Multimodal and retro-mode imaging in sclerochoroidal calcification: A case report.

We present a case report on sclerochoroidal calcification (SCC), a rare condition involving calcium pyrophosphate deposits in the posterior pole of the eye in a 70-year-old patient. We provide an account of the clinical presentation and its appearance in multimodal images, using color fundus photography, swept-source optical coherence tomography (SS-OCT), ocular ultrasound, and the novel retro-mode imaging (RMI) technique. Visual acuity was 20/25 in the right eye (OD) and 20/20 in the left eye (OS). Color fundus photography revealed yellowish deposits located in the upper temporal arcade of both eyes. SS-OCT demonstrated masses of scleral origin. Ocular ultrasounds confirmed the calcification of these masses. RMI detected hyper-reflective images with marked superficial elevation. Systemic laboratory results did not detect any abnormalities, leading to the diagnosis of bilateral idiopathic SCC.

Calcified Sclero-Choroidal Choristomas in Mosaic RASopathies: A Description of a New Imaging Sign.

PURPOSE: To evaluate the imaging and clinical features of unusual calcified lesions seen in the fundus of patients with mosaic RASopathy. DESIGN: Single-center retrospective observational study. SUBJECTS: Ten eyes with calcified fundus lesions in 7 patients with mosaic RASopathy. METHODS: The lesions were evaluated with fundus photography, oral fundus fluorescein angiography, B-scan ultrasonography, magnetic resonance imaging (MRI), and computed tomography (CT) scan where available. MAIN OUTCOME MEASURES: The imaging characteristics of calcified fundus lesions were assessed. RESULTS: We found 7 patients with mosaic RASopathies, 5 men and 2 women (3 with linear sebaceous nevus syndrome, 3 with oculoectodermal syndrome, and 1 with encephalocraniocutaneous lipomatosis) with molecular confirmation in 5 cases, all 5 having KRAS-pathogenic variants. Calcified fundus lesions were identified in 10 eyes (bilateral in 3 patients), appearing as slightly elevated, creamy-yellow lesions around or adjacent to the optic nerve, extending supero-nasally; all but 2 of these lesions involved both the choroid and sclera, with 2 of them only involving the sclera at the time of examination. One case developed a choroidal neovascular membrane necessitating intravitreal bevacizumab injections. All 7 patients had B-scan ultrasonography, and the lesion appeared as a hyperechogenic area with an acoustic shadow posteriorly despite reduced gain. Five patients had MRI, and where fundus lesions were present, there was a focal defect in the sclero-choroidal layer. Four patients had a CT scan, and all 4 showed calcifications affecting both the posteromedial sclero-choroid and adjacent medial rectus muscle. Two of these patients had normal eye movements, 1 had a unilateral fixed adducted eye and a vestigial fibrous medial rectus muscle seen in imaging and intraoperatively, and the fourth had marked exotropia with a right gaze deficit affecting both eyes. CONCLUSIONS: We propose that the lesions seen in this cohort are calcified sclero-choroidal choristomas and should be suspected in mosaic RASopathies when creamy-yellow lesions are seen in the fundus. If identified, the possibility of choroidal neovascularization should be considered during follow-up. In all cases where a CT scan was performed, a novel sign of sclero-muscular calcification involving the medial rectus muscle was seen. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Giant anterior scleral staphyloma caused by blunt ocular trauma: a case report.

BACKGROUND: Anterior scleral staphyloma is a relatively rare disease characterized by thinning and expansion of sclera. We described the clinical presentation, diagnosis and treatment of a case with giant anterior scleral staphyloma caused by blunt ocular trauma. CASE PRESENTATION: A 24-years-old male, presented with a black cyst-like mass protruding from the right eyeball for 9 years after a history of glass crush contusion. The ultrasound biomicroscopy examination showed two cysts in the right eyeball. The larger one was about 5.92 mm*4.69 mm in size and the scleral lacerations were connected to the posterior chamber below the cyst. For treatment, resection of the anterior scleral staphyloma and the scleral patch graft transplantation was performed. The vision of the patient was improved compared with that before surgery. There were no obvious complications. CONCLUSION: The clinical presentation, diagnosis, and treatment of the case with giant anterior scleral staphyloma can provide a reference for the management of anterior scleral staphyloma. Surgical resection and scleral patch graft should be a good option for the treatment of giant anterior scleral staphyloma.

Prevalence of Dome-Shaped Macula and Tilted Disc Syndrome in High Myopia.

BACKGROUND AND OBJECTIVE: Dome-shaped macula (DSM) and tilted disc syndrome (TDS) are two macular abnormalities that may occur in eyes with high myopia. The aim of this study was to determine the prevalence of both entities in our population. PATIENTS AND METHODS: This was a prospective and observational study. Optical coherence tomography of the macula was performed in eyes with high myopia (spherical equivalent [SE] of -8D or greater) to assess the prevalence of DSM and TDS. RESULTS: Sixty-eight eyes were included. Three eyes (4.41%) had DSM and 8 (11.76%) eyes had TDS. The most common macular anomaly was posterior staphyloma (PS) (12 [17.65%]). From the eyes with DSM (n = 3), only two presented PS. An older age and a higher SE were predisposing factors for PS (P = 0.003). CONCLUSIONS: A lower prevalence of DSM and a higher prevalence of TDS was observed in our population compared to those reported in literature. [Ophthalmic Surg Lasers Imaging Retina 2023;54:568-572.].

Successful Medical and Surgical Management of Recalcitrant Acanthamoeba Keratitis, Scleritis, and Culture-Positive Scleral Abscess.

PURPOSE: The purpose of this study was to describe the management of a case of recurrent scleritis and Acanthamoeba -positive scleral abscess in a patient after the use of miltefosine for recalcitrant Acanthamoeba keratitis. METHODS: This is a case study. RESULTS: In this study, we report a case of advanced Acanthamoeba keratitis with resultant corneal perforation with therapeutic keratoplasty and associated scleritis who later developed a scleral abscess after treatment with oral miltefosine. The scleral abscess was positive for Acanthamoeba cysts and trophozoites, and after treatment for an additional several months, the patient had complete resolution of her disease. CONCLUSIONS: Acanthamoeba scleritis is a rare complication associated with Acanthamoeba keratitis. It has traditionally been treated as an immune reaction and associated inflammation, especially with the use of miltefosine. Management can require a multitude of different approaches, and in this situation, it has been demonstrated that scleritis can be infectious and that conservative management can be effective.

Ocular surface squamous neoplasia with 360° limbal involvement: a study of 130 patients.

PURPOSE: To describe the risk factors, clinical features and management outcomes of ocular surface squamous neoplasia (OSSN) with 360° of limbal involvement (360-OSSN) and compare with segmental limbal involvement (SL-OSSN). METHODS: Retrospective comparative study of 360-OSSN vs SL-OSSN. All 360-OSSN and every 10th patient with SL-OSSN during the study period (2012-2020) were included. Lesions with uncertain diagnosis were excluded. RESULTS: Of 1250 patients diagnosed with OSSN during the study period, 30 (2%) had 360-OSSN. A total of 100 patients of OSSN with SL-OSSN were included for comparison. 360-OSSN patients more often had longer duration of symptoms (mean, 17 vs 8 months; p, 0.003), prior misdiagnosis (17% vs 6%, p, 0.13) and prior intervention (47% vs 13%; p, 0.0002) than patients with SL-OSSN. 360-OSSN had higher incidence of scleral fixity (57% vs 16%; p < 0.0001), corneal/scleral melt (17% vs 0%; p, 0.0005), intraocular tumor extension (17% vs 0%; p, 0.003), orbital tumor extension (33% vs 1%; p < 0.0001), and advanced T stage at presentation (Tis: 37% vs 76%, T1: 0% vs 15%; T2: 7% vs 4%; T3: 27% vs 4%; T4: 30% vs 1%; p < 0.001). Over a mean follow-up of 14 months, lymph node metastasis (8% vs 0%; p, 0.05) and distant metastasis (4% vs 0%; p, 0.23) were more common in 360-OSSN group compared to SL-OSSN group. CONCLUSION: Risk factors of 360-OSSN include prolonged symptoms, prior misdiagnosis and prior intervention. It represents an advanced form of disease with propensity for corneo-scleral melt and invasive disease which requires aggressive management.

Imaging characteristics of idiopathic scleroma: a retrospective case series and review of the literature.

BACKGROUND: Idiopathic scleroma (previously coined solitary idiopathic choroiditis or focal scleral nodule) is an innocuous lesion affecting the sclera with intraocular manifestations. It is often the basis of many misdiagnoses such as amelanotic choroidal melanoma, osteoma or metastatic lesions. Patients are often asymptomatic and the course is benign. With increasing use of community based imaging, more of such cases are being identified. This paper is a retrospective case series investigating the multi-modal imaging findings of idiopathic scleroma. METHODS: A retrospective analysis of prospectively collected data were analysed. Over the course of January 2008-January 2022, 44 patients diagnosed with idiopathic scleroma and imaged with wide-field colour fundus photography, fundus autofluorescence, ocular coherence tomography (OCT) and B-scan ultrasonography. Due to a poor image, only 43 images were included for OCT review. We also reviewed our patient's demographics, symptoms and baseline ophthalmic characteristics upon presentation. RESULTS: The mean age was 52 years (range 32-79) and there was no predilection towards gender. All lesions were post equatorial with the most common location being inferotemporal (n = 16, 36%); 32 lesions (73%) were yellow on fundus photography. 82% (n = 36/44) of lesions exhibited hyperautoflourescence and 43 lesions (98%) showed hyperechogenicity on B-scan ultrasonography. 100% of lesions originated from the sclera with no lesions showing active inflammation. 20 (47%) lesions had associated blood vessels overlying them on OCT. DISCUSSION: Idiopathic scleroma is a yellow, hyperautofluorescent, hyperechogeneic scleral lesion that has no signs of active inflammation. These characteristics help define them from other more sinister cause of amelanotic fundal lesions.

Scleral Discoloration Because of Minocycline Use: A Case Report and Review of the Literature.

In this case report, we highlight minocycline-induced scleral hyperpigmentation, combined with ear and fingernail discoloration that developed after over 15 years of use for rosacea in a 78-year-old male with multiple medical comorbidities. Minocycline, a tetracycline antibiotic, is used to treat rosacea and acne as well as some orthopedic infections. It is typically used for extended periods of time; long-term use of minocycline is associated with hyperpigmentation of the sclera, conjunctiva, retina, teeth, skin, subcutaneous fat, oral mucosa, tympanic membrane, and gingiva. This case highlights that hyperpigmentation is more likely to occur in older patients than in younger patients. Scleral hyperpigmentation is not associated with vision loss; however, cosmetic concerns can prompt discontinuation of minocycline. Nonetheless, after cessation, the lesions persist in some patients. Monitoring for hyperpigmentation in patients using minocycline is important, as the hyperpigmentation is more likely to be permanent with long-term use.

Modified Supportive Simple Limbal Epithelial Transplantation (M-SLET): A surgical technique modified for limbal stem cell deficiency.

This study aimed to develop and modify the surgical technique of simple limbal epithelial transplantation in patients with limbal stem cell deficiency to provide support to epithelial explants during the post-operative period. This is a case series of five eyes of five patients who underwent modified supportive simple limbal epithelial transplantation (M-SLET) surgery. The health and stability of the ocular surface were assessed based on clinical slit lamp examination; they were the main outcome measures. All patients had a stable ocular surface and healed epithelium during all the follow-up visits. The M-SLET technique provides additional support to limbal epithelial explants, adhering to the cornea, thus creating a stable epithelial surface. This is particularly important when there is a risk of explants being dislodged by eye rubbing.

Eyelid and scleral thermal injury following phacoemulsification in silicone oil: a case report.

BACKGROUND: Phacoemulsification has been the mainstay method for extracapsular cataract extraction surgery in the anterior segment; for cases of posterior drop of lens fragments into the vitreous, a posterior segment phacoemulsification instrument (fragmatome; Alcon, Inc., Fort Worth, TX) can be employed to remove the dislocated lens materials. Studies have reported on thermal injury to the cornea during phacmoemulsification of the anterior segment. However, few studies have investigated thermal burn in the simultaneous sclera and eyelid induced by the fragmatome. Currently, there is no reports and lack of optimal strategy for the management of nucleus drop in a vitreous cavity filled with silicon oil. CASE PRESENTATION: We present the case of a 53-year-old male patient with a thermal burn wound on the upper eyelid and sclera following phacoemulsification for a dropped lens in a silicone oil-filled vitreous. We further designed an experiment to verify our hypothesis that thermal injury could be induced by the high temperature of the metal tip during phacoemulsification in silicone oil. In our experiment, during 420 s of continuous ultrasonic wave, the temperature of the fragmatome tip in the balanced salt solution (BSS) increased from 22.0 to 24.0 ºC, while the temperature of the fragmatome tip in the silicone oil group increased from 22.0 to 43.0 ºC. CONCLUSIONS: The temperature of the fragmatome tip increased significantly in silicone oil compared to BSS in the experiment. Thus, physicians should be aware of possible thermal complications when using fragmatome in eyes filled with silicone oil.

ALT (allogeneic limbal transplantation): a new surgical technique for limbal stem cell deficiency.

PURPOSE: Limbal stem cell deficiency (LSCD) is a rare but extremely relevant disease of the eye. LSCD patients often require a variety of surgical procedures, including keratoplasty in some cases. However, the outcome of these surgeries, including opacification and revascularization, is often frustrating due to LSCD relapse. METHODS: We developed a new surgical technique for the treatment of LSCD in which partial allogenic limbal transplantation (ALT) is carried out as part of penetrating keratoplasty (PK). After the PK, 1-8 slices from the limbal tissue of the donor graft are prepared and placed under the double running sutures attaching the corneal graft. This procedure was performed on 14 patients with LSCD, caused by severe ocular burn in 5 cases and by infection in 9. Between one and eight limbal transplants were used depending on the extension of the LSCD. RESULTS: All 14 patients showed stable or increased visual acuity after the ALT surgery compared to their preoperative visual acuity. All of the grafts were integrated into the superficial corneal layers without progression of corneal vascularization beyond the limbal grafts. The median follow-up period was 12 months on average. CONCLUSION: The ALT method seems to be a promising surgical procedure for the treatment of patients with LSCD. It can be properly carried out in the context of keratoplasty and does not require a separate donor tissue. The ALT grafts may offer the possibility of constructing a new limbal region, resulting in stable or even increased visual acuity and the absence of corneal vascularization.

Anterior staphyloma repair following trauma and surgery - A retrospective review of techniques and outcomes over 25 years.

Purpose: To review surgical options, techniques, and outcomes of anterior staphyloma repair done following trauma and surgery. Methods: This was a retrospective case study of patients who underwent staphyloma repair with scleral or tibial periosteal patch grafts following trauma and surgery with a minimum follow-up of 3 months postoperatively. Preoperative risk factors, choice of graft materials, surgical details, and outcomes in terms of graft uptake and tectonic integrity were analyzed. Results: Seventeen eyes of 17 patients underwent successful staphyloma repair (scleral 15, tibial periosteal two). Mean follow-up was 47.1 months (3-159 months). Postoperative intraocular pressure rise noted in four eyes was controlled medically or surgically. Three patients underwent successful repeat patch grafting (graft melt one and recurrent ectasia two). Tectonic integrity of the eyeball was restored and maintained in all patients at the final follow-up. Conclusion: Comprehensive evaluation of the risk factors, control of ocular comorbid conditions, and early and meticulous surgery can optimize results.

Silicone Oil Tamponade-Retina Contact in Highly Myopic Eyes With and Without Encircling Bands: A Computational Fluid Dynamics Study.

Purpose: To investigate the behavior of silicone oil (SiO) at a steady equilibrium and during saccades in pseudophakic highly myopic eyes with posterior staphyloma with and without an encircling band and compare it to behavior in emmetropic eyes. The SiO-retina contact area and shear stress were calculated by computational fluid dynamics. Methods: A numerical model of an emmetropic eye and a myopic eye with and without scleral band underwent a saccade of 50°/0.137 s. The vitreous chamber surface was divided into superior and inferior 180° sectors: lens, pre-equator, post-equator, and macula. SiO-retina contact was evaluated as a function of fill percentages between 80% and 90% for standing, 45° upward tilt, and supine patients. Maximum and average shear stress were calculated. Results: Overall, SiO-retina contact ranged between 40% and 83%; fill percentage varied between 80% and 95%. Neither the encircling scleral band nor the staphyloma significantly affected the SiO-retina contact area, although the presence of a scleral band proved disadvantageous when gazing 45° upward. The inferior retina-SiO contact remained below 40% despite 95% SiO fill. The SS significantly increased at the scleral band indentation and decreased elsewhere. The staphyloma greatly reduced shear stress at the macula. Conclusions: The presence of a myopic staphyloma reduces shear stress at the macula but does not alter SiO-retina contact significantly. The apposition of a 360° scleral band may reduce SiO-retina contact at least in some postures and increases the SS at the indentation. Translational Relevance: Assessing SiO-retina contact when vitreous chamber geometry changes according to pathologic or iatrogenic modifications allows accurate prediction of real-life tamponade behavior and helps explain surgical outcomes.

Intercalary staphyloma after strabismus surgery in a patient with Marfan syndrome: A case report.

RATIONALE: A few cases of intercalary staphyloma have been reported in patients with Marfan syndrome, but we believe that this is the first case of intercalary staphyloma in Marfan syndrome developing after strabismus surgery. PATIENT CONCERNS: A 9-year-old girl diagnosed with Marfan syndrome visited a strabismus clinic for treatment of esotropia. Both eyes were aphakic and had 60 prism diopter esotropia at distance and 55 prism diopter esotropia at near. There were no corneal, conjunctival, or scleral abnormalities. Six millimeters of recession was performed on both medial rectus muscles via an inferonasal fornix approach under general anesthesia. 5 days after surgery, a dark gray protruding lesion was observed on the upper nasal side of the left eye. DIAGNOSES: Intraocular ultrasonography showed no bleeding, retinal detachment, or other abnormal findings. Computed tomography showed a conical protrusion of the scleral wall which was diagnosed as intercalary staphyloma. INTERVENTIONS: To reduce risk of progression of the staphyloma in the left eye and to reduce risk of development of a new staphyloma, intraocular pressure lowering eye drops were administered. OUTCOMES: We just observed it without any intervention except the intraocular pressures lowering eye drops. It remained stable for 12 months. LESSONS: Clinicians need to be alert to the possibility of this serious complication in Marfan syndrome patients after minor surgical trauma, which can occur during uneventful strabismus surgery.