Therapeutic keratoplasty in a case of fungal keratitis with lattice dystrophy

V Nishant Janardhana Raju; Shashidhar Banigallapati

doi:10.4103/tjosr.tjosr_64_21

CASE REPORT

Year : 2022 | Volume : 60 | Issue : 1 | Page : 92-95

Therapeutic keratoplasty in a case of fungal keratitis with lattice dystrophy

V Nishant Janardhana Raju, Shashidhar Banigallapati
Department of Cornea and Refractive Surgery, Sankara Eye Hospital, Guntur, Andhra Pradesh, India

Date of Submission	15-May-2021
Date of Decision	29-Dec-2021
Date of Acceptance	05-Jan-2022
Date of Web Publication	22-Mar-2022

Correspondence Address:
Dr. V Nishant Janardhana Raju
Sankara Eye Hospital, Vijayawada, Guntur Expressway, Pedakakani, Guntur - 522 509, Andhra Pradesh
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_64_21

Abstract

We report a case of fungal keratitis in a patient with lattice dystrophy who underwent successful therapeutic keratoplasty. A 30-year-old female presented with advanced microbial keratitis in left eye, with lattice dystrophy in both eyes. Scrapping revealed fungal filaments and culture grew Aspergillus flavus. With limbus-to-limbus infiltrate, the patient was posted for emergency left eye therapeutic keratoplasty. At 6-month postoperative period, the patient had a good ambulatory vision with no recurrence. Here, we try to make a point that patients with lattice dystrophy are prone to corneal erosions, making them susceptible to microbial keratitis. Patients should be made aware of this fact and counseled for immediate medical help even after trivial trauma. Therapeutic keratoplasty will be inevitable if they present at an advanced stage.

Keywords: Aspergillus flavus, fungal keratitis, lattice dystrophy, therapeutic keratoplasty

How to cite this article:
Janardhana Raju V N, Banigallapati S. Therapeutic keratoplasty in a case of fungal keratitis with lattice dystrophy. TNOA J Ophthalmic Sci Res 2022;60:92-5

How to cite this URL:
Janardhana Raju V N, Banigallapati S. Therapeutic keratoplasty in a case of fungal keratitis with lattice dystrophy. TNOA J Ophthalmic Sci Res [serial online] 2022 [cited 2022 Jul 2];60:92-5. Available from: https://www.tnoajosr.com/text.asp?2022/60/1/92/340370

Introduction

Lattice corneal dystrophy (LCD) is the most common corneal stromal dystrophy characterized by the deposition of amyloid resulting in steadily progressive loss of vision. These deposits create linear, “lattice-like” opacities arising primarily in the central cornea, while the peripheral cornea is often spared. Optical computed tomography shows atrophic epithelium, disruption of the Bowman layer, and inclusions extending from the Bowman layer into the anterior stroma sparing deep stroma, Descemet's membrane, and endothelial layers. Lattice-like opacities are radially oriented and are accompanied by gradual, superficial opacification of the cornea. Recurrent epithelial erosions are often present, causing ocular irritation and making them prone to infections.^{[1],[2],[3],[4]} Type I LCD (LCD1), also known as classic LCD or Biber-Haab-Dimmer dystrophy, is the primary form of LCD. It is autosomal dominant and results from mutations in the transforming human growth factor beta-induced (TGFBI) gene, located on chromosome 5 at locus 5q. This gene is also called BIGH3.^[5],[6] It usually presents in the first or second decade of life, equivocally affecting both males and females.^[4] LCD variants are subtypes of LCD caused by a variety of mutations on the TGFBI gene. LCD type II is no longer included among the corneal dystrophies as it is a primarily systemic disorder with ophthalmologic features.^[6] Recurrent erosions and an unhealthy epithelium in lattice dystrophy may predispose the cornea to microbial infections, bacterial and viral infections being more common in literature.^{[7],[8],[9],[10]} We present a rare clinical scenario of advanced fungal keratitis in a patient with lattice dystrophy and how timely therapeutic keratoplasty helped in the restoration of the patient's vision.

Case Report

A 30-year-old female homemaker by profession presented with complaints of pain, watering, redness, and diminution of vision in the left eye for the past 1 month. The patient gave a history of fall of dust in the left eye during her household work, following which she developed the above symptoms. She used over-the-counter medication before presenting it to us. Vision acuity in the right eye was 20/20 and the left eye was counting fingers close to face (CF-CF). Regurgitation on pressure over the lacrimal sac was negative on both sides with blepharitis on the left side. On slit-lamp examination, the right eye had multiple refractile linear lines in the anterior stroma extending to the periphery [Figure 1]. The left eye had limbus-to-limbus infiltrate with faintly detectable refractile lines [Figure 2]. Intraocular pressure in the right was 16 mmHg with noncontact tonometry and the left eye was digitally hard. Corneal scraping from the left eye revealed multiple septate fungal filaments in 10% potassium hydroxide preparation [Figure 3]. Anterior segment optical coherence tomography of the left eye showed spread-out hyperreflective material at the level of the Bowman layer extending to the anterior stroma, the epithelium was partially thinned, and the Bowman layer was disrupted [Figure 4]. The patient was diagnosed with lattice dystrophy in both eyes and advanced fungal keratitis in the left eye. The patient was started on voriconazole 1% eye drops hourly, atropine 1% eye drops three times a day, and oral ketoconazole 200 mg twice a day. Considering the advanced status of the ulcer which was threatening to perforate, the patient was posted for therapeutic keratoplasty in the left eye on the very next day. Postoperatively, the patient was put on oral ketoconazole 200 mg twice daily, eye drops voriconazole 1% 8 times a day, eye drops atropine 1% three times a day, and eye ointment itraconazole 1% three times a day for 2 weeks. After 1 week of incubation, Sabouraud's dextrose agar grew raised yellowish-green powdery colonies suggestive of Aspergillus flavus [Figure 5]. At 2-week postoperative period, the patient had no signs of reinfection, graft was clear, and the anterior chamber was quite with early lenticular changes. The patient was asked to stop voriconazole 1% eye drops and started on eye drops prednisolone acetate 1% six times a day and asked to taper every 2 weeks. In addition, the patient was put on eye drops atropine 1% two times a day, eye drops carboxymethyl cellulose 1% eight times a day, and eye ointment hydroxypropyl methylcellulose 0.3% at night. The patient was followed up regularly and was put on the maintenance dose of eye drops prednisolone acetate 1% twice daily along with carboxymethyl cellulose 1% eye drops. At 6-month postoperative period, the patient had an unaided vision 0f 20/200 with pinhole improving to 20/100. On slit-lamp examination, graft–host junction was well opposed, graft was clear with sutures in situ, and anterior chamber was well formed and quite. Lens showed early changes [Figure 6]. Intraocular pressure was 16 mmHg with Goldmann applanation tonometry, and fundus was normal.

Figure 1: Slit lamp photograph of right eye showing multiple refractile linear lines in the anterior stroma extending to the periphery

Click here to view

Figure 2: Slit-lamp photograph of the left eye showing limbus-to-limbus infiltrate involving sclera; inset shows faintly detectable refractile lines

Click here to view

Figure 3: 10% potassium hydroxide preparation showing multiple septate fungal filaments

Click here to view

Figure 4: Anterior segment optical coherence tomography of the right eye showing spread-out hyperreflective material at the level of the Bowman layer extending to the anterior stroma, partially thinned out epithelium and disrupted Bowman layer

Click here to view

Figure 5: Sabouraud dextrose agar medium showing raised yellowish-green powdery colonies of Aspergillus flavus

Click here to view

Figure 6: Slit-lamp photograph of the left eye at postoperative 6-month period showing well-opposed host–graft junction, clear 11 mm × 11 mm graft, well-formed anterior chamber with early lenticular changes

Click here to view

Discussion

Abnormal basement membrane complexes causing poor epithelial-stromal adhesions, incapable of maintaining structural integrity postulated to be the reason for recurrent corneal erosions in lattice dystrophy.^[11] Recurrent erosions make the cornea susceptible to infections due to jeopardized normal epithelial barrier with delayed healing process of the diseased epithelial layer.

In literature, we found Goodall et al. report a case series with mixed bacterial infections in patients with lattice dystrophy, whereas others have described bacterial or viral infections in lattice dystrophy. Chatterjee and Agrawal report a case of lattice dystrophy with fungal keratitis (Alternaria alternata) in a farmer, which was successfully treated medically not requiring any surgical intervention.^[12] Poor prognostic factors such as limbus-to-limbus infiltrate, scleral involvement, and usage of over-the-counter medication made us to plan for emergency therapeutic keratoplasty. Although infections are common in patients with lattice dystrophy, fungal keratitis is rare, and our case will be very next to the case reported by Chatterjee and Agrawal.

Here, we like to add that patients with lattice dystrophy should seek immediate medical help even after trivial ocular trauma, as there is a unhealthy epithelial barrier with delayed repairing process making them more prone to infections when compared to normal population. Patients should be discouraged for over-the-counter medication without reaching an ophthalmologist because in India, usually, the pharmacists dispense steroid or steroid-antibiotic combination for redness of eyes resulting in fulminant fungal keratitis. In the case reported by Chatterjee and Agrawal, the patient presented early and was treated successfully without any surgical intervention, but in our case, the patient presented to us 1 month after the onset of symptoms with advanced fungal keratitis, using over-the-counter medication making therapeutic keratoplasty inevitable.

Hence, we would like to conclude that patients with lattice dystrophy or any dystrophy causing recurrent corneal erosions should be counseled to use protecting eye gear while working, to seek immediate medical help even after trivial ocular trauma and to abstain from the practice of over-the-counter medication usage. Ophthalmologists should consider fungi as a potent cause for microbial keratitis in patients with lattice dystrophy.

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

1.	Munier FL, Korvatska E, Djemaï A, Le Paslier D, Zografos L, Pescia G, et al. Kerato-epithelin mutations in four 5q31-linked corneal dystrophies. Nat Genet 1997;15:247-51.
2.	Kannabiran C, Klintworth GK. TGFBI gene mutations in corneal dystrophies. Hum Mutat 2006;27:615-25.
3.	Klintworth GK. Corneal dystrophies. Orphanet J Rare Dis 2009;4:7.
4.	Siebelmann S, Scholz P, Sonnenschein S, Bachmann B, Matthaei M, Cursiefen C, et al. Anterior segment optical coherence tomography for the diagnosis of corneal dystrophies according to the IC3D classification. Surv Ophthalmol 2018;63:365-80.
5.	Klintworth GK. The molecular genetics of the corneal dystrophies – Current status. Front Biosci 2003;8:d687-713.
6.	Weiss JS, Møller HU, Aldave AJ, Seitz B, Bredrup C, Kivelä T, et al. IC3D classification of corneal dystrophies – Edition 2. Cornea 2015;34:117-59.
7.	Goodall K, Brahma A, Ridgway A. Lattice dystrophy and corneal ulceration. Eye (Lond) 1995;9:531-2.
8.	Pham-Vang S, Hardten DR. Recurrent corneal erosions causing bacterial keratitis. Optometry 2008;79:505-11.
9.	Chronister CL, Wasilauski ME. Recurrent corneal erosions (RCE) secondary to lattice dystrophy in a patient with acquired immune deficiency syndrome (AIDS). Optometry 2005;76:713-9.
10.	Sridhar MS, Sridhar U, Vemuganti GK, Mandal AK. Lattice corneal dystrophy type III with corneal fistula. A case report. Indian J Ophthalmol 2004;52:236-8. [PUBMED] [Full text]
11.	Bishop PN, Bonshek RE, Jones CJ, Ridgway AE, Stoddart RW. Lectin binding sites in normal, scarred, and lattice dystrophy corneas. Br J Ophthalmol 1991;75:22-7.
12.	Chatterjee S, Agrawal D. Fungal keratitis in lattice dystrophy. Indian J Ophthalmol 2010;58:162-4. [PUBMED] [Full text]