Telangiectatic Vessels Research Articles

Progressive ascending telangiectasias

Progressive ascending telangiectasias

Optical coherence tomography angiography in intermediate uveitis-related cystoid macular edema.

Cystoid macular edema (CME) is the leading cause of permanent visual impairment in patients with uveitis, particularly in patients with intermediate uveitis (IU). This study was aimed at comparing the changes in the macular microvasculature in patients with IU with uveitic non-responsive CME and without macular edema. In this case-control study, 55 eyes of patients with IU were assessed for macular microvascular structures, including vascular density, foveal avascular zone (FAZ) measurement, and vascular morphological changes, using spectral-domain optical coherence tomography angiography (OCT-A) with the AngioVue OCT-A system. We divided patients into the following two groups: the case group, including 30 eyes with IU-related non-responsive CME, and the control group, including 25 eyes with IU without macular edema. Participants in the case and control groups had comparable age (P = 0.753) and sex (P = 0.124) distributions. Superficial capillary plexus vessel density in the case group was significantly decreased in the whole image (P = 0.027) and the parafoveal area (P = 0.001) compared to the control group. However, there were no statistically significant differences between the two groups in terms of foveal superficial vessel density, deep capillary plexus vessel density, FAZ area, FAZ perimeter, FAZ acircularity index, or foveal vessel density in a 300-µm-wide annulus around the FAZ (all P > 0.05). Vascular morphological changes, such as the capillary tuft, telangiectatic vessels, or micro-aneurism, were not different in the overview images of the OCT-A printout between the two groups. The mean superficial capillary plexus vessel density was lower in eyes with IU-related nonresponsive CME than in those without macular edema. We observed more cystoid spaces in SCP than in DCP. Microcystic changes in the inner retina and ischemia may be the underlying cause in eyes with nonresponsive CME. Future prospective longitudinal studies with healthy, matched controls are warranted to confirm our findings.

Refractory bladder haemorrhage managed with cystoscopic diathermy in a patient with Ataxia-Telangiectasia and intercurrent BK polyomavirus infection

Ataxia telangiectasia (A-T) is a rare neurodegenerative immunodeficiency syndrome, characterised eponymously by progressive cerebellar ataxia and telangiectatic vessels, most often cutaneous. A-T displays autosomal recessive inheritance, is characterised by defective DNA repair due to mutations within the ATM gene on chromosome 11 [ 1 ]; and a lack of native ATM protein gives rise to the aforementioned hallmarks of the disease. With A-T comes a predisposition to haematological malignancy and humoral immunodeficiency, and as such these patients often suffer truncated lifespans [ 1 , 2 ]. Urological manifestations are rare, and intravesical involvement comprising bladder telangiectasia has only been described in a handful of cases, all of which have previously been treated with cyclophosphamide for malignancies, a known instigator of bladder haemorrhage [ [3] , [4] , [5] ]. Similarly, BK polyomavirus is a known precipitant of nephropathy and haemorrhagic cystitis in actively immunosuppressed renal transplant patients, though has not been established as such in genetically immunodeficient patients with native urological tissue [ 6 ]. Here we present, to our knowledge, the first case of refractory haemorrhagic cystitis in an A-T patient with intercurrent BK polyoma virus infection, who had previously been treated with cyclophosphamide for leukaemia, and propose that recurrent cystoscopic diathermy is a robust and relatively conservative surgical option to treat haemorrhagic cystitis secondary to bladder wall telangiectasia.

Mature Placental Teratoma: A Case Report

Introduction: Placental teratoma is an uncommon, non-trophoblastic benign tumor. Since the first description of this type of tumor by Morvilli in 1925, only a few cases of this abnormality have been diagnosed and reported. Case presentation: We describe here a 35-year-old pregnant woman admitted for an elective cesarean section. The placenta and membranes were delivered spontaneously. After delivering the placenta, a tumor mass containing hair was detected between the extraplacental amnion and chorion. On gross examination, a singleton placenta weighing 173 g, measuring 15 × 11 cm in area and 5.5 cm thick, with an attached cord and membranes, was observed. Microscopic examination showed orthokeratinized stratified squamous epithelium with underlying hair follicles, telangiectatic vessels, and sebaceous glands accompanied by subcutaneous fat. The fat also contained fibrocollagenous bundles. In addition, interconnecting bone trabecula rimmed by osteoblasts and attached mature cartilage were also found. Conclusion: An awareness of placental teratoma from a pathological perspective is needed to include this anomaly in the differential diagnosis of benign placental nodules, obtain more information about this unusual lesion, and to conduct more studies on this prenatal abnormality.

Diagnosis and Excision of a Solitary Trichoepithelioma

Background: Solitary trichoepithelioma is a rare benign tumor of hair follicle origin. Although trichoepithelioma has a predilection for the face and neck, it is not usually found on the eyelids. It has similar clinicopathological features to basal cell carcinoma. Surgical excision and histopathologic analysis is helpful for correct diagnosis. Case Report: A 59-year-old Caucasian female presented with a raised, reddish oval mass on the right lower eyelid. The mass had telangiectatic blood vessels on its surface, was non-pigmented and slightly umbilicated. It was in close approximation of the eyelid margin causing distortion and poor apposition of the lid to the globe. The mass was completely removed under local anesthesia using the tissue-sparing surgical technique known as shave biopsy. Histopathologic analysis confirmed the mass to be a trichoepithelioma. Conclusion: Optometrists should be able to differentiate benign trichoepithelioma from basal cell carcinoma.

Keloid-like dermatofibroma

Dermatofibroma is a common benign skin tumor, mainly occurring in young to middle-aged females. It is frequently localized in the lower extremities. A typical dermatofibroma usually presents itself as a single firm papule or nodule, of variable color, bluish, brownish, or pinkish. Its clinical, dermoscopic, and histological features usually allow easy diagnosis [1]. However, it is possible to observe some variations of these typical features. Keloid-like dermatofibroma is one of these atypical presentations rarely reported in the literature [2]. A 40-year-old patient with no previous medical history presented to our dermatology department with a lumbosacral lesion evolving for several months. A physical examination revealed a firm, well-demarcated, asymptomatic erythematous nodule, 5 × 12 mm in size, localized in the lumbosacral area (Fig. 1). The patient denied any trauma preceding the onset of the lesion. There was no personal or familial history of keloidal scars. A dermoscopic examination revealed erythema, telangiectatic vessels, a shiny white streak, and a brownish-yellow pigmentation (Fig. 2). A biopsy was performed. A histological examination revealed an atrophic epidermis. The dermis contained a fibroblastic proliferation of low cell density haphazardly arranged, located on a fibromatous background (Figs. 3 and 4). Dermatofibroma with a keloidal presentation was the diagnosis.

Periorbital Mass in a 2-month-old Girl.

Periorbital Mass in a 2-month-old Girl.

Diffuse infiltrating retinoblastoma: A diagnostic conundrum

Diffuse infiltrating retinoblastoma (DIR) is characterized by absence of intraocular mass, lack of calcification. It may mimic inflammatory uveitis or exudative retinopathy. An eight-years-old boy presented with progressive loss of vision in left eye. Clinical evaluation revealed neovascular glaucoma with a yellow–gray fundal glow, exudative retinal detachment, subretinal exudation and telangiectatic vessels. The presentation was consistent with exudative retinopathy (Coat’s disease) but for the presence of a family history of retinoblastoma in the younger sibling. Despite the absence of an intraocular mass or calcification on multimodal imaging, the enucleation was done on the basis of clinical suspicion of retinoblastoma. Histopathology confirmed a diagnosis of DIR.: DIR can pose a diagnostic challenge due to its non-characteristic clinical and imaging features and atypical presentation. A high index of suspicion along with a positive family history was key to diagnosis in our case; histopathology was confirmatory.

Dermoscopy in Leprosy: A Clinical and Histopathological Correlation Study

Leprosy, an insidious infectious granulomatous disease, is diagnosed traditionally through clinical examination coupled with skin smears and histopathology. It has myriad clinical presentations that pose diagnostic challenges. Lately, dermoscopy has emerged as a rapid, noninvasive diagnostic modality for many dermatoses. We evaluated the dermoscopic findings of various manifestations of leprosy and correlated them with clinical and histopathological features. This prospective, cross-sectional study was conducted in our skin outpatient department for a period of 1 year. Patients newly diagnosed as having leprosy or those undergoing leprosy treatment for less than 6 months were included. The most representative lesion was dermoscopically evaluated and later biopsied. We included 73 patients in the study. Results indicated an obvious correlation between dermoscopic findings and histopathology. We noted orangish yellow and white structureless areas, steadily throughout the spectrum, depicting dermal granuloma. Additionally, we observed focal vascular structures such as branching, linear, and crown vessels that result from the pressure of granuloma pushing the dilated vessels upwards. The relative absence of skin appendages aided in differentiating leprosy from other granulomatous disorders. Novel findings of our study were the detection of a branch-like pattern of clofazimine-induced pigmentation on dermoscopy and orange globules on onychoscopy. Other unique findings included violaceous structureless areas, characteristic large telangiectatic vessels, follicular plugging, star-shaped silvery-white scaling, and white globules in type 1 reaction; white shiny steaks were observed in patients with borderline lepromatous leprosy, and central white dots and keratotic plugs were observed in patients with histoid leprosy. Dermoscopy, as a noninvasive modality, could aid in the quick diagnosis of leprosy and should be used as a handy tool to complement other investigative tools for this disease.

Imaging the pediatric retina: An overview.

Recent decade has seen a shift in the causes of childhood blinding diseases from anterior segment to retinal disease in both developed and developing countries. The common retinal disorders are retinopathy of prematurity and vitreoretinal infections in neonates, congenital anomalies in infants, and vascular retinopathies including type 1 diabetes, tumors, and inherited retinal diseases in children (up to 12 years). Retinal imaging helps in diagnosis, management, follow up and prognostication in all these disorders. These imaging modalities include fundus photography, fluorescein angiography, ultrasonography, retinal vascular and structural studies, and electrodiagnosis. Over the decades there has been tremendous advances both in design (compact, multifunctional, tele-consult capable) and technology (wide- and ultra-wide field and noninvasive retinal angiography). These new advances have application in most of the pediatric retinal diseases though at most times the designs of new devices have remained confined to use in adults. Poor patient cooperation and insufficient attention span in children demand careful crafting of the devices. The newer attempts of hand-held retinal diagnostic devices are welcome additions in this direction. While much has been done, there is still much to do in the coming years. One of the compelling and immediate needs is the pediatric version of optical coherence tomography angiography. These needs and demands would increase many folds in future. A sound policy could be the simultaneous development of adult and pediatric version of all ophthalmic diagnostic devices, coupled with capacity building of trained medical personnel.

Long-standing purpuric exanthema

A 68-year-old woman presented with a chronic, progressive, mildly pruritic erythematous-purpuric exanthema developing over the past 3 years. The physical examination revealed slightly infiltrated, telangiectatic papules and macules on her trunk, extremities, and on her cheeks (Fig 1). Histopathologic examination revealed an increase of telangiectatic dilated vessels in the dermis without relevant inflammatory infiltrates, as well as small fibrin thrombi within some of the vessels (Fig 2).

Net-like superficial lymphatic malformation.

The net-like superficial lymphatic malformation (LM) is a newly described entity with distinctive clinical, dermoscopic, and histologic characteristics. Clinical picture consists of red to purplish macules with a finely reticulated pattern of vascular structures. Dermoscopy shows arborizing telangiectatic vessels. Histology is characterized by a vascular proliferation composed of thin-walled vessels, located in the upper dermis, that stains positive with podoplanin (D2-40). We report a new case of LM with an additional clinical feature, hypopigmented areas.

Demographic and Multimodal Imaging Features of Macular Telangiectasia Type 2: Korean Macular Telangiectasia Type 2 Study – Report No. 2

ABSTRACT Purpose: To investigate the demographic and multimodal imaging features of macular telangiectasia (MacTel) type 2 in Korea and their relationship with visual acuity and the clinical stage. Methods: A retrospective multicentre cross-sectional study was conducted in six tertiary hospitals in Korea and the study included 84 patients. Demographic data and imaging data of fundus photography, fundus autofluorescence (FAF), confocal blue-light reflectance (CBR), fluorescein angiography (FAG), and optical coherence tomography (OCT) were collected. Results: The Korean patients with MacTel type 2 were predominantly female (75%), and the mean logMAR visual acuity was 0.282 ± 0.280 at initial presentation. Most commonly presented signs were the loss of retinal transparency in fundus photographs (68.3%); increased autofluorescence in FAF (83.6%); increased blue reflectance involving the centre in CBR (68.0%); telangiectatic vessels in FAG (88.2%); and hyporeflective cavities in OCT (77.7%). The eyes diagnosed in the first half of the study period (2009–2014) showed a tendency to be diagnosed at more advanced severe stages than those diagnosed in the second half of the study period (2015–2019), using new severity scales based on FAG, FAF and OCT findings. Conclusion: The clinical features of MacTel type 2 in Korean patients assessed by newer imaging modalities suggest that Korean patients and the Caucasian-dominant population show similar presentations. This study showed that MacTel type 2 can be diagnosed in the earlier phase of the disease by using new imaging modalities and through better understanding of the disease.

As the worm turns: An infant with exotropia

A two-year-old full-term boy with a history of an intermittent exotropia presented to the pediatric ophthalmology clinic for routine follow-up. He was found to have a stable sensorimotor examination however dilated funduscopic examination of the right eye was significant for a dim foveal reflex with a new discrete, white, elevated retinal lesion superotemporal to the fovea with surrounding subretinal exudates. An examination under anesthesia (EUA) was performed two days later that revealed a subretinal posterior pole granuloma with hyperfluorescence and late leakage from the lesion without telangiectatic vessels on fluorescein angiography. A repeat EUA one month later demonstrated an increase in surrounding subretinal and overlying intraretinal fluid. The patient was started on topical 1% prednisolone with a presumed diagnosis of Toxocara granuloma. At EUA, three months later, the subretinal and intraretinal fluid had resolved. The topical steroids were tapered, and the patient continues to be followed closely.

A rare case report of Coats’ disease

Coats’ disease was first described by George Coats, as a unilateral retinal vascular abnormality.Coats’ disease is a nonhereditary, idiopathic disease presenting with vascular telangiectasia withintraretinal and subretinal exudation with no racial preponderance and systemic associations. Coats’disease presents with a wide ambit of clinical features- vision loss, strabismus, leukocoria, ornystagmus. The three classical features that are pathognomonic of Coats’ are exudative retinaldetachment, telangiectatic vessels, and peripheral retinal ischemia. The modalities for treatment ofCoats disease that can be used are laser photocoagulation, anti-VEGF agents, or a combination ofboth and cryotherapy. This article describes a case report of a 10-year-old male child withcomplaints of painless loss of vision, his ophthalmological evaluation, and the treatment isundertaken.

Surgical Approach to Control Advanced Coats Disease

Coats disease, first described by George Coats in 1908, is commonly a unilateral clinical entity characterized by leaky telangiectatic vessels leading to progressive subretinal exudation and exudative retinal detachment (ERD).1 It is generally seen in pediatric population and has a distinct male predilection of over 90%. Shields et al.2 classified Coats disease into five stages as follows: Stage 1: presence of retinal telangiectasia only, Stage 2: telangiectasia and exudation (2A: extrafoveal exudation, 2B: foveal exudation), Stage 3: exudative RD (3A: subtotal RD, 3B: total RD), Stage 4: total RD and neovascular glaucoma, Stage 5: advanced end-stage disease.
 Disease progression can be controlled with ablative therapies such as laser photocoagulation (LFK) and cryotherapy in the early stages of Coats disease. However, ablative therapies are not effective in the late stages (Stage 3 – 4) due to excessive subretinal exudation and ERD. Therefore, adjunctive surgical approaches have become popular in the treatment of advanced Coats disease. This helps in effective ablation of leaky abnormal vessels.

EVALUATION OF PHOTORECEPTORS, RETINAL CAPILLARY PLEXUSES, AND CHORIOCAPILLARIS IN PATIENTS WITH BIRDSHOT CHORIORETINOPATHY.

To assess the status of the cone photoreceptors using adaptive optics flood illumination ophthalmoscopy in patients with birdshot chorioretinopathy and to study their relationship with optical coherence tomography (OCT) angiography. Seventeen patients with birdshot chorioretinopathy were studied using standard automated perimetry, color vision testing, fluorescein angiography, indocyanine green angiography, spectral domain OCT, enhanced depth imaging OCT, and adaptive optics flood illumination ophthalmoscopy (rtx1, Imagine Eyes, Orsay, France) in a 90 × 90-μm area at 1.5° temporal of the fovea and OCT angiography (Spectralis OCT2, Heidelberg Engineering, Heidelberg, Germany). For adaptive optics flood illumination ophthalmoscopy, spectral domain OCT, and OCT angiography, a control group of 12 healthy patients (12 eyes) matched for age, sex, and refractive error was included. After excluding low-quality adaptive optics flood illumination ophthalmoscopy images, 12 eyes (12 patients, 7 women, mean age 53.5 ± 10.8 years) were analyzed. Best-corrected visual acuity was 0.01 ± 0.03 LogMAR (20/25 Snellen equivalent), and foveal threshold at standard automated perimetry was 35.2 ± 3.0. Cone density at 1.5° of the fovea in the birdshot chorioretinopathy group (11,435.25 ± 4,342.9 cells/mm) was significantly smaller than in the control group (24,594.04 ± 4,764.3 cells/mm, P < 0.001). In the same area, birdshot chorioretinopathy eyes showed disruption of the ellipsoid/interdigitation zone and vascular abnormalities, such as capillary loops (58.3%), increased superficial intercapillary spaces (75%), increased deep intercapillary spaces (58.3%), capillary dilations (58.3%), telangiectatic vessels (66.6%), and absence of choriocapillary flow (58%). Cone density at 1.5° was associated with duration of the disease (Spearman's rho -0.8, P = 0.01), although it was not associated with OCT angiography abnormalities in the same area. Birdshot chorioretinopathy can result in a reduction in cone density and development of macular vascular abnormalities even in the presence of preserved visual function.

A Case of Kaposi Sarcoma Co-infected with Cytomegalovirus

Background: Kaposi sarcoma (KS) is the most common AIDS-associated neoplasm. It is a vascular neoplasm that occurs as a result of infection with a human herpesvirus (HHV-8). Cytomegalovirus (CMV) and HHV-8 both belong to Herpesviridae, a family of DNA viruses. CMV is highly prevalent in the general population and can cause localized or disseminated disease in AIDS patients.Case: A 42-year-old male with an HIV infection presented with a painful ulcerated growing white nodule with overlying telangiectatic vessels on the right third toe that he noticed 4 weeks ago. A tangential biopsy revealed a vascular proliferation which was diffusely positive for HHV-8. In addition, scattered inclusion bodies were observed, indicating co-infection with CMV.Conclusion: This case reinforces the importance of considering KS as a potential diagnosis in all AIDS patients with unusual exophytic growths to avoid potential misdiagnosis and improper management.

Bilateral internal carotid artery occlusion with compensatory perfusion in a HIV-infected patient

HIV infection occlusive arteriopathies may result in neurological symptoms. We report a case of bilateral complete occlusion of the extracranial portions of the internal carotid arteries in a HIV+ve patient who presented with a syncopal episode due to intraventricular haemorrhage. Compensatory blood flow from the posterior cerebral circulation via the circle of Willis resulted in small telangiectatic vessels arising from the posterior cerebral circulation which probably accounted for this rare haemorrhagic complication of an occlusive arteriopathy.

Efficacy of Aflibercept in Bevacizumab-Resistant Macular Edema in Adult-Onset Coats Disease

Purpose: This case is the first to our knowledge that demonstrates the efficacy of intravitreal aflibercept in bevacizumab-resistant, laser-naive macular edema (ME) in adult-onset Coats disease. Methods: A retrospective case report is presented. Results: A 74-year-old woman presented with central visual distortion in her right eye. Imaging and exam were consistent with adult-onset Coats disease with unilateral telangiectatic vessels, numerous macroaneurysms, and peripheral capillary dropout. There were also ME, lipid exudate, and localized hemorrhage. ME failed to respond to 4 intravitreal injections of bevacizumab but dramatically improved following initiation of aflibercept, with patient deferral of laser photocoagulation. Conclusions: To our knowledge, this is the first reported case of aflibercept as the sole treatment of bevacizumab-resistant ME in adult-onset Coats disease. Given the localized macular involvement more common to adult-onset Coats disease, antivascular endothelial growth factor (anti-VEGF) therapy may be sufficient, compared with classic childhood Coats disease, in which anti-VEGF therapy serves as an adjunct to laser photocoagulation or cryotherapy.