLeod SD. Spontaneous corneal hydrops and perforation in both eyes of a patient with pellucid marginal degeneration. Cornea 2003;22:705-6.  Back to cited text no. 2
Ramamurthy B, Mittal V, Rani A, Ram M, Sangwan VS. Spontaneous hydrops in pellucid marginal degeneration: Documentation by OCT- III. Clin Experiment Ophthalmol 2006;34:616-7.  Back to cited text no. 3
Vanathi M, Behera G, Vengayil S, Panda A, Khokhar S. Intracameral SF6 injection and anterior segment OCT-based documentation for acute hydrops management in pellucid marginal corneal degeneration. Cont Lens Anterior Eye 2008;31:164-6.  Back to cited text no. 4


  [Figure 1], [Figure 2]


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