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 Table of Contents  
PHOTO ESSAY
Year : 2023  |  Volume : 3  |  Issue : 1  |  Page : 216-218

Multimodal imaging of bilateral basal laminar drusen


Department of Vitreo - Retina Services, Giridhar Eye Institute, Kochi, Kerala, India

Date of Submission10-Jun-2022
Date of Acceptance01-Oct-2022
Date of Web Publication20-Jan-2023

Correspondence Address:
Mahak Bhandari
Flat No. B2, First Floor, Jomer Retreat Apartment, Oppo. Giridhar Eye Institute, Kochi, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1389_22

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  Abstract 


Keywords: Basal laminar drusen, multi-modal imaging, serous pigment epithelial detachment


How to cite this article:
Bhandari M, Chandran K, Giridhar A. Multimodal imaging of bilateral basal laminar drusen. Indian J Ophthalmol Case Rep 2023;3:216-8

How to cite this URL:
Bhandari M, Chandran K, Giridhar A. Multimodal imaging of bilateral basal laminar drusen. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Feb 1];3:216-8. Available from: https://www.ijoreports.in/text.asp?2023/3/1/216/368138



A hypertensive 62-year-old male presented with diminution of vision in the right eye (RE) for 6 years. The best corrected visual acuity (BCVA) in RE was 2/60, < N36, and that in the left eye was 6/6, N6. Fundus examination, including color photos and multi-color photos showed numerous, round, elevated, large, well-demarcated bilaterally symmetrical retinal pigment epithelial detachment (RPED) [Figure 1]. Multi-modal imaging was performed using a Heidelberg Retinal Angiogram-2 (HRA-2; Heidelberg Engineering GmBH, Dossenheim, Germany). Spectral domain optical coherence tomography (SD–OCT) showed multiple RPED with hypo-reflective content and atrophy at the peak along with pachyvessels. RE had sub-foveal coalescent RPED with retinal pigment epithelium (RPE) and photoreceptor disintegration [Figure 2]. Fundus auto-fluorescence showed a hypo-auto-fluorescent center surrounded by hyper-auto-fluorescence [Figure 3]. Fundus fluorescence angiography revealed hyper-fluorescent lesion starting from an early arterio-venous phase giving a starry sky appearance bilaterally. RE also showed staining in the macula [Figure 4]. The enface OCT image showed a hypo-reflective center with a hyper-reflective border [Figure 5].
Figure 1: (a and b) Color fundus photo showing bilateral symmetrical circular, multiple, well demarcated but occasionally coalescent lesions suggestive of multiple retina pigment epithelial detachments (RPED) in the macula, posterior pole, mid periphery and periphery. (c and d) Multicolor photo showing circular elevated lesions suggestive of PED which appear greenish with an orange outer rim and the central macula (yellow arrow) demonstrating a bright orange appearance due to enhanced visualization of underlying bright orange choroidal vessels as a result of retinal pigment epithelial atrophy

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Figure 2: (a-d) Spectral domain optical coherence tomography (SD – OCT) scans shows multiple RPEDs in the posterior pole spreading to the periphery. (a and b) RPEDs are large with steep sides and hypo reflective content with retinal pigment epithelium (RPE) atrophy at the peak with back scattering (white arrow). The width of the lesions is in red and height in yellow. Pachyvessels can be seen bilaterally (green arrow). (a) Right eye (RE) has subfoveal coalescent PEDs along with RPE and photoreceptor disintegration. (c and d) The size of the lesions in the periphery are smaller than the posterior pole

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Figure 3: (a and b) Fundus autofluorescence (FAF) shows ring-pattern lesions (outnumbering those seen clinically) with hyperautofluorescent circle and hypoautofluorescent center. This pattern is the result of central retinal pigment epithelium erosion from triangular elevations of the RPE-basal lamina

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Figure 4: (a) Shows hyperfluorescent lesions in the early phase of Fundus Fluorescence angiography (FFA) in LE due to the RPE atrophy. (b) Venous phase of the Left eye (LE) shows starry sky appearance. (c) RE late venous phase has similar appearance and staining in the macula due to loss of the outer retinal layers (d) Indocyanine green angiography (ICG) shows hypercyanescent center surrounded by hypocyanescence. FFA and ICG was performed to come to a diagnosis and rule out other differential diagnosis such as Malattia leventinese, large colloidal drusen and soft drusen. The poor vision in the RE raised a suspicion of a choroidal neovascular membrane which was resolved by FFA and ICG

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Figure 5: (a and b) Pebbles in the fundus- Enface optical coherence tomography at the level of the lesions show hyporeflective content with hyper reflective border bilaterally. (a) RE shows confluence of lesions in the subfoveal area along with RPE and photoreceptor disintegration causing the hyperreflective center

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Systemic investigations including a complete blood picture, retinal function tests, and complement C3 were performed (normal results) because basal laminar drusen is associated with membranoproliferative glomerulonephritis type II. The patient has been kept on regular follow-up.


  Discussion Top


Basal laminar drusen or “cuticular drusen” is early onset drusen that is present in approximately 10% cases with age-related macular degeneration (ARMD).[1] It has three phenotypic variations.[2]

In its natural course, these lesions form confluent drusen in the central macular area and further development of serous pigment epithelial detachments,[3] which can be confused with idiopathic multiple RPED reported before.[4],[5] Diagnostic dilemma in such cases should be resolved by multi-modal imaging, which will help us predict a course towards ARMD rather than a pachychoroid spectrum, which is seen to be associated with idiopathic multi-focal serous RPEDs.[4],[5]

The RPE atrophy at the peak of these lesions and the occasional coalescence is striking and along with other characteristic features on multi-modal imaging should point toward the diagnosis of basal laminar drusen and help to differentiate it from simulating conditions such as idiopathic multiple RPED.

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.
Van de Ven JPH, Boon CJF, Fauser S, Hoefsloot LH, Smailhodzic D, Schoenmaker-Koller F, et al. Clinical evaluation of 3 families with basal laminar drusen caused by novel mutations in the complement factor H gene. Arch Ophthalmol 2012;130:1038-47.  Back to cited text no. 1
    
2.
Balaratnasingam C, Cherepanoff S, Dolz-Marco R, Killingsworth M, Chen FK, Mendis R, et al. Cuticular drusen: Clinical phenotypes and natural history defined using multimodal imaging. Ophthalmology 2018;125:100-18.  Back to cited text no. 2
    
3.
Boon CJ, Klevering BJ, Hoyng CB, Zonneveld-Vrieling MN, Nabuurs SB, Blokland E, et al. Basal laminar drusen caused by compound heterozygous variants in the CFH gene. Am J Hum Genet 2008;82:516-23.  Back to cited text no. 3
    
4.
Nagesha CK, Megbelayin EO. Bilateral multifocal retinal pigment epithelium detachment and pachychoroidopathy. Indian J Ophthalmol 2018;66:570-1.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Rauchegger T, Osl A, Teuchner B, Haas G. Symptomatic idiopathic bilateral multifocal retinal pigment epithelial detachments. Am J Ophthalmol Case Rep 2022;25:101336. doi: 10.1016/j.ajoc. 2022.101336.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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