Main Ocular Manifestations Research Articles

Ocular involvement in autoimmune bullous diseases

Autoimmune bullous diseases represent a heterogenous group of disorders caused by autoantibodies against adhesion molecules; the location of the target protein determines the level of cleft formation. The spectrum of ocular lesions in autoimmune bullous diseases can range from mild symptoms to severe involvement with sight impairment and even, in some cases, blindness. In pemphigus vulgaris, the prevalence of ocular involvement has been reported to be between 7% and 26%. The most common clinical sign of ocular pemphigus vulgaris is bilateral conjunctivitis with hyperemia. Ocular involvement also occurs in 41% to 70% of patients with paraneoplastic pemphigus. The main ocular manifestations are bilateral cicatrizing conjunctivitis with symblepharon formation, and shortening of the fornices. In mucous membrane pemphigoid, ocular involvement is seen in 61% to 70% of patients; the most frequent ocular finding is cicatricial conjunctivitis. Patients with autoimmune bullous diseases having common ocular involvement should be assessed by an ophthalmologist to avoid serious complications. Diagnostic procedures and treatment require multidisciplinary care based on the close cooperation between dermatologists and ophthalmologists.

Ocular manifestations in psoriasis: a narrative bibliographic review / Manifestações oculares na psoríase: uma revisão bibliográfica narrativa

Introduction: Psoriasis is an immune-mediated, inflammatory and chronic disease, being considered systemic for the wide range of body systems it can affect. Ocular involvement is present in 10-12% of all psoriatic patients, but is often a neglected and underappreciated manifestation. Objective: This article aims to elucidate the main ocular manifestations related to psoriasis. Development: The research was based on a narrative bibliographic review of reports from the National Library of Medicine (NLM), the Scientific Electronic Library Online (Scielo), Google Scholars and Federal University of Bahia databases. As a result, the authors could describe ocular manifestations in psoriasis, related to specific eye sites: eyelids, conjunctiva, cornea, uvea, and lens- and their main symptoms. Uveitis, conjunctivitis, blepharitis, xerosi, and keratoconjunctivitis sicca can be highlighted as the most prevalent ocular symptoms. Final Considerations: Ocular manifestations in psoriatic patients are a prevalent site of involvement and may lead to ophthalmologic complications. To guarantee psoriatic patients a better prognosis and quality of life, it is of considerable importance to make a detailed investigation of ocular symptoms by both dermatologists and ophthalmologists in those patients.

Ocular manifestations of rheumatoid arthritis and autoantibodies positivity: a systematic review

Background: Ocular involvement represents about 40% of extra-articular manifestations of Rheumatoid Arthritis, pointing to impairment in patients’ quality of life. Anti-cyclic citrullinated peptide antibody and rheumatoid factor are serological markers to laboratorial diagnosis of disease. Objective: Identify main ocular manifestations of Rheumatoid Arthritis and its relationship between positivity of rheumatoid factor and anti-cyclic citrullinated peptide antibody, through a Systematic Review of literature. Methods: Systematic Review was conducted on database search, including: PubMed, Scopus, Web of Science and SciELO until January, 2018. Inclusion and exclusion criteria were applied and data of select studies were extracted and organized in tables and graphics. Methodological appraisal and statistical analysis, including meta-analyses, were performed. Selected studies were stored in software Endnote X8 student (Serial Number: 3151802521 e Product Key: L899B-8N8FJ-SX9JW-BEQ58-U9HCD). Statistical analyses were performed with Review Manager software version 5.3 (free software). Results: From 1,985 studies found by database search, four studies were selected and analyzed. Sicca syndrome and secondary Sjögren’s syndrome represented about 50% of ocular manifestations. Meta-analyses applied in two studies demonstrated no statistical significative risk association between anti-cyclic citrullinated peptide and development of ocular manifestations. Conclusion: From extra-articular manifestations of Rheumatoid Arthritis, ocular manifestations correspond to a significative amount. Nevertheless, there were no statistical significative risk association between autoantibodies and these manifestations.

Clinical manifestations of congenital rubella syndrome at the Korle-Bu Teaching Hospital, Ghana

Background: Congenital rubella syndrome (CRS) is a variable constellation of birth defects related to intrauterine rubella infection which may result in visual, hearing, intellectual and cardiac impairments. Objective: The objective of this study is to describe the clinical manifestations in patients presenting with CRS at the Korle Bu Teaching Hospital using the World Health Organization criteria. Methods: A retrospective analysis of the medical charts of 16 children under 16 years old who presented with CRS at the Lions International Eye and the National Cardiothoracic Centres of the Korle Bu Teaching Hospital from 2012 to 2015, was done. Ocular, cardiac and other systemic clinical and laboratory findings were documented for each patient using predesigned forms. Results: Sixteen cases of CRS were recorded over the period (2012-2015) comprising 9 males and 7 females. The median (interquartile range) age at diagnosis was 4.0 months (2.0-7.0 months), age range at diagnosis was 1.0-24.0 months. The main ocular manifestations were; cataract 10 (62.5 %), microcornea 10 (62.5 %), microphthalmia 9 (56.3 %), nystagmus 7 (43.8 %) and strabismus 4 (25.0 %). The main cardiac defects included patent ductus arteriosus 12 (75.0 %), pulmonary stenosis 5 (31.3 %), ventricular septal defect 5 (31.3 %) and small branch pulmonary artery5 (31.3 %). Systemic conditions recorded included hearing defects 7 (43.8 %), microcephaly 7 (43.8 %), failure to thrive 5 (31.3 %) and mental retardation 2 (12.5 %). Conclusion: Cataract, microcornea and microphthalmia were the main ocular manifestations in children presenting with CRS at the Korle Bu Teaching Hospital. Patent ductus arteriosus and hearing defects were the main cardiac and systemic defects among children presenting with CRS at the Korle Bu Teaching Hospital.

Findings and Graduation of Sarcoidosis-Related Uveitis: A Single-Center Study.

Ocular involvement is present in up to 79% of sarcoid patients. Uveitis is the main ocular manifestation and presents as a chronic intraocular inflammatory condition with potentially detrimental effects on visual acuity and quality of life. This retrospective study was conducted to explore the incidence and characteristics of ocular sarcoidosis in a single tertiary ophthalmology center. Medical records of 84 patients presenting between June 2007 and March 2021 were analyzed. Based on the “International Workshop on Ocular Sarcoidosis” (IWOS) criteria, ocular sarcoidosis was determined as: definite (n = 24; 28.6%), presumed (n = 33; 39.3%), probable (n = 10; 11.9%), and indefinite (n = 17; 20.2%) in our study population. In 43.9% of the definite and presumed cases, the eye was primarily affected. In addition to specific ocular findings, the diagnosis was supported by biopsy (28.6%) and chest x-ray or computer tomography (66.7%). Moreover, an increased soluble interleukin-2 receptor (sIL-2R) expression (76.2%), elevated angiotensin-converting enzyme (ACE) levels (34.8%), and lymphocytopenia (35.1%) were valuable laboratory findings. Co-affected organs were lungs (60.7%), skin (15.5%), and central nervous system (8.3%). Our findings support the prominent role of the eye in the early detection of sarcoidosis. In addition to the IWOS criteria, sIL-2R, in particular, was shown to be relevant in establishing the diagnosis.

Structural and microvascular changes of the peripapillary retinal nerve fiber layer in Von Hippel\u2013Lindau disease: an OCT and OCT angiography study

Von Hippel–Lindau (VHL) disease is an autosomal dominant genetic disease caused by VHL gene mutation. Retinal hemangioblastomas (RH) are vascularized tumors and represent the main ocular manifestation of the disease. Histopathologically, RH are composed of capillary vessels and stromal cells, the neoplastic population of the lesion. The origin of these stromal cells remains controversial, even if they are hypothesized to be glial cells. The aim of the present study was to investigate neuronal and microvascular changes of the peripapillary retinal nerve fiber layer, in which glial cells, neurons and capillaries (the radial peripapillary capillary plexus) interact. VHL patients with or without peripheral RH were enrolled and compared to healthy controls. Mean peripapillary retinal nerve fiber layer (pRNFL) thickness was measured by means of optical coherence tomography (OCT). The following vascular parameters of the radial peripapillary capillary plexus were quantified using OCT angiography: Vessel Area Density,Vessel Length Fraction, Vessel Diameter Index and Fractal Dimension. One hundred and nine eyes of 61 patients, and 56 eyes of 28 controls were consecutively studied. Mean pRNFL was significantly thinner in VHL eyes without RH versus eyes with RH and controls. Mean pRNFL thickness did not differ between VHL eyes with RH and controls. All OCTA vascular parameters were reduced in VHL eyes with or without RH versus controls, with significative difference for Vessel Diameter Index. The same OCTA parameters did not significantly differ between VHL eyes with or without RH. In VHL eyes without RH, pRNFL thinning may be the consequence of impaired perfusion of the radial peripapillary capillary plexus, while the increase of pRNFL thickness in VHL eyes with RH may depend on possible activation and proliferation of the other RNFL resident cells, the glial cells.

Examination of corneal deposits in nephropathic cystinosis using in vivo confocal microscopy and anterior segment optical coherence tomography: an age-dependent cross sectional study

BackgroundPresence of corneal cystine crystals is the main ocular manifestation of cystinosis, although controversial findings concerning the corneal layer with the highest density have been reported. The aim of this study was the analysis of the characteristics of crystal arrangement in different corneal layers and the assessment of corneal morphological changes with age.MethodsA cross sectional study was carried out in three children and three adults who had nephropathic cystinosis and corneal cystine depositions. All patients underwent a comprehensive ophthalmological examination including best corrected distance visual acuity, slit-lamp examination, in vivo confocal microscopy and anterior segment optical coherence tomography. An evaluation of the depth of crystal deposits and crystal density in different corneal layers was also performed. Due to the low number of subjects no statistical comparison was performed.ResultsAnterior segment optical coherence tomography images revealed deposition of hyperreflective crystals from limbus to limbus in each patient. Crystals appeared as randomly oriented hyperreflective, elongated structures on in vivo confocal microscopy images in all corneal layers except the endothelium. In children the deposits occurred predominantly in the anterior stroma, while in adults, the crystals were mostly localized in the posterior corneal stroma with the depth of crystal deposition showing an increasing tendency with age (mean depth of crystal density was 353.17 ± 49.23 μm in children and it was 555.75 ± 25.27 μm in adults). Mean crystal density of the epithelium was 1.47 ± 1.17 (median: 1.5; interquartile range: 0.3–2.4). Mean crystal density of the anterior and posterior stroma of children and adults was 3.37 ± 0.34 (median: 3.4; interquartile range: 3.25–3.55) vs. 1.23 ± 0.23 (median: 1.2; interquartile range: 1.05–1.35) and 0.76 ± 0.49 (median: 0.7; interquartile range: 0.4–1.15) vs. 3.63 ± 0.29 (median: 3.7; interquartile range: 3.45–3.8), respectively. Endothelium had intact structure in all cases. Some hexagonal crystals were observed in two subjects.ConclusionsIn vivo confocal microscopy and anterior segment optical coherence tomography confirmed an age-related pattern of crystal deposition. In children, crystals tend to locate anteriorly, while in adults, deposits are found posteriorly in corneal stroma.

A FBN1 variant manifesting as non-syndromic ectopia lentis with retinal detachment: clinical and genetic characteristics

Background/objectivesFibrillin-1 (FBN1) mutations cause connective tissue dysgenesis the main ocular manifestation being ectopia lentis (EL), which may be syndromic or non-syndromic. We describe a pedigree with a FBN1 mutation causing non-syndromic EL with retinal detachment (RRD) and their management.Subjects/methodsPatients with familial EL with RRD were invited to participate (vitreoretinopathy branch of Target 5000, the Irish inherited retinal degeneration study). All patients signed full informed consent. The study was approved by the Institutional Review Board of the Mater Hospital, Dublin and abided by the Declaration of Helsinki.ResultsSeven adults were affected with bilateral EL. All subjects had RRD with bilateral non-synchronous RRD in 57%.ConclusionsThe FBN1 variant described herein confers an increased risk of both EL and RRD and can now be upgraded to ‘pathogenic’ ACMG status.

The ocular involvement in the transthyretin-related familial amyloid polyneuropathy

Transthyretin (TTR)-related familial amyloid polyneuropathy (FAP), which is caused by mutant TTR, is a rare but fatal autosomal dominant disease. TTR is synthesized by the liver (95%) , the choroid plexus of the brain and the retinal pigment epithelium. FAP leads to peripheral neuropathy, and the main ocular manifestations are vitreous opacity (yellowish cotton-like), secondary glaucoma and keratoconjunctivitis sicca. Liver transplantation has proven to be the most effective treatment for TTR-FAP. Nowadays, tafamidis is the only drug approved for TTR-FAP (early stage). However, neither liver transplantation nor tafamidis is capable to halt the progression of ocular involvement. Panretinal photocoagulation could damage the retinal pigment epithelium, and thus prevent the progression. Recent investigations on TTR-FAP and its ocular involvement are reviewed in this article. (Chin J Ophthalmol, 2017, 53: 783-785).

Ocular Manifestations and Therapeutic Options in Patients with Familial Amyloid Polyneuropathy: A Systematic Review.

Purpose. This paper aims to review the morphological and functional characteristics of patients affected by familial amyloid polyneuropathy (FAP), with greater focus on type I and its progression after liver transplantation. We also analyse therapeutic options for the ophthalmic manifestations. Methods. The literature from 2002 through 2015 was reviewed, with a total of 45 articles studied, using the key terms related to amyloidosis and its therapeutic approaches. Information was collated, evaluated, critically assessed, and then summarised in its present form. Pathophysiology and Treatment. FAP results from mutation of the transthyretin gene, with Val30Met being the most frequent substitution. The symptoms are those typical of a sensorimotor autonomic neuropathy and can be halted with liver transplantation. Nowadays there are new medical therapies that delay the progression of the systemic neuropathy. However, there are still no options to avoid ocular disease. Conclusion. The main ocular manifestations in patients with FAP type I are amyloid deposition in the vitreous, dry eye, and secondary glaucoma. Despite liver transplantation, eye synthesis of amyloid persists and is associated with progressive ocular manifestations, which require continued ophthalmologic follow-up. New therapeutic strategies are therefore needed, particularly to target the ocular synthesis of the abnormal protein.

Enhanced depth imaging optical coherence tomography findings associated with serous retinal detachment in preeclampsia

Preeclampsia is characterized by hypertension, proteinuria, and edema occurring in the second half of pregnancy. The main ocular manifestations are related to dysfunction of both retinal and choroidal circulation [1, 2]. Similarly to hypertensive retinopathy, cotton wool spots, hemorrhages, edema, papilloedema, serous retinal detachment and lesions of the retinal pigment epithelium (RPE) may develop in preeclampsia. Fluorescein angiography shows areas of choroidal hypoand nonperfusion (Elschnig spots) in preeclamptic women with serous retinal detachment [3, 4]. It has been proposed that intense spasm of the choroidal arterioles may be responsible for choroidal ischemia, resulting in increased vascular permeability, and subsequently, accumulation of serous fluid in the subretinal space [4]. We investigate the changes in the outer retina and choroid by means of enhanced depth imaging (EDI) [5] spectral-domain optical coherence tomography (SD-OCT) in a preeclamptic women with serous retinal detachment. A 38-year-old woman developed hypertension (190/ 110 mmHg), proteinuria, and generalized edema in the 35th week of pregnancy. Two days after undergoing a cesarean section, the patient was referred to our department for impairment of vision in both eyes. Best-corrected visual acuity was 20/25 in the right eye (RE) and 20/80 in the left eye (LE). Fundus biomicroscopy showed a peripapillary serous retinal detachment bilaterally, with involvement of the LE macula. Blue fundus autofluorescence (b-FAF) showed inhomogeneous increased autofluorescence in the peripapillary area, while near-infrared fundus autofluorescence (nirFAF) revealed peripapillary areas of hypoautofluorescence together with focal points of intensely decreased nir-FAF (Fig. 1). EDI SD-OCT scans through the areas of abnormal autofluorescence and the macula showed peripapillary serous retinal detachment bilaterally with focal RPE elevations, and serous macular detachment in the LE (Fig. 2). Choroidal thickness in the scan passing through the macula was 475 lm RE, 361 lm LE subfoveally, and 263 lm RE, 367 lm LE in the peripapillary area (Fig. 2) (increased compared with reference values) [5]. Two weeks later, BCVA improved to 20/20 in both eyes, and fundus biomicroscopy revealed disappearance of serous retinal detachment. b-FAF still showed increased autofluorescence in the peripapillary area, while nir-FAF revealed almost complete disappearance of both peripapillary areas of hypoautofluorescence and focal points of decreased nir-FAF (Fig. 1). EDI SD-OCT scans confirmed the disappearance of serous retinal detachment in both eyes, and showed persistence of focal RPE elevations (Fig. 2). Choroidal thickness in the scan passing through the macula was unchanged subfoveally, while decreased to 186 lm RE, 284 lm LE in the peripapillary area (Fig. 2). Previous reports have documented, by time domain OCT, the presence of serous retinal detachment in preeclamptic women [6]. To the best of our knowledge this is the first case study to describe the SD-OCT features of serous retinal detachment in preeclampsia. SD-OCT revealed focal RPE elevations in the peripapillary areas of abnormal FAF (both b-FAF and nir-FAF) suggesting compromised fluid transport (choroid-RPE-retina) in the acute phase of the disease, which persisted also after the resolution of both serous retinal detachment and focal L. Querques G. Querques (&) F. Loperfido R. Lattanzio F. Bandello Department of Ophthalmology, University Vita Salute San Raffaele, Via Olgettina 60, 20132 Milan, Italy e-mail: giuseppe.querques@hotmail.it

Hepatitis C and interferon-associated retinopathy: A case report

Background Hepatitis C is a common virus affecting approximately 170 million people worldwide. The main ocular manifestations of hepatitis C virus include a Sjögren-type of dry eye syndrome and an ischemic retinopathy secondary to treatment with interferon or a result of a systemic vasculitis induced by the infection itself. Current treatment for hepatitis C is a combination therapy using pegylated (long-lasting) interferon and ribavirin. This treatment, however, is not without ocular sequelae. Interferon-associated retinopathy is present in > 50% of patients taking the drug and is mainly characterized by cotton wool spots and retinal hemorrhages. The pathogenesis of this disorder is not specifically known. Fortunately, many patients remain asymptomatic, and the retinal changes are usually reversible. Case Report A 48-year-old black man presented to the clinic complaining of decreased vision in both eyes for the previous few weeks. His medical history was positive for hypertension and hepatitis C for which he was being treated. At his examination, he had interferon retinopathy diagnosed. Soon thereafter, his treatment for hepatitis C was discontinued, and when he returned for follow-up 4 months later, his retinal findings had resolved. Conclusions An overview of hepatitis C and its recommended therapy and possible ocular side effects are reviewed, as well as the differential diagnosis of interferon-associated retinopathy, 1 of the main side effects of treatment.

Uveíte anterior na ausência de esclerite em paciente com artrite reumatóide: relato de caso

Rheumatoid arthritis is the most common collagenosis and affects almost 0.6% of Brazilian population. It is an important cause of articular deformities. The main ocular manifestation of rheumatoid arthritis is dry eyes (secondary Sjögren's syndrome), followed by scleritis, peripheral ulcerative keratitis and uveitis. The aim of this paper is to present a case of anterior uveitis in the absence of scleritis in a patient with rheumatoid arthritis, a very rare presentation in this type of patient. Female patient, 55 years old, with rheumatoid arthritis, presenting suddenly ocular pain and low vision in the right eye. Her exam showed anterior chamber reaction with hypopion, peripheral corneal keratitis and intraocular pressure of 32 mmHg. She was diagnosed with hypertensive anterior uveitis and peripheral corneal keratitis and treated with systemic and topical corticosteroids, topical antibiotic, topic and systemic ocular hypotensive and mydriatic drops. Anterior uveitis is common in rheumatological diseases, especially in those seronegative arthropathies related to HLA B27. In this paper we present a patient with rheumathoid arthritis and anterior uveitis in the absence of scleritis, a rare presentation in actual medical literature.

Conventional Therapy of Sjogren’s Syndrome

Sjogren's syndrome (SS) is a chronic autoimmune disorder affecting mainly middle-aged women. It is characterized by lymphocytic infiltration and destruction of the exocrine glands (mainly the salivary and lacrimal glands), resulting in dry mouth and eyes. Symptoms of SS are chronic and sometimes devastating, compromising the quality of life at a major extent. Despite its autoimmune nature, evidence for the use of immunosuppressive agents, which are the mainstay of therapy of diseases of autoimmune origin, is limited. Keratoconjunctivitis sicca (KCS), the main ocular manifestation of SS, is managed with tear substitutes, as well as local and systemic stimulators of tear secretion and supportive surgical procedures. Management of oral manifestations includes intense oral hygiene, prevention and treatment of oral infections, use of saliva substitutes, and local and systematic stimulation of salivary secretion. Cholinergic agents, such as pilocarpine and cevimeline are the cornerstone of current therapy in SS. Corticosteroids, cyclophoshamide, and nucleoside analogues are reserved for severe extraglandular manifestations of SS. The role of anti-B-cell therapy is a promising option for glandular and extraglandular manifestations of the disease, as well as for the management of SS-associated lymphoma.

Ocular Involvement Associated With an Epidemic Outbreak of Chikungunya Virus Infection

To study the range of ocular symptoms in a cohort of patients with chikungunya infection. Retrospective, observational case series. Patients attending a tertiary eye care hospital in South India were included in the study. We included adult patients with serologically confirmed chikungunya virus infection who received clinical care at the Aravind Eye Hospital, Madurai, South India. They were assessed for demographic characteristics, ocular symptoms, laboratory parameters, and chikungunya virus infection severity. Patients underwent a complete ophthalmologic examination that included visual acuity, slit-lamp examination, and indirect funduscopic examination. Visual outcome at the end of three months was the main outcome measure. The charts of 37 patients were analyzed based on the clinical picture and the serologic results. Forty patients were included as controls and tested negative. There were 21 males and 16 females with a mean age of 44.17 years. The main ocular symptoms included granulomatous and nongranulomatous anterior uveitis, optic neuritis retrobulbar neuritis, and dendritic lesions. Of the 26 patients who were followed up for three months, the visual acuity improved in 11 patients (42.3%), remained the same in 12 patients (46.15%), and worsened in three patients (11.5%). The main ocular manifestation associated with the recent epidemic outbreak of chikungunya virus infection in South India included granulomatous and nongranulomatous anterior uveitis, optic neuritis, retrobulbar neuritis, and dendritic lesions. The visual prognosis generally was good, with most patients recovering good vision. Further studies are needed to understand the pathogenesis of this disease.

Uvéites et maladies inflammatoires chroniques de l’intestin : à propos de 3 cas

The most frequent inflammatory chronic bowel diseases are Crohn disease and ulcerative colitis, which present extra intestinal manifestations, particularly, arthritis and ocular inflammation. Anterior uveitis is the main ocular manifestation and remains exclusively an inflammatory process. [corrected] We report three cases of bilateral uveitis, with ulcerative colitis in two cases and Crohn disease in the last case. In the two first cases, uveitis occurred after 8 years of bowel disease which was stabilized by sulfasalazopyridine and 6 months after bowel surgery. In the third case, the patient was followed for Crohn disease for 3 years with anterior uveitis relapse concomitant to bowel relapse. In the two first cases, there was no recurrence after corticosteroids topical therapy. In the third case, topical and systemic corticosteroids provided adequate control of ocular inflammation, but the follow-up was marked by pupillary and inflammatory glaucoma, which required gonio-surgery. Anterior uveitis is the most frequent ocular manifestation in chronic inflammatory bowel disease: approximately 1.9-4.9% in Crohn disease and 1.6% in ulcerative colitis. The uveitis course is synchronous with bowel relapse and its treatment requires anti-inflammatory agents. Nevertheless, in severe relapse, recovery is obtained after the resection of the entire involved bowel segment.

Troubles oculaires de la myasthénie

Ocular disorders due to myasthenia gravis are frequent; they often represent the first manifestation of this autoimmune disease. Sometimes, they are the sole functional sign. Ptosis and diplopia are the main ocular manifestations. They result from a dysfunction in the neuromuscular junctions of extra-ocular skeletal and palpebral muscles. The frequent involvement of extra-ocular muscles is subject to different pathophysiological hypotheses. The diagnosis is mainly clinical; fluctuations, worsening of symptoms with fatigue, absence of neurological systematisation are characteristic. Diagnosing may be difficult in those cases with only ocular manifestations. Any ocular motility disturbance may be duplicated by ocular myasthenia gravis. Diagnosis tests are inconstantly positive and their negativity does not rule out diagnosis. Ocular myasthenia gravis can cause visual disability and dysfunction in daily living activities. Treatment is symptomatic. Optical devices must be proposed but they are often partly effective. Acetylcholinesterase inhibitors are the first line medical treatment. Corticosteroids in isolation or combined with other immunosuppressive therapies are indicated in case of failure of the previous therapy. Indications and modalities of corticosteroids for the prevention of disease generalization remain to be studied.

Análise do custo-benefício da avaliação ocular de pacientes portadores de rosácea

To observe the main ocular manifestations of patients with rosacea and to evaluate the cost-benefit of supplementary diagnostic examinations. Twenty patients with acne rosacea from the Dermatology Service of the São Paulo Federal University (UNIFESP) were interviewed through a questionnaire, submitted to ophthalmologic examination and tested with Schirmer I, fluorescein and rose Bengal staining, impression cytology and conjunctival scrub. The costs of these examinations were abstracted from the Public Health System's values (SUS), Brazilian Medical Association values (AMB) and values charged by a private service. Twenty patients were enrolled initially in the study, but only 16 completed it. Eleven patients were female (55%), 19 were white (95%) and the average age was 56 years. All patients had at least one complaint related to rosacea. The most common symptoms were itching (40%), tearing (35%), foreign body sensation (30%). The most common signs were telangiectasis of lid margins (70%), hyperemia of lid margins (70%), meibomitis (65%) and blepharitis (50%). Most subsidiary examination showed normal results. There was difference of costs between the different paying sources. The main ocular manifestations in rosacea were related with meibomian gland disease. Considering the subsidiary examination costs and their results, the ophthalmologic check-up presents the best cost-benefit for the patient with rosacea.

Ocular manifestations of keratitis– ichthyosis–deafness (KID) syndrome

Keratitis-ichthyosis-deafness (KID) syndrome is a rare congenital ectodermal dysplasia characterized by the association of hyperkeratotic skin lesions, moderate to profound sensorineural hearing loss and vascularizing keratitis. Mutations in the GJB2 gene coding for connexin 26, a component of gap junctions in epithelial cells, have been observed in several KID patients. Variable ocular manifestations of the disease in 3 patients with molecular genetically confirmed KID syndrome are reported. Retrospective case series. Clinical examination and molecular genetic analysis for mutations in the GJB2 gene were performed in 3 patients with KID syndrome ages 5, 13, and 41 years. Visual acuity ranged from normal to severe visual loss. The ocular signs included loss of eyebrows and lashes, thickened and keratinized lids, trichiasis, recurrent corneal epithelial defects, superficial and deep corneal stromal vascularization with scarring, keratoconjunctivitis sicca, and, in one patient, presumed limbal insufficiency. Whereas ocular surface integrity could be maintained with artificial tears in one patient, and an epithelial defect healed under conservative treatment in the second patient, multiple surgical procedures including superficial keratectomies, limbal allograft transplantation with systemic immunosuppression, amniotic membrane transplantation, lateral tarsorrhaphies, and lamellar keratoplasty could not preserve useful vision in the third patient. KID syndrome may affect the ocular adnexae and surface with variable severity independent of the age of the patient. Lid abnormalities, corneal surface instability, limbal stem cell deficiency with resulting corneal complications, and dry eye are the main ocular manifestations.

HIV seroprevalence in ophthalmologic patients of Cameroon

It is difficult to estimate the exact HIV infection rates in countries such as Cameroon because of diagnostic and statistical problems. The majority of people seek help from traditional healers outside the health system. A screening for human immunodeficiency virus type 1 (HIV-1) and type 2 (HIV-2) was performed on 2452 patients of the western province of Cameroon. All data were evaluated regarding HIV seroprevalence and ophthalmological findings in HIV-seropositive patients. The test covered all patients who came for cataract surgery (group 1), all outpatients with suspicious ophthalmological findings (group 2), and all remarkable patients of the collaborating department of general medicine (group 3) between 20 September 2000 and 20 September 2001. Of the 2452 screened patients, 467 (19.0%) were HIV seropositive. A positive test result was obtained in 29 (5.5%) of the 525 patients in group 1, 154 (35.6%) of the 433 patients in group 2, and 284 (19.0%) of the 1494 patients in group 3. The main ocular manifestations of the 154 HIV-seropositive patients in group 2 were uveitis (17.6%), squamous cell carcinoma of the conjunctiva (14.9%), zoster ophthalmicus (14.9%), and corneal ulcers (11.0%). This study shows that the seroprevalence of the screened population of Cameroon lies between 5.5% (results of group 1) and 19.0% (results of group 3).