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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 57-58

Mooren's ulcer with nonrhegmatogenous Descemet's membrane detachment (type-1), persisting after cataract surgery with clear cornea


1 Consultant, Department of Ophthalmology, Wagh Eye Clinic and Lasik Centre, Ghole Road, Shivaji Nagar; Grant Medical Foundation, Ruby Hall, Pune, Maharashtra, India
2 Department of Ophthalmology, Section of Academic Ophthalmology, Division of Clinical Neuroscience, University of Nottingham, Nottingham, UK

Date of Submission21-Feb-2021
Date of Acceptance17-Jun-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Dr. Sangeeta Wagh
Consultant Dept. of Ophthalmology, Wagh Eye Clinic and Lasik Centre, 1194 Ghole Road, Shivaji Nagar, Pune - 411 005, Maharashtra; Grant Medical Foundation, Ruby Hall, Pune - 411 001 Clinic, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_387_21

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  Abstract 


Acute hydrops is very rarely reported in the setting of Mooren's ulcer. Our patient with bilateral Mooren's ulcer, being managed with immunomodulation, presented with sudden decrease in vision in the left eye, and was diagnosed to have acute hydrops with a type1 non-rhegmatogenous pre-Descemet's layer and Descemet's membrane detachment.(DMD) Her corneal stromal edema resolved with conservative management and she regained a vision of 20/80 following cataract surgery, with an evident persistent DMD and a clear cornea.

Keywords: Mooren's ulcer, Descemet's Detachment, Hydrops, Dua's layer


How to cite this article:
Wagh S, Dua HS. Mooren's ulcer with nonrhegmatogenous Descemet's membrane detachment (type-1), persisting after cataract surgery with clear cornea. Indian J Ophthalmol Case Rep 2022;2:57-8

How to cite this URL:
Wagh S, Dua HS. Mooren's ulcer with nonrhegmatogenous Descemet's membrane detachment (type-1), persisting after cataract surgery with clear cornea. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Jan 19];2:57-8. Available from: https://www.ijoreports.in/text.asp?2022/2/1/57/334948



Acute hydrops typically occurs in keratoconus as a result of rupture in the Descemet's membrane and the pre-Descemet's layer (PDL). Herein we present a case of bilateral Mooren's ulcer with a type 1 Descemet's membrane detachmentt (DMD) in one eye.


  Case Report Top


A woman in her 50s with high myopia and bilateral Mooren's ulceration was treated with oral methotrexate and topical steroids. Her vision was 20/20 in the right eye and 20/200 in the left [Figure 1]a. She presented 12 weeks from diagnosis, with sudden loss of vision in the left eye, down to “hand motion” with marked central corneal edema. A DMD spread taut like a trampoline, extending for 6–7 mm across the corneal center [Figure 1]b, was noted. Conservative management with and immunosuppression was continued. Three months later, the eye was quiet, the cornea had cleared with resolution of edema [Figure 1]c. The vision was 20/200. The DMD persisted [Figure 1]d with some superficial stromal haze [Figure 1]c.
Figure 1: Active Mooren's ulcer (peripheral ulcerative keratitis) left eye, 270 degrees with an overhanging edge. (a) Corneal edema with type-I Descemet's membrane detachment (DMD). (b) Persistent DMD in spite of resolution of edema (c). Clear cornea, with DMD, persistent DM folds and cataract (d)

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Two years from presentation, she underwent phacoemulsification with intraocular lens implant in the right eye and attained BCVA of 20/20. Three years later, she presented with cataract in the left eye. The DMD had persisted as a taut membrane. She underwent an uneventful left phacoemulsification procedure without endothelial keratoplasty. Postoperatively, the DMD could be seen on the slit lamp as a taut membrane and on optical coherence tomography (OCT) as a “chord of a circle” [Figure 2]a and [Figure 2]b. BCVA in the left eye was 20/80. At last follow-up 6 years following cataract surgery, vision was maintained and the DMD persisted [Figure 2]c. On specular microscopy, endothelial cell morphology could be vaguely discerned in the left cornea [Figure 2]d but was normal on the right.
Figure 2: Persistent DMD after phacoemulsification (a). OCT showing classic type-I DMD (b). Six years postcataract surgery under the DMD. The type-I DMD persists with overlying cornea of normal thickness. (c) Specular endothelial microscopy demonstrating indistinct outline of endothelial cells on the posterior corneal surface (d)

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  Discussion Top


Mooren's ulcer is an chronic progressive ulceration of the peripheral cornea, not associated with any systemic condition.[1] It results from an (auto)immune response to an altered or normal corneal antigen.[1] A case of “acute hydrops” in Mooren's ulcer was described by Acharya et al.[2], which resolved on injection of perfluoropropane. Acute hydrops associated with keratoconus is known to be associated with a rupture in the DM and PDL (Dua's layer)[3],[4] in the context of the altered collagen and ground substance in the corneal stroma.[5] Extensive corneal edema of rapid onset and acute hydrops are not the same clinical entity. In acute hydrops, distinct fluid lacunae appear in the edematous cornea due to rapid ingress of aqueous through demonstrable detachment with tears in DM and PDL. In rapid-onset corneal edema, there is diffuse hydration of the stroma with no fluid lacunae, secondary to endothelial dysfunction, which may be associated with DMD without a tear as demonstrated in this and the previous case.[2]

The clinical and OCT appearance of the DMD in this case has the characteristics of a type-1 DMD wherein both the PDL and DM are detached.[6] However, it is not clear how the corneal edema resolved despite persistence of the DMD. In acute hydrops, endothelial cells migrate from the torn edges of the DM and cover the exposed stroma between the torn edges. These cells then produce a new basement membrane and restore endothelial cell function in the gap created by the tear, with resolution of the hydrops. In Acharya's case,[2] DMD was presumed and treated with intracameral injection of C3F8 gas. In our case, though DMD was clearly seen, there was no demonstrable tear making it difficult to understand how endothelial cells could have covered the posterior stroma from where the DM and PDL had detached. One has to presume that a small tear existed, from where the cells could access the posterior stromal surface.


  Conclusion Top


This case illustrates that resolution of stromal edema can occur despite persistence of a nonrhegmatogenous DMD. It is expected that interventions such as gas tamponade and endothelial keratoplasty can achieve this more quickly.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Garg P, Sangwan VS. Mooren's ulcer. In: Krachmer JH, editor. Cornea. 3rd ed. Philadelphia: Mosby Elsevier; 2011. p. 937-8.  Back to cited text no. 1
    
2.
Acharya M, Dave A, Mathur U. Unusual case of acute corneal hydrops in Mooren's ulcer treated with intracameral injection of perfluoropropane. Middle East Afr J Ophthalmol 2017;24:106-8.  Back to cited text no. 2
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3.
Dua HS, Faraj LA, Said DG. Dua's layer: Discovery, characteristics, clinical applications, controversy and potential relevance to glaucoma. Expert Rev Ophthalmol 2015;10:531-47.  Back to cited text no. 3
    
4.
Parker J, Birbal RS, van Dijk K, Oellerich S, Dapena I, Melles GRJ. Are descemet membrane ruptures the root cause of corneal hydrops in keratoconic Eyes? Am J Ophthalmol 2019;205:204-5.  Back to cited text no. 4
    
5.
Ting DSJ, Said DG, Dua HS. Are descemet membrane ruptures the root cause of corneal hydrops in keratoconic eyes? Am J Ophthalmol 2019;205:204.  Back to cited text no. 5
    
6.
Dua HS, Sinha R, D'Souza S, Potgieter F, Ross A, Kenawy M, et al. 'Descemet's membrane detachment': A novel concept in diagnosis and classification. Am J Ophthalmol 2020;218:84-98.  Back to cited text no. 6
    


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