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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 632-633

Bilateral progressive familial idiopathic annular lipid keratopathy – A case series


Gurunanak Eye Centre, Maulana Azad Medical College, New Delhi, India

Date of Submission14-Feb-2021
Date of Acceptance31-May-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Abhilasha Sanoria
G-242, Sector 22, Noida - 201 301, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_323_21

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  Abstract 


Lipid keratopathy (LK) is a disease in which fat deposits accumulate in the cornea, leading to opacification and decrease of visual acuity. It can be idiopathic, i.e. without signs of previous corneal disease or secondary to ocular or systemic diseases. It is usually associated with abnormal vascularization of the cornea, and the lipid classically deposits adjacent to these vessels. There have been case reports of annular LK in literature, but to the best of our knowledge, familial association has not been reported with it. In this case series, we thus present a family of six patients with bilateral annular LK.

Keywords: Familial association, idiopathic, lipid keratopathy


How to cite this article:
Jain P, Arora R, Sanoria A, Gupta I. Bilateral progressive familial idiopathic annular lipid keratopathy – A case series. Indian J Ophthalmol Case Rep 2021;1:632-3

How to cite this URL:
Jain P, Arora R, Sanoria A, Gupta I. Bilateral progressive familial idiopathic annular lipid keratopathy – A case series. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 26];1:632-3. Available from: https://www.ijoreports.in/text.asp?2021/1/4/632/327735



Lipid keratopathy (LK) is characterized by the opacification of the cornea due to lipid deposition. LK may be idiopathic, with no evidence of systemic or local disease, or secondary to ocular infection, inflammation, or trauma. Most commonly, LK occurs in regions of corneal neovascularization and scarring.[1] These lesions can be progressive and threaten the visual axis.[2]


  Case Report Top


We report a family of six patients (aged 35, 40, 42, 47, 50, 52 years, respectively) with bilateral paracentral annular deep stromal lipid deposits. Our patients had history of red eyes, pain, and photophobia showing on examination low-to-moderate inflammation at the corneo-scleral limbus (limbitis). Three (aged 35, 50, 52) out of the family of six had bilateral diffuse corneal infiltration with limbal involvement and had visual acuity of less than 20/400. Patients aged 40 and 42 years had a visual acuity of 20/200 in both eyes and had bilateral annular deposits with no limbitis. Vision was to some extent found to be preserved in the 47-year-old patient (20/80); hence, refraction and follow up were done for this patient. There was no history of previous ocular trauma, systemic diseases, or any similar family history. Fasting lipid profile, ECG, echocardiogram, liver function tests, kidney function tests, and carotid Doppler did not reveal any abnormality. Cholesterol crystals were observed in the paracentral area in all the cases [Figure 1], associated with deep corneal blood vessels. In advanced cases (five out of the family of six with BCVA</=6/60), penetrating keratoplasty was performed for visual rehabilitation and the histopathology of the corneal buttons showed the presence of focal stromal vascularization and staining for lipids [Figure 2] and [Figure 3].
Figure 1: (a), (b) ASOCT showing diffuse stromal deposits in a 35-year-old patient (B/E). (c) Corneal infiltration with limbal involvement and vascularization in the right eye. (d) The left eye showing paracentral involvement in the same patient

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Figure 2: Corneal photograph of a 50-year-old patient showing diffuse corneal infiltration (a) R/E, (b) L/E, (c) R/E ASOCT showing diffuse stromal infiltration, and (d) R/E post PK

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Figure 3: Corneal photographs of a 52-year-old patient (R/E) showing paracentral infiltration (a and b) ASOCT showing mid stromal infiltration, and (c) R/E post PK

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  Discussion Top


To the best of our knowledge, familial association of idiopathic annular LK has not been reported from the Asian subcontinent. Deposition of lipids in the cornea has been described in several pathological conditions, including LK, lipoidal Schnyder's crystalline dystrophy, corneal xanthoma, corneal arcus, and corneal xanthogranuloma.[3] Occasionally, lipids may be deposited in the cornea secondary to systemic lipoprotein disorders, including Tangier disease, lecithin cholesterol acyl transferase deficiency, and familial high-density lipoprotein deficiencies.[4] In lipoprotein disorders, the involvement is usually bilateral, but corneal vascularization is absent. In our patients, no systemic or ocular disorders were present to explain the lipid deposition in the cornea. The pathogenesis remains unclear, but we think that corneoscleral microvascular changes with incompetent limbus blood vessels may have accounted for corneal lipid infiltration in these cases. There are isolated case reports with a similar pattern of presentation, but to the best of our knowledge, a familial association from the Asian subcontinent has not been reported yet.[3],[5],[6]


  Conclusion Top


There have been reports of idiopathic LK; however, to the best of our knowledge, till date its familial association has not been reported in the literature. In this case series, we thus report a family of six patients with bilateral annular LK.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Alfonso E, Arrellanes L, Boruchoff SA, Ormerod LD, Albert DM. Idiopathic bilateral lipid keratopathy. Br J Ophthalmol 1988;72:338-43.  Back to cited text no. 1
    
2.
Hall MN, Moshirfar M, Amin-Javaheri A, Ouano DP, Ronquillo Y, Hoopes PC. Lipid keratopathy: A review of pathophysiology, differential diagnosis, and management. Ophthalmol Ther 2020;9:833-52.  Back to cited text no. 2
    
3.
Ghanem RC, Ghanem VC, Victor G, Alves MR. Bilateral progressive idiopathic annular lipid keratopathy. Case Rep Ophthalmol Med 2012;2012:731413. doi: 10.1155/2012/731413.  Back to cited text no. 3
    
4.
Barchiesi BJ, Eckel RH, Ellis PP. The cornea and disorders of lipid metabolism. Surv Ophthalmol 1991;36:1-22.  Back to cited text no. 4
    
5.
Durán JA, Rodriguez-Ares MT. Idiopathic lipid corneal degeneration. Cornea 1991;10:166-9.  Back to cited text no. 5
    
6.
Castro-Rebollo M, Montes-Mollón MA, Pérez-Rico C. [Primary bilateral lipid keratopathy]. Arch Soc Esp Oftalmol 2009;84:263-5.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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