7th Residency Announcement

Exciting Residency Announcement!

ACGME approves seventh resident complement; first increase in over 30 years!

Grand Rounds

Grand Rounds and Case Studies

Check out our weekly presentations

OTEP

OTEP

Ophthalmic Technician Education Program

UpClose Banner cropped

­­UpClose Fall Newsletter 2017

Keep up with the latest USC Roski news

LARGEST STUDY ON CHINESE AMERICANS PUBLISHED

LARGEST STUDY ON CHINESE
AMERICANS PUBLISHED

USC Ophthalmology Researchers Find More
Effective Treatments For Blinding Eye Diseases

EDUCATION

Case Study: Sowing Seeds of Doubt

Mustafi Kim
Presenter: Zachary Koretz, BSc
Visiting Medical Student
Discussants: Jonathan Kim, MD & Alena Reznik, MD
 

History

  • 75-year-old male presents with one week of blurry vision in the right eye
  • Blurriness has been constant, without pain, flashes or floaters
  • Past ocular history: Uveal melanoma in the right eye, arising from ciliary body and extending anteriorly into the iris and angle
    • Treated with plaque brachytherapy seven months prior to this presentation
    • Stable tumor size on last ultrasound biomicroscopy examination
  • Past medical history: hypertension (controlled)
  • Family history: no family history of ocular problems
  • Social history: former smoker

Exam Findings

  • VA
    • OD: 20/50 (prior 20/30)
    • OS: 20/25-1
  • Pupils 4mm, corectopic, fixed, no RAPD || 3mm, round, reactive, no RAPD
  • IOP (Ta) 40 mmHg || 10 mmHg
  • Extraocular movements intact
  • Anterior segment exam
    • Lids/Lashes/Adnexa: normal, both eyes
    • Conjunctiva/Sclera: normal, both eyes
    • Cornea: diffuse endopigment || clear
    • AC: 1/2+ pigmented cells, no white blood cells || deep and quiet
    • Iris: inferotemporal peripheral pigmented mass || flat and round
    • Lens: 1-2+ nuclear sclerosis, central posterior subcapsular cataract || 1-2+ nuclear sclerosis
    • Gonioscopy
      • OD
        • Heavily pigmented trabecular meshwork
        • Open angle except area of pigmented ciliary body lesion extending anteriorly/inferiorly into the angle around 8:00 clock hour, +peripheral anterior synechiae
      • OS
        • Non-pigmented trabecular meshwork
        • Open angle 360˚
  • Posterior segment exam
    • Cup-to-disc ratio 0.35 x 0.35 || 0.3 x 0.3
    • Disc flat, pink, no pallor || flat, pink, no pallor
    • Macula normal, both eyes
    • Vessels normal, both eyes
    • Periphery temporal chorioretinal atrophy || normal

Figure 1
Figure 1: External photo of the right eye prior to treatment demonstrates pigmented lesion from 7:00 to 9:00 clock hours (left). External photo of the right eye at current presentation demonstrates corectopia and decreased appearance of uveal melanoma from 7:00 to 9:00 clock hours, status-post brachytherapy 7 months prior (right).

Figure 1
Figure 2: Slit-lamp photo of the right eye demonstrating deformation of iris at 7:00 clock hour, before treatment (left) and status-post brachytherapy 7 months prior (right).

Figure 1
Figure 3: Example image of pigment on corneal endothelium (Krukenberg’s spindle), similar to what was seen on patient’s exam. (Photo courtesy of Sarwat Salim, MD, University of Tennessee).


Figure 1
Figure 4: Example gonioscopy images demonstrating heavily pigmented trabecular meshwork in the right eye (A) and non-pigmented trabecular meshwork in the left eye (B), similar to what was seen on the patient’s exam. (Yijie (Brittany) Lin, MD, & Joseph F. Panarelli, MD, 2015).

 

Differential Diagnosis

  • Open angle glaucoma
    • Secondary pigmentary glaucoma
    • Melanomalytic glaucoma
    • Direct infiltration of angle by untreated melanoma cells
  • Closed angle glaucoma
    • Secondary to peripheral anterior synechiae
    • Secondary to posterior synechiae with iris bombe
    • Secondary to anterior displacement of ciliary body
    • Secondary neovascular glaucoma

Additional Investigations


Figure 1
Figure 5: Ultrasound biomicroscopy image demonstrating uveal melanoma extending from ciliary body, prior to brachytherapy (top) and 7 months after brachytherapy (bottom).

 


Figure 1
Figure 6: OCT optic nerve head and retinal nerve fiber layer (RNFL) demonstrates a superotemporal RNFL defect in the right eye.

 


Figure 1
Figure 7: OCT demonstrates possible defect of ganglion cell/inner plexiform layer in the right eye.

 


Figure 1
Figure 8: OCT demonstrates possible defect of ganglion cell/inner plexiform layer in the right eye.

 

Diagnosis

  • Secondary pigmentary glaucoma

Pathophysiology

  • There are two proposed etiologies for pigmentary glaucoma secondary to uveal melanoma. In this case, the presumed etiology involves pigment release from a discohesive or necrotic tumor, which then clogs the trabecular meshwork and obstructs aqueous outflow. Since this pigment originates from the tumor, it is not associated with iris transillumination defects. Alternately, it is possible for the tumor to distort the anterior segment anatomy and induce iris rubbing with release of iris pigment, leading to a more classic pigmentary glaucoma.

Treatments

  • Topical/oral medication therapy
    • IOP did not decrease with trial of timolol/simbrinza
    • Prostaglandin analogs increase uveoscleral outflow
      • Theoretical increased risk of metastasis
      • Inflammation in setting of possible melanomalytic component
    • Oral carbonic anhydrase inhibitor
      • Side-effects preclude long-term use
      • Appetite/taste, paresthesias, fatigue, electrolyte disturbances
  • Laser trabeculoplasty
    • May increase pigment dispersion
    • Ability to visualize trabecular meshwork may be limited by peripheral anterior synechiae
    • May incite further inflammation/scarring
  • Trabeculectomy
    • Concern for altered healing/failure/scarring in the setting of previously irradiated tissue
    • Risk of local metastasis
      • Probably does not increase risk of remote metastasis
  • Glaucoma drainage device
    • Ahmed glaucoma valve vs. Baerveldt shunt
      • Surgeon’s preference
      • To preserve vision in this patient, Ahmed valve is preferable due to ability to lower IOP immediately
    • Risk of local metastasis
      • Probably does not increase risk of remote metastasis
  • Enucleation
    • Offered to this patient; however, patient declined due to strong preference to preserve binocular vision.
  • Note, in this case, cyclodestruction was not considered as a viable treatment option due to concern for disruption of blood-ocular barrier.

Prognosis and Future Directions

  • The prognosis of glaucoma secondary to uveal melanoma is variable, depending on the size and location of the tumor, the oncologic treatment and the mechanism of secondary glaucoma.
  • When evaluating treatment options, it is important to consider patient preferences and risk tolerance, as well as the visual potential of the eye. In this case, after failure of medical therapy the patient declined enucleation and chose to proceed with incisional (Ahmed glaucoma valve) surgery despite the potential risk for local recurrence.
  • There is currently no consensus regarding the appropriate management of glaucoma secondary to uveal melanoma. Although there are multiple case reports, there is a paucity of evidentiary studies. Future prospective studies may provide useful evidence to guide treatment selection.

Mechanisms for glaucoma in uveal melanoma patients:

  • Neovascular Glaucoma
    • Retinal ischemia (chronic RD or radiotherapy)
    • Anterior segment ischemia
    • 2 to 23 percent of patients undergoing brachytherapy
  • Anterior rotation of the ciliary body
  • Pigment clogging of the trabecular meshwork
    • Both iris melanomas and iris nevi
  • Posterior synechiae and iris bombe
  • Direct infiltration of the angle
    • Iris melanomas (tapioca, ring melanomas)
  • Tumor necrosis syndrome
  • Unrelated primary open angle glaucoma or narrow angle glaucoma

Glaucoma surgery in uveal melanoma patients:

  • Uveal melanoma is a common setting for glaucoma
  • Glaucoma surgery safest >2 years after brachytherapy
  • Good visual potential (20/100 or better)
  • Avoid trabeculectomy
  • Opposite quadrant
  • Overall risk of orbital spread is low but patients should be aware of the need for careful monitoring with orbital MRI

References

  • European Glaucoma Society Terminology and Guidelines for Glaucoma, 4th Edition – Chapter 2: Classification and terminology. Br J of Ophthalmol. 2017;101:73-127.
  • Grossniklaus HE, Brown RH, Stulting RD, Blasberg RD. Iris melanoma seeding through a trabeculectomy site. Arch Ophthalmol. 1990 Sep;108(9):1287-90.
  • Kaliki S, Eagle RC, Grossniklaus HE, Campbell RJ, Shields CL, Shields JA. Inadvertent implantation of aqueous tube shunts in glaucomatous eyes with unrecognized intraocular neoplasms: report of 5 cases. JAMA Ophthalmol. 2013 Jul;131(7):925-8.
  • Kiratli H, Koç İ, Tarlan B. Orbital extension of an unsuspected choroidal melanoma presumably through an aqueous tube shunt. Ocul Oncol Pathol. 2016 Apr;2(3):144-7.
  • Lin Y, Panarelli JF. Uveal melanoma masquerading as pigment dispersion syndrome. Glaucoma Today. 2015;13(1):39-41.
  • Radcliffe NM, Finger PT. Eye cancer related glaucoma: current concepts. Surv Ophthalmol. 2009 Jan-Feb 28;54(1):47-73.
  • Riechardt AI, Cordini D, Rehak M, Hager A, Seibel I, Böker A, Gundlach E, Heufelder J, Joussen AM. Trabeculectomy in patients with uveal melanoma after proton beam therapy. Graefe’s Archive for Clinical and Experimental Ophthalmology. 2016 Jul;254(7):1379-85.
  • Sharkawi E, Oleszczuk JD, Bergin C, Zografos L. Baerveldt shunts in the treatment of glaucoma secondary to anterior uveal melanoma and proton beam radiotherapy. Br J Ophthalmol. 2012;96:1104-1107.
  • Shields CL, Shields JA, Shields MB, Augsburger JJ. Prevalence and mechanisms of secondary intraocular pressure elevation in eyes with intraocular tumors. Ophthalmology. 1987 Jul;94(7):839-46.
  • Sweeney AR, Keene CD, Klesert TR, Jian-Amadi A, Chen PP. Orbital extension of anterior uveal melanoma after Baerveldt tube shunt implantation. Canadian Journal of Ophthalmology. 2014 Dec 1;49(6):e133-5.
  • Tan AN, Hoevenaars JG, Webers CA, D’amato B, Beckers HJ. Baerveldt implant for secondary glaucoma due to iris melanoma. Clinical ophthalmology (Auckland, NZ). 2010;4:407-409.
  • Tay E, Cree IA, Hungerford J, Franks W. Recurrence of treated ciliary body melanoma following trabeculectomy. Clin Exp Ophthalmol. 2009 Jul;37(5):503-5.

Contact

Section Editors

 

Produced by: Monica Chavez, John Daniel and Mellissa Linton
Scroll Up To Top
View Full Desktop Version