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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 1  |  Page : 114-116

A novel presentation of atypical bilateral Chandler syndrome with an insight into management


Glaucoma Services, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

Date of Submission19-Apr-2020
Date of Acceptance28-Jul-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Dr. Dewang Angmo
Assistant Professor of Ophthalmology, Room 374, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1045_20

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  Abstract 


We report an atypical presentation of bilateral Chandler's syndrome with secondary angle-closure glaucoma. Right eye (RE) AS-OCT showed higher lens vault. Left eye (LE) had decompensated cornea. Intraocular pressure (IOP) in RE was 18–20 mm Hg on four topical glaucoma medications. RE cup-disk-ratio was 0.8–0.9:1. Specular and confocal microscopy aided in diagnosis. As trabeculectomy had poor prognosis (due to increased lens vault), RE phacoemulsification was done in the first stage. Postoperatively IOP in the RE was 14 mm Hg on topical glaucoma medications at 3 months follow-up. Parameters like corneal status, lens vault and IOP control must be taken into account before planning for filtering surgery.

Keywords: Chandler syndrome, iridocorneal endothelial syndrome cells, lens vault, peripheral anterior synechia


How to cite this article:
Mahalingam K, Yadav M, Nathiya V, Angmo D. A novel presentation of atypical bilateral Chandler syndrome with an insight into management. Indian J Ophthalmol Case Rep 2021;1:114-6

How to cite this URL:
Mahalingam K, Yadav M, Nathiya V, Angmo D. A novel presentation of atypical bilateral Chandler syndrome with an insight into management. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Mar 3];1:114-6. Available from: https://www.ijoreports.in/text.asp?2021/1/1/114/305471



Iridocorneal endothelial syndrome (ICE) is described as a rare entity that consists of an endothelial abnormality with associated angle and iris changes which presents with variable corneal decompensation and glaucoma. It usually presents in middle-aged young females and has been described to be mostly unilateral in presentation.[1] Three clinical variants have been described viz Essential iris atrophy, Chandler syndrome and Cogan Reese syndrome. We describe an atypical presentation of bilateral Chandler's syndrome with secondary angle-closure glaucoma in a male patient and an insight into its management.


  Case Report Top


A 62-year-old male, presented with chief complaints of diminution of vision in both eyes (left > right) since 4 years, which deteriorated further in the left eye after ocular surgery. Left eye (LE) phacoemulsification with intraocular lens was done 3 months back. Best-corrected visual acuity of right eye (RE) 6/12 and LE finger counting close to face, with projection of rays accurate in all quadrants. On examination, RE showed relatively clear cornea [Figure 1]a, multiple peripheral anterior synechiae (PAS) [Figure 1]b and grade 2 nuclear sclerosis of lens. The LE cornea showed the presence of diffuse stromal oedema along with epithelial bullae [Figure 1]c and [Figure 1]d with posterior chamber intraocular lens. RE fundus showed cup-disc-ratio of 0.8-0.9:1 with thinning of the inferior and superior neuro-retinal rim [Figure 2]a with corresponding advanced visual field changes [Figure 2]b. LE media was hazy, ultrasound B scan was anechoic. Intraocular pressure (IOP) in RE was 18-20 mmHg and in LE was 24 mmHg (measured with tonopen) on four topical glaucoma medications.
Figure 1: Slitlamp image (a) of RE showing apparently clear cornea with multiple PAS on gonioscopy (b). Slit.lamp image (c) of LE showing decompensated cornea with diffuse corneal edema and epithelial bullae (d)

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Figure 2: Fundus image (a) of RE showing CDR 0.8 to 0.9:1 with HVF 10-2 (b) showing corresponding field defects

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Anterior segment OCT of RE showed PAS along with increased lens vault (720 μm) [Figure 3]. Central corneal thickness was 480 μm in RE eye, LE was not capturable due to corneal decompensation. Specular microscopy showed pleomorphism and polymegathism along with dark cells with central light spot with light borders characteristic of ICE cell [Figure 4]a. Confocal microscopy showed activated keratocytes, degeneration of subbasal corneal nerves and endothelium [Figure 4]b, [Figure 4]c, [Figure 4]d.
Figure 3: Anterior Segment OCT of RE showing lens vault of 720 μm (relatively high)

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Figure 4: Specular microscopy (a) of RE showing pleomorphism and polymegathism accompanied by dark cells with central light spot with light borders characteristic of ICE cell, confocal microscopy showing activated keratocytes, degeneration of subbasal corneal nerves and endothelium. (b-d)

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A diagnosis of atypical chandlers syndrome with secondary angle-closure glaucoma was made based on specular microscopy features suggestive of ICE cells and clinical examination. It was atypical because of bilaterality and male sex.

Oral glaucoma medications were started and RE phacoemulsification with posterior chamber intraocular lens implantation was done as lens vault was increased. In a study from our centre, the average lens vault in primary angle-closure glaucoma patients was found to be 841 ± 17 μm.[2] In the left eye Descemet's membrane endothelial keratoplasty (DMEK) was planned. Postoperatively IOP in RE was 14 mm Hg and LE was 12 mm Hg on four topical glaucoma medications at 3 months follow-up.


  Discussion Top


Chandler's syndrome is characterised predominantly by corneal endothelial abnormality with minimal iris changes. Though mostly a unilateral entity, bilaterality of this disease has been reported in a few cases in the past.[3],[4],[5] In a retrospective study among 203 patients of ICE syndrome, bilaterality was seen in 10% of cases.[6] Corneal decompensation and glaucoma, the two important manifestations seem to be the consequences of progressive anterior segment morphological changes that occur in eye namely transformation of endothelial cells to epithelioid like features called as ICE cells.[7] Migration of abnormal endothelial cells and PAS formation leads to obstruction of aqueous outflow and glaucoma.[1]

A total of 50%–70% of patients with ICE syndrome had associated glaucoma.[6],[8] Glaucoma is refractory and more than three-fourths of them required surgery. Primary trabeculectomy had a success rate of 60% and 21% at the end of 1st and 5th year, respectively.[8] While dealing with angle-closure glaucoma patients with higher lens vault, phacoemulsification even in the presence of insignificant cataract can be considered as it helps in removing non-pupillary block mechanism of angle-closure recalcitrant to iridotomy.[9] In a study from our centre on phacoemulsification in primary angle-closure glaucoma patients, there was a significant reduction in IOP post-operatively especially in patients with high lens vault, reducing the number of glaucoma medications needed.[2],[10] We also noted, higher the lens vault, greater is the reduction in IOP postoperatively.[2] Moreover, trabeculectomy in patients with secondary angle-closure glaucoma patients with high lens vault and extensive PAS has a poorer prognosis as there are higher chances of fibrosis and failure along with the higher rate of postoperative shallow anterior chamber compared to primary open-angle glaucoma patients. Also, combined surgery in such eyes would be difficult and therefore sort with complications and it has a poorer longterm success too. Due to progressive nature of the Chandler's syndrome along with age-related endothelial loss and increase in lens thickness, the patient may have a higher risk of corneal decompensation if cataract surgery is required at a later stage. So, in our patient with an increased lens vault, phacoemulsification was done in the first stage. At the time of presentation, the patient did not use medications in LE regularly (as he lost vision). A proper counselling regarding the timings and importance of regular usage of medications was given to the patient, which helped to control the IOP on follow-up. The patient was also counselled for regular lifelong follow-up and will be considered for filtering surgery in future if indicated.


  Conclusion Top


Although rare, bilateral presentation of ICE syndrome must be kept in mind while diagnosing such patients of corneal decompensation with PAS. Specular and confocal microscopy aids in confirming the diagnosis. Parameters like corneal status, lens vault and control of IOP must be taken into account before planning for filtering surgery in such secondary angle-closure glaucoma cases as it has a higher failure rate and a poor long-term prognosis. Therefore, the management of such atypical cases needs to be individualised on case to case basis.

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.
Silva L, Najafi A, Suwan Y, Teekhasaenee C, Ritch R. The iridocorneal endothelial syndrome. Surv Ophthalmol 2018;63:665-76.  Back to cited text no. 1
    
2.
Angmo D, Shakrawal J, Gupta B, Yadav S, Pandey RM, Dada T. Comparative evaluation of phacoemulsification alone versus phacoemulsification with goniosynechialysis in primary Angle-Closure glaucoma: A randomized controlled trial. Ophthalmol Glaucoma 2019;2:346-56.  Back to cited text no. 2
    
3.
Huna R, Barak A, Melamed S. Bilateral iridocorneal endothelial syndrome presented as Cogan-Reese and Chandler's syndrome. J Glaucoma 1996;5:60-2.  Back to cited text no. 3
    
4.
Islam F, Azad N, Khan A. Bilateral iridocorneal endothelial (ICE) syndrome with microspherophakia. J Coll Physicians Surg Pak 2011;21:374-5.  Back to cited text no. 4
    
5.
Gupta V, Kumar R, Gupta R, Srinivasan G, Sihota R. Bilateral iridocorneal endothelial syndrome in a young girl with Down's syndrome. Indian J Ophthalmol 2009;57:61-3.  Back to cited text no. 5
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6.
Chandran P, Rao HL, Mandal AK, Choudhari NS, Garudadri CS, Senthil S. Glaucoma associated with iridocorneal endothelial syndrome in 203 Indian subjects. PLoS One 2017;12:e0171884.  Back to cited text no. 6
    
7.
Walkden A, Au L. Iridocorneal endothelial syndrome: Clinical perspectives. Clin Ophthalmol 2018;12:657-64.  Back to cited text no. 7
    
8.
Laganowski HC, Kerr Muir MG, Hitchings RA. Glaucoma and the iridocorneal endothelial syndrome. Arch Ophthalmol 1992;110:346-50.  Back to cited text no. 8
    
9.
Nongpiur ME, Singhal S, Stewart S, Htoon HM, Narayanaswamy AK, Wong TY, et al. The impact of lens vault on visual acuity and refractive error: Implications for management of primary Angle-closure glaucoma. J Glaucoma 2016;25:e236-40.  Back to cited text no. 9
    
10.
Selvan H, Angmo D, Tomar AS, Yadav S, Sharma A, Dada T. Changes in intraocular pressure and angle status after phacoemulsification in primary angle closure hypertension. J Glaucoma 2019;28:105-10.  Back to cited text no. 10
    


    Figures

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



 

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