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One of the most challenging aspects of vitreoretinal surgery is the overlap with posterior segment ocular oncology. The diagnostic and therapeutic exigences in tackling neoplastic conditions of the retina and choroid require close collaboration between ophthalmic subspecialist teams. In the following case, technical nuances in the surgical management of underlying choroidal melanoma are highlighted. Furthermore, the role of inter-specialty communication is shown to be front and center in the pursuit of the best possible outcomes in complex oncology cases requiring vitreoretinal surgery.
CASE REPORT
A 31-year-old woman was referred to the Retina division of the Wills Eye Hospital in Philadelphia for evaluation of new floaters and vision loss in the left eye. Upon initial examination, she was found to have counting fingers vision in the affected eye, with dense vitreous hemorrhage precluding visualization of the fundus. B-scan ultrasonography revealed an inferonasal choroidal mass (14 mm basal diameter, 5 mm thickness) with relative internal echolucency and a mushroom-shaped configuration, concerning for possible choroidal melanoma (Figure 1). A lack of spontaneous vascular pulsations on dynamic B-scan, as well as an early cleft deep to the mass left the possibility of an evolving multilayered hemorrhage secondary to valsalva retinopathy in the differential diagnosis (although the patient denied any straining). The contralateral, uninvolved right eye had 20/20 VA, and ophthalmic examination was within normal limits.
Figure 1. B-scan ultrasonography demonstrates an internally echolucent choroidal mass with overlying echogenic vitreous opacity, concerning for possible choroidal melanoma and vitreous hemorrhage.
Consultation was requested of the Wills Ocular Oncology division, whose prompt evaluation confirmed the findings above. Given the partially equivocal findings, MRI imaging with contrast was obtained and showed enhancement of the lesion, which favored a neoplastic process. Prompt surgery was recommended to clear the vitreous hemorrhage and allow visualization for further diagnostic and therapeutic intervention.
Two weeks after initial presentation, the patient was taken for diagnostic and therapeutic pars plana vitrectomy, where intraoperative findings revealed a large pigmented choroidal/subretinal mass in the inferonasal near periphery with hemorrhagic eruption through the neurosensory retina into the vitreous cavity (Figure 2). Intraoperative consultation with ocular oncology was requested, and choroidal melanoma with hemorrhagic sequelae remained the leading and working diagnosis.
Figure 2. Pseudocolor ultra-widefield imaging of the left fundus on postoperative day 1 following vitrectomy and silicone oil placement. Hemorrhagic rupture of the choroidal mass into the vitreous cavity is seen just inferior to the inferotemporal arcade.
Given findings consistent with active malignant process, further surgical manipulation of the lesion was deferred, silicone oil was instilled into the eye to tamponade the full-thickness retinal defect, and the sclerotomy sites were treated with triple freeze-thaw cryotherapy and sutured closed. An undiluted vitreous sample showed chronic hemorrhagic and glial elements on pathologic analysis without neoplastic cells.
With a clear view to the posterior pole, the ocular oncology team monitored the patient postoperatively and obtained a fine-needle aspiration biopsy of the lesion that showed atypical spindle cells suggestive of melanocytic proliferation. Although final pathologic assessment was deemed insufficient for a definitive diagnosis, the collective findings were most consistent with malignant melanoma, and the ocular oncology team elected to treat the lesion with iodine-125 plaque brachytherapy.
In the post-radiation period of follow-up, full regression of the choroidal tumor was confirmed by ocular oncology, but the patient’s course was complicated by ghost cell glaucoma (requiring topical ocular antihypertensive therapy and close follow-up with the glaucoma service) and expected progression of posterior subcapsular cataract. VA progressed to hand motions, and adequate visualization of the posterior segment was lost to the progressing cataract and ultrasonographic artifact from silicone fill.
Following cataract consultation with our anterior segment specialists, the decision was made to again intervene surgically (2 months post primary vitrectomy), with a multispecialty approach, including cataract extraction by anterior segment colleagues, anterior chamber washout, silicone oil exchange, and complex combined tractional and rhegmatogenous retinal detachment repair to promote rehabilitation of the affected eye.
Intraoperatively, inspection of the fundus following silicone oil removal revealed a persistent full-thickness retinal defect overlying the regressed tumor. The surrounding retina was fibrotic, with elevation and subretinal fluid extending into the inferior macula from mixed tractional change and mechanical elevation from persistent dehemoglobinized subretinal hemorrhage. Using the 23-gauge vitreous cutter, fibrotic and hemorrhagic elements were peeled, sectioned, and aspirated to achieve sufficient retinal pigment epithelium-retinal apposition to allow for complete laser retinopexy around the retinal defect. Efforts were not made to remove absolutely all subretinal material, as the risks of choroidal violation (namely hemorrhage and/or exposure to neoplastic elements), especially in an area of prior malignancy, were understood.
Six months following this definitive procedure, and at the time of publication of this report, the patient has done well, with improvement in VA under silicone oil to 20/200, adequate control of IOP with topical antihypertensives, and progressive reduction in mass-effect-related subretinal fluid with spontaneous regression of residual subretinal clot (Figures 3 and 4). Silicone oil removal with prophylactic panretinal photocoagulation to limit sequelae of radiation retinopathy complications is planned within the next several months.
Figure 3. Pseudocolor ultra-widefield images of the left eye on postoperative day 1 (left) and postoperative month 4 (right) showing evolution of laser scarring and progressive absorption of subretinal hemorrhage after the patient’s second vitrectomy.
Figure 4. Vertical raster OCT images through the fovea of the left eye taken at postoperative week 1 (POW1), postoperative month 4 (POM4), and postoperative month 6 (POM6), demonstrating gradual resorption of subretinal hemorrhage and decreasing subretinal fluid with observation under silicone oil tamponade.
DISCUSSION
This case displays the complexity in diagnosis and management of posterior segment ocular neoplasms requiring vitreoretinal surgery. A cooperative multidisciplinary approach is essential, and the clinical course for eyes presenting with these serious conditions is better characterized as a marathon than a sprint.
The potential overlap in fundus appearance of choroidal melanoma and choroidal hemorrhage has been well-discussed in the literature.1-3 The hemorrhagic sequelae of our patient’s ocular mass added further uncertainty as to the definitive causative pathology. Nevertheless, with a collaborative approach between the vitreoretinal and ocular oncology teams, multimodal imaging, and pathologic assessment, sufficient features could be identified to proceed with prompt treatment and excellent regression of the underlying malignancy.
It is important to highlight the cautious, staged approach to this patient’s surgical interventions. During the first vitrectomy, no manipulation of the underlying mass was performed, nor was endolaser retinopexy performed, despite the presence of a full-thickness retinal rupture. Rather, the use of silicone oil tamponade alone provided the temporization needed prior to definitive intervention. A study in ex vivo cadaveric eyes suggests that the presence of silicone oil may also limit the amount of undesired exposure of healthy adjacent ocular tissue to brachytherapy radiation and thus may reduce the risk of radiation retinopathy,4 although this was not the primary intent of silicone oil instillation in this case. Cryotherapy to the sclerotomies and frequent postoperative monitoring reflect an awareness of the risk of tumor seeding in vitrectomies in eyes with active choroidal melanomas.5,6 Vitrectomy in eyes that have undergone plaque brachytherapy treatment has been studied and is generally considered to be safe.7-9 During our patient’s second vitrectomy (post plaque therapy), more definitive retinal detachment repair could therefore be completed. Care was still taken to accomplish adequate retinal apposition and retinopexy with as conservative an amount of dissection and clot removal from over the tumor bed as possible. Indeed, aggressive surgical invasion into the underlying choroid is known to increase risk of tumor spread, severe hemorrhagic complications, and even air embolus and death.10
CONCLUSIONS
In summary, management of eyes with concurrent choroidal melanomas and secondary vitreoretinal complications, such as vitreous hemorrhage and retinal detachment, requires a thoughtful approach and collaboration among multiple ophthalmic subspecialists. While patients must be counseled on the severity and guarded prognosis of their ocular condition despite optimum therapy, the potential for good anatomic and functional outcomes remains.
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2. Honig SE, Srinivasan A, Shields CL. Suprachoroidal hemorrhage simulating melanoma in idiopathic thrombocytopenic purpura. Ocul Oncol Pathol. 2019;5(3):162-166.
3. Marous CL, Sioufi K, Shields CL, et al. Coughing-induced suprachoroidal hemorrhage simulating melanoma in two cases. Retin Cases Brief Rep. 2018;12(4):336-341.
4. Oliver SC, Leu MY, DeMarco JJ, et al. Attenuation of iodine 125 radiation with vitreous substitutes in the treatment of uveal melanoma. Arch Ophthalmol. 2010;128(7):888-893.
5. Azarcon CP, Williams PJ, Grossniklaus HE, et al. Infusion port site tumor seeding after pars plana vitrectomy-assisted biopsy of choroidal melanoma [published online ahead of print, 2022 Dec 26]. Retin Cases Brief Rep. 2022;10.1097/ICB.0000000000001396.
6. Mashayekhi A, Shields CL, Eagle RC Jr, Shields JA. Vitreous and preretinal seeding after transvitreal fine needle aspiration biopsy of choroidal melanoma. Retin Cases Brief Rep. 2021;15(6):713-717.
7. Beykin G, Pe’er J, Hemo Y, et al. Pars plana vitrectomy to repair retinal detachment following brachytherapy for uveal melanoma. Br J Ophthalmol. 2013;97(12):1534-1537.
8. Foster WJ, Harbour JW, Holekamp NM, et al. Pars plana vitrectomy in eyes containing a treated posterior uveal melanoma. Am J Ophthalmol. 2003;136(3):471-476.
9. Lonngi M, Houston SK, Murray TG, et al. Microincisional vitrectomy for retinal detachment in I-125 brachytherapy-treated patients with posterior uveal malignant melanoma. Clin Ophthalmol. 2013;7:427-435.
10. Reichstein D, Karan K. Endoresection utilizing pars plana vitrectomy for benign and malignant intraocular tumors. Curr Opin Ophthalmol. 2019;30(3):151-158.
