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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 489-491

Ultrasound biomicroscopy findings and histopathological characteristics of anterior chamber cholesterolosis: A case report and literature review


1 Department of Cornea and Refractive Surgery, Instituto de Oftalmología, Conde de Valenciana, Mexico City, Mexico
2 Department of Oculoplastics, Instituto de Oftalmología, Conde de Valenciana, Mexico City, Mexico
3 Department of Glaucoma, Instituto de Oftalmología, Conde de Valenciana, Mexico City, Mexico

Date of Submission01-Oct-2020
Date of Acceptance25-Feb-2021
Date of Web Publication02-Jul-2021

Correspondence Address:
Dr. Arturo Ramirez-Miranda
Department of Cornea and Refractive Surgery, Instituto de Oftalmologia “Conde de Valenciana”, Chimalpopoca 14, Mexico City, CDMX, 06800
Mexico
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_3105_20

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  Abstract 


Anterior chamber cholesterolosis is an unusual finding, commonly caused by ocular trauma. Pathogenesis is still unknown; therefore, cases should be reported to determine correlations between past medical history and suggest probable etiologies. A 27-year-old male presented with normal vision in the right eye and no light perception in the left after suffering blunt ocular trauma. Ocular examination showed freely movable, highly refractile crystals forming a pseudohypopyon in the anterior chamber. B-scan ultrasonography revealed close funnel retinal detachment, ultrasound biomicroscopy showed cholesterol crystals in the anterior chamber and irideocorneal angle. Anterior chamber washout was performed, and aspirate indicated rhomboidal cholesterol crystals.

Keywords: Anterior chamber, cholesterol crystals, cholesterolosis, ultrabiomicroscopy


How to cite this article:
Mangwani-Mordani S, Ramirez-Miranda A, Ruiz-Gonzalez GC, Gurria LU, Graue-Hernandez EO. Ultrasound biomicroscopy findings and histopathological characteristics of anterior chamber cholesterolosis: A case report and literature review. Indian J Ophthalmol Case Rep 2021;1:489-91

How to cite this URL:
Mangwani-Mordani S, Ramirez-Miranda A, Ruiz-Gonzalez GC, Gurria LU, Graue-Hernandez EO. Ultrasound biomicroscopy findings and histopathological characteristics of anterior chamber cholesterolosis: A case report and literature review. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 26];1:489-91. Available from: https://www.ijoreports.in/text.asp?2021/1/3/489/320105



Cholesterol crystals found in the vitreous humor are known as synchysis scintillans or cholesterolosis bulbi, a rare condition first described in 1828 by Parfait Landrau.[1],[2] Since 1975, 88 cases have been reported, yet pathogenesis is unknown.[3] Several etiologies such as ocular trauma, aphakia, phacolysis, chronic retinal detachments, and hypermature cataracts have led to the formation of rhomboidal cholesterol crystals and their migration toward the anterior chamber (AC). Ultrasound biomicroscopy (UBM) is useful to visualize AC posterior to trauma, as well as angle closure.[4],[5] We present the case of a young patient with cholesterol crystals in the AC seen by UBM after suffering blunt ocular trauma.


  Case Report Top


A 27-year-old male presented to the emergency department referring to a sudden loss of vision in the left eye secondary to blunt trauma with 5 days of evolution. His past medical history was unremarkable. On examination, he presented with an uncorrected distance visual acuity (UDVA) of 20/20 in the right eye and no light perception (NLP) in the left. Both eyes had intraocular pressure (IOP) of 18 mmHg. Slit-lamp examination was normal in the right eye. In the left eye, the patient presented with hyperemic conjunctiva, ciliary injection, clear cornea, posterior synechiae, and a formed AC with freely movable, highly refractile crystals forming a pseudohypopyon [Figure 1]a and [Figure 1]b. Indirect gonioscopy could not be performed as a result of a large number of crystals seen. The left fundus exam was deferred due to poor mydriasis and posterior synechiae.
Figure 1: (a) Clinical photograph showing multiple cholesterol crystals in the anterior chamber forming pseudohypopyon. (b) Close up shows freely movable and highly refractile crystals

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B-scan ultrasonography of the left eye revealed retinal detachment with a close funnel as well as subretinal hemorrhage [Figure 2]. UBM of the AC with a 50 mHz probe allowed visualization of intact anterior and posterior capsules. Inferotemporal zonular dialysis and an obstructed irideocorneal angle with crystals were also seen [Figure 3]a and [Figure 3]b. AC washout was carried out, and the extracted material was sent for histopathological analysis. Rhomboidal highly refringent cholesterol crystals were reported [Figure 4]a and [Figure 4]b. The patient signed informed consent to publish this case, images of his eye, and results obtained by echography and UBM.
Figure 2: Mode B echography shows closed funnel retinal detachment and subretinal hemorrhage

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Figure 3: (a) Ultrabiomicroscopy images shows zonular dialysis. (b) Ultrabiomicroscopy images shows obstructed irideocorneal angle with cholesterol crystals in the anterior chamber

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Figure 4: (a and b) Histopathologic analysis reported rhomboidal highly refringent cholesterol crystals

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  Discussion Top


Several etiologies for the presence of cholesterolosis crystals in the AC have been acknowledged. Kennedy suggests that AC cholesterolosis does not occur as a primary phenomenon but rather as an indication of significant underlying ocular disease, which should be identified and studied.[6] Numerous cases have been subjected to severe trauma consequently leading to chronic retinal detachment, vitreous hemorrhage, and protracted inflammation.[6],[7] Cholesterol is released due to erythrocyte cell membrane breakdown and cholesterol crystals formed can eventually diffuse into the AC.[3],[6] Besides, aphakia and lens luxation can facilitate the migration of crystals to the AC.[6],[7] Other causes of cholesterol crystals in the AC are Coats and Eales disease.[8],[9]

Phacolysis has been identified as a possible etiology of cholesterolosis.[3],[6] Pathogenesis is unknown but is hypothesized to be caused due to degradation of intralenticular cell membranes.[7] Bartholomew and Rebello reported AC cholesterolosis in two patients with phacolytic glaucoma.[3] Jang et al. reported a case of synchysis scintillans mimicking phacolytic glaucoma.[10]

Mature (e.g Morgagnian cataracts) have been associated with increased cholesterol within the lens. Spontaneous rupture of such a lens due to weakened capsules or extraction of the latter can flood the AC with rhomboidal cholesterol crystals.[3]

In this case report, the patient presented with complete retinal detachment and subretinal hemorrhage with no visualization of vitreous hemorrhage. These findings could have contributed to the formation of cholesterol crystals due to breakdown of erythrocyte membranes, subsequently releasing cholesterol and increasing diffusion of crystals into the AC as hypothesized by Kennedy.[6],[7],[11] Furthermore, abrupt development of NLP was noted after suffering blunt ocular trauma. Literature search suggests that two-thirds of AC cholesterolosis cases present with NLP, indicating its importance as a common finding.[3] A vast majority of cases documented in the literature indicate the incidence of AC crystals several years after suffering severe trauma rather than few days posterior to ocular trauma, as seen in this case.[6],[8],[11] Finally, given the abundant crystals seen, an unidentified underlying pathology such as hypercholesteremia could be suspected.

UBM has been used to visualize the anatomy of the anterior segment of the eye as well as pathologies such as angle trauma, ciliary body damage, and angle-closure glaucoma.[5] It is also useful in detecting zonular defects, particularly after trauma. In this case report, zonular dialysis was seen secondary to blunt trauma. Pavlin et al. described the incidence of zonular abnormalities as direct signs, including “missing zonular fibers, increased zonular length, and zonular remanent on the lens capsule."[4] Angle closure can also be appreciated with UBM, in this case, abundant cholesterol crystals were seen in the angle; nonetheless, IOP remained normal.

Treatment options for AC cholesterolosis are limited, particularly in cases with funnel retinal detachments. As many of these eyes are blind, excision of the globe may be the most viable option, since it prevents sympathetic ophthalmia of the healthy eye.[1]


  Conclusion Top


UBM and B-scan ultrasonography in a case of anterior chamber cholesterolosis can help determine the etiology and the causative mechanism.

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 Top

1.
Kumar S. Cholesterol crystals in the anterior chamber. Br J Ophthalmol 1963;47:295-9.  Back to cited text no. 1
    
2.
Stacey AW, Borri M, Francesco S De, Antenore AS, Menicacci F, Hadjistilianou T. A case of anterior chamber cholesterolosis due to coats' disease and a review of reported cases. Open Ophthalmol J 2016;10:27-32.  Back to cited text no. 2
    
3.
Eagle RC Jr, Yanoff M. Cholesterolosis of the anterior chamber. Albrecht Von Graefes Arch Klin Exp Ophthalmol 1975;193:121-34.  Back to cited text no. 3
    
4.
Pavlin CJ, Buys YM, Pathmanathan T. Imaging zonular abnormalities using ultrasound biomicroscopy. Arch Ophthalmol 1998;116:854-7.  Back to cited text no. 4
    
5.
Liebmann JM. Ultrasound biomicroscopy of the anterior segment. J Glaucoma 2001;10:S53-5.  Back to cited text no. 5
    
6.
Kennedy CJ. The pathogenesis of polychromatic cholesterol crystals in the anterior chamber. Aust N Z J Ophthalmol 1996;24:267-73.  Back to cited text no. 6
    
7.
Sanmugasunderam S, Giligson A, Choi SB. A “sparkling” eye. CMAJ 2003;169:319.  Back to cited text no. 7
    
8.
Rishi P, Rishi E, Devulapally S. Cholesterolosis Bulbi. JAMA Ophthalmol [Internet]. 2017 Mar 9;135(3):e165458–e165458. Available from: https://doi.org/10.1001/jamaophthalmol.2016.5458.  Back to cited text no. 8
    
9.
Lu P, Huang J. Ultrasound findings in a case of Eales' disease and ocular trauma with anterior chamber cholesterolosis. BMC Ophthalmol [Internet]. 2020;20(1):393. Available from: https://doi.org/10.1186/s12886-020-01660-1.  Back to cited text no. 9
    
10.
Lo KJ, Huang YY, Hsu CC. Synchysis scintillans mimicking phacolytic glaucoma in a traumatic eye. Kaohsiung J Med Sci 2019;35:382-3.  Back to cited text no. 10
    
11.
Banc A, Stan C. Anterior chamber synchysis scintillans: A case report. Rom J Ophthalmol 2015;59:164-6.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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