Spheroidal Degeneration Research Articles

Clinical demographic characteristics and ocular Co-morbidities associated with Secondary lipid keratopathy at a tertiary care center

ObjectiveTo describe the demographics and clinical profile of Secondary Lipid Keratopathy in patients presenting to a multi-tier ophthalmology hospital network in India. MethodsThis cross-sectional hospital-based study included 3,082,727 new patients presenting between August 2010 and December 2021. Patients with a clinical diagnosis of Secondary Lipid Keratopathy in at least one eye were included as cases. The data were collected using an electronic medical record system. ResultsOverall, 389 (0.01%) patients were diagnosed with Secondary Lipid Keratopathy. Majority were males (53.5%) and had a unilateral affliction (82.8%). The most common age group at presentation was during the sixth decade of life with 76 (19.5%) patients. The overall prevalence was higher in patients from a lower socio-economic status (0.02%) presenting from the rural geography (0.01%) and in individuals related to agriculture work/ manual labor (0.02%). Of the 456 eyes, mild or no visual impairment (<20/70) was noted in 194 (42.5%) eyes followed by moderate visual impairment (>20/70 to 20/200) in 81 (17.8%) eyes. At presentation, the mean visual acuity was 0.96 ± 1.07 log MAR units. The most associated corneal condition was non-HSV corneal scar (36.8%), followed by spheroidal degeneration (19.7%) and HSV keratitis in (18.6%). ConclusionSecondary lipid keratopathy more commonly affected males presenting during the sixth decade of life. Most (42.5%) of the eyes had mild or no visual impairment. Corneal scar (non-HSV) was the most associated corneal condition in both unilateral and bilateral cases. HSV keratitis was the commonest corneal condition in those between 20 and 40 years, followed by those above 40 years.

Incidence of ocular surface squamous neoplasia in pterygium specimens

ObjectifLe ptérygion et la néoplasie squameuse de la surface oculaire (NSSO) seraient vraisemblablement des affections connexes qui partagent des facteurs de risque semblables, notamment l'exposition aux rayons UV et l'inflammation chronique. Notre étude avait pour objectif de relever l'incidence de NSSO dans des échantillons anatomopathologiques de ptérygion prélevés dans un centre de soins tertiaires entre 2010 et 2022. MéthodesIl s'agit d'une revue rétrospective des dossiers médicaux de patients qui ont subi une intervention en raison d'un ptérygion entre 2010 et 2022 au Centre hospitalier de l'Université de Montréal (CHUM). Les données recueillies comprenaient les caractéristiques démographiques de départ, les résultats de l'examen des échantillons anatomopathologiques et les données cliniques des sujets chez lesquels l'examen a mis au jour une NSSO. RésultatsAu total, 1559 patients ont subi une intervention en raison d'un diagnostic clinique de ptérygion entre 2010 et 2022; de ce nombre, 854 patients (55 %) étaient de sexe masculin. On disposait des rapports d'anatomopathologie pour 1142 échantillons; dans la vaste majorité des cas, il s'agissait effectivement d'un ptérygion (1105 sur 1142; 97 %). Trois cas de NSSO (3 sur 1142; 0,3 %) ont été découverts de manière fortuite. Outre le ptérygion, d'autres diagnostics ont été posés dans 3 % des échantillons (34 sur 1142), dont le nævus (n = 12), la dégénérescence sphéroïde (n = 3), le granulome pyogénique (n = 3) et la lymphangiectasie (n = 2). Les 3 cas de NSSO ont été découverts chez un homme de 81 ans d'origine canadienne-française, un homme de 52 ans d'origine sud-asiatique et une femme de 59 ans d'origine canadienne-française. Selon l'examen anatomopathologique, il s'agissait d'une néoplasie conjonctivale intra-épithéliale (CIN) de grade 3, d'une CIN de grade 2 et d'une CIN de grade 2, respectivement. ConclusionLa découverte d'une NSSO dans un ptérygion est rare au sein de notre population, et la NSSO peut être difficile à identifier d'après le seul examen clinique. Voilà pourquoi il est important d'envoyer tous les échantillons de ptérygion à l'anatomopathologiste.

Conjunctival Ocular Surface Squamous Neoplasia with Spheroidal Degeneration: A Rare Case of UV-B Associated Ocular Neoplasia and Keratopathy

Ocular Surface Squamous Neoplasia (OSSN) is an umbrella term that includes a broad spectrum of conjunctival and corneal malignancies, ranging from mild epithelial dysplasia to invasive squamous cell carcinoma. These are uncommon ocular surface lesions but harbour the potential to cause significant ocular morbidity and rarely mortality. Human Papilloma Viruses (HPV) and Ultraviolet-B light (UV) have been identified as important risk factors in aetiopathogenesis. The present OSSN patient is a 48- year-old male with redness, growth, and a foreign body sensation in his left eye over the past four months. Examination of the left eye revealed a growth in the nasal limbus with a feeder vessel measuring 0.9x0.3x0.1 cm in size. Diagnosis was confirmed by anterior segment Optical Coherence Tomography (OCT). The patient was admitted for an excision biopsy accompanied by intraoperative Mitomycin-C. The lesion’s histopathology revealed grade 2 in situ carcinoma with spheroidal formation. This case is considered rare due to the infrequent association between Conjunctival Intraepithelial Neoplasia (CIN) and spheroidal degeneration.

Complications in deep anterior lamellar keratoplasty - A retrospective cross sectional interventional analysis in a large series.

To analyse complications in patients managed with deep anterior lamellar keratoplasty (DALK) for diseases of anterior corneal stroma. This was a retrospective analysis of all the patients who underwent DALK in a tertiary care center in South India from 2010 to 2021. A total of 484 eyes in 378 patients were included in the study. Patients who underwent DALK for advanced keratoconus, keratoconus with Bowman's membrane scar, healed hydrops, macular corneal opacity, macular corneal dystrophy, granular corneal dystrophy, spheroidal degeneration, pellucid marginal degeneration, post-LASIK ectasia, descemetocele, postcollagen cross-linking aborted melt and dense scar, and postradial keratotomy were included in the study. The patients were followed up for 17.6±9.4 months(1-10years). Complications noted in the surgery were intraoperatively Descemet's membrane perforation in 32 eyes (6.6%), postoperatively secondary glaucoma in 16 eyes (3.31%), cataract in 7 eyes (1.45%), suture-related complications in 5 eyes (1.03%), graft rejection in 3 eyes (0.61%), traumatic dehiscence in 2 eyes (0.41%), filamentary keratitis in 2 eyes (0.41%), interface infiltrate in 1 eye (0.21%), and recurrence of disease in 4 eyes (8.77%) out of 57 eyes with corneal dystrophy. DALK as an alternative to penetrating keratoplasty for anterior corneal stromal diseases has proven to be better time and again. It has become an automatic choice for diseases of anterior cornea requiring keratoplasty. Complications occurring at any stage of surgery can be identified and managed effectively resulting in optimal outcome. This article compiles complications post DALK.

Complications in deep anterior lamellar keratoplasty - A retrospective interventional analysis in a large series.

To analyze the complications in patients managed with deep anterior lamellar keratoplasty (DALK) for diseases of the anterior corneal stroma. This is a retrospective analysis of all the patients who underwent DALK in a tertiary care center in South India from 2010 to 2020. A total of 474 eyes in 373 patients were included in the study. Patients who underwent DALK for advanced keratoconus, keratoconus with Bowman's membrane scar, healed hydrops, macular corneal opacity, macular corneal dystrophy, granular corneal dystrophy, spheroidal degeneration, pellucid marginal degeneration, post-laser-assisted in situ keratomileusis ectasia, descematocele, post-collagen cross-linking aborted melt and dense scar, and post-radial keratotomy were included in the study. The patients were followed up for 17.2 +/- 9.2 months (1-9 years). Complications noted in the surgery were intra-operatively Descemet's membrane perforation in 31 eyes (6.54%), post-operatively secondary glaucoma in 16 eyes (3.37%), cataract in seven eyes (1.47%), suture-related complications in five eyes (1.05%), graft rejection in three eyes (0.63%), traumatic dehiscence in two eyes (0.42%), filamentary keratitis in two eyes (0.42%), interface infiltrate in one eye (0.21%), and recurrence of disease in four eyes (7.14%) out of 57 eyes with corneal dystrophy. DALK as an alternative to penetrating keratoplasty for anterior corneal stromal diseases. It has become an automatic choice for diseases of the anterior cornea requiring keratoplasty. Complications can occur at any stage of surgery; however, if identified and managed early, they can result in optimal outcome.

A case of conjunctival intraepithelial neoplasia with spheroidal degeneration: a clinicopathological study.

A case of conjunctival intraepithelial neoplasia with spheroidal degeneration: a clinicopathological study.

Clinical profile and demographic distribution of band shaped keratopathy in India: A study of 8801 patients

Purpose:To describe the clinical profile and demographic distribution of band-shaped keratopathy (BSK) in patients presenting to a multitier ophthalmology hospital network in India.Methods:This cross-sectional hospital-based study included 2,664,906 new patients presenting between January 2011 and January 2021 (10-year period). Patients with a clinical diagnosis of BSK in at least one eye were included as cases. The data were collected using an electronic medical record system.Results:Overall, 8801 (0.33%) patients were diagnosed with BSK. The prevalence rates were 0.47% in children (age: <16 years) and 0.31% in adults. The majority of patients were males (62.87%) with unilateral affliction (85.21%). The mean age of the patients was 40.43 ± 23.14 years. The majority (16.93%) of the patients were in the age bracket of 11–20 years. A larger proportion of the patients were from higher socioeconomic status (60.46%) and the urban region (45.9%). Of the 10,103 eyes affected with BSK, the common ocular comorbidities were status post-vitreoretinal surgery (20.55%) and uveitis (12.7%) in children and corneal scar (41.23%) and spheroidal degeneration (13.7%) in adults. Most of the eyes had mild or no visual impairment (24.74%). Among the eyes that needed surgical intervention, chelation with ethylenediaminetetraacetic acid (EDTA) was the most performed surgical procedure (1.68%) along with phototherapeutic keratectomy (0.32%).Conclusion:BSK commonly affects adult males and is unilateral in nature. The majority of the patients in this cohort belonged to higher socioeconomic strata and urban geography. At initial presentation, visual impairment was mild to moderate in a vast majority of the patients, and the most common surgical intervention performed was chelation with EDTA during the study period.

Spheroidal Degeneration in Two Siblings: Clinical and Histopathological Features

Spheroidal corneal degeneration is predominantly seen in advanced age and hereditary predisposition to this disorder is very rare. This report describes the occurrence of bilateral band-shaped spheroidal corneal degeneration in two siblings.

English

BACKGROUND Leprosy is a chronic granulomatous infectious disease caused by Mycobacterium leprae. Several ocular manifestations occur before and during multi drug therapy (MDT) and they can continue to occur even after the completion in bacteriologically cured patients. Blindness is a catastrophic event that can occur by several mechanisms at any stage. We need to learn and recognise the early signs and symptoms of ocular complications, so that we can treat the patient early. We need to evaluate the pattern of ocular manifestation in leprosy patients on MDT (Multi Drug Therapy). METHODS A cross-sectional study was conducted over a duration of six months on diagnosed cases of leprosy and those cured from leprosy. Data was collected by clinical history with slit lamp examination, fundus examination, and laboratory investigation. RESULTS Total 30 patients were included in the study in which 13 patients were on MDT, 7 newly detected cases and 10 treated cases. 22 were males and 8 were females with male - female ratio of 2.75:1. Average age of presentation was 46 years with range from 18 - 80 years. 56 % had ocular manifestations in this study. Ocular lesions were more in cases who had leprosy for ≥ 16 years. Visual acuity ranged from 6 / 6 to PL + ve. Lagophthalmos was noted in 4 cases, cataract in 6 cases, exposure keratitis in 2, chalky white deposits on cornea in 2, spheroidal degeneration in 2, iris atrophy in 2, and pterygium in 2 cases. CONCLUSIONS Patients who completed treatment for MDT require periodic monitoring to detect ocular morbidity early and to prevent visual loss. KEYWORDS Leprosy, Multidrug Therapy, Exposure Keratitis, Lagophthalmos

Phototherapeutic keratectomy: Indications, methods and decision making.

Phototherapeutic keratectomy (PTK) involves treating anterior corneal lesions by superficial corneal ablation using an excimer laser (193 nm). Some of the commonly treated conditions include recurrent corneal erosions (RCE), corneal dystrophies, spheroidal degeneration, keratoconus, and corneal scars. We discuss various techniques of PTK including large area PTK, focal PTK, and multifocal PTK and alternatives to PTK. Masking agents like hyaluronate, methylcellulose, and dextran are recommended to help achieve a better outcome when ablating irregular corneal surfaces. Antifibrotic agents like mitomycin C reduce the chances of recurrence of the disease, apart from minimizing the postoperative scarring. Some of the complications include induced hyperopia and irregular astigmatism, haze, recurrence, and corneal thinning. However, earlier postoperative recovery, possibility of a repeat procedure, and ability to control the depth of ablation make PTK a promising, minimally invasive alternative to keratoplasty in cases with anterior corneal pathologies.

Corneal degenerations in central India: Clinical profile and visual impact

Corneal degenerations in central India: Clinical profile and visual impact - IJCEO- Print ISSN No: - 2395-1443 Online ISSN No:- 2395-1451 Article DOI No:- 10.18231/j.ijceo.2019.115, Indian Journal of Clinical and Experimental Ophthalmology-Indian J Clin Exp Ophthalmol

Association of spheroidal degeneration in pseudoexfoliation cases

Purpose: To correlate the association of pseudoexfoliation with spheroidal degeneration, and to identify the type and stage of spheroidal degeneration in pseudoexfoliation patients. Material and Methods: Total of 64 patients between the age group of 40 to 70 years, attending Ophthalmology out-patient department were included in this study. After taking informed consent, demographic details and detailed history were taken from these patients. Comprehensive ophthalmic examination, which included visual assessment by Snellens chart and anterior segment evaluation by slit lamp examination was done. Results: A total of 64 patients (41 male and 23 female), in the age group between 40 and 70years, with pseudoexfoliation were included. Out of the 64patientswith pseudoexfoliation who were screened in our outpatient department, 44(68.75%) patients were diagnosed to havespheroidal degeneration. Among these 28(43.75%) patients were having stage 1, 7(10.93%) patients were having stage 2, 5(7.81%) patients had stage 3 and, 4(6.25%) patients had stage 4 spheroidal degeneration. Conclusion: Pseudoexfoliation cases have higher incidence of spheroidal degeneration association as compared to general population. It was also observed that droplet type (stage 1) of spheroidal degeneration was commonly seen in pseudoexfoliation cases, and the pupillary margin was the common site for pseudoexfoliation material deposition in most of the cases. This poses challenges to measure intraocular pressure effectively, as well as increase the chances of complications when intraocular procedures are done even with experienced hands. Keywords: Cataract, Droplet type, Exfoliation material, Pseudoexfoliation, Phacodonesis, Spheroidal degeneration.

Prevalence of spheroidal degeneration of cornea and its association with other eye diseases in tribes of Western Rajasthan.

Purpose:To determine the prevalence of spheroidal degeneration of cornea (SDC) and its association with other eye diseases in the tribes of South-West Rajasthan.Methods:A total of 5012 patients were examined on slit lamp for the diagnosis of SDC. Diagnosis of SDC was made based on presence of amber granules in the superficial stroma of peripheral interpalpebral cornea with increasing opacification, coalescence and central spread or nodular and hazy surrounding stroma and divided in three stages.Results:The prevalence of SDC was 10.7%. Around 55% of the total of 535 cases examined were found to have Stage I followed by Stage II (32%) and Stage III (13%). The prevalence is greatest in both men and women over 70 years of age. The severity of SDC is greater in men. SDC was significantly associated with pterygium and pseudocapsular exfoliation.Conclusion:Extreme temperature, low humidity, dust, high wind, and microtrauma caused by sand particles are the probable etiologies for higher prevalence of this kind of degeneration in this region.

Delayed onset of congenital hereditary endothelial dystrophy due to compound heterozygous SLC4A11 mutations

Background:Congenital hereditary endothelial dystrophy (CHED) is an autosomal recessive disorder characterized by bilateral, symmetrical, noninflammatory corneal clouding (edema) present at birth or shortly thereafter. This study reports on an unusual delayed presentation of CHED with compound heterozygous SLC4A11 mutations.Materials and Methods:A 45-year-old female, presenting with bilateral decreased vision since childhood that deteriorated in the last 5 years, was evaluated to rule out trauma, viral illness, chemical injury, glaucoma, and corneal endothelial dystrophies. Tear sample was sent for herpes simplex viral (HSV) antigen testing. Genomic DNA from peripheral blood was screened for mutations in all exons of SLC4A11 by direct sequencing. Full-thickness penetrating keratoplasty was done and corneal button was sent for histopathological examination.Results:Slit-lamp findings revealed bilateral diffuse corneal edema and left eye spheroidal degeneration with scarring. Increased corneal thickness (762 μm and 854 μm in the right and left eyes, respectively), normal intraocular pressure (12 mmHg and 16 mmHg in the right and left eyes, respectively), inconclusive confocal scan, and specular microscopy, near normal tear film parameters, were the other clinical features. HSV-polymerase chain reaction was negative. Histopathological examination revealed markedly thickened Descemet's membrane with subepithelial spheroidal degeneration. SLC4A11 screening showed a novel variant p.Ser415Asn, reported mutation p.Cys386Arg and two polymorphisms, all in the heterozygous state and not identified in 100 controls.Conclusions:The study shows, for the first time, compound heterozygous SLC4A11 mutations impair protein function leading to delayed onset of the disease.

Climatic droplet keratopathy: an old disease in new clothes.

Climatic droplet keratopathy (CDK) is an acquired and potentially handicapping cornea degenerative disease that is highly prevalent in certain rural communities around the world. It predominantly affects males over their forties. It has many other names such as Bietti's band-shaped nodular dystrophy, Labrador keratopathy, spheroidal degeneration, chronic actinic keratopathy, oil droplet degeneration, elastoid degeneration and keratinoid corneal degeneration. CDK is characterized by the haziness and opalescence of the cornea's most anterior layers which go through three stages with increasing severity. Globular deposits of different sizes may be histopathologically observed under the corneal epithelium by means of light and electron microscopy. The coalescence and increased volume of these spherules may cause the disruption of Bowman's membrane and the elevation and thinning of the corneal epithelium. The exact aetiology and pathogenesis of CDK are unknown, but they are possibly multifactorial. The only treatment in CDK advanced cases is a corneal transplantation, which in different impoverished regions of the world is not an available option. Many years ago, the clinical and histological aspects of this disease were described in several articles. This review highlights new scientific evidence of the expanding knowledge on CDK's pathogenesis which will open the prospect for new therapeutic interventions.

Phototherapeutic Keratectomy for Treatment of Corneal Diseases : Our Experience at King Hussein Medical Center

phototherapeutic keratectomy for various corneal conditions were included in the study. Patients with any systemic disease that would affect epithelial healing such as diabetes or systemic lupus were excluded from the study. Data was collected including patient's age, gender, visual acuity, primary diagnosis, refraction, corneal thickness using Pentacam (Oculus, Wetzlar, Germany) and presence of any systemic disease. All patients were followed after one week of treatment. Bandage contact lens was removed and the cornea checked for epithelial healing and the patients were followed up for at least 6 months after treatment. Results: One hundred and forty-six eyes of 146 patients with a mean age of 57 years (range 7 to 74 years) were enrolled in the study, 87 of them were males. The most common type of corneal pathology found was band keratopathy (37%) followed by old superficial corneal scars (22%), other pathologies found included: Salzmann nodules (12%), bullous keratopathy (9%), spheroidal degeneration (6%), keratoconus with nebula (6%), granular dystrophy (3%), Reis Buckler dystrophy (3%) and recurrent corneal erosions syndrome (3%). Ninety percent of patients experienced improvement in their vision. All patients showed satisfactory improvement in either visual acuity or symptoms of pain and discomfort in the immediate post operative period. Conclusion: Phototherapeutic keratectomy is a safe and very effective procedure to be used for treatment of various surface corneal lesions.

Simple method of restoration of vision with amniotic membrane transplantation in spheroidal degeneration

Spheroidal degeneration is known to cause symptoms of pain, watering and foreign body sensation and visual deterioration. Management includes lubricants, contact lens, lamellar and penetrating keratoplasty and phototherapeutic keratectomy. They have limitations in the form of availability of tissue and equipment, cost and post-op care. Amniotic membrane transplant (AMT) has been proven effective for ocular surface reconstruction. Therefore this study was done to evaluate the efficacy of superficial keratectomy with AMT in spheroidal degeneration in terms of symptomatic relief and visual improvement. Twenty three eyes of 20 patients were included in the study after complete clinical examination. Superficial keratectomy along with amniotic membrane grafting was done in all cases. Patients were evaluated for symptomatic relief and visual improvement. Post operatively symptomatic relief of one or more symptoms was seen in all eyes. Pre-operative watering, foreign body sensation and irritation was relieved in 73.3%, 86.5% and 79.9% cases respectively. Visual improvement by more than two lines occurred in 21 of the 23 eyes. Corneal haze in the form of a nebular macular opacity was noted in five cases. None of our patients required a repeat procedure. Superficial keratectomy with amniotic membrane grafting is a cheaper and easily available treatment option for providing smooth ocular surface with symptomatic and visual improvement in spheroidal degeneration.

In vivo confocal microscopy of climatic droplet keratopathy

We describe the corneal microstructural changes in a patient with spheroidal degeneration using in vivo confocal microscopy. Multiple hypo‐ and hyper‐reflective spherical lesions were observed in the anterior corneal stroma and Bowman's layer ranging from 45 to 220 μm in size. The corneal epithelium, posterior stroma and endothelium were otherwise unaffected. In vivo confocal microscopy demonstrates good correlation with excised histological samples in climatic droplet keratopathy. It provides a non‐invasive technique to examine the living cornea for degenerative disease and acts as a bridge between clinical and laboratory observations.

Indications of Penetrating Keratoplasty in Southern India

To study the indications of penetrating keratoplasty in Southern India. Retrospective study. Retrospective evaluation of eye bank records from December, 2002 to December, 2012 with respect to indications for penetrating keratoplasty. During the period considered for study, 102 penetrating keratoplasties were performed. The leading indications for penetrating keratoplasty were corneal scarring (60.7%), followed by regrafting (12.7%), spheroidal degeneration (9.8%), aphakic bullous keratopathy (5.88%), pseudophakic bullous keratopathy (2.94%), acute infectious keratitis (2.94%), corneal dystrophy and keratoconus (4.9%). Healed infectious keratitis (72.88%) was the most common subcategory among the eyes with corneal scarring followed by traumatic corneal scars (16.12%). Corneal scarring from healed infectious keratitis are the most common indication for keratoplasty in Southern India.

Phototherapeutic keratectomy

Phototherapeutic keratectomy (PTK) is done regularly for anterior corneal diseases such as corneal dystrophies, corneal degenerations, scars, and band-shaped keratopathy. The various indications include both therapeutic and visual. The aim of this article is to discuss the therapeutic indications for PTK, the specific technique pertaining to a specific etiology, the various other procedures like amniotic membrane graft combined with PTK or PTK being done for recurrences in the grafts, and PTK done before cataract surgery when the anterior corneal pathology coexists with the cataract. Post PTK management such as healing of an epithelial defect, use of steroids in the post PTK period, recurrences of primary disease pathology, and infections, will be discussed. Methods of literature search: A Medline search was carried out for articles in the English language, with the keywords, phototherapeutic keratectomy, band-shaped keratopathy, spheroidal degeneration, scars, bullous keratopathy, and corneal dystrophy. The relevant references are mentioned here.