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 Table of Contents  
CHALLENGING CASE
Year : 2015  |  Volume : 10  |  Issue : 3  |  Page : 348-352

Diagnosis and management of a bilateral macular problem


Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran

Date of Web Publication26-Nov-2015

Correspondence Address:
Alireza Ramezani
Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2008-322X.170346

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How to cite this article:
Ramezani A. Diagnosis and management of a bilateral macular problem. J Ophthalmic Vis Res 2015;10:348-52

How to cite this URL:
Ramezani A. Diagnosis and management of a bilateral macular problem. J Ophthalmic Vis Res [serial online] 2015 [cited 2019 Aug 21];10:348-52. Available from: http://www.jovr.org/text.asp?2015/10/3/348/170346




  Case Presentation Top


A 59-year-old gentleman presented with decreased visual acuity and metamorphopsia in both eyes for one year, worsening over the past three months. Except for a history of phacoemulsification and intraocular lens (IOL) implantation in his left eye, medical, surgical and ocular history was unremarkable.

Best corrected visual acuity was 3/10 and 6/10 with +3.50-0.75 × 180 and −0.50-0.75 × 180 in the right and left eyes, respectively. There was no relative afferent pupillary defect. Slit lamp examination revealed 2 + nuclear sclerosis (NS) and 2 + posterior subcapsular (PSC) cataract in the right eye and a well-positioned posterior chamber IOL (PCIOL) in his left eye. Intraocular pressure was 18 mmHg in both eyes. On the fundus examination, the media were clear and the optic discs had normal appearance. Vertical cup-to-disc ratio was about 0.4 in both eyes.

[Figure 1],[Figure 2],[Figure 3],[Figure 4] show color fundus photography, optical coherence tomography (OCT), fluorescein angiography (FA), and indocyanine green (ICG) angiography images of both eyes, respectively.
Figure 1. Color fundus photograph.

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Figure 2. Optical coherence tomography (OCT) and enhanced depth imaging (EDI) OCT of the right (a) and left (b) eyes.

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Figure 3. Infrared autofluorescence and angiography images of the right (a) and left (b) eyes.

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Figure 4. Indocyanine green of the right (a and b) and left (c and d) eyes.

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Question 1: How do you explain findings evident on the fundus photograph [Figure 1]a and [Figure 1]b, OCT [Figure 2]a and [Figure 2]b, FA [Figure 3]a and [Figure 3]b and ICG [Figure 4]a and [Figure 4]b?

Question 2: What is your diagnosis according to the findings?

Question 3: What are the differential diagnoses?

Question 4: What is your plan for this patient?

Question 5: What is the importance of Enhanced Depth Imaging (EDI) findings [Figure 2]?

Reza Karkhaneh, MD

The fundus photographs show concentric hypopigmented perifoveal lesions in the right and left eyes. There are also some small lesions in the posterior poles of both eyes, peripheral to these lesions. Fundus autofluorescence findings are unremarkable, except for a small hyperfluorescent spot between the disc and macula in the right eye, and a small hyperfluorescent spot at the fovea in the left eye.

FA in the mid-phase of the angiogram in the right eye indicates multiple hyperfluorescent spots in the posterior pole that have not changed in shape and size until the late phase, consistent with a transmission defect and some staining. In the left eye the lesions are similar, except for a hyperfluorescent spot that indicates leakage of dye, inferonasal to the macula. ICG angiography reveals diffuse hyperpermeability of the choroid and leakage of dye in both eyes.

OCT shows disruption of interdigitate and ellipsoid zones associated with intraretinal cystoid spaces in the outer and inner nuclear layers, and increased macular thickness in the both eyes. EDI-OCT reveals increased choroidal thickness.

These findings are consistent with chronic central serous chorioretinopathy (CSC)

(cystoid type) associated with cystoid retinal degeneration. Regarding the ICG findings, differential diagnoses such as polypoidal choroidal vasculopathy (PCV) could be considered, but the lack of other signs such as subretinal and intraretinal fluid, exudate and hemorrhage are inconsistent with this disease. OCT findings such as outer retinal disruption and intraretinal cystoid degeneration may be observed in parafoveal telangiectasia (type 2), but the absence of leakage on FA and increased foveal thickness on OCT are not consistent with this condition. Cystoid macular edema (CME) secondary to retinal vascular disease is another differential diagnosis. Absence of fluorescein leakage in the cystoid spaces within the retina is not in line with this disorder.

ICG-based photodynamic therapy (PDT) is the best treatment of choice for this case. Based on the hypothesis that choroidal hyperpermeabiliy is associated with increased expression of the vascular endothelial growth factor (VEGF), intravitreal bevacizumab may be another modality for management of the disease.

OCT shows degeneration of the foveal photoreceptor layer and foveal atrophy, without cystic changes, in most patients with chronic CSC. However, intraretinal cystoid spaces in the macula have been reported.[1],[2] These changes are not necessarily associated with severe reduction of visual acuity.[2] In 2003, Iida et al [1] reported cystoid macular degeneration in seven of eight cases with chronic CSC. Primarily, the intraretinal cystoid spaces are located in the outer retina. In some cases large cystoid spaces occupy most of the retinal thickness. In a study by Piccolino on 34 eyes with chronic CSC, cystoid involvement of the fovea was observed in 20 eyes (58.8%). In 16 eyes (47%), the greatest thickness of retina was because of a large cystoid space in the fovea. In contrast to cystoid macular edema secondary to retinal vascular disease, there is no fluorescein leakage in the cystoid spaces within the retina in patients with chronic CSC. OCT is the most reliable method for identifying these cavities.

Mehdi Modarreszadeh, MD

Answer 1: In the center of the fovea there is a dark spot (as expected) surrounded by a hyperpigmented halo that has created a bull's eye appearance in both eyes. In both eyes, especially the left one, the pigmented area surrounding the fovea has extended somewhat temporally. Any telangiectatic vessels, if present, are not readily visible on these photographs. The autofluorescence images of both eyes show that the foveal centers are hyperfluorescent, indicating deficiency of the overlying xanthophil pigment, probably because of retinal atrophy.

On fluorescein angiography (FA) of the right eye, there are hyperfluorescent spots, mostly on the temporal side of the fovea, which leak slightly during the course of time, recorded up to four minutes. The very center of the fovea is also hyperfluorescent. Elsewhere on the angiogram, there are hyperfluorescent spots, especially inferonasal to the fovea, which do not increase in size and seem to be retinal pigment epithelium (RPE) defects (window defects). In the FA of the left eye, the leaking points temporal to the fovea are more prominent. The center of the fovea and the hyperfluorescent dot inferonasal to it, as well as, some extrafoveal dots are again window defects, with some added element of RPE staining in the case of the inferonasal lesion.

In the ICG angiograms, some dilated choroidal vessels are seen in the posterior pole, occasionally, showing a beading appearance in some areas.

OCT images show cystic spaces inside the retinal tissue surrounding the fovea and a cystic space in the very center of the fovea, with draping of a superficial retina over it. On EDI-OCT, mild thickening of the choroid is in the left eye. The apparent thickening of the subfoveal choroid, however, may be related to its enhanced visibility due to atrophy of the retinal tissue.

Answer 2: Considering the leaking points surrounding the fovea, especially on the temporal side, the presence of cystic lesions inside the retina and the appearance of draping on the fovea, my first impression is parafoveal telangiectasia.

Answer 3: In differential diagnoses, polypoidal choroidal vasculopathy (PCV) or some kind of pachychoroid retinopathy must be considered. This is based on the appearance of dilated choroidal vessels on the posterior pole and suggestion of some choroidal thickening on the EDI-OCT. However, the presence of cystic spaces in the retina and the absence of pigment epithelial detachments are difficult to explain with this diagnosis. The relative hyperopia, however, is in its favor.

Answer 4: The nature of ocular findings should be discussed in detail with the patient. Intravitreal anti VEGF injections may be tried, which may address both tentative diagnoses. This may be repeated a few times to judge the effect. If telangiectatic vessels are present on careful clinical funduscopy, thermal laser photocoagulation may be tried. If these modalities of treatment fail, PDT may be tried, first for the right eye, which has lower vision and more prominent choroidal vessels.

I am not very impressed by the EDI findings. As stated above, the apparently increased thickness of the choroid in the very center of the fovea may be related to atrophy of the overlying retina. Elsewhere, the choroidal thickness (312, 328 micrometers) is not much different from the normal and some mildly increased thickness may be related to hyperopia.

Homayoun Tabandeh, MD

This patient is a 59-year-old man, who has decreased visual acuity and metamorphopsia in both eyes for one year. Past ocular history is notable for uncomplicated cataract surgery and intraocular lens implantation in the left eye. He has been in good general health. Corrected visual acuity is 3/10 OD and 6/10 OS.

Color fundus photograph shows a hazy view of the right fundus due to media opacity. There are small macular holes, subtle drusenoid deposits, and RPE changes in both eyes. The OCT shows a macular hole, hyporeflective cavities, and attached posterior hyaloid face in both eyes (OU). FA is consistent with macular telangiectasia (MacTel) and secondary leakage in the later phases of the angiogram, in both eyes. ICG angiography shows areas of irregularly dilated choroidal vessels and hyperpermeability in both eyes. EDI-OCT indicates choroidal thickening in the macular region, OU.

This is an interesting case with multiple abnormalities on multimodal imaging. To summarize, there is visually significant cataract in the right eye, the vision in the pseudophakic eye is relatively good. Other findings include small macular holes, MacTel, thickened choroid, and irregularly dilated hyperpermeable choroidal vessels on ICG, in both eyes.

Further evaluation could include OCT angiography, illustrating the retinal and choroidal vascular architecture. B-scan ultrasound would be useful to rule out subclinical posterior scleritis. A baseline systemic evaluation to rule out diabetes, hypertension, and inflammatory disorders, among others, would also be helpful.

This patient may be managed initially with observation. He has done well with cataract surgery in the left eye. Cataract surgery could be considered in the right eye, if he is significantly affected by blurred vision.

The macular holes are small in both eyes, presumably causing a similar degree of visual impairment (0.6). Macular hole surgery in the setting of MacTel is associated with variable outcomes and could be considered if the macular hole progresses, further reducing vision. Spontaneous closure of a small macular hole associated with MacTel has also been reported. The thickened choroid (pachychoroid), as demonstrated on EDI-OCT, may be idiopathic, inherited, associated with central serous chorioretinopathy (CSC), polypoidal choroidal vasculopathy or an inflammatory and infiltrative disorder. In this case, choroidal hyperpermeabilty and irregularly dilated choroidal vessels may be associated with pachychoroid or forme fruste CSC.


  Consultants Top


Reza Karkhaneh, MD. Eye Research Center, Farabi Eye Hospital, Tehran University of Medical Sciences Tehran, Iran.

Mehdi Modarreszadeh, MD. Eye Research Center, Rassoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran.

Homayoun Tabandeh, MD. Retina-Vitreous Associates Medical Group, Los Angeles, California, USA.

Financial Support and Sponsorship

Nil.

Conflicts of Interest

There are no conflicts of interest.

 
  References Top

1.
Iida T, Hagimura N, Sato T, Kishi S. Evaluation of central serous chorioretinopathy with optical coherence tomography. Am J Ophthalmol 2000;129:16-20.  Back to cited text no. 1
    
2.
Piccolino FC, De La Longrais RR, Manea M, Cicinelli S. Posterior cystoid retinal degeneration in central serous chorioretinopathy. Retina 2008;28:1008-1012.  Back to cited text no. 2
    


    Figures

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



 

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