[Angioid streaks in systemic disease]. / Szemfenéki angioid csíkok szisztémás betegségekben.

Angioid streaks are defined as the special morphological alteration of the fundus; the most common clinical manifestations are irregular, reddish brownish stripes around the optic nerve head or on the posterior pole. On the basis of histological examination, the cause of this phenomenon is the breaks and continuity deficiencies in the thin layer of Bruch membrane caused by the degeneration of elastic fibers. The aim of this study is to present the ocular complication of this rare entity through the description of three cases, and to draw attention to systemic diseases in the background. In our first and third cases, pseudoxanthoma elasticum (Grönblad-Strandberg syndrome) was in the background, while in our second case, hematological disease was confirmed. In our first and second cases, the ocular complication was the choroidal neovascularization, which we treated with intravitreal anti-VEGF injection. In our third case, the choroidal rupture was the ocular complication, caused by trauma. Angioid streaks on the fundus may be sub-phenomena of systemic diseases, the detection, differential diagnosis and treatment require interdisciplinary collaboration between associate physicians. Orv Hetil. 2019; 160(25): 994-1000.

The time course changes of choroidal neovascularization in angioid streaks.

PURPOSE: To assess the clinical course of choroidal neovascularization (CNV) in patients with angioid streaks using optical coherence tomography and fluorescein angiography/indocyanine green angiography. METHODS: We examined a consecutive series of 88 eyes of 44 patients with angioid streaks using color fundus photography, optical coherence tomography, and fluorescein angiography/indocyanine green angiography. RESULTS: At the initial visit, 33 eyes exhibited no CNV, 2 exhibited polypoidal choroidal vasculopathy, 8 exhibited Type 1 CNV, 32 exhibited active Type 2 CNV, and 13 exhibited a fibrotic scar. In addition to the 2 eyes that exhibited macular polypoidal choroidal vasculopathy at the initial visit, 3 exhibited peripapillary polypoidal lesions, and 2 exhibited polypoidal lesions at the edge of the preexisting Type 2 CNV/fibrosis. During the follow-up, Type 2 CNV developed in 4 eyes on the basis of Type 1 CNV. Visual acuity was worse in eyes with Type 2 CNV and fibrosis than in those with Type 1 CNV, while polypoidal choroidal vasculopathy did not affect the visual acuity. CONCLUSION: Eyes with angioid streaks can develop any form of CNV including polypoidal choroidal vasculopathy. Considering the worse visual acuity in eyes with Type 2 CNV and fibrosis, patients should be carefully observed so as to treat them promptly when Type 2 CNV occurred beneath the fovea.

[Angioid streaks complicated by choroidal neovascularization secondary to pseudoxanthoma elasticum: diagnosis and treatment. Case report]. / Stries angioïdes compliquées de néovaisseaux choroïdiens révélant un pseudoxanthome élastique : diagnostic et stratégie thérapeutique. À propos d'un cas.

Angioid streaks represent linear breaks in Bruch's membrane secondary to a change in the elastic layer. They are often associated with pseudoxanthoma elasticum. We report the case of a 36-year-old man with no prior history who was seen for a macular problem in the left eye, eventually involving the right eye after 3 months. He was diagnosed with pseudoxanthoma elasticum, associated with angioid streaks, complicated by choroidal neovascularization in both eyes. He was treated with intravitreal ranibizumab injections (0.5 mg/0.05 mL). His course in the right eye was remarkable for stable improvement at 3 months after the final injection. In the left eye, after initial improvement, recurrence was noted 2.5 months after injection, with subfoveal progression of the choroidal neovascularisation, unresponsive to a fourth ranibizumab injection. Angioid streaks represent a degenerative retinal pathology of elastic tissue with the potential for ingrowth of choroidal neovascularization. Various therapeutic approaches such as photodynamic therapy or laser photocoagulation have been proposed, with variable and sometimes limited results. Intravitreal ranibizumab injections currently remain the best treatment and should be studied with a longer-term, larger series.

[Angioid streaks]. / Les stries angioïdes.

Angioid streaks represent breaks in Bruch's membrane, appearing as dark or reddish radial streaks eminating from the optic disc. Usually asymptomatic, these streaks may develop neovascularisation and lead to a maculopathy with marked loss of vision. Some associations with systemic disease are classically described, especially pseudoxanthoma elasticum. This condition may involve cardiovascular complications. A mutation has been found in the ABCC6 gene, which encodes for a membrane transport protein involved in the synthesis of the extracellular matrix. Imaging allows for visualization of the extent of the streaks, and autofluorescence is particularly informative. Spectral domain OCT may also demonstrate early breaks in Bruch's membrane. Neovascular complications, previously responsible for inevitable visual impairment at some point after their occurrence, are now managed by intravitreal injections of anti-VEGFs with clear efficacity. The ophthalmologist must be aware of this condition, in order to guide the patient towards a systemic work-up if necessary, and also to insure quick and targeted treatment in the case of neovascular complications.

Angioid streaks - clinical course and photodynamic therapy. Case report.

Angioid streaks develop as a result of damage to the Bruch's membrane. They may occur as idiopatic angioid streaks or in association with pseudoxanthoma elasticum, Paget's disease and the haemoglobinopathies. Other pathologies of the fundus of eye may coexist together with angioid streaks, such as drusen of an optic disc and granular-like retina in posterior pole known as "peau d'orange". Subretinal neovascularization as well as atrophy in macula are also observed in the cases of long-lasting pathologies. The diagnosis is based mainly on clinical manifestations. Photodynamic therapy in treatment patients with secondary choroidal neovasularisation appeared to be effective.

Pseudoxanthoma elasticum: genetics, clinical manifestations and therapeutic approaches.

Pseudoxanthoma elasticum (PXE) is an inherited disorder that is associated with accumulation of mineralized and fragmented elastic fibers in the skin, vascular walls, and Bruch's membrane in the eye. Clinically, patients exhibit characteristic lesions of the posterior segment of the eye including peau d'orange, angioid streaks, and choroidal neovascularisations, of the skin including soft, ivory-colored papules in a reticular pattern that predominantly affect the neck and large flexor surfaces, and of the cardiovascular system with peripheral and coronary arterial occlusive disease as well as gastrointestinal bleedings. There is yet no definitive therapy. Recent studies suggest that PXE is inherited almost exclusively as an autosomal recessive trait. Its prevalence has been estimated to be 1:25,000-100,000. Very recently, the ABCC6 gene on chromosome 16p13.1 was found to be associated with the disease. Mutations within ABCC6 cause reduced or absent transmembraneous transport that leads to accumulation of extracellular material. Presumably, this mechanism causes calcification of elastic fibers. Despite the characteristic clinical features, the variability in phenotypic expressions, and the low prevalence may be responsible for the disease being underdiagnosed. This review compiles and summarizes current knowledge of PXE pathogenesis and clinical findings. Furthermore, different therapeutic strategies to treat retinal manifestations are discussed, including thermal laser coagulation, photodynamic therapy, and intravitreal injections of drugs inhibiting vascular endothelial growth factor.

Terapia fotodinámica en estrías angioides / Photodynamic therapy in angioid streaks

Objetivo: Evaluar la eficacia y seguridad de la Terapia Fotodinámica (TFD) en la neovascularización coroidea (NVC) asociada a estrías angioides (EA). Métodos: Se realiza un estudio retrospectivo de ocho pacientes (once ojos) diagnosticados de EA, que presentan neovascularización subfoveal, yuxtafoveal o extrafoveal, tratada al menos una vez con TFD. Se obtiene la mejor agudeza visual corregida (MAVC), retinografía así como angiografía con fluoresceína (AFG), antes y después del tratamiento para valorar su eficacia. El seguimiento medio fue de 13,2 meses. Resultados: Diez ojos presentaban membranas subfoveales, un ojo yuxtafoveal y otro extrafoveal. Tras la TFD, la AV se mantuvo igual en dos pacientes, aumentó en un paciente y disminuyó en ocho pacientes. En cuanto a la respuesta angiográfica, nueve ojos presentaron respuesta fibrótica tras una sesión de TFD, mientras que en dos ojos persistió exudación. Hubo tres casos de recidiva de la NVC. Conclusiones: La TFD en la NVC asociada a EA no parece alterar el curso de esta enfermedad, sólo enlentece su progresión

Objetive: To evaluate the efficacy and safety of photodynamic therapy in choroidal neovascularization associated with angioid streaks. Methods: We performed a retrospective study of eleven eyes (eight patients) with subfoveal, juxtafoveal or extrafoveal neovascularization, which received at least one session of photodynamic therapy. Treatment efficacy was evaluated by visual acuity (VA) testing, color photography and fluorescein angiography. The mean follow-up was 13.2 months. Results: Ten eyes presented subfoveal neovascularization, one eye presented juxtafoveal and another eye extrafoveal neovascularization. VA was unchanged in two patients, increased in one patient, and decreased in eight patients following photodynamic therapy. Nine eyes presented conversion to a fibrotic disciform lesion, whereas leakage persisted in two eyes after one session of photodynamic therapy. Recurrence of choroidal neovascularization appeared in three eyes. Conclusions: Photodynamic therapy in choroidal neovascularization related to angioid streaks does not appear to alter the course of this disease, but it does slow its progression

Tratamiento con terapia fotodinámica en las estrías angioides / Treatment of angioid streaks with photodynamic therapy

Caso clínico: Paciente con estrías angioides bilaterales complicadas con degeneración cicatricial en el ojo izquierdo y posterior aparición de una membrana neovascular subfoveal en el ojo derecho. Se plantea tratamiento con terapia fotodinámica y la paciente responde favorablemente con recuperación de la agudeza visual y regresión angiográfica de la lesión. Discusión: Las membranas neovasculares son la complicación más grave de las estrías angioides. Hoy en día uno de los posibles tratamientos de las membranas neovasculares subfoveales es la terapia fotodinámica, siendo ésta, también, un tratamiento eficaz en algunas membranas neovasculares subfoveales secundarias a estrías angioides

CClinical case: A patient had bilateral angioid streaks complicated by cicatricial degeneration in the left eye and subfoveal choroidal neovascularization in the right eye. Photodynamic therapy resulted in a favorable response with normalization of the visual acuity and angiographic resolution of the lesions. Discussion: The most serious complication of angioid streaks is choroidal neovascularization. Today photodynamic therapy is an effective treatment of subfoveal choroidal neovascularization. It also appears useful in the treatment of choroidal neovascularization in angioid streaks

Fundamentos da fisioterapia dermato-funcional: revisão de literatura / Grounds of aesthetic physical therapy: a review

A fisioterapia estética, recentemente renomeada como fisioterapia dermato-funcional, está cada vez mais em evidência. Afim de melhor definir essa área de atuação profissional, procedeu-se a uma revisão bibliográfica das patologias nas quais o fisioterapeuta pode atuar, como fibroedema gelóide (celulite),estrias, linfedema, no pré e pós-operatório de cirurgia plástica, queimaduras, cicatrizes hipertróficas e quelóides, flacidez, obesidade e lipodistrofia localizada.

Aesthetic physical therapy (which in Portuguese has been renamed 'dermatho-functional') has recently gotten more attention. In view of a better definition of the area, this paper reviews related literature to list pathologies that the physical therapist can treat - such as cellulitis, streach marks, obesity, lymphedema, burn care, hypertrophic scar and keloids flaccid skin and muscles, and local fat accumulation...

Periumbilical pseudoxanthoma elasticum associated with chronic renal failure and angioid streaks--apparent regression with hemodialysis.

Pseudoxanthoma elasticum (PXE) is a heritable connective tissue disease involving progressive fragmentation and dystrophic calcification of elastic fibers. Periumbilical disease as the exclusive site of cutaneous involvement is most commonly seen in the rare entity termed periumbilical perforating pseudoxanthoma elasticum (PPPXE). Patients with this disorder are generally obese, middle aged, multiparous black women with hypertension. The cutaneous lesions are well-demarcated, hyperpigmented, periumbilical plaques with keratotic papules on the periphery. Extracutaneous manifestations have rarely been described. We describe a patient with periumbilical PXE associated with chronic renal failure and bilateral angioid streaks. Histopathologic examination demonstrated typical calcification of elastic fibers with additional amorphous calcium deposits in the superficial dermis. Transepidermal elimination was not present. Normalization of the serum calcium-phosphate product resulted in regression of the lesions--both clinically and histopathologically. The relation between PPPXE and hereditary PXE is discussed. The role of chronic renal failure in precipitating PPPXE is considered.

Understanding angioid streaks.

BACKGROUND: Angioid streaks are defined as a series of linear, cracked-line dehiscences of Bruch's membrane, with secondary changes in the retinal pigment epithelium and choriocapillaris. They may be progressive or degenerative, with varied presentation, color, distribution, and retinal involvement. METHODS: The epidemiology, pathophysiology, diagnosis, and management of angioid streaks are described. In addition, the systemic diseases most commonly associated with the disease are reviewed. RESULTS: Optometrists need to be able to differentially diagnose angioid streaks and to refer for evaluation of underlying systemic disease. CONCLUSIONS: Angioid streaks are considered a rare disorder, associated with pseudoxanthoma elasticum, Paget's disease of the bone, sickle hemoglobinopathies. Marfan syndrome, and Ehlers-Danlos syndrome. Ocular complications include subretinal choroidal neovascular membrane formation. Clinicians should be aware of the disease's subtle ocular appearance, its association with systemic diseases, its potential for producing subretinal ocular complications, and correct management protocols and treatments.

Pseudoxanthoma elasticum and its complications: two case reports.

We describe 2 patients with pseudoxanthoma elasticum (PXE) and angioid streaks. In one patient PXE was complicated by cardiovascular disease. The other patient presented with acute visual decline in one eye after a minor blunt injury. Information about PXE and its complications is given.

Diagnosis and management of angioid streaks.

Angioid streaks are asymptomatic breaks in Bruch's membrane developing later in life. Secondary macular degeneration and other fundus abnormalities often accompany their development. Angioid streaks are frequently associated with systemic diseases such as pseudoxanthoma elasticum, Paget's disease of bone, and the sickle cell hemoglobinopathies. The clinical manifestations of angioid streaks, related fundus changes, and these three systemic disorders are discussed as well as principles of treatment and management. Two case reports are presented which illustrate some of the important characteristics of angioid streaks.

Angioid streaks.

Angioid streaks were first described by Doyne in 1889. Gronblad proposed in 1929 that they followed disruption of the elastic layer of Bruch's membrane, having noted the association between angioid streaks and pseudoxanthoma elasticum. Several other diseases have been associated with angioid streaks, including Paget's disease and sickle cell disease. Angioid streaks are found predominantly in the 20 to 50 year age-group and may be associated with minimal visual loss, but the problem is the high risk of rapid development of subretinal neovascularisation at the macula with resultant haemorrhage and scarring. Prophylactic light coagulation along the angioid streaks to prevent subretinal neovascularisation is not recommended. However, light coagulation treatment to foci of subretinal neovascularisation is possible if the network is not too close to fixation. As recurrence of neovascularisation is to be expected, very careful follow-up is necessary.

Angioid streaks: a case report.

A case of vision loss in a patient with angioid streaks and pseudoxanthoma elasticum is described. Discussion includes the natural course and clinical appearance of the disease, the role of trauma, and recommended optometric management.