A 40-year-old male presented with a history of diminution of vision in the right eye, noted a month ago. The visual acuity was hand motions in right eye (OD) and 6/6 in left eye (OS), intraocular pressure was 12 mmHg in OD and 14 mmHg in OS. Pupillary reaction was sluggish in OD. On examination, OD showed dilated episcleral vessels at 8:00 o’clock, scleral pigmentation from 7:00 to 8:00 o’ clock, diffuse pigmentation of the peripheral iris and anterior chamber angle from 3:00 to 9:00 o’clock and cortical and nuclear cataract [Fig. 1]. Fundus view was hazy in the RE. The left eye was normal.
What Will be the Next Step?
- Small-incision cataract surgery (SICS) OD
- Phacoemulsification in OD
- Ultrasound B-scan and ultrasound biomicroscopy (UBM) OD
- Fine needle biopsy OD
The patient underwent ultrasound B-scan and ultrasound biomicroscopy (UBM), which showed an inferotemporal ciliary body tumor extending into the choroid, measuring 16 mm base and 12 mm height. The patient was clinically diagnosed to have a large ciliochoroidal melanoma with possible intrascleral extension. and was counselled for en-bloc primary enucleation with an orbital implant, confirmation of diagnosis on histopathology and systemic evaluation. Surgery was uneventful. Histopathology confirmed the diagnosis of ciliochoroidal melanoma, mixed epithelioid and spindle B-cell type with mitotic figures 2/40 high power fields. Anterior chamber angle, peripheral iris and scleral patch showed only pigment deposit and no tumor cells. Systemic evaluation was unremarkable. The patient continues to do well on annual follow-up.
Correct answer: C
Dense cataract often precludes fundus examination. Before proceeding with the surgery, it is very important to perform an ultrasound B-scan (and an UBM in a suspected cilary body tumor) to rule out an intraocular tumour. Shields et al. reported a series of 20 cases of pseudomelanoma in patients with hypermature cataract based on ultrasound findings of non-contiguity with the choroid, postural change in location of lesion, ciliary body mass in all 4 quadrants on UBM and absence of sentinel vessels and transillumination shadow on examination. Uveal melanoma, in contrast, presents as a dome shaped mass, contiguous with uveal tissue with characteristic moderate to low internal reflectivity. Atypical unilateral cataract in a young patient without any predisposing factors, sentinel episcleral vessels, scleral pigmentation, iris bump or sectoral shallow anterior chamber, anterior chamber angle pigmentation, iris neovascularization, elevated intraocular pressure, and sectoral zonular dehiscence should be the red flags to suspect an underlying ciliary body melanoma. In such cases, gonioscopy, UBM and ultrasound B-scan (magnetic resonance imaging and positron emission tomography scan if indicated) are mandatory before planning further management. Based on the size and location of the tumour, the treatment may be conservative with plaque brachytherapy or enucleation. If one proceeds with plaque brachytherapy, safety-enhanced phacoemulsification with a clear corneal approach and preservation of posterior capsule is recommended to visualize the tumor to plan plaque brachytherapy and monitor response to treatment. Any violation of the conjunctiva and sclera in SICS, trabeculectomy, or glaucoma drainage device predisposes the patient to develop extraocular extension of melanoma and it becomes medicolegally imperative to proceed with enucleation with en-bloc excision of the incision area. In conclusion, patients with atypical unilateral cataracts should be carefully investigated to rule out an occult intraocular tumor before considering cataract surgery.
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.
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