Adult linear scleroderma en coup de sabre with epileptic seizure and Coats-like response: a case report and literature review / 中华神经科杂志

Objective:To report the clinical characteristics, diagnosis and treatment of a patient with linear scleroderma en coup de sabre (LSCS), and review the relevant literature in order to provide the basis for early diagnosis and timely treatment of the disease.Methods:The clinical data and treatment process of a patient with LSCS admitted to Hangzhou Traditional Chinese Medicine Hospital Affiliated to Zhejiang Chinese Medical University on September 22, 2022 were summarized, and the case reports or case series studies related to LSCS with epilepsy or Coats-like response at home and abroad were systematically analyzed. The gender, age, onset time, clinical manifestations, treatment and prognosis of this type of patients were summarized.Results:The patient is a 22 years old female with a history of scalp patchy alopecia and ipsilofrontal en coup de sabre for over 10 years and was diagnosed as Coasts disease due to decreased vision in the right eye 5 years ago, and now she is blind. This visit was due to "episodic loss of consciousness for more than 2 hours" with epileptic seizures and Coats-like response of the left eye. Treatment with antiepileptic drugs, glucocorticoids and immunosuppressants showed satisfactory results. The clinical data of all 20 patients with LSCS reported in domestic and foreign literature were analyzed. The age of onset was 11.00 (6.75, 20.50) years, with a male to female ratio of 1∶1. The imaging findings of patients with LSCS with epilepsy were mainly manifested as multiple brain calcifications, soft tissue atrophy and skull thinning on the focal side. The results of fundus examination and fundus fluorescein angiography in patients with LSCS with Coats-like response were mainly exudative inflammation and retinal detachment, including 1 case with cerebral cerebrovascular inflammation. In terms of treatment, most of the patients with LSCS with epilepsy were treated with antiepileptic drugs, glucocorticoids combined with immunosuppressant, interleukin-6 inhibitor tozizumab, and the other 2 cases were treated with surgery. Patients with LSCS with Coats-like response were treated with intravitreal bevacizumab in combination with glucocorticoids and immunosuppressive therapy or retinal targeted photocoagulation or local laser therapy with triamcinolone. The above treatment can control the patient′s refractory epilepsy and improve the vision loss.Conclusions:The main manifestations of LSCS are en coup de sabre lesion with pigmentation on the forehead above the eyelid, accompanied by Coats-like response of the eye, epilepsy, and brain imaging abnormalities. The above clinical features may appear successively or simultaneously. In some patients, these symptoms may progress slowly, and can lead to blindness and refractory epilepsy severely. Glucocorticoids combined with immunosuppressive therapy should be given as early as possible, and intravitreal bevacizumab therapy can improve visual loss of LSCS patients.

Dexamethasone intravitreal implant as an adjuvant treatment for pediatric patients with Coats' disease / Implante intravítreo de dexametasona como tratamento adjuvante em pacientes pediátricos com doença de Coats

ABSTRACT We report two cases of stage 3A unilateral Coats' disease in pediatric patients. In both cases, disease control was achieved using a dexamethasone intravitreal implant in addition to other treatments. The treatment improved visual acuity in one patient and prevented the worsening of the decline in visual acuity in the other patient during follow-up periods of 7 and 3 years, respectively. One of the patients presented an increase in intraocular pressure, which was controlled with topical antiglaucoma medication, but developed a cataract that required surgery. In conclusion, dexamethasone intravitreal implant may be a useful adjuvant treatment to consider in some pediatric cases with Coats' disease.

RESUMO Relatamos dois casos de doença de Coats em estágio 3A unilateral em pacientes pediátricos. Em ambos os casos, o controle da doença foi obtido com implante intravítreo de dexametasona, além de outros tratamentos, com melhora da acuidade visual em um caso e sem piora da visão no outro, durante um período de acompanhamento de 7 e 3 anos. Um dos casos apresentou elevação da pressão intraocular controlada com medicação antiglaucoma tópica e desenvolveu catarata que exigiu cirurgia. Em conclusão, o implante intravítreo de dexametasona pode ser um tratamento adjuvante útil a ser considerado em alguns casos pediátricos com doença de Coats.

Cirugía por radiofrecuencia una alternativa terapéutica en la Insuficiencia Venosa Crónica / Radiofrequency surgery a therapeutic alternative in Chronic Venous Insufficiency / Cirurgia de Radiofrequência uma alternativa terapêutica na Insuficiência Venosa Crônica

La insuficiencia venosa crónica es una patología caracterizada por dificultar el retorno venoso hacia el corazón, se estima que el 90% de la población, padecerá de esta patología en algún momento de la vida, no obstante, su impacto toca la esfera socioeconómica, laboral e inclusive estética del paciente. Clínicamente se manifiesta con telangiectasias, venas varicosas e inclusive úlceras venosas por estasis, el cuadro se acompaña de dolor, cansancio, sensación de pesadez a nivel de miembros inferiores. Objetivo. Establecer los beneficios de la ablación por radiofrecuencia en el tratamiento de la insuficiencia venosa crónica estadificada de acuerdo con la CEAP (Clasificación clínica, etiológica, anatómica, fisiopatológica) en el Hospital Instituto Ecuatoriano de Seguridad Social en la ciudad de Riobamba, en el período 2018-2020. Materiales y Métodos. Se presenta un estudio retrospectivo de corte transversal, realizado en 75 pacientes intervenidos con radiofrecuencia en el Instituto Ecuatoriano de Seguridad Social (IESS) Riobamba, en el período 2018-2020 quienes voluntariamente decidieron participar en el estudio. Resultados. Se obtuvo que esta patología se presenta en un 68% en el sexo femenino y en un 32% en el sexo masculino, de la población total el 60% presentó sobrepeso y el 40% presentó obesidad, mediante la clasificación CEAP se categorizó en C3 al 58.7 % seguido de C2 al 32%, los pacientes de esta última categoría mostraron mejoría dentro de los 6 meses posteriores a la intervención. Conclusiones. La ablación por radiofrecuencia es una técnica quirúrgica efectiva en el tratamiento de los troncos safenos insuficientes, con excelentes resultados postquirúrgicos y en la calidad de vida del paciente.

Chronic venous insufficiency is a pathology characterized by hindering venous return to the heart, it is estimated that 90% of the population will suffer from this pathology at some point in life, however, its impact touches the socioeconomic, labor and even aesthetic sphere of the patient. Clinically it manifests with telangiectasias, varicose veins and even venous stasis ulcers, the picture is accompanied by pain, fatigue, feeling of heaviness in the lower limbs. Objective. To establish the benefits of radiofrequency ablation in the treatment of chronic venous insufficiency staged according to the CEAP (Clinical, etiological, anatomical, pathophysiological classification) in the Hospital Instituto Ecuatoriano de Seguridad Social in the city of Riobamba, in the period 2018-2020. Materials and Methods. A retrospective cross-sectional study is presented, performed in 75 patients intervened with radiofrequency at the Instituto Ecuatoriano de Seguridad Social (IESS) Riobamba, in the period 2018-2020 who voluntarily decided to participate in the study. Results. It was obtained that this pathology is presented in 68% in the female sex and 32% in the male sex, of the total population 60% presented overweight and 40% presented obesity, by CEAP classification was categorized in C3 to 58.7% followed by C2 to 32%, the patients of this last category showed improvement within 6 months after the intervention. Conclusions. Radiofrequency ablation is an effective surgical technique in the treatment of insufficient saphenous trunks, with excellent post-surgical results and in the patient's quality of life.

A insuficiência venosa crônica é uma patologia caracterizada pela dificuldade de retorno venoso ao coração. Estima-se que 90% da população sofrerá desta patologia em algum momento de suas vidas; entretanto, seu impacto afeta a esfera sócio-econômica, ocupacional e até estética do paciente. Clinicamente, manifesta-se com telangiectasias, varizes e até mesmo úlceras de estase venosa, acompanhadas de dor, cansaço e uma sensação de peso nos membros inferiores. Objetivo. Estabelecer os benefícios da ablação por radiofreqüência no tratamento da insuficiência venosa crônica encenada de acordo com a CEAP (classificação clínica, etiológica, anatômica, fisiopatológica) no Hospital Instituto Ecuatoriano de Seguridad Social na cidade de Riobamba, no período de 2018-2020. Materiais e métodos. É apresentado um estudo transversal retrospectivo, realizado em 75 pacientes que intervieram com radiofreqüência no Instituto Equatoriano de Seguridade Social (IESS) Riobamba, no período de 2018-2020, que voluntariamente decidiram participar do estudo. Resultados. Obteve-se que esta patologia ocorre em 68% no sexo feminino e 32% no sexo masculino, da população total 60% apresentava sobrepeso e 40% apresentava obesidade, pela classificação CEAP foi categorizada em C3 a 58,7% seguido por C2 a 32%, os pacientes desta última categoria apresentaram melhora em 6 meses após a intervenção. Conclusões. A ablação por radiofrequência é uma técnica cirúrgica eficaz no tratamento de troncos de safena insuficientes, com excelentes resultados pós-cirúrgicos e na qualidade de vida do paciente.

A prospective case-control study of intravitreal injection of anti-vascular endothelial factor drugs in the treatment of Coats disease / 中华眼底病杂志

Objective:To investigate the efficacy and safety of traditional laser photocoagulation, laser combined with intravitreal injection of anti-vascular endothelial factor (anti-VEGF) drugs and intravitreal injection of anti-VEGF drugs alone in Coats disease.Methods:The patients diagnosed as Coats disease stage 2B-3A2 in Department of Ophthalmology, Eye and ENT Hospital of Shanghai Medical College of Fudan University from December 2016 to November 2019 were included in this study. Patients were divided into three groups, including laser group, combined group and drug group, according to the different treatment. In the laser group, the initial treatment was traditional laser photocoagulation alone. In the drug group, the anti-VEGF drug was injected into vitreous once a month for three months. The initial treatment of the eyes in the combined group was laser combined with intravitreal injection of anti-VEGF drugs, or laser treatment within 1 week after anti-VEGF drug treatment. The follow-up time was more than 6 months, and best-corrected visual acuity (BCVA), ultra-wide-angle fundus photography, and fluorescein fundus angiography were performed during follow-up. The treatment efficiency, subretinal fluid (SRF), macular edema, BCVA and complications were compared among the three groups.Results:Among 60 patients (60 eyes), there were 55 males (55 eyes) and 5 females (5 eyes), with the mean age of 17.1±2.0 years. Among 60 eyes, there were 26 eyes in 2B stage, 23 eyes in 3A1 stage, and 11 eyes in 3A2 stage. Twenty patients (20 eyes) was in the laser group, combined group and drug group, respectively. After the initial treatment of all eyes in the drug group, the abnormal blood vessels did not regress significantly; the absorption and increase of SRF were 4 (20.0%, 4/20) and 5 (25.0%, 5/20) eyes, respectively. Supplementary laser therapy was given to 16 eyes, and vitrectomy (PPV) was given to 4 eyes. Among the 16 eyes treated by laser, 10 eyes were effective (50.0%, 10/20); vitreous hemorrhage, fibrous membrane hyperplasia, and complicated cataract occurred in 1, 1, and 2 eyes during the treatment, respectively, and PPV was given again in all eyes. Recurrent and persistent macular edema occurred in 4 and 1 eyes, respectively. Among the eyes in the combined group, treatment were effective in 11 eyes (55.0%, 11/20); 5, 2, and 2 eyes had SRF, fibrous membrane hyperplasia, and complicated cataract during the treatment, and PPV was given again; the edema was repeated and persisted in 1 eye, respectively. Among the affected eyes in the laser group, 15 eyes (75.0%, 15/20) were treated effectively; 2, 2, and 1 eyes developed a large number of vitreous hemorrhage, fibrous membrane hyperplasia, and complicated cataract during the treatment, and PPV was given again.Conclusions:Anti-VEGF drugs alone are ineffective in the treatment of Coats disease, and ablation of other abnormal blood vessels is needed. In the treatment of Coats disease, anti-VEGF drugs can not only promote the absorption of SRF, but also may lead to its increase, and the application should be cautious.

Macular vessel defects for Coats' disease associated with subfoveal fibrotic nodule / 中华眼底病杂志

Objective:To investigate macular microvascular abnormalities in eyes with subfoveal fibrotic nodules secondary to Coats' disease.Methods:A cross-sectional study. From January 1, 2018 to July 30, 2021, 45 eyes of 45 patients diagnosed with Coats' disease with or without subfoveal fibrotic nodules in Eye and ENT Hospital, Shanghai Medical College of Fudan University were included in this study. There were 40 eyes in 40 males and 5 eyes in 5 females. All were under 21 years old. According to the presence or absence of subfoveal fiber nodules, the patients were divided into fibrotic group (26 cases, 26 eyes) and non-fibrotic group (19 cases, 19 eyes). Optical coherence tomography angiography was used to scan 3 mm×3 mm or 6 mm×6 mm macular area of both eyes. The software of the device automatically processed the images. The presence of FAZ edge anastomotic vascular arch ring breakage and abnormal microvascular branch (AMB) in the foveal avascular zone (FAZ) were observed.Results:In 26 eyes of fibrosis group, AMB originating from the parafoveal retinal capillary network was observed, which grew into and destroyed the integrity of the vascular arch ring at the edge of FAZ. AMB was crisscrossing and winding, and its curvature expands. B-scan images showed the blood flow signal in the subfoveal fiber nodule, and the blood flow signal traversed between the inner retina and the fiber nodule in 23 eyes (88.46%, 23/26). In the non-fibrosis group, all the vascular abnormalities were characterized by capillary dilation and defect, and no breakage of FAZ anastomotic vascular arch ring or AMB was observed.Conclusions:In Coats' disease with subfoveal fiber nodules, staggered and dilated AMBs emerge from the parafoveal vascular network, grow into and destroy the integrity of the vascular arch ring at the edge of FAZ, and grow down longitudinally into the fiber nodules.

Multicolor scanning laser imaging in macular telangiectasia / 中华实验眼科杂志

Objective:To observe the characteristics of macular telangiectasia (MacTel) in multi-color and multi-mode fundus images.Methods:An abservational case series study was conducted.Sixteen eyes of 12 patients diagnosed with MacTel by fluorescein fundus angiography (FFA) from January to November 2019 in Shandong Eye Hospital were analyzed.There were 8 cases (8 eyes) with MacTel type Ⅰ, among which 4 cases were male and 4 cases were female, with an average age of (62.3±12.5) years.The other 4 cases (8 eyes) had MacTel type Ⅱ, all of which were female, with an average age of (58.7±10.5) years.Best corrected visual acuity, slit lamp microscopy, color fundus photography, multicolor scanning laser imaging, FFA, optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) were carried out in all the patients.This study adhered to the Declaration of Helsinki.The study protocol was approved by the Ethics Committee of Shandong Eye Institute (No.2019S003).Results:In color fundus images of MacTel type Ⅰ eyes, annular macular exudation with macular edema occurred in 6 eyes, macular edema without hard exudates in 1 eye, and hard macular exudates without macular edema in 1 eye.However, the transparency of retina in temporal fovea in MacTel type Ⅱ eyes decreased, showing a gray color.In multi-color fundus images of MacTel type Ⅰ eyes, punctate granular yellow macular exudation and yellow-green macular edema were observed, which were clearer than those in color fundus images.Punctate exudation was seen in both the blue and green reflectance images, which was clearest in green reflectance image, followed by blue reflectance image and then the infrared reflectance image.In OCT images of MacTel type Ⅰ eyes, cystoid edema of inner retina or uneven reflection signal of outer plexiform retina were observed.Loss of inner and outer retinal structures and cavities were observable in MacTel type Ⅱ eyes, and outer retinal atrophy appeared in 2 eyes.In OCTA images, the destruction of superficial and deep capillary plexus in macular area were observed in both MacTel typeⅠand type Ⅱ eyes, and the destruction of deep capillary plexus was more obvious.In addition, more obviously increased vascular space, decreased vessel density, and increased foveal avascular zone were found in MacTel type Ⅱ eyes.In early stage of FFA, delayed capillary filling near fovea was seen in MacTel typeⅠeyes, and dilated temporal vessels in fovea, some of which showed tumor-like dilation, and the limited tumor-like dilation was enhanced in the later stage.Different degrees of dilated parafoveal blood vessels in the early stage, and the capillary in the temporal side of the macula showing diffuse strong fluorescence in the late stage of FFA was observed in MacTel type Ⅱ eyes.Conclusions:Multi-color scanning laser imaging can be used to observe the morphological characteristics of MacTel, and the imaging features of different types of MacTel are significantly different.

Coats disease with retinal neovascularization under subfoveal nodule: optical coherence tomography-angiography findings before and after ranibizumab treatment / Doença de Coats com neovascularização da retina sob nódulo subfoveal: achados da angiografia com tomografia de coerência óptica (OCT) antes e depois do ranibizumabe

ABSTRACT We conducted retinal neovascularization under subfoveal fibrotic nodule for Coats disease by using optic coherence tomography-angiography before and after ranibizumab treatment. Our patient was an 8-year-old boy who was referred with suspicious left retinal mass. His visual acuity was 20/400 in the left eye and 20/20 in the right eye at the time of admission. Posterior segment evaluation of the left eye revealed telengiectatic vessels at the inferotemporal region of the peripheral retina with hard exudates around the optic disc and macula typical for Coats disease. His optic coherence tomography revealed a subfoveal fibrotic nodule after ranibizumab injections and laser photocoagulation treatment. The optic coherence tomography-angiography results revealed neovascularization under the subfoveal nodule at the superficial vascular complex layer. After 3 intravitreal ranibizumab injections, his neovascularization regressed on optic coherence tomography-angiography and his visual acuity improved. To the best of our knowledge, this is the first report demonstrating neovascularization under the subfoveal fibrotic nodule in Coats disease on the basis of comparative with the help of optic coherence tomography-angiography before and after the treatment.

RESUMO Demonstramos uma neovascularização da retina sob o nódulo fibrótico subfoveal na doença de Coats com a ajuda da Angiotomografia de Coerência Óptica (OCT-A) antes e após o tratamento com ranibizumabe. Paciente do sexo masculino de 8 anos foi encaminhado com suspeita de massa retiniana no olho esquerdo. A acuidade visual foi de 20/400 no olho esquerdo e de 20/20 no olho direito. A avaliação do segmento posterior do olho esquerdo revelou vasos telengiectáticos na região inferotemporal da retina periférica e exsudados duros em torno do disco óptico e mácula típica da doença de Coats. A angiotomografia de coerência óptica apresentou nódulo fibrótico subfoveal após injeções de ranibizumabe e tratamento com fotocoagulação a laser. A angiotomografia de coerência óptica mostrou neovascularização sob o nódulo subfoveal na camada superficial do complexo vascular. Após três injeções de ranibizumabe intravítreo, a neovascularização regrediu na angiografia por tomografia de coerência óptica e a acuidade visual melhorou. onde sabemos, este é o primeiro relato a mostrar neovascularização sob nódulo fibrótico subfoveal na Doença de Coats com a ajuda da angiografia por tomografia de coerência óptica antes e após o tratamento.

A case of cutaneous collagenous vasculopathy / 中华皮肤科杂志

A 22-year-old female patient presented with skin flushing in the bilateral legs for 4 years, which gradually spread throughout the whole lower limbs and forearms 6 months ago. Skin examination showed diffuse flushing and dilated capillaries in the lower limbs and both forearms, and the flushing faded after a press. Histopathological examination of the skin lesion on the leg showed hyperkeratosis in a basket-like shape, increased pigmentation in the basal layer, infiltration of the superficial dermis with scattered lymphocytes, with no obvious red blood cell overflow; periodic acid-Schiff staining showed thickened and homogeneous deposits around the blood vessels; immunohistochemical staining showed thickened blood vessel walls and positive staining for type Ⅳ collagen. Diagnosis: cutaneous collagenous vasculopathy.

Efficacy of focal photocoagulation on Coats disease / 中华眼底病杂志

Objective:To observe the effect of local retinal laser photocoagulation (local photocoagulation) on Coats disease.Methods:A retrospective clinical study. From January 1, 2006 to August 1, 2020, 48 patients (48 eyes) who were diagnosed as Coats disease and received focal photocoagulation at The Eye Hospital Affiliated to Wenzhou Medical University were included in the study. Among them, there were 40 males (40 eyes) and 8 females (8 eyes). The average age was 32.46±22.41 years old. Nine eyes were in stage 2A, and 39 eyes were in stage 2B. All affected eyes underwent best corrected visual acuity (BCVA), color fundus photography and fluorescein fundus angiography (FFA).The BCVA was carried out using a standard logarithmic visual acuity chart, which was converted into the logarithmic minimum angle of resolution (logMAR) visual acuity during statistics. According to age, patients were divided into the adolescent group (age≤ 20 years old) and the adult group (age> 21 years old), with 18 eyes in 18 cases and 30 eyes in 30 cases, respectively. In the adolescent group, 18 eyes of 18 cases were all male; the average age was 11.17±3.31 years; the average logMAR BCVA was 0.83±0.60. Among the 30 patients in the adult group, 22 patients were male and 8 patients were female. the average age was 49.26±15.26 years old; the average logMAR BCVA was 0.82±0.59. All the affected eyes were treated with focal photocoagulation. Laser parameters were as followed: wavelength 577 nm, spot diameter 500 μm, exposure time 0.5 to 0.7 s, spot intensity level Ⅲ. FFA was FFA was performed 3 to 4 months after the first laser photocoagulation, and laser treatment was repeated as needed. The average follow-up after first treatment was 36.88±36.92 months. The changes in BCVA, abnormal blood vessels in the fundus, and hard exudation were observed.Results:Among 48 eyes, 36 eyes (75.00%, 36/48) received multiple local photocoagulation treatments. At the last follow-up, 36 eyes (75.00%, 36/48) had an improved or stable vision, and 17 eyes (35.42%, 17/48) had BCVA ≤ 0.32 logMAR units (≥ 0.5). The average logMAR BCVA of eyes in the adolescent group was 0.66±0.54, which was higher than the baseline, but the difference was not statistically significant ( Z=-1.126, P=0.260). The average logMAR BCVA of the eyes in the adult group was 0.96±0.79, which was lower than the baseline, but the difference was not statistically significant ( Z=-0.482, P=0.630). Among 48 eyes, abnormal blood vessels were completely or partially occluded in 42 eyes (87.50%, 42/48); of which, 29 eyes were completely occluded (60.42%, 29/48), and 13 eyes were partially occluded (27.08%, 13/48)). The hard exudation at macula or peripheral retina were completely absorbed or obviously absorbed in 40 eyes (83.33%, 40/48); among them, the complete and obvious absorption were 11 (22.92%, 11/48) and 29 (60.42%, 29/48) eyes. Conclusion:The treatment of focal photocoagulation with a larger spot, long exposure and weak level Ⅲ spot can effectively seal abnormal blood vessels in the eyes of Coats disease,reduce hard exudation and improve or stabilize vision.

Observation on the imaging characteristics of optical coherence tomography angiography in eyes with macular telangiectasia type 2 / 中华眼底病杂志

Objective:To observe the imaging characteristics of optical coherence tomography angiography in macular telangiectasia type 2 (Mac-Tel 2).Methods:A retrospective case analysis. From October 2017 to June 2021, 11 patients (22 eyes) diagnosed as Mac-Tel type 2 by multi-modal imaging in Nanjing Medical University Eye Hospital were included in this study. There were 5 males (10 eyes) and 6 females (12 eyes). The age were 41.61±11.32 years old. All patients underwent the examinations of best corrected visual acuity, indirect ophthalmoscope, fundus color photography, fluorescein fundus angiography (FFA), optical coherence tomography (OCT), and OCT angiography (OCTA). The scope of 3 mm × 3 mm in macular area of eyes was scanned by OCTA. After automatic image processing, the system could provide the blood flow image of capillary layer, deep capillary layer, outer retina, choroidal capillary layer, and the B-scan image. The imaging characteristics were observed.Results:Among the 22 eyes, 14 eyes were in the early stage of the disease, and 8 eyes had secondary subretinal neovascularization (SRN) and/or choroidal neovascularization (CNV). FFA examination that in the early stage of the disease, the capillaries near the fovea were dilated, the blood vessels were stretched, and the late fluorescence was mainly stained; high-fluorescence leakage was seen when SRN and CNV were developed. OCTA examination showed that in the early stage of the disease, the temporal capillaries in the macular area were dilated and stretched, especially in the deep layer. The capillary space was enlarged, and the right-angled venules were seen to change and infiltrate into the deep layer; when the lesions invaded the outer retina, flower clusters-like SRN were seen; neovascularization was seen in the outer retina and choroidal capillary layer when CNV was developed. B-scan image showed that in the early stage of the disease, irregular weak reflex cavities and lamellar holes change between the neuroepithelial layers; secondary SRN and CNV showed strong little clumpy reflexes accompanied by abundant blood flow signals.Conclusion:The image characteristics of OCTA in the eyes of Mac-Tel 2 were dilated, stretched superficial and deep temporal capillaries in the macula area and right-angled changes in blood vessels.

The changes of macular blood flow density in patients of macular telangiectasis type 1 with macular edema before and after the treatment of anti-vascular endothelial growth factor / 中华眼底病杂志

Objective To observe the changes ofmacular blood flow density in patients ofmacular telangiectasis type 1 (Mac-Tel type 1) with macular edema before and after the treatment of anti-VEGF.Methods A retrospective clinical study.From January 2016 to December 2017,14 Mac-Tel type 1 patients (14 eyes) diagnosed in Nanjing Medical University Eye Hospital were included in the study.There were 6 males (6eyes) and 8 females (8 eyes),with the mean age of 35.3± 9.3 years.All patients underwent BCVA and OCT angiography examinations.The BCVA examination was performed using the Snellen visual acuity chart,which was converted into logMAR visual acuity.All the patients were received anti-VEGF injection treatment once a month for 3 consecutive months.The OCTA scanning region in the macular area was 3 mm × 3 mm.Macular blood flow density in the superficial capillary plexus (SCP) and deep capillary plexus (DCP),the vessel density within a 300 μm width ring surrounding the foveal avascular area (FD-300) and central macular thickness (CMT)were measured in all eyes.Paired samples t-test and Pearson correlation analysis were used in this study.Results At the baseline,logMAR BCVA was 0.69 ± 0.07,CMT was 468.43 ± 26.59 μm,SCP blood flow density was (50.99± 1.19)％,DCP blood flow density was (43.79± 1.44)％,FD-300 was (50.73 ± 1.16)％.Compared with the baseline,there were significant differences between logMAR BCVA,CMT,DCP blood flow density and FD-300 in 1 week,1 month,3 months after treatment and 2 months after cessation of treatment (logMAR BCVA:t=6.77,13.30,16.99,9.51;P=0.00,0.01,0.00,0.01.CMT:t=6.99,15.88,26.10,6.50;P=0.00,0.01,0.01,0.00.DCP:t=6.75,8.61,15.12,7.63;P=0.00,0.01,0.01,0.00.FD-300:t=11.86,13.08,14.36,4.41;P=0.00,0.01,0.01,0.03).There was no significant difference in blood flow density of SCP between baseline and 2 months after cessation of treatment (t=1.36,P=0.19),but there was significant difference at the other time points after treatment (t=5.50,6.84,6.27;P=0.00,0.01,0.01).The Pearson's correlation analysis showed that there was a significant positive correlation between FD-300 and CMT (r2=0.54,P=0.04).Conclusions There is no significant change in the SCP blood flow density in the patients of Mac-Tel type 1 with macular edema,while the DCP blood flow density decreased and FD-300 increased.After anti-VEGF treatment,DCP blood flow density increased and FD-300 decreased.FD-300 is positively correlated with CMT.

Uso de tartarato de brimonidina para resolução de matting telangiectásico: relato de caso / Use of brimonidine tartrate to resolve telangiectatic matting: case report

Resumo A escleroterapia é, atualmente, o tratamento de escolha para telangiectasias e veias reticulares, apresentando nível de recomendação 1ª pela diretriz europeia para escleroterapia. Os efeitos colaterais mais comuns desse procedimento são a hiperpigmentação e o matting telangiectásico, sendo este último um dos mais temidos em virtude do dano estético e da dificuldade de tratamento. O matting se refere a vasos com diâmetro inferior a 0,2 mm que podem surgir esporadicamente ou em áreas bem definidas, principalmente nos membros inferiores. Este relato apresenta um caso de matting tratado com o uso de tartarato de brimonidina tópico.

Abstract Sclerotherapy is currently the treatment of choice for telangiectasias and reticular veins, with grade 1A recommendation in the European Guideline for sclerotherapy. The most common side effects of this procedure are hyperpigmentation and telangiectatic matting, the second of which provokes great concern because of the esthetic damage and the difficulty of treatment. Matting refers to vessels with a diameter of less than 0.2 mm, which may emerge irregularly or in well-defined areas, especially on the lower limbs. This report presents a case of matting treated with topical Brimonidine Tartrate.

Clinical efficacy of 595-nm pulsed-dye laser in treatment of childhood facial spider nevi: a retrospective study of 110 patients / 中华医学杂志(英文版)

Background@#Spider nevi (SN) are quite common in children. SN are treated via different techniques, and complete removal often requires multiple treatments. However, few studies have evaluated the treatment of SN. The present study aimed to evaluate the therapeutic effect and safety of a 595-nm pulsed-dye laser (PDL) for treating facial SN in children.@*Methods@#A total of 110 children aged 0.2 to 12 years with facial SN were treated with a 595-nm PDL in a single institution from January 2016 to February 2018. In accordance with the treatment method, the patients were retrospectively divided into the small-spot-combined-with-large-spot group (SL-group) and the large-spot group (L-group). Patients with poor therapeutic results were retreated every 6 weeks until the lesions disappeared. The minimum follow-up period was 1 year. The groups were compared using independent-samples t tests, Mann-Whitney U test, Chi-square test, and Fisher exact probability test.@*Results@#The therapeutic efficacy was significantly higher in the SL-group than in the L-group, with clearance rates of 90.9% and 53.0% after the primary treatment, respectively (χ2= 17.937, P < 0.001). For skin lesions with a central spider body diameter ≥1 mm, the once-treatment cure rates were 100% in the SL-group and 34.8% in the L-group (χ2 = 20.780, P < 0.001). For skin lesions with a central spider body diameter <1 mm, the once-treatment cure rates were 82.6% in the SL-group and 62.8% in the L-group (χ2 = 3.961, P = 0.138). The rates of adverse reactions and recurrence did not differ between the two groups (P = 0.141 and P = 1.000, respectively).@*Conclusions@#The 595-nm PDL might be a safe and effective treatment option for facial SN in children. The small-spot-combined-with-large-spot method is especially suitable for SN with a central spider body diameter ≥1 mm.

Dosis atípica de bevacizumab en un paciente con síndrome de Osler-Weber-Rendu / Atypical dose of bevacizumab in a patient with Osler-Weber Rendu syndrome

Resumen El síndrome de Osler-Weber-Rendu es una enfermedad de transmisión autosómica dominante. Si bien el síntoma más común es la epistaxis, existen muchas alteraciones viscerales que deben considerarse al momento del estudio. Los anti-VEFG son un pilar en el tratamiento, aunque no existe consenso acerca de la dosis a administrar. Comunicamos el caso de un paciente con síndrome de Osler-Weber-Rendu atendido en el servicio de Medicina Interna del Hospital Regional de Puebla, al que se prescribió una dosis atípica de bevacizumab con buena respuesta terapéutica.

Abstract Osler-Weber-Rendu syndrome is an autosomical dominant condition. Although epistaxis is the most frequent symptom, many other visceral implications have to be considered while the workup takes place. While Anti-VEGF are the cornerstone of the treatment, there is no consensus regarding the specific dose to be administered. We communicate de case of a patient with Osler-Weber-Rendu syndrome attended at our Hospital in which we prescribed an atypical dose of bevacizumab with good outcome.

Progress in diagnosis and treatment of adult Coats disease / 中华眼底病杂志

Adult Coats disease is characterized by abnormal expansion of retinal capillaries,often accompanied by massive lipid exudation and exudative retinal detachment.Unlike Coats disease in young children,adult Coats disease is mostly limited to peripheral retina,with slow progress and better prognosis.Adult Coats disease should be identified with Coats-like diseases such as exudative age-related macular degeneration,diabetic retinopathy,obsolete retinal vein occlusion,idiopathic macular telangiectasia 1,obsolete posterior uveitis,retinal vasculitis,or acute retinal necrosis.Because the pathogenesis of Coats disease is not clear,it lacks specific treatment measures for the cause of disease.The purpose of simple or combined laser photocoagulation,freezing,vitreous intravitreal injection against vascular endothelial growth factor drugs or triamcinolone and surgery is to eliminate abnormal blood vessels and exudation,maintain visual function,which can also improve retinal detachment and prevent neovascular glaucoma and other complications.To explore the similarities and differences of adult Coats disease with Coats disease in young children,to further promote the study of the pathogenesis of adult Coats disease and to provide new targets for its treatment are the direction of future research.

Appropriate understanding the application of anti-vascular endothelial growth factor agents and laser photocoagulation for infants retinal vascular diseases / 中华眼底病杂志

Retinopathy ofprematurity,familial exudative vitreoretinopathy and Coats disease are the most common neonates and infants retinal vascular diseases,which may lead to severe visual damage because of either tractional retinal detachment caused by the proliferation of pathogenic neovascularization,or exudative retinal detachment due to the extremely leakage from abnormal retinal vessels.Classic treatment is retinal laser photocoagulation which could destroy these abnormal vessels or reduce non vascular areas to diminish the growth of new vessels,however the side effects induced by laser it self such as visual field damage,hemorrhage,retinal tear,fail to control the progression of the disease make the laser treatment hard to improve the vision of these young patients.Anti-vascular endothelial growth factor (VEGF) agents have been widely applied in various adult retinal and choroidal vascular diseases,they are even possible to replace the pan retinal photocoagulation in proliferative diabetic retinopathy,while there are still many unsolved problems in the applying in neonates and infants retinal vascular diseases,like dosage,timing,retreatment and systemic side effects.We should realize the importance of selecting the laser photocoagulation and anti-VEGF for neonates and infants retinal vascular diseases.

Fluorescein Angiographic Abnormalities in the Contralateral Eye with Normal Fundus in Children with Unilateral Coats' Disease

PURPOSE: We report the detection of peripheral retinal vascular abnormalities in the fellow eye with normal fundus in children with unilateral Coats' disease. METHODS: The clinical records of patients diagnosed with Coats' disease were retrospectively reviewed. We recorded the subjects' characteristics and obtained fundus photography and fluorescein angiography (FA) images. The main outcome measure was peripheral vascular abnormalities in the contralateral eye with normal fundus in children with unilateral Coats' disease, observed with FA. RESULTS: Out of 47 patients with Coats' disease, two (4.3%) were diagnosed with clinically bilateral Coats' disease. Of the 45 patients with presumed unilateral Coats' disease, four (8.9%) had bilateral abnormal peripheral vasculature in FA. The mean age of these four patients was 6.4 ± 5.4 years (range, 1 to 14 years), and three patients were male (75%). All four had peripheral retinal nonperfusion, and two (50%) received laser photocoagulation due to peripheral leakage with telangiectatic vessels. CONCLUSIONS: Coats' disease may more often be a bilateral disease with asymmetry than previously thought. Patients with Coats' disease should undergo careful examination of the fellow eye with FA in order to detect and treat vascular abnormalities that are not visible clinically.

Coats disease-like retinopathy and inherited metabolic disorders or syndromes / 中华眼底病杂志

Coats disease is a relatively rare and idiopathic disorder characterized by retinal telangiectasia and massive intra-retinal and (or) sub-retinal lipid accumulation, resulting in complications including retinal detachment and neovascular glaucoma. Previous reports have revealed that Coats disease can be associated with other disorders, especially some inherited diseases, such as retinitis pigmentosa (RP) and facioscapulohumeral muscular dystrophy (FSHD). Coats disease associated with other inherited disorders is generally called Coats-like retinopathy, which has some unique features that differs from the classic Coats disease, for example there is no sex and age preference, more bilateral cases, more severe cases and more genetic factors involved. Patients of Coats-like retinopathy with RP and FSHD may have mutations in Crumbs homologue gene 1 and D4Z4 genes.

Tratamiento quirúrgico de rinofima. Presentación de dos casos / Surgical treatment of rhinophyma. Report of two cases

El rinofima es una enfermedad de la piel de la nariz, consistente en una hipertrofia de las glándulas sebáceas locales que conlleva a una deformación característica. Se presenta con hipertrofia de la piel, inflamación crónica, aumento del tejido vascular, con múltiples telangiectasias y comedones, todo lo cual ocasiona una punta de la nariz deformada. Afecta con más frecuencia al sexo masculino y puede llegar a malignizarse. Se presentan dos pacientes afectados por rinofima, que fueron atendidos en el Servicio de Cirugía Maxilofacial del Hospital Universitario Dr. Gustavo Aldereguía Lima de Cienfuegos. Se describe la técnica quirúrgica empleada en ambos casos. Se trata de una enfermedad rara, por lo que se considera de interés su estudio y divulgación.

Rhinophyma is a nose skin disease consisting on a hypertrophy of the local sebaceous glands which leads to a characteristic deformation. It presents with skin hypertrophy, chronic inflammation and increase of the vascular tissue, with multiple telangiectasias y comedones, all of which causes a deformed tip of the nose. It more often affects the male sex and can get to a malignancy. Two patients affected by rhinophyma are presented who were assisted at the maxillofacial service of the General Teaching Hospital Dr Gustavo Aldereguía Lima, Cienfuegos. The surgical technique used in both cases is described. It is a rare disease therefore it is considered of interest for study and dissemination.

The status and progress of studies of idiopathic parafoveal telangiectasis / 中华眼底病杂志

Idiopathic parafoveal telangiectasis (IPT) is a retinal vascular disease which is characterized by foveal and parafoveal telangiectasia.The main clinical manifestations are retinal telangiectasis,reduced retinal transparency,retinal venular dilatation,yellow exudation,retinal pigment epithelial lesions,retinal hemorrhage,macular atrophy,macular hole or lamellar hole,subretinal neovascularization and retinal detachment.According to the clinical characteristics and features of fluorescein angiography,IPT can be divided into 3 types and 6 subtypes.Laser photocoagulation,photodynamic therapy,and intravitreal injection of glucocorticoid or anti-vascular endothelial growth factor drugs,can reduce the macular edema and neovascularization.However,due to the unclear etiology of IPT,the existing treatment measures are not specific for its etiology.We need to work hard to understand further the clinical features and pathogenesis of IPT and search the targeted treatments based on its pathogenesis mechanism.