Surgical Approach for Monoclonal Gammopathy of Undetermined Significance-Related Ocular Copper Deposition.

PURPOSE: The aim of this study was to present the surgical management of a patient with ocular copper deposition associated with monoclonal gammopathy of undetermined significance (MGUS). METHODS: This is a case report of a 44-year-old man with MGUS who presented to us with bilateral diffuse deposition of copper in the cornea and lens. RESULTS: Despite initiating systemic therapy for MGUS, no corneal clearing was observed. A decision was made to proceed with cataract extraction in the left eye given worsening vision. Despite trypan blue staining and a central descemetorhexis, visualization remained too poor to complete phacoemulsification. Pars plana lensectomy and vitrectomy to remove the residual lens material and placement of a posterior chamber intraocular lens in the sulcus with endoillumination was subsequently performed. As vision in the left eye steadily improved postoperatively, cataract surgery was then performed in the right eye. With use of trypan blue, creation of a 6-mm central descemetorhexis, and a retinal light pipe for endoillumination anteriorly to augment visualization, capsulorhexis, phacoemulsification, and insertion of intraocular lens in the bag were completed without difficulty. The patient's vision improved at subsequent follow-ups, reaching a final best-corrected visual acuity of 20/20-1 in the right eye and 20/25-1 in the left eye. CONCLUSIONS: Ocular copper deposition is a rare manifestation of MGUS. Cataract extraction is challenging, often requiring advanced techniques. Endoillumination is useful to improve visualization through the dense corneal copper deposition.

Tamoxifen retinopathy: A comprehensive review.

Tamoxifen is a selective estrogen receptor modulator used mainly for the treatment of breast cancer. Based on the case reports and studies performed to date on the retinal toxicity of tamoxifen, retinopathy appears to occur in as many as 12% of patients taking 20 mg tamoxifen a day for over 2 years. Of this 12%, as many as half develop symptomatic changes in visual acuity. Retinal changes consist primarily of crystalline deposits, cystoid macular edema, hyperreflective deposits in the inner retinal layers, and telangiectasia. Tamoxifen retinopathy is currently managed by discontinuing tamoxifen therapy as the cancer prognosis permits; however, discontinuing therapy demonstrates little to no improvement in visual acuity once visual changes have taken place. Intravitreal injections of steroids or antivascular endothelial growth factor therapy have been performed, but require further studying before conclusions can be made. Until then, optical coherence tomography screening for retinal changes should be performed every 6 months for patients who have been on tamoxifen therapy for 2 years or more. This way, patients can become aware of retinal changes, and their physicians can consider adjusting tamoxifen therapy before they risk developing changes in visual acuity.

Nyctalopia and Xerophthalmia in a Patient With Crohn's Induced Vitamin A Deficiency.

The vitamin A derivative, retinal, plays a pivotal role in scotopic and color vision. Although vitamin A deficiency (VAD) presents as a common cause of preventable blindness in areas with poor access to foods rich in vitamin A, it is uncommon in developed countries. We present a 56-year-old male with a history of Crohn's disease and pancreatitis who was referred to our ophthalmology office by optometry for severe dry eyes. He complained of a two-year history of constant blurred vision and nyctalopia. He stated that "images just appear dark." Examination demonstrated mildly decreased visual acuity with severe ocular surface disease and characteristic Bitot's spots in both eyes. Based on the patient's history and physical, a diagnosis of xerophthalmia in the setting of VAD was made. The patient was referred to his internist, he then underwent further evaluation and treatment with vitamin A intramuscular injections post-diagnosis.  This case illustrates the potential for VAD secondary to malabsorption from Crohn's disease and the importance of taking a full patient history so systemic causes of ophthalmic symptoms may be promptly identified and treated. VAD is extremely rare in the United States, however, patients at risk for VAD may benefit from regular vitamin A level checks and ophthalmologic evaluation.

Aggressive conjunctival Kaposi sarcoma as the initial manifestation of acquired immunodeficiency syndrome.

PURPOSE: To report a case of Kaposi sarcoma in a patient with previously undiagnosed Human Immunodeficiency Virus (HIV) infection. OBSERVATIONS: A 23-year-old male patient presented to the eye clinic with complaints of redness of his left eye for the past month. The patient had been seen one day prior to presentation in the emergency department for a neck mass and pneumonia. Exam was notable for a left hemorrhagic, nodular, bulbar conjunctival mass, a right hemorrhagic conjunctival lesion, and violaceous facial skin lesions. Due to suspicion for conjunctival Kaposi sarcoma, HIV and Acquired Immune Deficiency Syndrome (AIDS) serologies were obtained which returned positive. Biopsies of the ocular mass and axillary lymph nodes confirmed Kaposi sarcoma. The patient was started on anti-retroviral therapy and Doxorubicin. The left conjunctival mass initially progressed to cover most of his cornea but eventually regressed by 6 months. CONCLUSIONS AND IMPORTANCE: Ocular involvement of Kaposi sarcoma as the initial manifestation of HIV/AIDS is rare with only a few reported cases. Since the advent of highly active antiretroviral therapy, conjunctival or adnexal Kaposi sarcoma is not commonly encountered by healthcare providers. Concern for Kaposi sarcoma of the conjunctiva in this patient led to the evaluation for HIV/AIDS. It is vital for all healthcare providers to have Kaposi sarcoma in the differential diagnosis of a hemorrhagic conjunctival or adnexal mass and be familiar with its association with HIV/AIDS. If suspected, appropriate counseling and testing should be performed.