Purpose The PreserFlo MicroShunt (PMS) is a glaucoma drainage device designed to reduce intraocular pressure (IOP) with fewer complications than conventional filtering surgery. An intraluminal stent suture can prevent early postoperative hypotony; however, a potential infection risk exists. We report a rare case of bleb-related infection associated with an intraluminal stent suture in the PMS. Observation An 80-year-old man with secondary glaucoma due to bilateral cytomegalovirus anterior uveitis underwent bilateral PMS implantation with a 10-0 nylon intraluminal stent suture. The stent was removed from the right eye 1 month postoperatively but retained in the left eye because the IOP remained stable. At 3.5 months postoperatively, the left eye developed eyelid swelling, ocular pain, conjunctival hyperemia, and anterior chamber inflammation without bleb leakage. Infection related to the stent suture was suspected. After stent removal, suture culture grew Streptococcus agalactiae. Intensive topical and intracameral antibiotic therapy resolved the inflammation within 2 weeks. Transient hypotony and choroidal detachment occurred after removal but resolved conservatively, and the IOP stabilized. Conclusion and Importance Intraluminal stent sutures may effectively prevent early postoperative hypotony after PMS implantation, but their indication should be carefully considered while balancing the risks of hypotony and infection. Early stent removal and careful postoperative monitoring are essential.
Purpose Phelan-McDermid Syndrome (PHMDS) is a rare neurogenetic, highly heterogenous, disorder with hallmark signs of neonatal hypotonia, dysmorphic facial features, and developmental delay. We report a case of unilateral ocular hypertension with glaucomatous concern in a child with PHMDS. Observations/Interventions A 7-year-old female with genetically confirmed chromosome 22q13.3 deletion was referred for unilateral ocular hypertension and signs of elevated episcleral venous pressure (EVP). Given a complex genetic profile and past medical history, minimal topical medication was tolerated leaving a persistently elevated intraocular pressure (IOP) of 20mmHg in the right eye compared to 12mmHg in the left eye with concerns for glaucomatous cupping. Magnetic resonance imaging with angiography of the brain and orbits was unrevealing for a cause of her suspected unilateral elevated EVP. Transscleral diode cyclophotocoagulation was performed and achieved successful IOP control with limited topical therapy. Conclusions/Importance This case highlights the challenges of evaluating and managing ocular hypertension with glaucomatous concern in a medically complex child, as early recognition and individualized intervention can effectively manage glaucoma in the context of complex systemic comorbidities.
Purpose Ophthalmic malignancies are well described in DICER1 syndrome and include intraocular malignancies and, rarely, primary orbital tumors (or medulloepitheliomas). Here, we present the case of an aggressive primary orbital malignancy (embryonal tumor with multilayered rosettes [ETMR]) in a child with germline and somatic DICER1 abnormalities. Observations In a 1-month-old boy born at term, a computed tomography scan revealed an orbital mass initially thought to be consistent with a capillary hemangioma. After initial treatment with a 4-week course of oral propranolol (3 mg/kg/day) without response, a magnetic resonance imaging scan revealed an enlarging lobulated large enhancing left intraconal mass. A series of biopsies and DNA methylation array analyses showed the lesion to be an ETMR, atypical subclass. Germline analysis revealed a mutation in the DICER1 gene. The tumor itself had a second, different mutation in the DICER1 gene. ETMR has been rarely identified in the orbit and there are no accepted treatments, but overall survival of ETMR lesions is poor (<20%) with combinations of surgery, radiation, or brachytherapy. The family was offered exenteration but refused, so intraarterial chemotherapy was offered with the understanding that it had never been tried in DICER1-mutated orbital ETMR. After treatment in 4 monthly 2-hour outpatient sessions with intraarterial chemotherapy, we observed prompt regression of clinical findings. Serial imaging following the final cycle of treatment demonstrated no evidence of recurrent or progressive disease. Twenty-three months later, there has been no regrowth. Conclusion and importance A somatic and germline (“two hit”) DICER1-mutated orbital tumor was successfully managed without surgery, radiation, or systemic chemotherapy using the well-established intraarterial approach presently used for retinoblastoma. In the case of our patient, intraarterial chemotherapy proved to be an effective treatment for this tumor.
Background Reverse pupillary block (RPB) is an increasingly recognized complication following secondary scleral-fixated intraocular lens (IOL) implantation, particularly in vitrectomized eyes with high axial myopia. Although its clinical manifestations are well described, the underlying hydrodynamic mechanism remains poorly understood. We sought to visualize aqueous humor flow in vivo and determine whether altered intraocular fluid dynamics could explain the development of RPB. Setting Private practice with a single surgeon Design Retrospective chart review and observational study Methods Patients who underwent IOL exchange with intrascleral haptic fixation (ISHF) by Yamane technique by a single surgeon underwent slit lamp video microscopy postoperatively using a Haag-Streit BQ900 slit lamp with a IM900 imaging system. Results Six eyes of six patients (mean axial length 27.26 mm) demonstrated reproducible movement of suspended particles from the anterior chamber into the posterior segment through the iridolenticular channel. All six eyes underwent concurrent pars plana vitrectomy, although two had previously been vitrectomized. All eyes had one or more prophylactic peripheral iridotomies and none demonstrated active reverse pupillary block or elevated intraocular pressure at the time of observation. However, the observed flow pattern provides a physiological explanation for the reverse pupillary block that had occurred previously or was known to occur intermittently in these anatomically susceptible eyes. Conclusion In vitrectomized eyes with scleral-fixated IOLs, aqueous humor may preferentially flow from the anterior chamber toward the posterior segment. We propose that this reversed pressure gradient is driven by enhanced posterior aqueous outflow through the retinal pigment epithelium pump in eyes lacking the normal lens-zonule-vitreous barrier. This altered fluid dynamics provides a mechanistic explanation for reverse pupillary block and its relief following peripheral iridotomy.
Purpose We demonstrate the potential role of acalabrutinib, a second-generation selective Bruton tyrosine kinase (BTK) inhibitor, as monotherapy in treating optic nerve infiltration by chronic lymphocytic leukemia (CLL). Observations A 68-year-old male, initially diagnosed with CLL 13 years earlier, presented with a decrease in vision and intermittent floaters for 5 days. On fundus examination, there was optic disc swelling in both eyes (OU) concerning for CLL infiltration and vitreous haze in the left eye (OS). The MRI of the brain and orbit was normal, and his infectious and uveitis workups were unremarkable. He was prescribed acalabrutinib 100 mg twice daily by mouth. Five days later, the optic disc edema OU and vitreous haze OS improved and completely subsided two months later. Conclusions and importance Early initiation of acalabrutinib may be sufficient therapy for CLL optic nerve infiltration.
Purpose To report severe panuveitis with extensive retinal arteritis caused by prolonged retained lens fragments following cataract surgery, in the setting of delayed referral during the 2024 Korean medical crisis. Observations A 61-year-old woman presented with severe ocular pain and injection in the right eye two months after phacoemulsification cataract surgery performed at an outside hospital. The patient was unaware of any intraoperative complications, and no formal referral had been made. Vision was hand motion with elevated intraocular pressure (34 mmHg), hypopyon, and dense vitreous opacity suggesting panuveitis or endophthalmitis. Emergency 25-gauge pars plana vitrectomy was performed, during which retained lens particles were identified at the inferonasal periphery and removed using Ocutome® lensectomy. Intraoperative fundus examination revealed retinal arteries accompanied by granular, non-continuous peri-arterial precipitates. Correspondingly, fluorescein angiography demonstrated arterial beading, luminal stenosis, and peripheral non-perfusion, while optical coherence tomography confirmed hyperreflective thickening of the arterial wall consistent with arteritis. Following surgical removal of the antigenic lens material and oral corticosteroid therapy, ocular inflammation subsided, and the best-corrected visual acuity improved to 0.5 at 6-month. Conclusions and importance Prolonged lens retention can cause panuveitis with chronic phacoantigenic inflammation extending to retinal vessels, resulting in extensive arteritis. Early vitreoretinal referral after retained lens fragments is critical, particularly during healthcare crises requiring prioritized communication to prevent vision-threatening sequelae.
Purpose To describe a minimally invasive technique for managing intraluminal obstruction of an Ahmed glaucoma valve (AGV) tube using direct aspiration via the anterior chamber. Observations A 75-year-old man with AGV implantation for bilateral secondary glaucoma associated with chronic uveitis developed postoperative intraocular pressure (IOP) elevation in his left eye. Although incarceration at the tube tip was released with neodymium-doped yttrium aluminum garnet (Nd:YAG) laser treatment, IOP remained elevated. Anterior segment optical coherence tomography (AS-OCT) revealed tissue-like material within the tube lumen, confirming the diagnosis of intraluminal obstruction. A 30-gauge needle connected to a syringe was inserted into the tube lumen via the anterior chamber, and negative pressure was applied to aspirate and remove the obstructive tissue. IOP normalized promptly after the procedure, and postoperative AS-OCT confirmed disappearance of the occluding material with no recurrence during 1 year of follow-up. Conclusions and Importance Intraluminal aspiration via the anterior chamber may represent a minimally invasive therapeutic option for AGV tube obstruction that avoids conjunctival incision while preserving bleb function. AS-OCT may play a key role in identifying intraluminal obstruction and determining the indication for surgical intervention.
Purpose To describe a case of bilateral cystoid macular edema (CME) that developed following intracameral travoprost implantation, which then resolved after explantation, and to review the association of CME with prostaglandin analogues (PGA). Observations The patient developed CME after intracameral travoprost implantation in each eye. An extensive work up including blood work and aqueous biopsy did not reveal other inflammatory etiologies. Explantation of the travoprost led to resolution of the CME in each eye, suggestive of a temporal association of the implant and the CME. Conclusions & Importance The association of CME with intracameral PGA might differ from previously reported associations of CME with topical PGA, because intracameral medications provide a sustained dose closer to the retina. Surgeons should consider PGA as a possible etiology in cases of post-procedural CME.
Purpose:To report our experience with two cases of acrylate allergy confirmed by patch testing, wherein acrylic intraocular lens (IOL) implantation was performed and followed up for 1 year. Observations:A 78-year-old woman with an allergy to acrylic resin consulted a general allergist because of suspected depigmented contact dermatitis. A patch test was positive for methyl methacrylate and 2-hydroxyethyl methacrylate (2-HEMA), which are acrylic resins. Before cataract surgery, patch tests for the IOL and other acrylic resin materials were performed, all of which tested negative. Case 2: A 49-year-old woman was allergic to several substances, including acrylic resin. A patch test was positive for 2-HEMA and ethylene glycol dimethacrylate. We consulted an anesthesiologist regarding immediate-type allergy and explained to the patient about the possibility of IOL removal for delayed-type allergy. Intra-or postoperative complications were not observed in either patient. Postoperative inflammation was similar to that observed after conventional cataract surgeries. During the 1-year follow-up period, worsening of inflammation in the anterior chamber or other symptoms indicative of delayed hypersensitivity were not observed. Conclusions and importance:In two patients with cutaneous acrylate allergy, hydrophobic acrylic IOL implantation was uneventful through the 1-year follow-up period. Careful preoperative patch testing, allergist collaboration, informed consent about uncertain long-term risk, and close follow-up are advised.
Purpose:To illustrate the clinical consequences of prolonged loss to follow-up for annual screening in long-term hydroxychloroquine users through representative cases of hydroxychloroquine retinopathy. Observations:Three Korean women with systemic lupus erythematosus who had received hydroxychloroquine 200 mg daily for 12-20 years were evaluated after being lost to recommended annual retinal screening for 4-6 years. All patients showed pericentral retinopathy but were asymptomatic. Fundus autofluorescence demonstrated pericentral abnormalities ranging from localized temporal hyperautofluorescence to extensive pericentral hypoautofluorescence. Despite prompt discontinuation of hydroxychloroquine, follow-up imaging showed progression with expansion of abnormal pericentral autofluorescence in all cases. Conclusions and importance:These cases highlight that interruption of annual screening in long-term hydroxychloroquine users may allow silent progression to advanced retinopathy, which can continue despite cessation of therapy. The findings underscore the critical importance of sustained adherence to screening recommendations, particularly in patients with risk factors.
Purpose To describe a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) presenting with granulomatous panuveitis and to highlight multimodal ocular imaging findings useful for diagnosis. Observations A 20-year-old Japanese man presented with acute vision loss in the left eye. Best-corrected visual acuity was 20/20 in the right eye and 20/250 in the left eye. Slit-lamp examination revealed bilateral mutton-fat keratic precipitates with anterior chamber cells and anterior vitreous cells. Fundus examination showed multiple confluent yellowish-white placoid lesions bilaterally. Angiographic and OCT findings were characteristic of APMPPE. These included the angiographic reversal phenomenon on fluorescein angiography, persistent hypofluorescence on indocyanine green angiography, and the angular sign of Henle fiber layer hyperreflectivity on optical coherence tomography. Systemic evaluation, including syphilis serology, an interferon-gamma release assay for tuberculosis, serum angiotensin-converting enzyme and soluble interleukin-2 receptor levels, viral serology, and chest radiography, did not identify an associated infectious or inflammatory disease. Bilateral sub-Tenon triamcinolone acetonide injections, oral prednisolone, and topical corticosteroids led to rapid resolution of inflammation and recovery of visual acuity. The patient remained free of recurrence for 13 months after presentation, including more than seven months after cessation of therapy. Conclusions and importance APMPPE should remain in the differential diagnosis of granulomatous panuveitis when placoid fundus lesions are present. Careful ocular imaging and systemic evaluation are useful for distinguishing APMPPE from VKH disease, sarcoidosis, and infectious uveitis.
Purpose To describe a case of acute-onset bilateral angle-closure glaucoma secondary to ciliochoroidal effusions in a 43-year-old otherwise healthy female. An autoimmune and inflammatory work-up was negative, and the patient was not on any medications or supplements. She had been using a red-light therapy (RLT) mask over the past two weeks, including the morning of her presentation. Observations The underlying mechanism of the patient's presentation was most consistent with secondary angle-closure due to ciliochoroidal effusions and anterior rotation of the ciliary body. The only recent lifestyle change was the new use of RLT mask therapy for over two weeks. With prompt diagnosis, close outpatient monitoring, cessation of the RLT mask use, and careful titration of IOP-lowering, cycloplegic, and steroid medications, the patient returned to her baseline vision of 20/20 OU and had bilateral choroidal effusions resolve. Conclusion and importance Choroidal effusions have not been previously described in association with at-home RLT mask use; however, existing evidence suggests that RLT exposure may increase choroidal vascularity and thickness. Following cessation of RLT and appropriate medical management, the patient had complete resolution of the ciliochoroidal effusions and full recovery of vision. Further investigation is warranted to better characterize the ocular safety profile of RLT devices.
Purpose:To report the migration of perivascular opacities, in patients with retinal vasculopathy, with and without clinical evidence of ocular inflammation. Methods:Retrospective case series of four patients with either idiopathic retinal vasculitis or presumed retinal vascular inflammation. Patients were imaged with wide-field fundus photographs, infrared fundus images, fundus fluorescein angiography (FA), and flood-illumination adaptive optics (FIAO). Successive fundus images were registered and compared over follow-up periods ranging from 2 to 10 months. Displacement of identifiable perivascular opacities between imaging sessions was measured to estimate migration velocity. Results:Two patients presented with presumed retinal vasculopathy associated with retinal vein occlusion (RVO). Their fundus images showed disseminated perivenous opacities along veins. In both patients, time-lapse videos recapitulating several months of fundus observations revealed that most of the perivascular opacities shifted along veins with or against the flow at an estimated mean velocity of approximately 15 μm/day, calculated from displacement between imaging sessions. Two patients presented with retinal vasculopathy in the form of retinal vasculitis. Their fundus examination showed perivascular sheathing (arteritis and phlebitis). FIAO demonstrated multiple focal perivascular opacities in the same areas. All 4 patients imaged presented similar focal perivascular cell-like opacities either on infrared or FIAO fundus images. Conclusions:We describe the migration of presumed immune cell-like perivascular opacities in the human eye. The significance of this process remains to be fully explored, because evidence of ocular inflammation was clinically present in two patients. The close temporal and spatial relationship of these perivascular opacities associated with branch RVO in two of these patients suggests that they may have been involved in the occurrence of RVO.
Purpose To report a case of bilateral Roth spots in an adolescent, with a recent metapneumovirus viral infection. Observations A 17-year-old male consulted with painless floaters and blurred vision. Fundus examination showed bilateral Roth spots and segmental venous sheathing. An extensive systemic workup was unremarkable, including negative QuantiFERON-TB Gold testing. The patient had a recent history of viral infection, and a metapneumovirus was detected on PCR of a nasal swab. The retinal lesions resolved spontaneously by day 7, visual acuity was normal and Goldmann visual field testing showed no residual defect. At the one-year follow-up, complete resolution was sustained on both color fundus photography and fundus autofluorescence, with no residual changes of the retinal pigment epithelium. Conclusion and importance This case highlights a rare association between a viral respiratory infection and Roth spots. The lesions are presumed to be due to a post-viral immune-mediated process affecting the retinal microvasculature. The rapid spontaneous resolution and the absence of long-term sequelae suggest that similar cases may go unnoticed.
Purpose To describe a rare case of capillary hemangioma of the caruncle and to present a summary of previously published cases. Observations A 59-year-old woman presented with a reddish, indurated mass of the left caruncle following minor ocular trauma. Slit-lamp examination revealed a well-defined vascular lesion measuring 6 × 6 mm without deep extension. Orbital imaging confirmed a circumscribed, superficial lesion. Histopathologic evaluation of the excised lesion demonstrated lobular proliferation of thin-walled capillary vessels within fibrous stroma, consistent with capillary hemangioma. The lesion showed partial spontaneous regression and no recurrence following complete excision.A comprehensive literature search of PubMed, Scopus, Embase, Web of Science, and Google Scholar (from inception to October 10, 2025) identified only a few histologically confirmed capillary hemangiomas of the caruncle. Most vascular lesions reported at this site were classified as pyogenic granuloma (lobular capillary hemangioma), emphasizing the exceptional rarity of true capillary hemangioma. Conclusions and Importance Capillary hemangioma of the caruncle is exceedingly uncommon and may clinically mimic other vascular or inflammatory lesions. This case, together with the literature review, broadens the recognized clinicopathologic spectrum of caruncular vascular tumors and suggests that antecedent trauma may contribute to lesion development. Excisional biopsy remains the diagnostic and therapeutic gold standard.
Introduction Steroid-induced hypertensive response is a common, yet underdiagnosed complication of steroid therapy. Certain patients, referred to as high responders, can experience a rapid and severe rise in intraocular pressure (IOP) potentially reaching sight-threatening levels. This report presents two high-responder patients with multiple risk factors. Methods Medical records of two patients who underwent 360° angle surgery and experienced postoperative IOP spikes were reviewed, and clinical data was extracted. Results Two male patients aged 8 and 22 years old with juvenile open angle glaucoma (JOAG) with elevated IOP despite maximum medical therapy underwent gonioscopy-assisted transluminal trabeculotomy with canaloplasty in both eyes and were started postoperatively on prednisolone. Patient 1 had an IOP of 61 mmHg on postoperative day (POD) 6, prednisolone was discontinued, and treatment was switched to ketorolac, antiglaucoma drops and oral agents. Patient 2 had an IOP of 48 mmHg on POD 9 and IOP was controlled upon discontinuing prednisolone and glaucoma drops. His second eye was treated with fluorometholone after surgery but presented with an IOP of 39 mmHg on POD 8 and also switched to ketorolac. Both patients had controlled IOPs after steroid cessation. Conclusion Steroid response can occur even in procedures targeting the trabecular meshwork. Identifying risk factors, including a glaucoma diagnosis, family history, young age, and use of potent steroids enables closer monitoring. Early detection and cessation of steroid therapy are crucial to preventing sustained IOP elevation, underscoring the importance of vigilance in high-risk patients.
Purpose: To report a case of aqueous misdirection occurring shortly after intraluminal ripcord removal in a pseudophakic, previously vitrectomized eye implanted with a PAUL Glaucoma Implant. Case description: A 65-year-old Caucasian woman presented with primary angle-closure glaucoma and previous history of aqueous misdirection following a combined procedure of phacoemulsification and XEN45 gel stent implant, which was managed with pars plana vitrectomy elsewhere. Because of high IOP despite maximum tolerated medical treatment, a PAUL Glaucoma Implant was performed with positioning of a 6-0 Prolene® intraluminal ripcord. Shortly after ripcord removal 6 weeks postoperatively, she presented with significant myopic shift, uniformly shallow anterior chamber, and markedly raised IOP. Anterior-segment OCT showed patent tube in the superotemporal quadrant of the anterior chamber, suggesting the recurrence of aqueous misdirection. Revision of pars plana vitrectomy with extensive peripheral vitreous shaving and irido-zonulo-hyaloidectomy was required to restore anterior chamber depth, to re-establish forward aqueous flow, and to stabilize IOP. Conclusions and importance: This case suggests that intraluminal ripcord removal from a PAUL glaucoma implant can elicit aqueous misdirection in eyes at risk. Furthermore, the final outcomes of the present case suggest that thorough pars plana vitrectomy with irido-zonulo-hyaloidectomy may be the most effective surgical approach to aqueous misdirection.
Purpose:To report the anatomical and visual outcomes of autologous Tenon's capsule patch grafting for closure of a large post-vitrectomy full-thickness macular hole. Observations:A 63-year-old female with proliferative diabetic retinopathy presented with decreased vision in the right eye. Optical coherence tomography demonstrated a 1112 μm full-thickness macular hole. Due to prior internal limiting membrane peeling, insufficient tissue remained for conventional techniques, an autologous Tenon's capsule graft was harvested through a limited conjunctival peritomy and positioned on the macular defect. Silicone oil tamponade was used to stabilize the graft. One-month follow-up demonstrated anatomical closure of the macular hole, which remained stable at 6 months. Visual acuity improved from hand motion preoperatively to 1/60 at final follow-up. Conclusions and importance:Autologous Tenon's capsule patch grafting may serve as a useful surgical alternative for large post-vitrectomy macular holes when internal limiting membrane tissue is unavailable.
Purpose:To report a case of paracentral acute middle maculopathy (PAMM) after physical exertion and to explore possible underlying microvascular mechanisms. Observations:A 42-year-old Caucasian man presented with a persistent, unilateral, painless paracentral scotoma that appeared after an intense ski session seven days earlier. His medical history included an 11-year course of migraine with aura and recurrent transient scotomas in either eye, lasting from minutes to days, often triggered by fatigue, exertion, or altitude. At presentation, best-corrected visual acuity was 1.6 Snellen equivalent bilaterally (20/12), with unremarkable slit lamp and fundoscopy examination. Optical coherence tomography (OCT) revealed a focal hyperreflective band in the inner nuclear layer of the right eye, consistent with PAMM. Fundus autofluorescence revealed a hyperautofluorescent spot in the same area. Microperimetry confirmed a localized scotoma in the corresponding area. At follow-up, clinical examination before and after moderate exertion (90 minutes of cycling) showed no new scotomas or clinical abnormalities. However, en face OCT angiography (OCTA) of the deep capillary plexus revealed multifocal variations in capillary density, with some areas demonstrating reduced perfusion after exercise. Conclusions and importance:PAMM is an OCT finding of focal retinal ischemia associated with various vascular disorders. Migraine has also been reported as a risk factor. In this case, physical exertion may have contributed to transient hemodynamic changes, potentially unmasking an underlying retinal microvascular vulnerability. The multifocal post-exercise decrease in perfusion on OCTA despite stable symptoms emphasizes the dynamic, subclinical nature of microvascular injury.Multimodal imaging combining structural and functional assessment is critical for the early detection of ischemic retinal events, even in patients without classic vascular risk factors.
