Retinal Cases and Brief Reports最新文献

Purpose: Surgical outcome of presumed Autosomal Recessive Bestrophinopathy (ARB)with full thickness macular hole and retinal detachment managed by pars plana vitrectomy.

Methods: A retrospective observational case series of two eyes of two patients who underwent 25 G pars plana vitrectomy with peeling of internal limiting membrane and SF6 tamponade and were assessed with respect to best corrected visual acuity, fundus photographs and spectral domain optical coherence tomography.

Results: Type 1 closure of macular hole was achieved along with resolution of retinal detachment in both patients.

Conclusion: 25 G pars plana vitrectomy with peeling of internal limiting membrane and SF6 tamponade can result in favorable outcome in macular hole associated retinal detachment in patients with presumed Autosomal recessive Bestrophinopathy.

Purpose: To report a case of branch retinal vein occlusion (BRVO) causing retinal neovascularization (NV) and vitreous hemorrhage associated with astrocytic hamartoma (AH) involving the optic nerve head in a patient with tuberous sclerosis complex (TSC). We review the natural history of the clinical presentation and posit its etiology.

Methods: Electronic health records were reviewed for patient history and demographics and multimodal ophthalmic imaging including fundus photography, fluorescein angiography, and optical coherence tomography (OCT).

Results: Neovascularization emanating superiorly and inferiorly from a large AH of the optic nerve head was identified as the cause of a longstanding vitreous hemorrhage. Retinal nonperfusion was also found in superior peripheral quadrants, most notably distal to the superior NV. OCT was useful in visualizing small retinal AH in the fellow eye that was otherwise undetected on prior dilated fundus examinations. The patient was treated with intravitreal anti-VEGF and superior sectoral PRP in the involved eye.

Conclusion: BRVO and associated NV may represent rare vision-threatening sequela of AH in TSC and manifested in this case with diffuse peripheral retinal nonperfusion secondary to BRVO-induced ischemia. OCT is a valuable resource in identifying and monitoring AH of the retina.

Purpose: To describe a unique case of drusen resolution accompanying the spontaneous closure of an eccentric full-thickness macular hole (FTMH).

Methods: This study is a case report.

Results: A 71-year-old male exhibited confluent drusen in the right eye beneath the fovea. During regular follow-up, an eccentric FTMH developed in the right eye and closed spontaneously. Concurrently, the confluent drusen showed rapid regression without signs of geographic atrophy or macular neovascularization. Visual acuity remained stable throughout the monitoring period.

Conclusion: This case suggests that drusen regression can occur alongside spontaneous FTMH closure without surgical intervention. This adds to the current understanding of the pathology and treatment for drusen.

Purpose: The purpose of this article is to describe the novel surgical technique utilizing laser photocoagulation for the management of Candida chorioretinal lesions that are refractory to medical therapy.

Methods: This report presents the use of laser photocoagulation applied to a Candida chorioretinal lesion that was refractory to extensive systemic and intravitreal antifungal medications. The presenting lesion was in an immunosuppressed patient. Argon laser photocoagulation was applied onto and surrounding the Candida chorioretinal lesion.

Results: Following laser photocoagulation, the Candida chorioretinal lesion has remained resolved for over one year with no further reactivation, extensions, or satellites.

Conclusion: Candida chorioretinitis can be extremely therapeutically resistant. Current treatment involves systemic and intravitreal antifungals. There are currently limited options for chorioretinal lesions refractory to standard medical therapy. The authors report the novel application of laser photocoagulation as a potential treatment option in refractory cases of Candida chorioretinitis.

Purpose: The MER proto-oncogene tyrosine kinase (MERTK) helps maintain the homeostasis of the retinal pigmented epithelium (RPE). Additionally, MERTK regulates the innate immune system. As such inhibition of MERTK has gained recent interest as a target for cancer therapeutics. Herein, we present on MERTK inhibitor associated retinal toxicity in a human.

Methods: A 43-year-old male with past medical history of esophageal adenocarcinoma was enrolled in a trial studying the MERTK inhibitor PF-07265807. As part of the study protocol, the patient was seen every two to three weeks for dilated fundus exams and ancillary testing. Data on the patient's relevant past medical history, ophthalmic exam findings, and imaging performed at baseline and subsequent ophthalmology visits was obtained via retrospective chart review.

Results: At the patient's baseline visit, his dilated exam and testing were normal. Seven months after starting the MERTK inhibitor the patient developed subtle but reproducible signs of retinal toxicity with disruption of the extrafoveal ellipsoid zone on optical coherence tomography and extrafoveal hyper-autofluorescence on short wavelength fundus autofluorescence. The patient's vision remained stable throughout the study; however, the medication was stopped due to the unknown ocular effects as well as progression of the patient's cancer.

Discussion: Patients taking MERTK inhibitors should be monitored by an ophthalmologist while on the drug. If toxicity develops, discussion of whether to continue the medication should take place between the patient, ophthalmologist, and oncologist, with consideration of the risks of vision loss versus benefits of taking the medication from a cancer perspective.

Purpose: To describe remarkable choroidal thickness fluctuations corresponding to episodes of recurrent anterior uveitis with subretinal fluid development when exceeding a choroidal thickness threshold.

Methods: A patient with pachychoroid pigment epitheliopathy and unilateral acute anterior uveitis of the left eye was evaluated over a period of 3 years with multimodal retinal imaging including optical coherence tomography. Longitudinal changes in subfoveal choroidal thickness (CT) were measured and correlated with episodes of recurrent inflammation.

Results: Over the course of five recurrent episodes of inflammation in the left eye treated with oral antiviral and topical steroid therapy, subfoveal CT increased as much as 200 μm or more. By contrast, subfoveal CT in the fellow quiescent right eye was within normal limits and minimally changed throughout the follow-up. Increased CT occurred with each episode of anterior uveitis and decreased by 200 µ m or more during periods of quiescence in the affected left eye. Subretinal fluid and macular edema developed with a maximum CT of 486 μm and spontaneously resolved when CT decreased after treatment.

Conclusion: In eyes with pachychoroid disease, anterior segment inflammation may lead to marked increases in subfoveal CT and the development of subretinal fluid at a threshold thickness value.

Purpose: To describe the ocular pathology of a patient with fungal endophthalmitis with features mimicking sympathetic ophthalmia.

Methods: Review of medical records and histopathology of a single patient.

Results: A 72-year-old man who sustained penetrating injury to the left eye with an agave plant presented to our clinic 16 months after the initial injury. Before presentation, the patient had developed endophthalmitis and had undergone anterior chamber washout, vitrectomy, and intravitreal steroids, antibiotics, antifungals, and anti-vascular endothelial growth factor therapy. At presentation, the patient had a blind, painful eye and subsequently underwent enucleation. Histopathology demonstrated granulomatous inflammation with multinucleated giant cells in the iris and Dalen-Fuchs nodules with CD68-positive epithelioid histiocytes associated with the retinal pigment epithelium sparing the choriocapillaris. These findings were initially attributed to sympathetic ophthalmia. The fellow eye did not have any signs of inflammation, and Grocott methenamine silver stain was positive for filamentous fungal elements, leading to a diagnosis of fungal endophthalmitis.

Conclusions: Fungal endophthalmitis may develop histopathologic features that are similar to those observed in sympathetic ophthalmia. Recognition of the overlap between the histopathologic features of these diseases may reduce the possibility of misdiagnosis and unnecessary treatment of the fellow eye.

Purpose: The authors describe a case of a retinal capillary hemangioblastoma (RCH) in a pediatric patient with von Hippel-Lindau (VHL) syndrome that was successfully treated with systemic belzutifan.

Methods: The clinical course was documented with serial fundus examinations and multimodal imaging, including Optos widefield fundus photography and optical coherence tomography. A literature review was conducted to look for similar cases and/or discussion.

Results: A left RCH was noted on a standard VHL surveillance retinal examination of a then 15-year-old male patient with VHL syndrome. Over the course of 17 months, this RCH was treated with focal laser therapy, photodynamic therapy, cryotherapy, bevacizumab injection, and endolaser ablation. Complications of these treatments included subretinal fluid and vitreomacular traction necessitating laser retinopexy, scleral buckle, and pars plana vitrectomy with membrane stripping. After a 6-month interval from the last local therapy (endolaser treatment), there was minimal regression of the lesion, and many concerning features persisted. At 22 months from presentation, the patient started belzutifan 120 mg PO daily with subsequent regression in size and less perfusion to the hemangioblastoma within 4 months. The patient is tolerating the systemic belzutifan with only the expected normocytic anemia and has not required transfusion therapy after 12 months of treatment.

Conclusion: von Hippel-Lindau disease is a rare and serious condition associated with multiple types of benign and malignant tumors. Belzutifan is tolerated in the adolescent population and can provide a systemic treatment alternative for VHL-associated RCH.

Purpose: To report a rare case of a subretinal Phialophora richardsiae abscess in a patient with chronic granulomatous disease.

Methods: A 21-year-old man with chronic granulomatous disease and a history of invasive pulmonary aspergillosis presented with progressive loss of vision and pain in his left eye. He was found to have a subretinal abscess with a macula involving serous retinal detachment. A diagnostic and therapeutic pars plana vitrectomy, subretinal biopsy with debridement, inferior retinectomy, and silicone oil tamponade were performed. Intraoperative cultures grew Pleurostoma (Phialophora) richardsiae. He was treated with systemic liposomal amphotericin B and high-dose posaconazole. However, his eye eventually required enucleation.

Results: Preoperative visual acuity was light perception on the left eye and improved to count fingers at 4 weeks postoperative. However, VA rapidly declined to light perception, and he developed an opaque white cataract, iris neovascularization, posterior synechiae, and corectopia. The retina remained attached under silicone oil. Histopathology revealed granuloma formation and active fungal elements.

Discussion/conclusion: The case supports the importance of vitreoretinal surgery to determine a definitive systemic diagnosis. Treatment of Phialophora infection is surgically challenging to manage and has a poor visual prognosis in patients with chronic granulomatous disease.

Purpose: Pentosan polysulfate (PPS), a drug used for interstitial cystitis, has recently been detected to cause maculopathy in a dose-dependent manner. Outer retinal atrophy is the hallmark of this condition.

Methods: History, examination, and multimodal imaging were used to guide diagnosis and management.

Results: We report a case of PPS-related maculopathy in a 77-year-old lady, who presented with florid retinal atrophy at the posterior pole in both eyes, and a concurrent macular hole in the left eye. She had been diagnosed with interstitial cystitis several years before for which she was prescribed PPS (Elmiron). She had noticed a drop in vision 5 years after initiation of PPS and self-discontinued the drug after 24 years of use. A diagnosis of PPS-related maculopathy with a macular hole was made. She was counselled regarding the prognosis and was advised to avoid PPS. Surgery for macular hole was deferred in view of the severe retinal atrophy.

Conclusion: Pentosan polysulfate-related maculopathy can lead to severe retinal atrophy and a subsequent degenerative macular hole. A high index of suspicion is required for early detection and cessation of drug to prevent this irreversible vision loss.