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   Table of Contents      
Year : 2020  |  Volume : 68  |  Issue : 8  |  Page : 1519

The veiled monster

1 Orbit, Oculoplasty, Reconstructive and Aesthetic Services, Aditya Birla Sankara Nethralaya, Kolkata, West Bengal, India
2 Bhagwan Mahavir Vitreo-Retina Services, Sankara Nethralaya, Chennai, Tamil Nadu, India

Date of Web Publication24-Jul-2020

Correspondence Address:
Dr. Md Shahid Alam
Orbit, Oculoplasty, Reconstructive and Aesthetic Services, Aditya Birla, Sankara Nethralaya, Kolkata - 700099, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_472_20

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How to cite this article:
Alam MS, Roy SS, Khetan V. The veiled monster. Indian J Ophthalmol 2020;68:1519

How to cite this URL:
Alam MS, Roy SS, Khetan V. The veiled monster. Indian J Ophthalmol [serial online] 2020 [cited 2020 Dec 4];68:1519. Available from: https://www.ijo.in/text.asp?2020/68/8/1519/290451

  Case Top

A 74-year-old Asian lady presented with gradual diminution of vision and pain in the left eye (OS) for the past 6 months. She was diagnosed to have complicated cataract in the left eye and was advised cataract surgery elsewhere. Her best corrected visual acuity in the right eye was 20/60, while it was just perception of light in the left. Anterior segment evaluation of OS showed total cataract with multiple posterior synechiae and irregular nonreactive pupil [Figure 1]a. Multiple pigmented lesions were noted at 11 and 2:30'O clock position which was considered to be primary acquired melanosis (PAM). View of the fundus was completely obscured because of complicated total cataract. Right eye was otherwise normal.
Figure 1: (a) Diffuse slit lamp photograph showing total cataract, posterior synichiae with irregular pupil. (b) Ultrasound B scan showing large intraocular mass with high surface reflectively and diffuse internal low reflective echoes, suggestive of ciliochoroidal melanoma. (c) MRI axial cut showing a large intra ocular mass displaying T1 hyperintensity. (d) Microphotograph (Hematoxylin and Eosin 20X) showing a tumor arising from ciliary body and choroid. The tumor cells exhibit nuclear atypia and anaplasia, and are predominantly of epitheloid variety. Findings are suggestive of ciliochoroidal melanoma

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  What is Your Next Step? Top

  1. Consider for cataract surgery and plan for fundus evaluation thereafter
  2. Explain guarded visual prognosis and start on topical steroids
  3. Ultrasonography (USG) B scan
  4. Complete uveitis workup and plan cataract surgery under steroid cover.

  Findings Top

Ultrasound B-scan of OS revealed a large homogenous retino-choroidal mass filling the entire vitreous cavity associated with retinal detachment [Figure 1]b. Magnetic resonance imaging (MRI) of the orbit showed a large dome shaped cilio-choroidal lesion in the nasal and superior quadrant of OS, displaying hyperintense signal on T1 weighted images, suggestive of ciliochoroidal melanoma [Figure 1]c. The pigmented lesions over the sclera were now suspected to be extrascleral extension. The patient underwent enucleation with ball implant of OS under general anesthesia. The conjunctiva around the suspected pigmented lesions was left intact and peritomy was carried out with 5 mm clear margin. Histopathology showed a pigmented tumor arising from the ciliary body and choroid and extending over the optic nerve; however there was no invasion of the optic nerve. The tumor cells were predominantly composed of epitheloid cells with high nucleo-cytoplasmic ratio and prominent nucleoli [Figure 1]d. Extrascleral extension was noted however the overlying conjunctiva in these areas was spared. The findings were suggestive of cilio-choroidal melanoma. Metastatic workup in the form of chest X-ray, ultrasound abdomen and pelvis and liver function test was negative.

  Correct Answer: C. Top


Cilio-choroidal Melanoma (OS).

  Discussion Top

Choroidal melanoma is the most common primary intraocular malignancy in adults.[1] Usually melanoma can present with various manifestations and can mimic other lesions leading to a diagnostic ambiguity. Hence, a strong clinical suspicion with appropriate imaging is warranted.[2] Cases of uveal melanoma mimicking panophthalmitis and bleeding anterior staphyloma have been described earlier in literature.[3],[4] Khetan et al., reported a case of uveal melanoma presenting as staphyloma and complicated cataract in a 45-year-old female, in similarity with the present case.[5] Our case highlights the necessity of meticulous examination and importance of ultrasound in all cases presenting as dense cataract which preclude fundus examination. The possibility of harbouring an intraocular mass in all such cases cannot be totally ruled out and they should not be planned for cataract surgery unless an ultrasound screening has been done. With high mortality and a poorer prognosis, choroidal melanoma is indeed a tumor which needs early recognition with prompt treatment.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Singh AD, Turell ME, Topham AK. Uveal melanoma: Trends in incidence, treatment, and survival. Ophthalmology 2011;118:1881-5.  Back to cited text no. 1
Shields JA, Mashayekhi A, Ra S, Shields CL. Pseudomelanomas of the posterior uveal tract: The 2006 Taylor R. Smith Lecture. Retina 2005;25:767-71.  Back to cited text no. 2
Sood NN, Ratnaraj A. Malignant melanoma of Choroid presenting as panophthalmitis. J All India Ophthalmol Soc 1968;16:67-9.  Back to cited text no. 3
Alam MS, Tongbram A, Krishnakumar S, Mukherjee B. Ciliochoroidal melanoma presenting as bleeding anterior staphyloma: Report of a case and review of literature. Indian J Ophthalmol 2019;67:1162-3.  Back to cited text no. 4
[PUBMED]  [Full text]  
Khetan V, Gupta K, Mohan ER, Gopal L. Uveal melanoma presenting as cataract and staphyloma. Indian J Ophthalmol 2009;57:223-5.  Back to cited text no. 5
[PUBMED]  [Full text]  


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