|Year : 2021 | Volume
| Issue : 3 | Page : 270-272
Malady of lattice
Shobita Nair1, Venugopal Anitha1, Meenakshi Ravindran2, Vinit J Shah3
1 Cornea and Refractive Services, Aravind Eye Hospital, Tirunelveli, Tamil Nadu, India
2 Paediatric and Strabismus Services, Aravind Eye Hospital, Tirunelveli, Tamil Nadu, India
3 Retina Services, Aravind Eye Hospital, Tirunelveli, Tamil Nadu, India
|Date of Submission||23-Feb-2021|
|Date of Decision||18-Apr-2021|
|Date of Acceptance||03-Jul-2021|
|Date of Web Publication||09-Sep-2021|
Dr. Venugopal Anitha
Aravind Eye Hospital, S. N. High Road, Tirunelveli Junction, Tirunelveli - 627 001, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Lattice corneal dystrophy (LCD) is rare, bilateral, slowly progressive, inherited condition characterized by amyloid deposition in the corneal stroma. Recurrent corneal erosions most commonly occur with LCD due to abnormal basement membrane complexes incapable of providing normal structural integrity to the epithelial layer predisposing the cornea to microbial infections such as bacterial and fungal keratitis. Amyloid fibrils have also been postulated to cause tissue damage by stimulating local and nonlocal immune cell infiltration into the tissues and producing pro-inflammatory cytokines leading to sterile inflammation. This case is one of the few rare case reports of sterile infiltrates in LCD Type 1, patients identified after multiple treatment failures to the best of our knowledge.
Keywords: Amyloid, corneal melt, lattice corneal dystrophy, recurrent corneal erosions, sterile infiltrates
|How to cite this article:|
Nair S, Anitha V, Ravindran M, Shah VJ. Malady of lattice. TNOA J Ophthalmic Sci Res 2021;59:270-2
| Introduction|| |
Lattice corneal dystrophy (LCD) is a congenital hereditary condition where Types I, II, and IIIa are inherited as autosomal dominant, and Type III is autosomal recessive. Type II or Meretoja syndrome is associated with systemic amyloidosis, also known as familial amyloidotic polyneuropathy IV affecting skin, heart, kidneys, liver, cranial, and peripheral nerves. Symptoms usually begin in the first decade of life in the form of decreased vision, photophobia, recurrent corneal erosions (RCE), and delayed epithelial healing due to the accumulation of amyloid material in the subepithelial stroma., Our patient was a 50-year-old female who presented multiple episodes of RCE and infiltrates which was managed with topical steroids considering the inflammatory role of amyloid deposits in the stroma.
| Case Report|| |
A 50-year-old female, a known case of bilateral LCD Type 1, presented to us with multiple episodes of RCE in both eyes. She was conservatively treated with topical lubricants, hypertonic saline, and therapeutic bandage contact lens for RCE in both the eyes alternately. Later, she underwent phototherapeutic keratectomy (PTK) in the right eye alone when the conservative treatment failed. After this, she was completely asymptomatic in the right eye for 1 year. She was a known hypertensive and hypothyroid on treatment for 10 years. During her last visit, she presented to us again with pain, redness, defective vision in the left eye for 1 week.
Ocular examination revealed that best-corrected visual acuity (BCVA) in the right eye was 20/80 and left eye counting fingers close to the face. Anterior segment examination of the right eye showed lattice lesions in the corneal stroma with superficial scarring following PTK, altered limbal architecture with superficial vascularization in one quadrant 2 mm into the cornea. The left eye showed circumcorneal congestion, epithelial erosions, epithelial ridges, dense stromal haze, multiple stromal ring-shaped mid-peripheral infiltrates, and 360° deep vascularization 2 mm into the cornea with altered limbal architecture [Figure 1]a and [Figure 1]b. The rest of the anterior segment examinations were within normal limits. The right eye posterior segment was within normal limits, and the left eye B scan was normal.
|Figure 1: (a and b) Slit-lamp photographs of both eyes. (a) Right eye showing lattice corneal dystrophy with scarring postphototherapeutic keratectomy. (b) Left eye showing lattice corneal dystrophy with multiple stromal ring-shaped infiltrates with vascularization (fluorescein stained)|
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The patient was suspected of having infective keratitis and empirically started on topical and systemic antibiotics after corneal scrapping. Corneal scrapings and viral polymerase chain reaction were negative on multiple occasions. She also underwent left eye amniotic membrane grafting twice for a persistent epithelial defect, which failed to stabilize the ocular surface with persistent infiltrates. Later, considering the repeated negative microbiological reports, we did a thorough literature search for the role of inflammation and the amyloid component of the LCD. We started on topical steroid loteprednol at 0.5% and systemic anticollagenase inhibitor doxycycline 100 mg twice a day with close observation and tapered over a month. She responded well in the left eye but soon came with recurrence and another similar episode in the right eye, all treated similarly with longer tapering of topical steroids over 3 months along with 0.05% cyclosporine and hyperosmotic agents such as sodium chloride 6% eye ointment at night time. Both the eyes' cornea epithelized and scarred with lattice dystrophy, and the ocular surface stabilized over 8 months. The patient had BCVA of 20/80 in the right eye and 20/120 in the left eye during the final visit [Figure 2]a and [Figure 2]b.
|Figure 2: (a and b) Slit-lamp photographs of both eyes posttreatment showing lattice corneal dystrophy with scarred and epithelized cornea|
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| Discussion|| |
LCD Type 1 is characterized by amyloid deposits in the corneal stroma in a linear and radially oriented fashion, mostly involving the central cornea, causing progressive vision loss. Papathanassiou et al. said that subepithelial accumulations of fibrillar amyloid deposits distort the epithelial contour, basement membrane irregularity, and discontinuity leading to an irregular corneal surface and obstruction of reepithelialization. This poor epithelialization and corneal wound healing predispose the patient to RCE and corneal infiltrates.
LCD is one of many amyloid diseases characterized by abnormal accumulation of proteinaceous materials such as amyloid fibrils or plaques in tissues or organs. Amyloid causes tissue damage and stimulation of local inflammation by immune cell infiltration and the production of pro-inflammatory cytokines. Thus, a vicious cycle is initiated, which creates an environment of chronic inflammation causing tissue damage and loss of function, controlled only if promptly started on anti-inflammatory agents such as steroids. Serum amyloid is also a potent stimulus for releasing tumor necrosis factor-alpha, interleukin 1-beta, and interleukin 8. Amyloid beta-protein induces necrosis rather than apoptosis, initiating an inflammatory process. Patients with preexisting corneal pathology are susceptible to developing corneal infiltrates, which can be infective or sterile. Reduced corneal sensitivity and dry eyes can damage the epithelial integrity leading to persistent epithelial defect and chronic inflammation, which could progress to corneal melt. Thus, it is crucial to differentiate infective from sterile infiltrates. Infective infiltrates are characterized by acute painful presentation, lid edema, discharge, epithelial defect, large, central, single flat appearing infiltrate, anterior chamber reaction, hypopyon, culture positive, and responds well to treatment with antibiotics. While on the other hand, sterile infiltrates present with chronic, recurrent, less painful symptoms, small, multiple, peripheral, circumferential, elevated appearance, quiet anterior chamber, culture negative, and no response to topical antibiotics.
Corneal scraping to find the etiological agent is primarily done in all cases of corneal infiltrates and ulcers to rule out infective etiology. Goodall et al. proposed that lattice dystrophy may be particularly susceptible to microbial keratitis due to repeated epithelial disturbances caused by the irregularity of basement membrane complexes, leading to poor epithelial-stromal adhesion, which in turn predisposes to corneal erosion. Chatterjee and Agrawal reported a case of Alternaria alternata fungal infection in a patient with LCD. Ganger et al. reported fungal keratitis, namely, Aspergillus fumigatus and Curvularia lunata, in two patients with LCD. While Papathanassiou et al. reported corneal melt postcataract surgery in a patient with LCD.
In our patient, multiple culture-negative scrapings and multiple episodes of recurrence in both the eyes with the chronicity of the condition prompted us to consider chronic inflammation and sterile infiltrates in LCD. LCD with sterile infiltrates can rapidly progress to corneal melt if not treated promptly with systemic and topical steroids or immunomodulatory drugs, and anticollagenase agents have a protective role in such cases. Our patient responded well to the topical steroids and systemic anticollagenase agents, but close observation is needed to look for secondary infections.
| Conclusion|| |
In our case, initial corneal misdiagnosis delayed corneal healing, emphasizing the importance of reconsidering our diagnosis and our management strategy of ulcers, not responding to empirical treatment. It is essential to differentiate sterile and infective causes of corneal infiltrates. Hence, timely diagnosis and cautious initiation of early treatment with topical steroids with close follow-up for sterile infiltrate in LCD carry a good prognosis.
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
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]