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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 4  |  Page : 882-885

An unusual case of sequential corneal perforation in pellucid marginal degeneration: A case report


1 Department of Cornea and Refractive Services, The Eye Foundation Eye Hospital, Coimbatore, Tamil Nadu, India
2 Department of Cataract and Refractive Services, The Eye Foundation Eye Hospital, Coimbatore, Tamil Nadu, India

Date of Submission02-Feb-2022
Date of Acceptance13-Jun-2022
Date of Web Publication11-Oct-2022

Correspondence Address:
Dr. Shreesha Kumar Kodavoor
582-A, Diwan Bahadur Rd, R.S Puram West, Coimbatore - 641 002, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_334_22

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  Abstract 


Spontaneous and sequential corneal perforation is rare in pellucid marginal degeneration (PMD) in the seventh decade of life. Hence, the importance of repeated follow-up, clinical examination along with serial corneal topography even in the seventh and eighth decades of life is of utmost importance in PMD. We report a case of spontaneous corneal perforation in a 64-year-old male patient with PMD having normal corneal thickness in the right eye which was managed with primary suturing and compression sutures. The other eye was absolutely normal with normal topographic findings. Over a period of four years of follow-up, the patient developed progressive diminution of vision in both eyes and was diagnosed with cataract. Temporal phacoemulsification with toric IOL implantation was done in both eyes, following which the left eye developed spontaneous perforation after a period of two months which was also managed with primary suturing and compression sutures.

Keywords: Corneal collagen crosslinking, corneal perforation, pellucid marginal degeneration


How to cite this article:
Kodavoor SK, Patekar KB, Dandapani R. An unusual case of sequential corneal perforation in pellucid marginal degeneration: A case report. Indian J Ophthalmol Case Rep 2022;2:882-5

How to cite this URL:
Kodavoor SK, Patekar KB, Dandapani R. An unusual case of sequential corneal perforation in pellucid marginal degeneration: A case report. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Dec 5];2:882-5. Available from: https://www.ijoreports.in/text.asp?2022/2/4/882/358168



Pellucid marginal degeneration (PMD) is a rare, progressive, non-inflammatory ectatic disorder of the cornea.[1] This condition is more common in men in the fourth or fifth decades of life. In a majority, it occurs bilaterally; however unilateral cases have also been reported.[2] It affects the inferior cornea mainly in a crescentic form, separated from the limbus by a relatively uninvolved area of 1 to 2 mm in width, leading to corresponding steepening and thinning which is reflected as high “against the rule” astigmatism. The corneal protrusion is more marked, just superior to the area of thinning where the thickness of the cornea is usually normal.[3]


  Case Report Top


A 64-year-old male patient presented to the clinic with painless progressive diminution of vision in the right eye since one month and was found to have a UCVA of 3/60 in the right eye (OD) and 6/6 in the left eye (OS). With +7.00 Diopter sphere (DS) / -12.00 Diopter Cylinder (DC) × 65°, the visual acuity improved to 6/18 in OD. On examination, a crescentic arcuate reflex was seen 2 mm away from the limbus extending from 4 o' clock to 8 o'clock in the right eye. Fellow eye was normal on clinical examination. Corneal topography (Pentacam, OCULUS Optikgerate Gmbh) showed the “crab claw sign” with against-the-rule astigmatism in OD. K1 was 37.90 D and K2 was 51.10 D as shown in [Figure 1]a. Left eye was completely normal with normal topography as shown in [Figure 1]b. Patient was diagnosed with PMD in the right eye and scleral contact lens was advised, but patient did not use it and preferred spectacles. There was no history of any systemic illness and necessary blood tests were done to rule out collagen vascular disorder.
Figure 1: (a) Topographic image of the right eye at initial presentation. (b) Topographic image of the left eye at initial presentation

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Four months later, patient presented with sudden onset of pain and diminution of vision in the right eye and was diagnosed with full thickness corneal perforation with iris prolapse, as shown in [Figure 2]a, and was repaired with two primary sutures and additional compression sutures with 10-0 nylon with iris repositioning as shown in [Figure 2]b.
Figure 2: Slit-lamp image of (a) spontaneous perforation with iris prolapse in right eye. (b) Postoperative picture of right eye with compression sutures. (c) Topographic findings of right eye 1 month after the repair. (d) Topographic findings of left eye

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One-month post-op showed UCVA of 6/18 in OD improving to 6/9 with + 2.25 DS/−2.75 DC × 90°. OS UCVA was 6/6. On topography, K1 and K2 values of the right eye were 42.3 and 45.8, respectively, as shown in [Figure 2]c Left eye was completely normal as shown in [Figure 2]d.

Over a period of four years of follow-up, the patient developed progressive diminution of vision in both eyes and was diagnosed with cataract. OD showed UCVA of 1/60, not improving with refraction and OS showed UCVA of 6/9 not improving further. Keratometry readings were 39.4 D/50.2 D OD and 40.7 D/46.4 D OS, as shown in [Figure 3]a and [Figure 3]b.
Figure 3: (a) Topographic image of right eye after 4 years of follow-up. (b) Topographic image of left eye after 4 years of follow-up

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Patient was then operated for cataract in both eyes. Phacoemulsification with supero- temporal clear corneal incision was done and customized toric IOL was implanted in the bag; one-month post-op showed visual acuity of 6/36 improving to 6/9 with +2 DS/−1.25 DC × 90° in OD, and 5/60 improving to 6/9 with +2.5 DS/−5.5 DC × 130° in OS.

Two months after the phacoemulsification, the patient complained of redness, pain and diminution of vision in left eye and was diagnosed with left-sided infero-temporal perforation with shallow anterior chamber and iris incarceration, as shown in [Figure 4]a and [Figure 4]b; it was repaired with two primary sutures and additional compression sutures with iris repositioning, as shown in [Figure 4]c. Last visit showed UCVA of 6/24 in OD improving to 6/9 with +1 DS/−1.25 DC × 90°, and OS 6/24 improving to 6/9 with +1 DS/−1.75 DC × 135°.
Figure 4: Slit-lamp image of (a) left eye perforation at 6 o' clock position. (b) ASOCT showing left eye perforation. (c) Postoperative picture with compression sutures of left eye

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  Discussion Top


Spontaneous perforation and sequential eye involvement without any predisposing factor or systemic association is rare in PMD. Unilateral perforation cases should be watched closely for the development of progression in the fellow eye. Lucarelli et al.[1] were the first to report spontaneous perforation in PMD. Forooghian et al.[4] reported the case of sequential spontaneous corneal perforation in a patient with PMD with corneal perforation occurring a year apart between the affected eyes.

This case documents the very unusual presentation of progression of disease in the seventh decade of life. Aging is associated with increased non-enzymatic CXL that leads to increased corneal rigidity and prevents progression. Randleman et al.[5] suggested that age-related crosslinks might be protective against keratoconus. A potential for severe ocular morbidity should be considered in patients irrespective of age, despite a seemingly stable disease. Combining treatments such as intrastromal corneal ring segment (ICRS), CXL and refractive surgery is promising, but additional studies are needed to investigate their efficacy and safety. CXL is a proven modality in preventing progression of PMD, but there is paucity of literature on its efficacy in elderly patients. Steppat et al.[6] did not note any side effects and/or progression of the disease after an eighteen-month follow-up with 8 PMD patients treated with CXL.

Tuffaha, et al.[7] presented a case of progressive PMD treated with CXL after a previous unsuccessful implantation of intracorneal segments. After one year, an increase in corneal biomechanical properties accompanied by improvement in visual acuity was observed.

In our case, supero-temporal clear corneal phacoemulsification was done with 2.8-mm incision, and inferior side port incision was taken at the limbus away from the PMD thinned areas. We preferred phacoemulsification over small incision cataract surgery (SICS), as SICS may induce postoperative astigmatism and have unpredictable results, and patient preferred toric IOL implantation.

The study conducted by A Balestrazzi et al.[8] also showed that phacoemulsification with 2.2-mm clear corneal micro incision with implantation of hydrophobic toric IOL was a safe and effective surgical procedure to correct mild-to-moderately stable PMD.

CXL prior to cataract surgery was one of the options that could have been tried in this case, for strengthening the cornea and halting the progression of disease. Postoperative non-steroidal anti-inflammatory drugs (NSAIDS) can be avoided, as NSAID-induced corneal melt has been reported in compromised cornea.[9]


  Conclusion Top


Routine follow up of PMD cases is important in order to know the trend of the disease, as both eyes may get involved over a period of time and spontaneous perforation may take place even in the seventh decade of life.

The intent of this report was to raise awareness regarding the potential for severe vision-threatening complication associated with PMD even in a previously documented normal or stable eye, as in our case. There were no precipitating factors like coexisting keratoconus, contact lens usage and eye rubbing, yet still the disease progressed and thinning occurred in the seventh decade of life in near normal eye, which was not anticipated.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Lucarelli MJ, Gendelman DS, Talamo JH. Hydrops and spontaneous perforation in pellucid marginal corneal degeneration. Cornea 1997;16:232-4.  Back to cited text no. 1
    
2.
Orlin SE, Sulewski ME. Spontaneous corneal perforation in pellucid marginal degeneration. CLAO J 1998;24:186-7.  Back to cited text no. 2
    
3.
Shreesha Kumar K, Bijita D, Ramamoorthy D. An unusual presentation in pellucid marginal degeneration. JCRS Online Case Rep 2020;8:e00012.  Back to cited text no. 3
    
4.
Forooghian F, Assaad D, Dixon W. Successful conservative management of hydrops with perforation in pellucid marginal degeneration. Can J Ophthalmol 2006;41:74-7.  Back to cited text no. 4
    
5.
Randleman JB, Woodward M, Lynn MJ, Stulting RD. 2008.  Back to cited text no. 5
    
6.
Steppat MH, Raiskup F, Spoerl E. Collagen cross linking in patients with pellucid marginal degeneration. In Proceedings of the Association for Research in Vision and Ophthalmology Meeting, Ft Lauderdale, Fla, USA, April-May 2008.  Back to cited text no. 6
    
7.
Tuffaha BT, Alio JL, Piñero DP. Cross-linking for the management of pellucid marginal degeneration in a case of an unsuccessful implantation of intracorneal ring segments. Int J Keratoconus Ectatic Corneal Dis 2012;1:120-4.  Back to cited text no. 7
    
8.
Balestrazzi, A, Baiocchi S, Balestrazzi A, Cartocci G, Tosi GM, Martone G, et al. Mini-incision cataract surgery and toric lens implantation for the reduction of high myopic astigmatism in patients with pellucid marginal degeneration. Eye (Lond) 2015;29:637-42.  Back to cited text no. 8
    
9.
Rigas B, Huang W, Honkanen R. NSAID-induced corneal melt: Clinical importance, pathogenesis, and risk mitigation. Surv Ophthalmol 2020;65:1-11.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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