Left Eye Research Articles

Clinical characteristics and treatment outcomes of patients with IgG4-positive ocular adnexal marginal zone B-cell lymphoma.

To explore the clinicopathological characteristics of immunoglobulin G4 (IgG4)-positive ocular adnexal marginal zone B-cell lymphoma (OAML) and associated patient treatment outcomes. Medical records from patients diagnosed with IgG4-positive OAML treated at the West China Hospital between January 2016 and August 2023 were retrospectively analyzed. This study included data from 22 patients (11 males, 11 females), aged between 36 and 83 years, with disease durations from 1 month to 30 years. Sixteen cases exhibited unilateral ocular involvement (ten left eyes, six right eyes), while six exhibited bilateral involvement. Common clinical symptoms included ocular masses, eyelid swelling, and proptosis, with the orbit and lacrimal gland being the most commonly impacted sites. Among the 22 patients, 13 who were clinically suspected of having IgG4-related ophthalmic disease (IgG4-ROD) underwent serum IgG4 testing pre-operatively, revealing elevated IgG4 levels in 11 of these patients. The use of computed tomography and magnetic resonance imaging facilitated the evaluation of the location and size of lesions. All 22 patients received surgical treatment. Subsequently, 14 of these patients underwent local radiotherapy, five received post-operative chemotherapy, and three were closely observed. The follow-up period of patients in this study was 3-77 months, with an average follow-up time of 36 months. Except for one patient who died of disease progression, all others showed favorable prognoses with significant improvements. These results support the classification of IgG4-positive OAML as a distinct OAML sub-type with clinical features that partially overlap with IgG4-ROD. Therefore, accurate differentiation between OAML and IgG4-ROD is imperative, necessitating timely surgical intervention and precise pathological diagnosis to prevent diagnostic errors and inappropriate treatment. Currently, no standardized treatments for IgG4-positive OAML exist, but our results suggest that standard OAML therapies are generally efficacious.

Mydriasis in eastern box turtles (Terrapene carolina carolina) following topical administration of proparacaine, 10% phenylephrine, and rocuronium bromide.

To determine the mydriatic effect of topical 10% phenylephrine with 10 mg/mL rocuronium bromide and compare this protocol with and without pretreatment with proparacaine. Ten client-owned pet adult eastern box turtles (Terrapene carolina carolina). All turtles were sedated with 8 mg/kg alfaxalone intramuscularly. One group of four turtles received four 20 μL drops of 10% phenylephrine and four 20 μL drops of rocuronium bromide in the right eye. Another group of four turtles received one standard drop of proparacaine followed by four 20 μL drops of 10% phenylephrine and four 20 μL drops of rocuronium bromide in the right eye. Two control group turtles received four 20 μL drops of saline in the right eye. The left eye was untreated in all turtles. Drops of the same type were separated by 2 min while drops of different types were separated by 5 min. Pupil size was recorded at 0, 15, 30, 60, 90, 120, 180, 240, and 360 min after administration of the final drop. Treatment with 10% phenylephrine and rocuronium bromide resulted in pupil diameter changes from baseline that were statistically significant from zero at 60, 90, and 120 min in the non-proparacaine group and 90 min in the proparacaine group. The time to peak effect was 90 min in the proparacaine group and 75 min in the non-proparacaine group. Saline-treated pupils in the control group decreased in diameter over the study period. Overall, the treated eyes of the proparacaine group and non-proparacaine group were not different from each other, but both dilated more than the control group. Rocuronium bromide and 10% phenylephrine can produce effective and safe mydriasis in eastern box turtles, but there was wide interindividual variation in effectiveness. Proparacaine did not improve the mydriatic effect.

Improvement in Central Serous Chorioretinopathy Following Multiwavelength Photobiomodulation Treatment - Case Report.

Central serous chorioretinopathy (CSCR) is a condition where fluid build-up accumulates underneath the retina, resulting in retinal pigment epithelium (RPE) detachment and vision loss. Irreversible retinal functional and anatomical changes are possible consequences. Research into novel strategies to aid in recovery are of interest. Photobiomodulation (PBM) uses light wavelengths to improve cellular function and shows positive effects in several conditions including those with edema. This prospective case report details a 39-year-old woman with CSCR. Multiwavelength PBM treatment was initiated with the Valeda® Light Delivery System. A series of treatment included nine sessions delivered over 3-5weeks. Follow-up treatments were conducted. Optical coherence tomography (OCT) imaging and best-corrected visual acuity (BCVA) measures were taken. The patient has been followed for approximately 1year. The patient presented with blurred vision and a BCVA score of 65 letters in the left eye. After 3weeks of observation, the patient's vision had further declined two lines on the Early Treatment Diabetic Retinopathy Study (ETDRS) chart to 55 letters. Fluorescein angiography was performed, confirming CSCR diagnosis, and PBM was initiated. PBM treatment improved BCVA and fluid build-up in the RPE within 1week of treatment (three treatment sessions). Following the full series of treatment (nine PBM treatment sessions), fluid was completely resolved and BCVA scored at 80 letters. The patient had a repeat PBM treatmentseries ~ 6months later and has shown stable vision and no fluid present on OCT scan. The patient was seen again ~ 1year later with continued stable vision and no fluid detection. PBM is a non-invasive treatment option that may provide benefit in CSCR to resolve fluid build-up, macular change, and vision loss. Research into PBM as an immediate treatment option for CSCR, especially those with chronic presentations or those posed to have irreversible damage, is warranted to confirm effectiveness.

Recognition of a patient with neck autonomic dysfunction: findings from a rare case report of harlequin syndrome in direct access physiotherapy.

Harlequin syndrome is a rare autonomic condition consisting of unilateral facial flushing and sweating induced by heat, emotion or physical activity. The affected side presents anhidrosis and midline facial pallor due to denervation of the sympathetic fibers. This case describes a patient who reported right-side redness of the face associated with hyperhidrosis during physical activity. She had two previous major motor vehicle accidents. The patient demonstrated difficulties in the visual accommodation of the left eye, but cranial nerve assessment was unremarkable; the patient was then referred to an ophthalmologist, who excluded any autonomic dysfunction as the primary cause of convergence and visual acuity. A left-sided sympathetic dysfunction with Harlequin sign diagnosis was made followed by a progressive compensatory adaptation of the right face. The patient was educated and reassured about the benign nature of her problem. Knowledge of the autonomic nervous system is still limited in clinical practice. Although challenging, physiotherapists should develop the knowledge and ability needed to perform appropriate assessment of autonomic dysfunctions. A dispositional reasoning model should be considered in differential diagnosis.

Scleral contact lens for severe keratoconus with corneal hydrops: A case report

We describe a case of fitting a scleral contact lens for keratoconus management. A 37-year-old female has came for the keratoconus management opinion. She was using glasses for the last 20 years. She was presented with bilateral severe keratoconus with corneal hydrops, and it show high myopic. A standardized eye examination was thoroughly done in the hospital visual acuity, corneal oculyzer and slit lamp examination with IOP measurement. . The best spectacle corrected visual acuity was Right eye 6/60 with pinhole no improvement with the correction of -13.75/-2.00 x 35 and Left eye having best spectacle corrected visual acuity of counting finger- finger counting with pinhole no improvement with the correction of -14.00 dsph. Optimal fitting characteristics were found in terms of vault, centration and landing and coverage the final parameter that we selected base curve, diameter, power and sag value RE 7.34, 16.40 mm, -15.25/-1.00 X 40, 5.41; LE 6.75, 16.40, -19.25/-1.50 X 110, 5.68 respectively. With over refraction of this power visual acuity was 6/6p and 6/9 in right eye and left eye respectively. In this case we were fit the patient with miniscleral lens which are more comfortable, and the visual acuity was also improved and in the follow up of 3 months no ocular adverse effects were seen. This case report shows that in this patient a Maxim Scleral Lens could be fitted successfully for keratoconus management, providing good visual quality.

Evaluation of a new transpalpebral tonometer for self-measuring intraocular pressure.

We developed a novel transpalpebral self-tonometer called the TapEye tonometer (TET) based on palpation of the upper eyelid. Our goal was to evaluate a method for improving the accuracy of measuring intraocular pressure (IOP) through the eyelid. Participants underwent standardized training by technicians and were required to be able to use the TET for study inclusion. Subsequently, a noncontact tonometer and Goldmann applanation tonometer (GAT) were used. All participants were instructed to measure their IOPs using the three tonometers at baseline (visit 1) and at 1 month (visit 2). At visit 2, the corrected IOP value measured by the TET (c-TET) was calculated using the difference between the TET and GAT measurements obtained at visit 1. No significant correlations were found between the TET and GAT measurements at any visit, but the correlation between the c-TET and GAT measurements at visit 2 was significant. The mean difference between the c-TET and GAT measurements was 0.4 ± 3.7 mmHg in the right eye and 0.5 ± 3.4 mmHg in the left eye. After correcting the IOP based on the difference between the TET and GAT measurements at the initial visit, the corrected IOP value of the TET was correlated with that of the GAT at the second visit. The TET has the potential to address an unmet need by providing a tool for minimally invasive IOP measurements. Clinical trial registration number: jRCTs032220268.

Eyes of empowerment: A case study on the impact of custom ocular prosthesis in restoring quality of life

Ocular prostheses serve as invaluable tools in restoring both visual symmetry and psychological well-being for individuals who have experienced ocular defects. This article presents a comprehensive overview of the fabrication and placement of custom acrylic ocular prosthesis in a 39-year-old male patient who suffered left eye loss following a traumatic accident. The patient's history underscores the profound impact of ocular trauma on self-image and quality of life, highlighting the importance of prosthetic rehabilitation. Meticulous attention was paid to every step of the fabrication process, including impression taking, color matching, and fitting, to ensure optimal aesthetic outcome and functionality. The successful placement of the prosthesis resulted in significant improvements in the patient's confidence and overall well-being. This case report emphasizes the approach required for successful ocular prosthetic rehabilitation and underscores the transformative impact of custom ocular prostheses in restoring quality of life for individuals with ocular defects.

Visual dysfunction and neurodegeneration caused by severe inflammatory optic neuropathy after coil embolization of a paraclinoid aneurysm: illustrative case.

Visual impairment due to delayed optic neuropathy is a rare complication of the endovascular treatment of paraclinoid aneurysms. An inflammatory response induced in the treated aneurysm wall is a known mechanism underlying this pathophysiology. The authors describe a case with severe and progressive optic neuropathy leading to neuronal degeneration and severe visual dysfunction. A 42-year-old female with a history of surgical clipping for a paraclinoid aneurysm presented with a recurrence. Although the patient was unaware of any visual dysfunction, a preoperative ophthalmological examination revealed mild inferior quadrantanopia in the left eye. The coil embolization procedure was uneventful; however, the following day, the patient experienced progressive visual impairment, which worsened despite the initiation of steroid therapy. Ophthalmological examination revealed a severe decrease in visual acuity and further deterioration of the visual field. Magnetic resonance imaging showed remarkable swelling and edema of the left optic nerve adjacent to the treated aneurysm. Despite continued steroid therapy, the patient's visual function did not recover well due to subsequent optic nerve degeneration. Optic neuropathy after endovascular procedures can lead to severe visual dysfunction. Careful management is essential, particularly when treating a symptomatic paraclinoid aneurysm, even if symptoms are only apparent on detailed examination.

Clinical characteristics and surgical management outcomes of glaucoma secondary to congenital ectropion uveae: a preliminary analysis of penetrating Schlemm's canaloplasty

Objective: To investigate the clinical characteristics and treatment outcomes of glaucoma secondary to congenital ectropion uveae (CEU) using penetrating Schlemm's canaloplasty. Methods: This was a retrospective case series study. Medical records of patients diagnosed with glaucoma secondary to CEU and undergoing penetrating Schlemm's canaloplasty at the Eye Hospital of Wenzhou Medical University between August 2020 and December 2021 were collected. Clinical characteristics including the extent and location of iris ectropion, type of glaucoma, were analyzed. Follow-up visits were conducted at 1, 3, 6 months, and 1 year postoperatively. Visual acuity, intraocular pressure (IOP), anterior segment and fundus condition, filtering bleb morphology, use of IOP-lowering medications, ultrasound biomicroscopy results, and other indicators were analyzed to summarize surgical outcomes. Results: Six cases (6 eyes) of glaucoma secondary to CEU were included, all unilateral, with 3 left eyes and 3 right eyes; median age was 10.0 (5.3, 28.8) years; including 3 males and 3 females. Preoperative IOP was (31.7±10.0) mmHg (1 mmHg=0.133 kPa), and the preoperative number of IOP-lowering medications used was 2.0 (2.0, 3.2). The extent of iris ectropion in the 6 cases ranged from 270 ° to 360 °, with peripheral anterior synechiae corresponding to the location of iris ectropion, and angle closure with the degree of synechiae extending beyond Schwalbe's line. No surgical complications occurred in any of the 6 cases postoperatively. At 1 month postoperatively, the IOP was (16.4±3.2) mmHg, with a median of 0.0 (0.0, 1.5) medications used. At 3 months postoperatively, the IOP was (14.8±6.0) mmHg, with a median of 0.0 (0.0, 2.2) medications used. At 6 months postoperatively, the IOP was (18.1±6.1) mmHg, with a median of 0.0 (0.0, 0.5) medications used. Among them, 5 patients had a follow-up period of 1 year postoperatively, all achieving controlled IOP without the use of IOP-lowering medications, with an average IOP of (15.5±3.1) mmHg. No obvious filtering bleb formation was observed at the surgical site in all patients. Conclusions: Glaucoma secondary to CEU manifests primarily as closed-angle glaucoma, with a correspondence between the closure range of anterior iris adhesions in the angle and the extent of iris ectropion. Penetrating Schlemm's canaloplasty demonstrates favorable and stable efficacy for its treatment.

Glomangiopericytoma of the Nasal Sinus Mimicking Dacryocoele: A Case Report and Literature Review

Introduction: Glomangiopericytoma (GPC) of the nasal sinus is a rare mesenchymal tumor that can mimic various benign pathologies in this anatomical region. We report an unusual clinical case and describe the diagnostic and therapeutic challenges encountered. Case Presentation: A 62-year-old woman presented with swelling of the left inner eye angle, tearing, and ipsilateral nasal obstruction. Clinical examination and imaging initially suggested chronic dacryocystitis. A polypoid nasal sinus mass was detected on endoscopy and computed tomography. Complete excision was performed via endoscopic nasal sinus surgery. The histopathological analysis confirmed the definitive diagnosis of glomangiopericytoma. Discussion: Nasal sinus GPC is a rare tumor, typically benign, derived from perivascular cells. Its clinical presentation is nonspecific and can mimic more common pathologies. Diagnosis is based on imaging and histopathological analysis with immunohistochemistry. The treatment of choice is complete surgical excision, preferably via minimally invasive endoscopic approach. Long-term close monitoring is recommended due to the risk of local recurrence. Conclusion: This clinical case highlights the importance of considering glomangiopericytoma in the differential diagnosis of nasal sinus masses, even in cases of initial benign presentation. A multidisciplinary approach, involving imaging, surgery, and histopathological analysis, is essential for optimal management.

IGWAS: Image-based genome-wide association of self-supervised deep phenotyping of retina fundus images.

Existing imaging genetics studies have been mostly limited in scope by using imaging-derived phenotypes defined by human experts. Here, leveraging new breakthroughs in self-supervised deep representation learning, we propose a new approach, image-based genome-wide association study (iGWAS), for identifying genetic factors associated with phenotypes discovered from medical images using contrastive learning. Using retinal fundus photos, our model extracts a 128-dimensional vector representing features of the retina as phenotypes. After training the model on 40,000 images from the EyePACS dataset, we generated phenotypes from 130,329 images of 65,629 British White participants in the UK Biobank. We conducted GWAS on these phenotypes and identified 14 loci with genome-wide significance (p<5×10-8 and intersection of hits from left and right eyes). We also did GWAS on the retina color, the average color of the center region of the retinal fundus photos. The GWAS of retina colors identified 34 loci, 7 are overlapping with GWAS of raw image phenotype. Our results establish the feasibility of this new framework of genomic study based on self-supervised phenotyping of medical images.

Open-Globe Injury With Globe Penetration Leading to Complex Retinal Detachment After Intraoral Anesthetic Injection

Purpose: To report a case of a suspected open-globe injury and globe penetration complicated by a rhegmatogenous retinal detachment (RRD) after administration of intraoral anesthetic injections during an endodontic procedure. Methods: A retrospective chart review was performed. Results: A 34-year-old woman presented with acute onset of flashes, floaters, and a curtain-like shadow across her field of vision. Four days previously, she had multiple left-sided anesthetic injections during an endodontic procedure. The visual acuity (VA) in the left eye was 20/25. Ophthalmoscopy showed a suspected globe-penetration site inferiorly, associated multilayered hemorrhages, and adjacent subretinal fluid. Laser demarcation of the inferior RRD was performed at sequential clinical visits. Through 8 months, the multilayered hemorrhages improved while proliferative vitreoretinopathy–associated tissue formation over the posterior pole caused macular distortion and mild visual deterioration. The VA in the left eye was 20/40 at the last follow-up. Conclusions: Iatrogenic globe penetration during intraoral anesthesia delivery associated with an endodontic procedure may result in globe injury.

Medical staff dose estimation during pediatric cardiac interventional procedures.

The purpose of this study is to evaluate the occupational doses (eye lens, extremities and whole body) in paediatric cardiac interventional and diagnostic catheterization procedures performed in a paediatric reference hospital located in Recife, Pernambuco. For eye lens dosimetry, the results show that the left eye receives a higher dose than the right eye, and there is a small difference between the doses received during diagnostic (D) and therapeutic (T) procedures. The extrapolated annual values for the most exposed eye are close to the annual limit. For doses to the hands, it was observed that in a significant number of procedures (37 out of 45 therapeutic procedures, or 82%) at least one hand of the physician was exposed to the primary beam. During diagnostic procedures, the physician's hand was in the radiation field in 11 of the 17 catheterization procedures (65%). This resulted in a 10-fold increase in dose to the hands. The results underscore the need for optimization of radiation safety and continued efforts to engage staff in a radiation safety culture.&#xD.

Case report: Bilateral optic nerve sheath meningocele: clinical aspects

Optic nerve sheath meningocele is an enlargement of the sheath itself, consisting of a collection of cerebrospinal fluid along the perineural space. It should be considered primary if it is not associated with orbital–cerebral neoplasm or with cranio-orbital junction malformations. We report three cases of bilateral primary idiopathic optic nerve sheath meningocele, two of them with gradual vision loss. The first case presented a history of monocular blurred vision of the right eye and headache. It was initially treated with acetazolamide without any improvement, after which optic nerve sheath fenestration was required. The second case showed intermittent binocular diplopia with central 24-2 perimetry defects in the left eye. The third case was first presented as a subacute bilateral conjunctivitis with a suspected orbital pseudotumor. An incidental bilateral optic nerve sheath meningocele was found in the orbital imaging, being totally asymptomatic. In all the cases, orbital and cranial magnetic resonance with contrast and fat suppression was crucial in the diagnosis.

Neglected Spheno-orbital Meningioma Presenting with Unilateral Blindness

Sphenoorbital meningiomas (SOM) constitutes 9% of all intracranial meningiomas and are originated from sphenoid wing dura. They are also called en plaque meningioma. They cause hyperostosis of involved bone and their soft tissue growth may spread the orbit, the infratemporal fossa, and the temporal fossa. Most patients with SOM are middle- aged women and most commonly presents with unilateral, nonpulsating, progressive proptosis. Recently a woman with unilateral progressive proptosis from a remote area of Bangladesh presented to us with blindness of her left eye due to diagnostic delay. After thorough evaluation and imaging studies, she was diagnosed with a large spheno-orbital meningioma with extensive bony involvement of skull base and orbit. Objective: This report highlights the necessity of creating awareness among optometrists, undergraduate medical students as well as and ophthalmologists who are practicing in remote areas of Bangladesh about clinical presentation of sphenoorbital meningiomas which is a benign lesion but may be a cause of unilateral blindness if remain undiagnosed for long Bang. J Neurosurgery 2023; 13(1): 51-54

Surgical repair of macular fold in X-linked retinoschisis initially misdiagnosed as familial exudative vitreoretinopathy

Purpose: To describe the presentation and surgical management of a young boy initially thought to have familial exudative vitreoretinopathy who was ultimately diagnosed with an unusually aggressive form of X-linked retinoschisis that included rapidly progressive bullous retinoschisis and tractional macular fold. Methods: Retrospective case report Results: A 19-month-old boy with straightening of major arcades, peripheral retinal ischemia, and in the left eye, a large macular fold was initially diagnosed as familial exudative vitreoretinopathy. During follow-up, he developed a rapidly progressive bullous retinoschisis in the left eye involving the inferior macula extending superiorly up to the macular fold. This revised the working diagnosis to X-linked retinoschisis, which was confirmed by genetic testing. Pars plana vitrectomy, inner flap retinectomy, unrolling of the macular fold and inner flap retinectomy, and C3F8 gas tamponade were performed. This resolved the macular fold. The patient showed good anatomical results without surgical complications up to 18 months of post-operative follow-up. Conclusions: X-linked retinoschisis can rarely present in young children with macular fold and peripheral ischemia, mimicking familial exudative vitreoretinopathy. Rapidly progressive bullous retinoschisis in this setting can be treated with pars plana vitrectomy, inner wall retinectomy, and removal of the vitreous traction to improve visual prognosis.

Management of bilateral lower eyelid entropion in a black Angus bull by temporary tacking and modified Hotz Celsus

AbstractThe objective was to evaluate the progression and management of eyelid tacking sutures before permanent eyelid surgical correction. A 1‐year‐old Aberdeen black Angus bull presented with a 1‐month history of bilateral corneal ulcers. Ophthalmic examination revealed an infected stromal corneal ulcer on the left eye and bilateral lower eyelid entropion. Lower eyelid tacking sutures were placed bilaterally to allow corneal healing, prevent recurrent corneal ulceration and allow resolution of concurrent moist dermatitis. Approximately 2 weeks after eyelid tacking, permanent entropion repair using a modified Hotz Celsus technique was performed on both lower eyelids. This report details the successful treatment of bilateral lower eyelid entropion in a bull using temporary tacking sutures to stabilise corneal and eyelid disease before permanent eyelid surgical correction.

Estimating brain and eye lens dose for the cardiologist in interventional cardiology-are the dose levels of concern?

To establish conversion coefficients (CCs), between mean absorbed dose to the brain and eye lens of the cardiologist and the air kerma-area product, PKA, for a set of projections in cardiac interventional procedures. Furthermore, by taking clinical data into account, a method to estimate the doses per procedure, or annual dose, is presented. Thermoluminescence dosimeters (TLDs) were used together with anthropomorphic phantoms, simulating a cardiologist performing an interventional cardiac procedure, to estimate the CCs for the brain and eye lens dose for nine standard projections, and change in patient size and x-ray spectrum. Additionally, a single CC has been estimated, accounting for each projections fraction of use in the clinic and associated PKA using clinical data from the dose monitoring system in our hospital. The maximum CCs for the eye lens and segment of the brain, is 5.47 μGy/Gycm2 (left eye lens) and 1.71 μGy/Gycm2 (left brain segment). The corresponding weighted CC: s are 3.39 μGy/Gycm2 and 0.89 μGy/Gycm2 respectively. Conversion coefficients have been established under actual scatter conditions, showing higher doses on the left side of the operator. Using modern interventional x-ray equipment, interventional cardiac procedures will not cause high radiation dose levels to the operator when a ceiling mounted shield is used, otherwise there is a risk that the threshold dose values for cataract will be reached. In addition to the CCs for the different projections, methods for deriving a single CC per cardiac interventional procedure and dose per year were introduced.

Thickened Retinal Nerve Fiber Layer Without Hypermyelination in Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay

Purpose: To report the retinal findings in a patient with autosomal recessive spastic ataxia of Charlevoix-Saguenay. Methods: A case was evaluated. Results: A 16-year-old male patient with a known diagnosis of autosomal recessive spastic ataxia of Charlevoix-Saguenay was referred for evaluation of retinal hypermyelination given its frequent association with the condition. The patient was asymptomatic with a best-corrected visual acuity of 20/20. Optical coherence tomography of the peripapillary retinal nerve fiber layer (RNFL) showed bilateral thickening in each eye (average thicknesses: 180 µm, right eye; 177 µm, left eye). An examination showed no myelinization of the RNFL. Conclusions: Most studies to date describe RNFL thickening secondary to hypermyelination as a characteristic finding in autosomal recessive spastic ataxia of Charlevoix-Saguenay. This case provides evidence that this thickening may be a result of hypertrophy rather than hypermyelination. Further investigation is needed to define the pathophysiologic cause of RNFL thickening in autosomal recessive spastic ataxia of Charlevoix-Saguenay.

Ophthalmic artery ischemia in hemophagocytic lymphohistiocytosis: A case report and review of current literature

Introduction: This case report describes the ocular manifestations of hemophagocytic lymphohistiocytosis in a 35-year-old male with suspected Adult-onset Still’s Disease. Documented ocular presentations of hemophagocytic lymphohistiocytosis are variable, and the pathophysiology remains unclear. A complete review of the current literature suggests that posterior segment findings, including retinal and vitreous hemorrhage, are most common. Case Report: Here we describe the first published case of bilateral combined choroidal and retinal ischemia in a patient with ocular hemophagocytic lymphohistiocytosis. This is suggestive of complete ophthalmic artery occlusion driving proliferative retinopathy. Mechanistic hypotheses include anemia, thrombocytopenia, coagulopathy, and histiocytic infiltration. Conclusion: In this case, pre-retinal hemorrhage, retinal hemorrhage, and vitreous hemorrhage were treated with panretinal photocoagulation, pars plana vitrectomy, and bevacizumab at different stages in each eye. Unusually for ocular hemophagocytic lymphohistiocytosis, the patient developed a unilateral tractional retinal detachment, later thought to be due to early administration bevacizumab prior to vitrectomy in the left eye. Final visual acuity in this patient remains poor, and worse in the left eye. We therefore suggest early vitrectomy to maintain adequate views of the fundus before bevacizumab or panretinal photocoagulation in this patient cohort.