Dr. Samyak Gupta, Dr. Prabhat Nangia, Dr. Vinay Nangia
Suraj Eye Institute, Nagpur
Case Description
A male, 41 years of age came to our institute for eye pain and blurring of vision. His visual acuity was 6/9, N10 in both eyes (OU). Anterior segment examination showed refractile stromal lattice lines with scarring in both eyes. Intra-ocular pressure was 16 mmHg in right eye and 20 mmHg in left eye. He had a positive family history of the similar disease with his brother being affected.
Figure 1(b) – Anterior segment photograph of left eye under diffuse illumination showing a linear corneal scar superiorly(yellow arrow)
Figure 2(b) – Left eye anterior segment photograph under parallelepiped illumination showing epithelial corneal scars (yellow arrows)
Figure 3(b) – Left eye anterior segment photograph under retroillumination showing multiple intrastromal refractile lattice lines (yellow arrows)
Discussion
| Lattice corneal dystrophy (LCD) is an inherited disorder involving the corneal stroma. It is characterized by amyloid deposition within the stroma resulting in linear lattices. It is caused by mutations in transforming human growth factor beta-induced (TGFBI) gene located on chromosome 5q31. Lattice dystrophies show an autosomal dominant inheritance pattern. They are characterized by multiple eosinophilic amyloid inclusions between epithelium, Bowman layer and the corneal stroma. Histologically, the amyloid material in LCD stains with Congo red with an apple green birefringence when viewed using polarised light. It is one of the common causes of recurrent corneal erosions among corneal dystrophies. In our case, the patient had presence of lattice lines (yellow arrows Fig. 3) within the corneal stroma. He also had a history of recurrent episodes of pain and redness with several epithelial corneal scars (yellow arrows in Fig. 1 and Fig. 2) suggesting that he may have had recurrent corneal erosions in the past. His AS-OCT showed hyper-reflective material in the anterior stroma(yellow arrows in Fig.4) and few areas with loss of Bowman’s layer (white arrows in Fig.4(b) and Fig.5(a)) which were consistent with the appearance of LCD. However as the patient had good visual acuity and his eye was quiet, we decided to manage the patient with lubricants and regularly follow up the patient for monitoring of the disease. The patient also had a positive family history with his brother affected with a similar condition. His brother underwent keratoplasty in one eye due to diminished vision. Histopathological examination of the corneal tissue post surgery had revealed the presence of Lattice corneal dystrophy, hence further confirming our diagnosis in this patient. Patient denied presence of similar symptoms in other members of the family. Lattice lines in LCD by themselves do not cause a decrease in vision. Any loss of vision in such cases is usually seen due to scarring. Surgical options include Deep anterior lamellar keratoplasty, penetrating keratoplasty, phototherapeutic keratectomy and femtosecond laser assisted lamellar keratoplasty. Prognosis is good in cases of LCD although there is tendency for amyloid deposition within the grafted cornea resulting in recurrence. |
ReadWise
- Moshirfar M, West W, Ronquillo Y. Lattice Corneal Dystrophy. https://europepmc.org/article/MED/32310559/NBK430685#free-full-text
- Sauvageot P, Julio G, Bolaños JV, Carrera M, de Toledo JÁ, Barraquer RI. Recurrence and Visual Outcomes of Phototherapeutic Keratectomy in Lattice Corneal Dystrophy: A Cohort Study. Journal of Refractive Surgery. 2022 Jan 1;38(1):43-9. https://doi.org/10.3928/1081597X-20211104-01
- Carrwik C, Stenevi U. Lattice corneal dystrophy, gelsolin type (Meretoja’s syndrome). Acta ophthalmologica. 2009 Dec;87(8):813-9. https://doi.org/10.1111/j.1755-3768.2009.01686.x
- Siebelmann S, Scholz P, Sonnenschein S, Bachmann B, Matthaei M, Cursiefen C, Heindl LM. Anterior segment optical coherence tomography for the diagnosis of corneal dystrophies according to the IC3D classification. Survey of ophthalmology. 2018 May 1;63(3):365-80. https://doi.org/10.1016/j.survophthal.2017.08.001
Correspondence
Dr Prabhat Nangia
DNB, FICO, FMRF, FAICO
Consultant
Department of Cornea and Ocular surface
Suraj Eye Institute
Email – education@surajeye.org
QuizWise
Q1. Lattice corneal dystrophy occurs at which layer of cornea:
A) Epithelium
B) Stroma
C) Endothelium
D) Epithelial-Stromal
Q2. Which is correct regarding order of recurrence in corneal graft for corneal dystrophies :
A) Reis Buckler > Lattice > Granular > Macular
B) Lattice > Reis Buckler > Granular > Macular
C) Reis Buckler > Granular > Lattice > Macular
D) Macular > Reis Buckler > Lattice > Granular
Q3. Which stain is used to identify LCD:
A) Alcian blue
B) Congo red
C) Masson Trichrome
D) None of the above
Q4. Not a treatment for LCD?
A) Photherapeutic keratectomy
B) Deep anterior lamellar keratoplasty
C) Penetrating keratoplasty
D) Descemet stripping endothelial keratoplasty
Q5. Which is not a cause of recurrent corneal erosions?
A) Fuchs endothelial dystrophy
B) Lattice corneal dystrophy
C) Granular dystrophy
D) Reis Buckler dystrophy