Retinal Cases and Brief Reports最新文献

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.

Purpose: The purpose of this study was to describe two cases of Cutibacterium acnes endophthalmitis that reinforce the importance of performing both bacterial culture and 16S polymerase chain reaction when the causative pathogen is unclear or difficult to culture, such as C. acnes . A case of C. acnes endophthalmitis complicated by subbuckle scleral perforation is illustrated with intraoperative photography.

Methods: This is a two-case series.

Results: Case 1 describes a case of C. acnes endophthalmitis in a longstanding pseudophakic patient after multiple vitrectomies for recurrent retinal detachment, complicated by subbuckle scleral perforation. Bacterial culture revealed C. acnes while 16S PCR was negative. Conversely, Case 2 demonstrates a case of chronic endophthalmitis diagnosed one year after cataract surgery. PCR (with repeat tap for confirmation) revealed C. acnes with a negative culture.

Conclusion: When the causative pathogen of endophthalmitis is unclear, dual testing of microbial culture and C. acnes 16S PCR improves the diagnostic yield of investigations for fastidious pathogens. C. acnes can present as an indolent or virulent endophthalmitis.

Purpose: Postoperative endophthalmitis is a relatively uncommon, but potentially visually devastating, complication associated with cataract surgery. Specific microbial causes of endophthalmitis are characteristically associated with particular disease time courses. Although Cutibacterium acnes is typically associated with an indolent course of inflammation, we report a case of C. acnes endophthalmitis with onset on postoperative day (POD) 1 and a positive culture from POD 2.

Methods: This is a case report.

Results: A 56-year-old man underwent cataract extraction and posterior chamber intraocular lens placement in his left eye. On POD 1, he presented with severe discomfort, reduced visual acuity, and significant inflammation. On POD 2, his anterior chamber was tapped and injected with broad-spectrum antibiotics and steroids. The inflammation ultimately resolved, and his visual acuity improved to 20/20.

Conclusion: C. acnes is a rare cause of hyperacute-onset postoperative endophthalmitis. Maintaining a high clinical suspicion and initiating prompt treatment can help to optimize long-term visual outcomes.

Background/purpose: To report a case of fovea plana with fundus hypopigmentation in a patient with Prader-Willi syndrome (PWS).

Methods: Case report.

Results: During a routine examination, fovea plana and fundus hypopigmentation were observed in both eyes in a 34-year-old male patient with PWS and documented with fundus photography, spectral domain optical coherence tomography and optical coherence tomography-angiography.

Conclusion: Fovea plana and fundus hypopigmentation may be associated with PWS. Indeed, both PWS and oculocutaneous albinism may be explained by the deletion of the same genomic region on chromosome 15. The present case of a patient with PWS with fundus hypopigmentation supports the genetic and clinical overlap between PWS and oculocutaneous albinism.

Background/purpose: Bilateral diffuse uveal melanocytic proliferation is a paraneoplastic syndrome affecting the eye that is a sign of poor prognosis of underlying malignancy. This is the first documented case to show serial and sustained improvement of bilateral diffuse uveal melanocytic proliferation after immunotherapy in the setting of primary non-small-cell carcinoma of the lung.

Methods: Single-center, case report.

Results: A 65-year-old man reported a gradual decrease in vision and floaters in the right eye after cataract surgery. Fundus examination demonstrated diffuse multiple brown subretinal lesions bilaterally. Next-generation sequencing of melanocytic tissue of the patient described in this case revealed a specific RB1 c.411A>T (p.Glu137Asp) variant with an allele frequency of 44.8%, consistent with heterozygosity. Plasma samples from the patient and a control patient with no history of cancer and/or paraneoplastic syndrome were cultured with neonatal melanocytes, which revealed a >180% increase in proliferation of normal neonatal melanocytes compared with the control. Pembrolizumab therapy was initiated, which resulted in shrinkage and stabilization of the lesions documented in serial diagnostic testing.

Conclusion: In conclusion, we report a cytologically and serologically confirmed case of bilateral diffuse uveal melanocytic proliferation in a patient with a primary non-small-cell carcinoma of the lung. Next-generation sequencing of melanocytic tissue of the patient described in this case revealed a specific RB1 c.411A>T (p.Glu137Asp) variant with an allele frequency of 44.8%, consistent with heterozygosity. Furthermore, we show documented serial improvement in the patient's ocular and systemic disease with treatment. This case as one of the longest surviving confirmed cases of a patient with bilateral diffuse uveal melanocytic proliferation.

Purpose: The purpose of this study was to describe the management of a hemorrhagic bacillary layer detachment (BALAD) in a patient with neovascular age-related macular degeneration.

Methods: The clinical records and imaging were reviewed.

Results: A 74-year-old woman presented with acute-onset hemorrhagic neovascular age-related macular degeneration with a large hemorrhagic BALAD. The intra-BALAD hemorrhage was amenable to displacement with SF 6 pneumatic displacement with subsequent VA recovery.

Conclusion: Sulfur hexafluoride (SF 6 ) pneumatic displacement in combination with aflibercept injection is a viable means by which to manage a hemorrhagic BALAD in the context of neovascular age-related macular degeneration. Displacement of large intra-BALAD hemorrhages can result in good visual recovery.

Purpose: The purpose of this study was to highlight a potential alternative to additional surgery for management of retinal redetachment through the use of additional facedown positioning with silicone oil tamponade.

Methods: Retrospective case series of two patients evaluated with examination, multimodal imaging, including fundus photography, optical coherence tomography, and fluorescein angiography.

Results: In Case 1, a 70-year-old female patient underwent surgery for a full-thickness macular hole with associated macula-off retinal detachment, but experienced a recurrent detachment and underwent a second surgery with silicone oil placement. Another recurrent detachment was found. The case was managed conservatively with facedown positioning, resulting in resolution of subretinal fluid and improvement in vision. At follow-up, the retina remained attached with stable vision. In Case 2, a 25-year-old male patient underwent a surgical repair for proliferative vitreoretinopathy retinal detachment with a scleral buckle, cryotherapy, and external drainage. After multiple redetachment surgeries with retinectomy and oil placement, another tractional redetachment of the fovea was noted. Management was with facedown positioning, and follow-up evaluation showed resolution of the subretinal fluid and improvement in vision with stability for greater than 2 months.

Conclusion: For recurrent retinal redetachments with silicone oil in place, an additional week of facedown positioning can result in anatomical success and be a viable alternative or bridge to invasive surgical interventions. This approach may have greatest utility for patients who are poor surgical candidates without new peripheral pathology.