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Year : 2004  |  Volume : 52  |  Issue : 3  |  Page : 236-8

Lattice corneal dystrophy type III with corneal fistula. A case report.

Cornea Centre, L V Prasad Eye Institute, Hyderabad, India

Date of Submission18-Jan-2003
Date of Acceptance13-Jun-2003

Correspondence Address:
Mittanamalli S Sridhar
Cornea Centre, L V Prasad Eye Institute, Hyderabad
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Source of Support: None, Conflict of Interest: None

PMID: 15510466

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Lattice corneal dystrophy is a distinct clinical entity characterised by amyloid deposits in the corneal stroma. We report a patient who presented with a corneal fistula in the right eye and thick lattice lines involving the peripheral cornea in both eyes suggestive of type III lattice dystrophy. The association of corneal fistula with lattice corneal dystrophy type III makes this a unique case.

Keywords: Lattice corneal dystrophy, amyloid deposits, corneal fistula

How to cite this article:
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

How to cite this URL:
Sridhar MS, Sridhar U, Vemuganti GK, Mandal AK. Lattice corneal dystrophy type III with corneal fistula. A case report. Indian J Ophthalmol [serial online] 2004 [cited 2020 Jun 6];52:236. Available from: http://www.ijo.in/text.asp?2004/52/3/236/14583

Lattice corneal dystrophies (LCDs) are bilateral inherited forms of corneal stromal amyloidosis.[1] LCDs have been classified into four distinct subtypes based on the age of presentation, clinical features and histopathology. [2],[3],[4] To the best of our knowledge, lattice corneal dystrophy type III has not been reported from the Indian population (Medline search). We report a case of LCD type III, associated with corneal fistula in one eye.

  Case report Top

A 50-year-old gentleman presented to us with a complaint of decreased vision in both eyes of one year's duration. He gave a history of redness, watering, pain and diminution of vision in the right eye after dust had fallen into that eye. Past ocular history and family history were unremarkable. His visual acuity was perception of light in the right eye and 6/24 in the left eye. Intraocular pressure (IOP) was normal by digital examination in the right eye and was 14 mm Hg in the left eye. The right eye corneal examination showed diffuse opacity and thick lattice lines in the periphery. Centrally, the scar was dense and there was a fistula, confirmed by a positive forced Seidel's test [Figure - 1]. There were blood vessels in the peripheral cornea. Anterior chamber details could not be visualised. In the left eye, the cornea showed thick branching lattice lines in the mid-stroma. The lines involved the peripheral cornea and were distinctly seen on direct illumination [Figure - 2]a and b. There was an area of dense scarring involving the inferior cornea. The lens showed nuclear cataract.

Penetrating keratoplasty with synechiolysis and extracapsular cataract extraction was performed in the right eye under local anaesthesia. An 8.0mm graft on a 7.5mm bed was sutured with sixteen 10-0 monofilament nylon interrupted sutures . On the first postoperative day, the IOP was 60 mm Hg with tonopen. He was treated with topical timolol maleate 0.5%, brimonidine tartrate 0.2%, tablet acetazolamide 250mg four times a day and injection intravenous mannitol. Transcleral diode laser cyclophoto-coagulation (41 spots of 2490 mw and 2000 ms duration) was performed on the seventh postoperative day. At last follow-up, four weeks after surgery, the visual acuity was 6/60 with +10 D Sph in the right eye and the intraocular pressure was 8 mm Hg (without anti-glaucoma medication). There was graft oedema. Fundus examination revealed hazy media with a cup-disc ratio of 0.5:1.

Histopathology of the corneal button of the right eye revealed irregular cornea with marked thinning in the center. The epithelium was irregular with complete disruption of the Bowman's layer. There was loss of corneal stroma in the central region, while the surrounding cornea showed scarring with irregular fibroblasts [Figure - 3]a. The deeper stroma revealed vascular channels and few round cell infiltrates. Few linear, pale, waxy eosinophilic stromal deposits were seen in the deeper layers, which were congophilic and emitted apple green birefringence when viewed under polarized filters [Figure - 3]b.

  Discussion Top

Lattice corneal dystrophy (LCD) was first described by Biber in 1890 as "glitterage keratitis" in three unrelated females.[4] The nature of the deposit was identified as amyloid by Klintworth in 1967.[5] LCD type I has onset early in childhood, and the central lattice lines that are difficult to visualise with direct illumination are characteristic of this entity.[2] LCD Type II (amyloidosis type V) is associated with Meretoja's syndrome.[3] In this type, the lattice lines are coarse and extend from the limbus towards the central cornea, involving the mid-stroma at the periphery and more superficial stroma centrally. LCD Type III was first reported by Hida et al in 1984.[4] In this type, the onset is usually after 40 years of age and lattice lines extending from limbus to limbus, the lattice lines are thicker and more easily seen with direct illumination than in LCD type I. LCD type IIIA differs from type III by presence of corneal erosions, the occurrence in Caucasians and the autosomal dominant inheritance pattern.

We diagnosed the patient reported here as LCD type III based on the late onset of presentation, clearly visible lattice lines on direct illumination, involvement of the limbus and lack of evidence of systemic amyloidosis. An interesting feature in our case was the association of corneal fistula in one eye. Based on the patient's history of acute symptoms following dust entry, we presume that he had a corneal perforation secondary to infection. Corneal ulceration has been reported with LCD.[6] Corneal fistula is a rare clinical entity in which there is a chronic communication between the anterior chamber and the corneal surface, resulting from the mal-apposition of corneal tissue.

In the case report, because of the associated corneal scar, the fistula was managed by penetrating keratoplasty. Following the penetrating keratoplasty there was expected rise in IOP, necessitating trans-scleral diode laser cyclophotocoagulation. In a fistula in which there has been a chronically or recurrently shallow anterior chamber, concomitant prophylactic glaucoma surgery may be indicated at the time of fistula closure. In such cases, the fistula may have served as a filtration site that is lost at the time of closure. The presence of normotensive eye with a Seidel's positive fistula should alert one to the possibility of a pressure increase after the fistula is closed.

To conclude, we report a rare case of lattice corneal dystrophy type III with corneal fistula in one eye of an Asian Indian. The association of corneal fistula with LCD type III has makes this case unique.

  References Top

Stock EL, Feder RS, O' Grady RB, Sugar J, Roth SI. Lattice corneal dystrophy type IIIA. Clinical and histopathological correlations. Arch Ophthalmol 1991;109:354-58.  Back to cited text no. 1
Mannis MJ, Desouza LB, Gross RH. The stromal dystrophies in:Krachmer JH, Holland EJ, Mannis MJ, editors. Diseases of Cornea . St. Louis: Mosby, 1997:1043-62.  Back to cited text no. 2
Meretoja J. Familial systemic paramyloidosis with lattice dystrophy of cornea, progressive corneal neuropathy, skin changes and various internal symptom: a previously unrecognized heritable syndrome. Ann Clin Res 1969;1:314-24.  Back to cited text no. 3
Hida T, Proia AD, Kigasawa K, Sanfilippo FP, Burchette JL, Akiya S, et al. Histopasthologic and immunochemical features of lattice corneal dystrophy III. Am J Ophthalmol 1987;104:249-54.  Back to cited text no. 4
Klintworth GK. Lattice corneal dystrophy. An inherited variety of systemic corneal amyloidosis restricted to the cornea. Am J Pathol 1967;50:371.   Back to cited text no. 5
Goodall K, Brahma A, Ridgway A. Lattice dystrophy and corneal ulceration. Eye 1995;9:531-32.  Back to cited text no. 6


  [Figure - 1], [Figure - 2], [Figure - 3]

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Chatterjee, S., Agrawal, D.
Indian Journal of Ophthalmology. 2010; 58(2): 162-164


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