Cavernous hemangioma of the retina and retinal telangiectasis. Distinct or related vascular malformations?

Cavernous hemangioma of the retina and retinal telangiectasis are two rare developmental vascular lesions of the retina. They are considered to be distinct clinical entities, but they share some common features, so that the differential diagnosis of the two diseases is an important clinical challenge. In the right eye of a young woman, a cavernous hemangioma of the retina close to an area of retinal telangiectasis was found. This association supports the possibility that these developmental anomalies could be strictly related.     

伴眼部症状的颅内静脉窦血栓形成眼科漏诊、误诊临床分析

目的 分析伴眼部症状的颅内静脉窦血栓形成(cranial venous sinus thrombosis,CVST)眼科漏诊、误诊情况,眼科临床特点及CVST确诊方法.方法 回顾性分析19例伴眼部症状的眼科漏诊、误诊CVST患者的眼科临床特点及影像学检查结果.结果 19例伴眼部表现的CVST患者均伴双侧视盘水肿.所有患者均伴有颅内压增高,13例高于300 mmH2O,双眼视力下降18例,双眼复视7例,一过性黑矇1例.其中,10例误诊为双眼视盘炎,5例误诊为双侧视盘血管炎,误诊为前部缺血性视神经病变、Vogt-小柳原田综合征伴外展神经麻痹、Leber遗传性视神经病变、双侧外展神经麻痹各1例.19例患者中,经数字减影血管造影确诊12例,经磁共振(magnetic resonance imaging,MRI)+磁共振血管成像确诊6例,经MRI确诊1例.结论 伴视力下降、复视、视盘水肿等眼部表现的CVST易被漏诊或误诊为其他眼科疾病.MRI+磁共振血管成像可做为确诊CVST首选检查方法,数字减影血管造影确诊CYST更为准确.眼科医师遇到伴双侧视盘水肿的患者应考虑到CVST的可能.     

Distensible venous malformations of the orbit: clinical and hemodynamic features and a new technique of management.

OBJECTIVE: To investigate distensible venous malformations of the orbit (DVMO) as part of a spectrum of orbital vascular malformations, including some that involved periorbital skin, extraorbital sites (central nervous system or nasal sinuses), or combinations of these. The authors also investigated the effectiveness of a new technique of management for selected cases. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Thirty patients had distensible venous anomalies, of which four were combined distensible venous-lymphatic vascular malformations. Distensible lesions were defined as those showing clinical or radiographic expansion with Valsalva maneuver or when the head was placed in a dependent position. These lesions were then classified as superficial (anterior to the equator of the globe), deep (posterior to the globe's equator), combined (deep and superficial), or complex (with intracranial or major extraorbital involvement). INTERVENTION: Surgery was performed on 15 patients (50%), mainly for pain or for cosmetic indications. Six patients underwent this new technique, which involved intraoperative direct venography with control of outflow via pressure at the superior or inferior orbital fissure. The venous malformation was then embolized (by use of cyanoacrylate glue) and excised. RESULTS: The mean age at presentation was 28.2 years (range, 8 months to 75 years). Sixty-six percent of cases involved the left orbit. Superior and medial orbital involvement was most common. Three cases (10%) were classified as superficial, and 13 (43%) as deep. Six patients (20%) had combined superficial and deep components. Eight (27%) had major extraorbital involvement (4 intracranial, 2 facial, and 2 paranasal sinus). Direct venography demonstrated complex multichannel anomalies draining to various sites, including the face and pterygopalatine fossa, without necessarily having a direct connection to the major orbital venous circulation. CONCLUSIONS: Distensible venous malformations of the orbit are part of a spectrum of developmental venous malformations that may be localized to the orbit or involve it as part of a more extensive lesion. The authors describe their clinical and radiologic features and report a new technique of management for selected cases. This method of vascular isolation and embolization of lesions may greatly facilitate excision.     

The predictive value of subjective symptoms and clinical signs for the presence of treatment‐requiring exudative age‐related macular degeneration

Purpose: The introduction of vascular endothelial growth factor inhibitors for the treatment of exudative age‐related macular degeneration (AMD) has increased the referral rates of AMD patients with visual symptoms to treating centres considerably. However, a large proportion of the referred patients do not qualify for treatment implying that considerable resources could be saved if these patients could be identified on the basis of the clinical data available in the referring nonspecialized setting. Methods: A prospective observational study of 1682 consecutive patients referred with suspicion of exudative AMD qualifying for intravitreal angiostatic treatment. On the basis of the structured interviewing about symptoms, ophthalmoscopy, optical coherence tomography scanning, and fluorescein angiography, the patients were divided into two groups: one qualifying for and another not qualifying for treatment. Multiple logistic regression was used to identify independent parameters predicting the need for treatment. Results: The presence of metamorphopsia, dyschromatopsia, retinal haemorrhages and exudates, central retinal thickness, and the absence of micropsia were highly significant individual determinants of treatment‐requiring AMD. Sudden onset and worsening of symptoms and the presence of a central dark spot covaried with the occurrence of retinal haemorrhages, whereas reduced visual acuity and blurred vision covaried with the presence of both haemorrhages and exudates. Conclusion: Patients with treatment‐requiring AMD can be reliably identified by questioning about the presence of metamorphopsia and dyschromatopsia and the absence of micropsia, combined with ophthalmoscopical detection of retinal haemorrhages and exudates. This information may improve the triage of patients considered for referral.     

Correlations between commonly used objective signs and symptoms for the diagnosis of dry eye disease: clinical implications

Purpose: To evaluate the relationship between signs and symptoms of dry eye disease (DED) in a clinic‐based population. Methods: In a retrospective analysis, clinical signs and symptoms were evaluated for 344 subjects (n = 82, normal; n = 263, dry eye), across 11 sites from the EU and United States. Pearson correlations between signs and symptoms (r²) and an independent components analysis (ICA) mixing matrix were derived from the data set. Similar analysis was performed on an independent data set from 200 subjects in a previous study in Munich, Germany. Results: No correlations above r² = 0.17 were found between any signs and symptoms, except for corneal and conjunctival staining, which reported an r² = 0.36. In the multisite study, the average r² for osmolarity (0.07), tear breakup time (0.12), Schirmer test (0.09), corneal (0.16) and conjunctival staining (0.17), meibomian grading (0.11) and Ocular Surface Disease Index^® (0.11) were consistently low. Among patients who showed evidence of DED by consensus of clinical signs, only 57% reported symptoms consistent with a diagnosis of DED. Similar results were observed in the Munich‐based study data set. Each component of the ICA mixing matrix exhibited minimal residual information. Conclusions: No consistent relationship was found between common signs and symptoms of DED. Each type of measurement provides distinct information about the condition of the ocular surface. These results also demonstrate that symptoms alone are insufficient for the diagnosis and management of DED and argue for a consensus of clinical signs that better reflect all aspects of the disease.     

Tyrosinemia type II: nine cases of ocular signs and symptoms

PURPOSE: To report nine cases of tyrosinemia type II, with ocular signs and symptoms. METHODS: Participants included nine patients (18 eyes) who were followed for a mean follow-up period of 6.5 years (range, 2 to 8 years). Intervention included dietary restriction of tyrosine and phenylalanine, which led to resolution of ocular and cutaneous lesions, improved behavior in one patient, and may have prevented developmental delay in others. The main outcome measures were visual acuity and serum tyrosine levels to determine the response to dietary therapy. Intelligence testing and developmental screening were performed when appropriate. RESULTS: All patients presented with ocular signs and symptoms as the primary manifestation of disease and serve to illustrate the typical ocular findings of this syndrome. Three patients presented with cutaneous manifestations, and one patient had mild mental impairment at the time of diagnosis. CONCLUSIONS: Recalcitrant pseudodendritic keratitis may be the presenting sign in tyrosinemia type II. Awareness of the presenting signs and symptoms may accelerate the diagnosis and dietary intervention. Initiation of a tyrosine-restricted and phenylalanine-restricted diet in infancy is most effective in preventing cognitive impairment.     

Three cases of isolated mucoceles and one case of an Aspergillus infection in the sphenoid sinus with orbital signs and symptoms

Four patients with an isolated sphenoid lesion: three with a mucocele and one with an Aspergillus infection of the sphenoid sinus in whom the orbital impairment had a rhinogenic cause, are described. They all presented with one or more of the following features: visual loss, motility disturbances, visual field defects, and sensitivity loss of the infraorbital nerve. In one patient, optic disc edema was seen. Diagnostic imaging technologies such as computer tomography (CT) and magnetic resonance (MRI) make an early diagnosis possible but nevertheless, very large mucoceles with major functional loss were detected at a late stage of the disease. The Aspergillus infection was detected at an early stage without delay because of its specific appearance on CT and MRI. The signs and symptoms of these lesions are more striking in the orbital and neuro-ophthalmological field than in the ear-nose-throat (ENT) field, but nevertheless, primary treatment should be marsupialization by an ENT-surgeon.     

Three cases of isolated mucoceles and one case of an Aspergillus infection in the sphenoid sinus with orbital signs and symptoms

Four patients with an isolated sphenoid lesion: three with a mucocele and one with an Aspergillus infection of the sphenoid sinus in whom the orbital impairment had a rhinogenic cause, are described. They all presented with one or more of the following features: visual loss, motility disturbances, visual field defects, and sensitivity loss of the infraorbital nerve. In one patient, optic disc edema was seen. Diagnostic imaging technologies such as computer tomography (CT) and magnetic resonance (MRI) make an early diagnosis possible but nevertheless, very large mucoceles with major functional loss were detected at a late stage of the disease. The Aspergillus infection was detected at an early stage without delay because of its specific appearance on CT and MRI. The signs and symptoms of these lesions are more striking in the orbital and neuro-ophthalmological field than in the ear-nose-throat (ENT) field, but nevertheless, primary treatment should be marsupialization by an ENT-surgeon.     

Dural arteriovenous fistula of the sigmoid sinus with a clinical expression on the opposite eye: A case report

We report the case of an 87-year-old patient who developed a dural arteriovenous fistula of the sigmoid sinus with progressive exophthalmia in the opposite eye. The patient had a unilateral decrease in visual acuity; the vessels of the conjunctiva were dilated, and a progressive central retinal vein occlusion occurred in the left eye. A dural carotid cavernous fistula of the right sigmoid sinus was diagnosed with an angiography exam and was successfully treated with embolization of the arteriovenous shunt using Onyx(?). A central partial facial nerve paralysis occurred after embolization and spontaneously resolved in 6 months. Six months after the treatment, visual acuity improved to 7/10 and the exophthalmia and central retinal vein occlusion signs regressed. Dural arteriovenous fistulae are abnormal shunts between a collateral vessel of the carotid artery and the cavernous sinus; the unilateral pulsating exophthalmia is the most frequent associated symptom. Cerebral MRI is useful to analyze indirect signs of the arteriovenous shunt showing an abnormal dilatation of the veins, but angiography of the carotid artery is essential to confirm the diagnosis. Furthermore, it allows treatment of the fistula at the same time, the main complication being the embolization of normal vessels close to the abnormal shunt. The clinical expression of the dural arteriovenous fistula on the opposite eye is infrequent and is related to the presence of complex septae within the cavernous sinus, resulting in a bilateral drainage of the cerebral venous system. The early diagnosis and management of the disease is necessary to improve the visual prognosis.     

The relationship between habitual patient-reported symptoms and clinical signs among patients with dry eye of varying severity

PURPOSE: To investigate symptom profiles and clinical signs in subjects with dry eye and normal subjects in a cross-sectional multicenter study. METHODS: Subjects aged 35 to 65 were recruited according to dry eye diagnostic codes and telephone interview and completed the Dry Eye Questionnaire 2001, among others, and underwent dry eye clinical tests. RESULTS: Subjects (122) included 28 control subjects (C), 73 with non-Sj?gren's keratoconjunctivitis sicca (non-SS KCS) and 21 with Sj?gren's syndrome (SS). Subjects with SS or non-SS KCS reported discomfort and dryness most frequently and that many symptoms worsened over the day and were quite bothersome. Groups were significantly different in corneal fluorescein staining, conjunctival lissamine green staining, Schirmer 1 tear test, and tear break-up time (TBUT; chi2 and Kruskal-Wallis, P<0.0001). Statistically significant, but moderate, correlations were found between the frequency and evening intensity of dryness and discomfort and TBUT, Schirmer's tear test, overall corneal fluorescein staining, and temporal lissamine green conjunctival staining (Spearman r=0.31-0.45, P<0.01). Symptoms were moderately to highly correlated with the clinician's global grading of severity and highly correlated to patient's self-assessment of severity (r=0.46-0.86, P<0.0001), whereas signs showed lower correlations (r=0.22-0.46, P<0.0001). CONCLUSIONS: Subjects with SS or non-SS KCS reported frequent and intense ocular surface symptoms in the evening, some of which correlated moderately with clinical test results. The global clinician grade of dry eye correlated more highly with patient symptoms than did clinical signs, suggesting that patient symptoms influence dry eye diagnosis and grading of dry eye more than clinical test results.     

Presenting signs and symptoms of choroidal melanoma: what do they mean?

Presenting signs and symptoms of 193 patients with choroidal melanomas (followed for up to 152 months) were recorded using retrospective chart review. Eighty patients (41%) had no symptoms (the melanoma was found during routine ophthalmologic examination in 65 patients and during treatment for other eye problem in 15 patients). We found 113 patients (59%) had symptoms (visual acuity or visual field defects, 77 patients; flashes or floaters, 30 patients; pain, 5 patients, and metastatic disease, 1 patient). The tumor size was related to the presence or absence of symptoms (chi-square, 10.6; P = .005). More tumors that presented with symptoms were medium sized (64%) than large (27%). Men had significantly more symptoms (chi-square, 4.1; P = .04). The right eye was more likely to be involved in patients with symptoms (chi-square, 7.3; P = .007). Kaplan-Meier survival analysis for age, sex, presence of tumor or retinal detachment in the macula, tumor location, presence of symptoms, laterality, and tumor size showed that only tumor size was related to a difference in incidence of metastasis (log-rank chi-square, 12.9; P = .002). With increased tumor height, the probability of developing metastasis was greater.     

Dronabinol reduces signs and symptoms of idiopathic intracranial hypertension: a case report.

A case is presented in which a woman diagnosed with a longstanding history of idiopathic intracranial hypertension reported improvement of frontal headaches, photophobia, transient blindness, enlarged blind spots, and tinnitus after smoking marijuana. All these symptoms and signs were associated with increased intracranial pressure (220-425 mm of water). Treatment with dronabinol at a dose of 10 mg twice a day, then reduced to 5 mg twice a day, relieved all of her symptoms. Previously noted papilledema and enlargement of blind spots also resolved, and this, in the absence of psychoactive effect or weight gain.     

Night blindness, yellow vision, and yellow skin: symptoms and signs of malabsorption

BACKGROUND: Rapidly progressing bilateral night blindness in an elderly patient suggests primarily a diagnosis of paraneoplastic retinopathy. Occasionally diffuse rod dysfunction can result from vitamin A deficiency. HISTORY AND SIGNS: A 70-year-old man complained of progressive night blindness and xanthopsia for the past 6 months. Visual acuity was 0.8 in both eyes with severe dyschromatopsia. Slit-lamp and fundus examination were normal. Visual field disclosed bilateral depression. Scotopic full-field ERG was severely reduced. The patient's medical history revealed an acute pancreatitis one year ago, followed by chronic jaundice and an increased blood bilirubin. Serum vitamin A level was decreased to 0.1 micromol/L (normal range 1.5 to 4.0). THERAPY AND OUTCOME: Intramuscular injections of vitamin A were provided. Subjective visual improvement was reported already one day after initiation of therapy. Scotopic full-field ERG was markedly improved 3 days after the injection and was only slightly subnormal 3 months later. CONCLUSIONS: In developed countries, vitamin A deficiency usually results from malabsorption syndromes and manifests initially by rod more than cone dysfunction. This diagnosis should be entertained early as vitamin A supplementation induces a rapid restoration of vision.     

Management of endophthalmitis related to glaucoma drainage devices: review of the literature and our experience

Glaucoma drainage device (GDD)-related endophthalmitis is a devastating complication of device implantation. There are no guidelines in the literature to assist clinicians in deciding if the GDD should be explanted or if patients require pars plana vitrectomy (PPV). This study compares the outcomes of GDD explantation with device retention and also independently compares the outcomes of PPV versus intravitreal antibiotics alone in patients with GDD-related endophthalmitis. A literature search for studies discussing GDD-related endophthalmitis from 2005 to 2019 was performed; 30 articles were included. The visual acuity/anatomical outcomes were compared between GDD explantation and retention, and between patients that received a PPV and those that did not. These outcomes were combined with a medical records review of 13 patients with GDD-related endophthalmitis from an academic institution. A total of 88 eyes were included. 70.5% underwent GDD explantation, while 37.8% received a PPV. GDD explantation was associated with a lower rate of evisceration/enucleation when compared to GDD retention (4.8% versus 19.2%, OR 0.22, 95% CI 0.05–1.01, p = 0.05), but visual acuity outcomes were similar. No eyes that received an immediate vitrectomy became phthisic or required evisceration/enucleation, compared an evisceration/enucleation rate of 15.2% in eyes treated solely with intravitreal antibiotics (OR 1.18, 95% CI 1.04–1.33, p = 0.04). Explantation of the GDD and immediate vitrectomy are both associated with better anatomical outcomes in GDD-related endophthalmitis. Further research is needed to provide more definitive guidelines in the ideal management of these patients.Subject terms: Retinal diseases, Glaucoma     

Cavernous sinus thrombosis with central retinal artey occlusion in COVID-19: A case report and review of literature

Coronavirus disease-2019 (COVID-19) is showing a wide spectrum of ocular manifestations. They are creeping from vision sparing to irreversible visual loss as a result of its thromboembolic events. Hypercoagulability associated with COVID-19 is also called “sepsis-induced coagulopathy” and may predispose to thromboembolic phenomenon that decides the morbidity and mortality of this pandemic. A 37-year-old man presented with no perception of light in the left eye with optic atrophy and macular pucker on fundus examination. Visual evoked potential showed extinguished P 100 wave. His past medical history revealed severe pneumonia secondary to severe acute respiratory syndrome-coronavirus-2 (SARS-CoV 2) infection about 3 months back.