What do Cochrane systematic reviews say about congenital vascular anomalies and hemangiomas? A narrative review

ABSTRACT BACKGROUND: Congenital vascular anomalies and hemangiomas (CVAH) such as infantile hemangiomas, port-wine stains and brain arteriovenous malformations (AVMs) impair patients' lives and may require treatment if complications occur. However, a great variety of treatments for those conditions exist and the best interventions remain under discussion. OBJECTIVE: To summarize Cochrane systematic review (SR) evidence on treatments for CVAH. DESIGN AND SETTING: Review of SRs conducted in the Division of Vascular and Endovascular Surgery of Universidade Federal de São Paulo, Brazil. METHODS: A broad search was conducted on March 9, 2021, in the Cochrane Database of Systematic Reviews to retrieve any Cochrane SRs that assessed treatments for CVAH. The key characteristics and results of all SRs included were summarized and discussed. RESULTS: A total of three SRs fulfilled the inclusion criteria and were presented as a qualitative synthesis. One SR reported a significant clinical reduction of skin redness by at least 20%, with more pain, among 103 participants with port-wine stains. One SR reported that propranolol improved the likelihood of clearance 13 to 16-fold among 312 children with hemangiomas. One SR reported that the relative risk of death or dependence was 2.53 times greater in the intervention arm than with conservative management, among 218 participants with brain AVMs. CONCLUSION: Cochrane reviews suggest that treatment of port-wine stains with pulsed-dye laser improves redness; propranolol remains the best option for infantile hemangiomas; and conservative management seems to be superior to surgical intervention for treating brain AVMs.

Clinical significance of lesion distribution in children with Sturge-Weber syndrome / 中华皮肤科杂志

Objective:To analyze distribution characteristics of facial port-wine stains and brain imaging features in children with Sturge-Weber syndrome (SWS) .Methods:Clinical and imaging data were collected from 22 children with confirmed SWS at Department of Dermatology, Children′s Hospital Affiliated to Capital Institute of Pediatrics from July 2017 to August 2020, and retrospectively analyzed. The distribution characteristics of port-wine stains along the facial trigeminal nerve and brain imaging features were investigated in these children with SWS.Results:Among the 22 children, 10 were males and 12 were females. Their age ranged from 0.08 to 9.92 years, and the median age was 1.67 years. There were 13 cases of SWS type Ⅰ and 9 cases of SWS type Ⅱ. In terms of the port-wine stain type, 4 children aged from 0.50 to 2.17 years were diagnosed with pink port-wine stains; 14 children aged from 0.08 to 8.83 years were diagnosed with purple port-wine stains; 4 children aged from 4.92 to 9.92 years were diagnosed with thickened port-wine stains. Port-wine stains were distributed in the ophthalmic (V1) division of the trigeminal nerve in 22 patients, in the maxillary (V2) division in 20 patients, and in the mandibular (V3) division in 8 patients. There were 17 children with ocular abnormalities, aged from 0.08 to 9.92 years, including 11 with glaucoma, 5 with elevated intraocular pressure and 2 with visual impairment. Among the children with glaucoma, 7 developed glaucoma within 2 years of age, 8 suffered from unilateral glaucoma, and 3 from bilateral glaucoma, and glaucoma occurred on the same side as port-wine stains. Brain imaging abnormalities were observed in 12 children, and mainly included vascular malformations in the cerebral cortex involving the frontal, parietal, temporal and occipital lobes, as well as cerebral atrophy, punctate hemorrhage, calcification, sulcal widening, midline deviation and increased intraventricular choroid plexus vessels. Eleven children had symptoms of epilepsy and other neurological manifestations, including developmental delay, mental retardation and physical impairment.Conclusion:Port-wine stains distributed in the V1 and V2 divisions of the facial trigeminal nerve may indicate a higher risk of SWS, and ophthalmic and brain imaging screening as well as long-term follow-up should be performed.

Efficacy of medical cold patches in relieving burning pain and restoring skin homeostasis after hematoporphyrin monomethyl ether-based photodynamic therapy for the treatment of port-wine stains / 中华皮肤科杂志

Objective:To evaluate the efficacy of medical cold patches in relieving burning pain and restoring skin homeostasis after hematoporphyrin monomethyl ether-based photodynamic therapy (HMME-PDT) for the treatment of port-wine stains.Methods:Forty patients with port-wine stains in the middle face, who met the inclusion and exclusion criteria, were collected from Department of Dermatology, the Seventh Medical Center of Chinese PLA General Hospital from November 2019 to April 2021, and randomly and equally divided into test group and control group. Patients in the test group received cold compress with medical cold patches at treatment sites for 1 hour immediately after HMME-PDT, and then once a day for 3 consecutive days, while those in the control group received no special treatment and experienced a spontaneous recovery. Pain numeric rating scale (NRS) scores were recorded immediately, 0.5, 1 and 12 hours after HMME-PDT. Skin surface temperature was measured 10 minutes before, and immediately, 30 minutes and 1 hour after HMME-PDT. Transepidermal water loss (TEWL) and water content of the stratum corneum (WCSC) were measured 10 minutes before, and immediately, 24, 48 and 72 hours after HMME-PDT. The scabbing rate was calculated at weeks 1, 2 and 3 after HMME-PDT. Two-way repeated measures analysis of variance was used for comparisons of observation indicators at different time points before and after treatment, and Bonferroni or Sidak′s test was used for comparisons between groups and within groups.Results:There were no significant differences in age, gender composition, TEWL or WCSC between the test group and control group before HMME-PDT (all P > 0.05) . Immediately after HMME-PDT, no significant difference in the NRS score was observed between the test group and control group (8.00 ± 1.17 vs. 8.20 ± 1.06, F = 0.30, P = 0.592) ; at 0.5 and 1 hour after HMME-PDT, the NRS score was significantly lower in the test group (6.25 ± 1.29, 4.80 ± 0.77, respectively) than in the control group (7.15 ± 0.99, 6.50 ± 0.69, respectively, both P < 0.05) . Immediately after HMME-PDT, the skin surface temperature in the test group and control group increased to 35.21 ± 1.333 ℃ and 35.64 ± 0.832 ℃, respectively, and there was no significant difference between the two groups ( P = 0.062) ; at 30 and 60 minutes after HMME-PDT, the skin surface temperature in the test group was 29.11 ± 1.59 ℃ and 32.46 ± 1.07 ℃ respectively, which were significantly lower than those in the control group (35.01 ± 0.91 ℃, 34.86 ± 0.74 ℃, F = 212.63, 100.20, respectively, both P < 0.001) . At 48 and 72 hours after HMME-PDT, the TEWL in the test group was 12.44 ± 0.67 g·h -1·m -2 and 10.85 ± 0.81 g·h -1·m -2 respectively, which were significantly lower than those in the control group (14.61 ± 0.34 g·h -1·m -2, 14.93 ± 0.24 g·h -1·m -2, F = 195.87, 520.54, respectively, both P < 0.001) , while the WCSC was significantly higher in the test group (57.83 ± 9.29 AU, 52.64 ± 8.09 AU, respectively) than in the control group (43.87 ± 4.82 AU, 38.68 ± 5.33 AU, F = 24.41, 49.22, respectively, both P < 0.001) . At 1 week after HMME-PDT, scab formation was observed in 3 cases in the test group, as well as in 6 cases in the control group, and there was no significant difference in the scabbing rate between the two groups ( P = 0.451) . Conclusion:The application of medical cold patches after HMME-PDT for the treatment of port-wine stains can reduce skin surface temperature, exert analgesic effects, shorten duration of postoperative pain, and promote the recovery of skin permeability barrier function.

Clinical and imaging features of 27 cases of childhood Sturge-Weber syndrome / 中华皮肤科杂志

Objective:To analyze clinical and imaging features of Sturge-Weber syndrome in children.Methods:Clinical data were collected from 27 children with Sturge-Weber syndrome in Xuzhou Children′s Hospital, Xuzhou Medical University from July 2013 to December 2019, and analyzed retrospectively.Results:Among the 27 children, 17 were males and 10 were females. Their age at the clinic visit ranged from 2 days to 10 years and 7 months, and averaged 2.54 years. All the 27 patients presented with facial port-wine stains of varied color from light red to purple red, which were all distributed across the facial midline, including 21 with predominantly unilateral port-wine stains and 6 with bilateral symmetrical port-wine stains. There were 17 patients with ocular choroidal vascular malformations, including 14 with congenital glaucoma, 5 with high intraocular pressure, and 1 with optic nerve atrophy accompanied by transient blindness. Neurological impairment occurred in 12 patients, and all manifested as epilepsy. All the 27 children underwent imaging examination, and abnormalities were found in 20. Among the 10 patients with abnormal computed tomography images, local calcification was observed in 8, and local thickening of the skull on the side affected by skin lesions in 8; 13 of 14 patients with abnormal magnetic resonance imaging scan results had signs of brain atrophy, 9 showed enhanced gyrus-like blood vessel formation by enhanced magnetic resonance imaging, and 5 showed decreased branches of the anterior and middle cerebral artery on the affected facial side by magnetic resonance angiography.Conclusions:Children with Sturge-Weber syndrome are clinically characterized by predominantly unilateral port wine stains on the face, some of whom are accompanied by epilepsy, glaucoma or mental retardation, and imaging examinations mainly show local calcification, brain atrophy, local thickening of the skull plate, enhanced gyrus-like blood vessel formation, etc. Early definite diagnosis and comprehensive systemic treatment are needed to reduce disability and mortality rates in patients with Sturge-Weber syndrome, and long-term follow-up should be considered.

Polydocanol percutaneous sclerotherapy for the treatment of giant venular malformation of the lips and cheeks in adults / 口腔疾病防治

Objective @#To investigate the clinical effect of polidocanol sclerotherapy in the treatment of giant venular malformations of the lips and cheeks in adults.@*Methods@# From September 2019 to September 2020, 5 patients with huge venular malformations of the lips and cheeks (4 males, 1 female) admitted to Xuzhou Central Hospital were included in the study. All the patients were treated with local injection of polidocanol foam scleroagent, and all patients were followed up with a 3-week treatment course. If the clinical symptoms were not alleviated and the MRI examination showed that > 25% of the lesion remained, or it relapsed again after symptoms are stable, the patient needed to be treated again. The endpoints of treatment were: ①subsidence of clinical symptoms and MRI showing residual lesions < 25% in size; ②continuous treatment for 4 times without relief or aggravation of symptoms; ③a discontinuation of treatment. @* Results@#All 5 patients successfully completed the treatment and were injected 2 to 4 times during treatment. The curative effect was evaluated according to the Achauer standard, including grade Ⅰcurative effects in 1 patient, grade Ⅱ in 2 patients, grade Ⅲ in 2 patients. Among them, one patient suffered from erosion and bleeding in the lesion before the operation, and the symptoms were significantly improved postoperatively. No serious side effects were found except skin pigmentation in 1 case. @*Conclusion@#Local injection of polidocanol foam scleroagent is a safe and effective treatment method for adult giant venular malformations of the lips and cheeks, and it has a hemostatic effect on spontaneous bleeding invenular malformations.

Facomatosis tipo Cesioflammea: Reporte de caso de neonato con Manchas Mongólicas y Vino oporto. Una presentación simple de una condición dermatológica compleja.

La facomatosis pigmentovascular es un conjunto de signos y síntomas caracterizado por la coexistencia de malformaciones vasculares capilares con una lesión pigmentaria cutánea congénita (nevo epidérmico, nevo spilus o melanosis dérmica) asociada o no, a manifestaciones sistémicas. Existen varios grupos de facomatosis pigmentovasculares según el tipo de nevo asociado a la lesión capilar vascular. Puede existir solo la afectación cutánea o tener manifestaciones sistémicas, entre ellas, traumatológicas, neurológicas, oftalmológicas, inmunológicas, renales, vasculares, linfáticas, entre otras. Exponemos el caso de un neonato masculino, quien presenta manchas vino oporto facial bilateral, cuello y tórax anterior y posterior, junto a melanocitosis dérmicas en espalda y ambos miembros inferiores, asociado a glaucoma congénito del ojo derecho. Debido a las manifestaciones clínicas cutáneas y sistémicas, se diagnostica facomatosis pigmentovascular, realizándose evaluación multidisciplinaria por radiología, dermatología, oftalmología, genética y neurología, para descartar afectación extracutánea. Los estudios complementarios son de vital importancia para detectar alteraciones extracutáneas, como se presentaron en el paciente.

Phakomatosis pigmentovascularis is set of signs and symptoms characterized by the association of a vascular nevus with a congenital pigmented lesion (epidermal nevus, nevus spilus, and dermal melanocytosis) associated or not to systemic malformations. There are different types of phakomatosis pigmentovascularis according to the pigmentary nevus associated with the vascular malformation. There may be only the cutaneous condition or have systemic manifestations such as, trauma, neurological, ophthalmological, inmunological, renal, vascular, lymphatic, among others disorders. We present the case of a male newborn who had a port wine stain located bilaterally on the face, anterior and posterior thorax and neck, together with dermal melanocitosis in the back and lower extremities, associated with congenital glaucoma of the right eye. Due the clinical manifestations, the diagnosis of phakomatosis pigmentovascularis was made and multidisciplinary evaluation is requested by radiology, dermatology, ophthalmology, genetics and neurology to rule out extracutaneous involvement. Complementary studies are essential to detect extracutaneous alterations as presented in the patient.

Sturge-Weber Syndrome – A Case Report

Encephalo-trigeminal angiomatosis otherwise known as struge Weber syndrome is characterised by port-wine stain. Here wereport a case with typical charecteristics of the same and description about the patients clinical presentations

Clinical efficacy of hematoporphyrin monomethyl ether-based photodynamic therapy for the treatment of port-wine stain and its sonographic changes / 中华皮肤科杂志

Objective@#To evaluate the clinical efficacy of hematoporphyrin monomethyl ether-based photodynamic (HMME-PDT) therapy for the treatment of port-wine stain (PWS) and its sonographic changes.@*Methods@#A total of 45 patients with confirmed PWS were enrolled from the Department of Dermatology, Third Affiliated Hospital of Soochow University from March 2017 to June 2018, including 5 with pink PWS, 39 with purplish red PWS and 1 with thickened PWS. All the patients received 3 sessions of HMME-PDT therapy. The skin thickness and density were compared before and after the treatment by using high-frequency ultrasound. Ranked data were analyzed by using nonparametric test. Measurement data were expressed as mean ± standard deviation, and analyzed using ony-way analysis of variance. Multiple comparisons were performed using Student-Newman-Keuls-q (SNK-q) test. The results were considered to be statistically significant if P < 0.05.@*Results@#Among the 45 patients with PWS who completed the treatment and follow-up, 10 were cured, 21 received marked improvement, 12 received improvement, and 1 showed no response. The total response rate was 97.78%, and the response rate in the patients with pink PWS was higher than that in the patients with purplish red PWS (U = 12.50, P < 0.001) . The difference value of the skin thickness or skin density before and after the treatment significantly differed among the cured patients, patients receiving marked improvement and those receiving improvement (skin thickness:0.65 ± 0.21, 0.56 ± 0.88, 0.37 ± 0.12 mm respectively; skin density: -8.65 ± 2.19, -6.86 ± 2.79, -4.92 ± 2.91 g/cm3 respectively; F = 14.528, 5.428 respectively, both P < 0.001) , and the difference values of the skin thickness and density were significantly higher in the cured patients than in those receiving improvement (q = 5.82, 4.63, both P < 0.05) . Erythematous swelling to different extents occurred at the laser-exposed sites in the zygomatic and cheek region in 23 patients with PWS and in the frontal-zygomatic region in 6 with PWS after the HMME-PDT therapy, but gradually regressed about 1 week later. Pale brown crusts were observed at the laser-exposed sites in 35 patients, and shed spontaneously about 3 weeks later. Post-inflammatory hyperpigmentation at the laser-exposed sites was observed in 4 patients, and gradually regressed after 2-month follow-up.@*Conclusions@#HMME-PDT therapy is effective for the treatment of PWS, with high safety and few adverse reactions. High-frequency ultrasound can be used for objectively evaluating the clinical efficacy of HMME-PDT therapy.

Clinical efficacy of hematoporphyrin monomethyl ether-based photodynamic therapy for the treatment of port-wine stain and its sonographic changes / 中华皮肤科杂志

Objective To evaluate the clinical efficacy of hematoporphyrin monomethyl etherbased photodynamic (HMME-PDT) therapy for the treatment of port-wine stain (PWS) and its sonographic changes.Methods A total of 45 patients with confirmed PWS were enrolled from the Department of Dermatology,Third Affiliated Hospital of Soochow University from March 2017 to June 2018,including 5 with pink PWS,39 with purplish red PWS and 1 with thickened PWS.All the patients received 3 sessions of HMME-PDT therapy.The skin thickness and density were compared before and after the treatment by using high-frequency ultrasound.Ranked data were analyzed by using nonparametric test.Measurement data were expressed as mean ± standard deviation,and analyzed using ony-way analysis of variance.Multiple comparisons were performed using Student-Newman-Keuls-q (SNK-q) test.The results were considered to be statistically significant if P ＜ 0.05.Results Among the 45 patients with PWS who completed the treatment and follow-up,10 were cured,21 received marked improvement,12 received improvement,and 1 showed no response.The total response rate was 97.78％,and the response rate in the patients with pink PWS was higher than that in the patients with purplish red PWS (U =12.50,P ＜ 0.001).The difference value of the skin thickness or skin density before and after the treatment significantly differed among the cured patients,patients receiving marked improvement and those receiving improvement (skin thickness:0.65 ± 0.21,0.56 ± 0.88,0.37 ± 0.12 mm respectively;skin density:-8.65 ± 2.19,-6.86 ± 2.79,-4.92 ± 2.91 g/cm3 respectively;F =14.528,5.428 respectively,both P ＜ 0.001),and the difference values of the skin thickness and density were significantly higher in the cured patients than in those receiving improvement (q =5.82,4.63,both P ＜ 0.05).Erythematous swelling to different extents occurred at the laser-exposed sites in the zygomatic and cheek region in 23 patients with PWS and in the frontal-zygomatic region in 6 with PWS after the HMME-PDT therapy,but gradually regressed about 1 week later.Pale brown crusts were observed at the laser-exposed sites in 35 patients,and shed spontaneously about 3 weeks later.Post-inflammatory hyperpigmentation at the laser-exposed sites was observed in 4 patients,and gradually regressed after 2-month follow-up.Conclusions HMME-PDT therapy is effective for the treatment of PWS,with high safety and few adverse reactions.High-frequency ultrasound can be used for objectively evaluating the clinical efficacy of HMME-PDT therapy.

Tratamiento con Pulsed Dye Laser de mancha de vino oporto: seguimiento a 10 años / Treatment with Pulsed Dye Laser of wine stain Porto: 10-year follow-up

Las manchas de vino de oporto son un tipo de malformación capilar que afecta del 0,3 al 0,5% de la población. Están presentes desde el nacimiento como máculas o placas eritematosas en la piel o mucosas. Sin tratamiento, las lesiones tienden a oscurecerse con la edad, tornándose rojizas o color púrpura, y pueden desarrollar engrosamiento nodular o un granuloma piógeno asociado. Los tratamientos con láser proporcionan mejoría mediante la destrucción selectiva de la vasculatura. Se han utilizado una variedad de láseres vasculares selectivos, pero el tratamiento de primera elección es el láser de colorante pulsado. Los mejores resultados se obtienen cuando el tratamiento es instaurado tempranamente. (AU)

Port wine stains are a type of vascular malformation that affects 0.3% to 0.5% of the population. They are present from birth as erythematous macules or plaques on the skin or mucous membranes. Without treatment, these lesions tend to darken with age, becoming reddish or purple and may develop nodular thickening or an associated pyogenic granuloma. Laser treatments might provide an improvement by selective destruction of the vasculature. A variety of selective vascular lásers may be employed, with the pulsed dye laser as the gold standard treatment. Better results are obtained when the treatment is established early. (AU)

Recurrent pyogenic granuloma associated with port-wine stain: a case report / Granuloma Piogênico recorrente associado à Mancha Vinho do Porto: um relato de caso

O granuloma piogênico (GP) é um processo proliferativo não neoplásico. Este termo entretanto, é equivocado, uma vez que não está relacionado a qualquer infecção, não contém pus e não é um verdadeiro granuloma. A ocorrência do GP recorrente associado a lesões vasculares na pele, tais como manchas de vinho do porto, tem sido descrita na literatura. Paciente do gênero masculino com 23 anos de idade foi atendido na Universidade Federal do Maranhão apresentando uma lesão com características de granuloma piogênico na vestibular do primeiro molar, associada à mancha vinho do porto. O diagnóstico foi confirmado através de biopsia excisional e análise histopatológica. Devido à recorrência da lesão após a abordagem cirúrgica, injeções intralesionais de corticoides foram realizadas três vezes. Após dois anos de acompanhamento, não houve recorrência. Pode-se concluir que, apesar da alta prevalência de recorrência do granuloma piogênico, injeções intralesionais com corticoides podem ser uma alternativa ao tratamento cirúrgico convencional.

Pyogenic granuloma (PG) is a non-neoplastic proliferative lesion. This term, however, is a misconception, since it is not related to any infection, does not contain pus and it is not a true granuloma. The occurrence of recurrent PG associated with vascular lesions on the skin, such as port-wine stains, has been described in the literature. A 23 years old male patient was attended in the Federal University of Maranhão presenting a lesion with pyogenic granuloma characteristics in the buccal side of the first molar associated with port-wine stain. The diagnosis was confirmed per an excisional biopsy, along with histopathologic analysis. Due to the recurrence of the lesion after the surgical approach, intralesional corticosteroid injections was performed three times. After two-years no signs of recurrence could be observed. In conclusion, despite of the high prevalence of Pyogenic Granuloma recurrence, intralesional corticosteroid injections may be an alternative option to conventional surgical treatment.

Analysis of ultrasound images of port-wine stains / 中华皮肤科杂志

Objective To investigate ultrasound features of port-wine stains (PWS),and to evaluate the diagnostic value of ultrasound imaging.Methods A total of 128 patients with pathologically or clinically confirmed PWS (162 lesions),who were also subjected to ultrasound examination,were collected from Shanghai Ninth People's Hospital affiliated to Shanghai Jiao Tong University School of Medicine between January 2015 and January 2016.According to ultrasound findings,these PWS lesions were divided into 3 types:flat type,hypertrophic type and nodular type,and the ultrasound features were retrospectively analyzed.Results For 95 patients with flat PWS (118 lesions),the skin thickness was significantly higher in the lesion areas than in the normal areas,but most of the thickness difference was less than 0.2 mm.Of the 118 lesions,79 (67％) showed hypoechoic areas,75 (64％) showed no blood flow signal,and the vein spectrum was detected in 15,with a peak of (3.33 ± 1.80) cm/s.For 17 patients with hypertrophic PWS (24 lesions),the lesion areas showed significantly increased skin thickness compared with the normal areas (1.80 ± 0.70 mm vs.1.14 ± 0.43 mm,t =6.834,P ＜ 0.001).All (100％) of the 24 lesions showed hypoechoic areas,low blood flow signals were observed in 15 (62％),and the vein spectrum was detected in 18 lesions,with a peak velocity of (6.61 ± 3.87) cm/s.For 16 patients with nodular PWS (20 lesions),the skin thickness in the lesion areas was (6.45 ± 4.68) mm.Of the 20 lesions,18 (90％) showed hypoechoic areas,and abundant blood flow signals were observed in 15 (75％).Additionally,the vein spectrum was detected in 15 lesions,with a peak velocity of (10.00 ± 5.39) cm/s,and the artery spectrum was detected in 19 lesions,with a systolic peak velocity of (24.58 ± 13.82) cm/s and a resistance index of 0.59 ± 0.13.The skin lesions was significantly thicker in the hypertrophic PWS group and nodular PWS group than in the flat PWS group (both P ＜ 0.05),and significantly thicker in the nodular PWS group than in the hypertrophic PWS group (P ＜ 0.05).The peak velocity of vein spectrum significantly differed among the 3 groups (F =10.630,P ＜ 0.001),and was significantly higher in the hypertrophic PWS group and nodular PWS group than in the flat PWS group (both P ＜ 0.05).However,no significant difference was observed between the hypertrophic PWS group and nodular PWS group (P ＞ 0.05).Conclusion Ultrasonography is,to a certain extent,valuable in the diagnosis of PWS.

Clinical Presentation and the Treatment of Glaucoma in Patients with a Facial Port-wine Stain

PURPOSE: To characterize the development of glaucoma, age of glaucoma onset, and treatments for patients with a facial port-wine stain (PWS). METHODS: We performed a retrospective analysis of the medical records of 58 patients (116 eyes) with facial PWS between January 2000 and August 2016. We noted patients' age at the initial examination, cup-to-disc ratio, corneal diameter, occurrence of ocular hypertension, development of glaucoma, age of glaucoma onset, and treatments. We compared the clinical features of eyes that developed glaucoma with those that did not develop glaucoma. Among those eyes with glaucoma, we investigated the differences between eyes that underwent surgery and those that did not undergo surgery. RESULTS: Among the 58 patients with a facial PWS (116 eyes), glaucoma was diagnosed in 38 patients (46 eyes; 39.66%). Of these, 26 patients (27 eyes; 58.69%) underwent glaucoma surgery. PWS-associated glaucoma usually developed by the age of 2 years (85.61%). In all patients, glaucoma developed on the same side of the face as the PWS. Of the 58 patients, 19 (32.76%) showed neurological symptoms, including seizures, developmental delays, intellectual disabilities, or hemiplegia, and 32 (55.17%) were diagnosed with Sturge-Weber syndrome. The mean number of glaucoma surgeries was 1.55 ± 0.93. The initial surgery included trabeculectomy (7 eyes), trabeculotomy (5 eyes), combined trabeculotomy/trabeculectomy (13 eyes), and aqueous drainage device insertion (2 eyes). The mean age at the first surgery was 35.14 ± 50.91 months. In 18 of 27 eyes (66.67%), the postoperative intraocular pressure (IOP) was controlled to below 21 mmHg, but 9 eyes (33.33%) showed elevated IOP and required a reoperation. CONCLUSIONS: PWS can be accompanied by ocular hypertension or glaucoma, so patients require regular ophthalmic examinations. When glaucoma occurs, it often does not respond to medication, making it difficult in some cases to control the IOP, so appropriate glaucoma surgery is necessary.

Phacomatosis pigmentovascularis of cesioflammea type

Abstract Phacomatosis pigmentovascularis is a rare syndrome, defined as the simultaneous presence of vascular nevus and melanocytic nevus in the same patient. We report the case of a 53-year-old woman presented with dermal melanosis and extensive vascular nevus, which match the typical manifestations of phakomatosis pigmetovascularis of cesioflammea type, according to Happle's classification. The rare occurrence of this genodermatosis and the clinical exuberance of the skin lesions motivated this case report.

Treatment of intractable port-wine stains / 中华皮肤科杂志

Port-wine stains(PWS)are a common congenital capillary malformation. The lesions of PWS appear to gradually darken in color and thicken with age, and even progress into nodules in late stage. Based on the principles of selective photothermolysis, pulsed dye laser (PDL)has become a standard treatment for PWS. However, for some intractable PWS, single PDL treatment shows unsatisfactory and even no effects. Considering this state, the authors summarize multiple treatment strategies for intractable PWS with thick or nodular lesions, providing a reference for their treatment.

Clinical comparison between photodynamic therapy and pulsed dye laser for the treatment of port wine stains / 中华皮肤科杂志

Objective To compare the clinical efficacy and adverse effects of photodynamic therapy (PDT) versus pulsed dye laser(PDL)for the treatment of port wine stains(PWS). Methods Forty?five patients with PWS were enrolled in this study. The PWS lesions in each patient were randomly divided into PDT and PDL areas. Hematoporphyrin monomethyl ether of 5 mg/kg was injected intravenously into the PDT area protected from light, followed by 20?minute irradiation with a 532?nm, solid?state, continuous?wave laser(power density:80-100 mw/cm2;spot diameter: 7 cm)10 minutes later. The PDL area was treated with a single session of 595?nm pulsed dye laser radiation(spot diameter:7 mm;pulse width:10 ms;energy density:10-12 J/cm2). The interval between PDT and PDL treatment was no shorter than two months. Follow up visits were scheduled on day 4 and week 8 after each treatment. Adverse reactions were recorded, and photographs were taken before and 8 weeks after the treatment for evaluation of lesion regression. Results In the case of PDT area, 10 cases(22.22%)were nearly cured, 22(48.89%)achieved marked improvement, 9(20.00%)improvement, 4(8.89%)no improvement. As far as the PDL area is concerned, 6 cases(13.33%)were nearly cured, 16(35.56%)achieved marked improvement, 18(40.00%)improvement, and 5 (11.11%)no improvement. The response rate was significantly higher in the PDT area than in the PDL area(Z=2.48, P0.05). Conclusion For the treatment of PWS, both PDT and PDL are effective and safe, and single?session PDT appears to be superior to single?session PDL.

The case of a boy with nevus of Ota, extensive Mongolian spot, nevus flammeus, nevus anemicus and cutis marmorata telangiectatica congenita: a unique instance of phacomatosis pigmentovascularis

Phacomatosis pigmentovascularis is a rare, congenital condition characterized by a combination of cutaneous melanocytic lesions and vascular malformation. We discuss an entirely unique case of Phacomatosis pigmentovascularis with nevus of Ota, extensive Mongolian spot, nevus flammeus, nevus anemicus and cutis marmorata telangiectatica congenita, which may represent a heretofore undescribed variant of phacomatosis pigmentovascularis.

Bilateral Sturge-Weber syndrome and glaucoma controlled with ahmed valve implant

Sturge-Weber Syndrome is a rare neuro-oculocutaneous disorder. The authors describe the case of a 13 years old boy, presented with bilateral Sturge-Weber Syndrome and glaucoma. Surgical treatment with Ahmed valve implantation in both eyes was carried out achieving lower levels of intraocular pressure.

A síndrome de Sturge-Weber trata-se de uma doença neuro-óculocutânea rara. Os autores relatam o caso de um paciente do sexo masculino, de 13 anos de idade, que se apresentou com Síndrome de Sturge-Weber bilateral e glaucoma. Foi realizado o tratamento cirúrgico com implante de válvula de Ahmed em ambos os olhos e alcançado a redução da pressão intraocular bilateral.

Analysis of hypertrophy-related factors in 82 adult patients with hypertrophic port-wine stains / 中华皮肤科杂志

Objective To analyze clinical features of and hypertrophy-related factors in patients with hypertrophic port-wine stains (PWS). Methods Patients with PWS were enrolled into this study from Anhui Provincial Hospital and the First Affiliated Hospital of Anhui Medical University between January 2010 and August 2014. Clinical features of hypertrophic PWS were investigated. The factors related to hypertrophy in PWS were analyzed by univariate and multivariate unconditional logistic regression analyses. Results A total of 262 patients with PWS were enrolled, 82 (30 males and 52 females)of whom had hypertrophic PWS with a median age of 32.5 years (range, 18 - 54 years). Among the 82 patients, 66(80.48%)had plaque-like hypertrophic PWS, 9(10.98%)had papular or nodular type, and 7 (8.54%)had mixed type; 56.10% (46/82)were aged ≥ 30 years, 41.46% (34/82)varied from 11 to 30 cm2 in lesional area, and 85.36% (70/82)showed purple lesions. There was a significant difference between patients with hypertrophic PWS and those with flat PWS in the distribution of age, lesional area and color(χ2 = 25.559, 10.580, 90.630, respectively, all P 0.05). Multivariate unconditional logistic regression analysis revealed that an age ≥ 30 years(OR = 2.889, 95%CI: 1.459 - 5.721)and purple lesions(OR = 19.984, 95% CI: 5.704 - 70.023)were factors related to skin hypertrophy in PWS. Conclusion An age ≥ 30 years and purple lesions seem to be hypertrophy-related factors in PWS.

Effects of aminolevulinic acid-based photodynamic therapy on chicken combs, an animal model for port wine stains / 中华皮肤科杂志

Objective To investigate the effects of aminolevulinic acid-based photodynamic therapy(ALA-PDT) on chicken combs, an animal model for port wine stains (PWS), and to explore the feasibility of PWS treatment with ALA-PDT. Methods A total of 80 leghorns were randomly and equally divided into 10 groups: blank control group receiving no treatment, ALA group treated with ALA alone, four single laser groups irradiated with 630-nm red laser at 75, 100, 150 and 200 J/cm2 respectively, four ALA-PDT groups pretreated with ALA followed by 630-nm red laser radiation at 75, 100, 150 and 200 J/cm2 respectively. An area sized 1 cm × 1 cm were marked at one side of combs in all these leghorns, and served as the experiment area to receive corresponding treatment, with that in the other side as the control area. Tissue specimens were obtained on the 14th and 28th days after treatment followed by the observation of morphological and histological changes, calculation of decrement rate in capillary number, and determination of apoptosis index in vascular endothelial cells (VECs) in chicken combs. Results In all the four ALA-PDT groups, the combs became lighter in color with apoptosis of some VECs as well as a decrease in capillary count and diameter in the dermis of the experiment areas. The decrement rate in capillary number was 33.53% ± 4.89%, 52.02% ± 2.77%, 67.48% ± 5.58%and 88.96% ± 2.47% respectively, and apoptosis index in VECs was 63.44 ± 1.09, 88.50 ± 6.11, 94.32 ± 3.67 and 113.76 ± 10.57 respectively, in the 75-, 100-, 150- and 200-J/cm2 ALA-PDT groups on the 14th day after treatment, and both the decrement rate and apoptosis index in each of these groups were significantly different from those in the blank control group, ALA group, single laser groups receiving red laser radiation at the corresponding dose, and the other ALA-PDT groups (all P < 0.01)separately. The apoptosis depth of VECs, defined as the vertical distance from the basal layer to the deepest level at which VEC apoptosis occurred, was 201.19 ± 0.33 μm, 266.15 ± 1.02 μm, 546.09 ± 2.45 μm and 766.37 ± 1.08 μm respectively in the 75-, 100-, 150- and 200-J/cm2 ALA-PDT groups on the 14th day, with significant differences between these four groups (all P < 0.01). Conclusions ALA-PDT can markedly damage capillaries in the animal model of port wine stains, chicken combs, with the degree and depth of capillary damage associated with red light energy density. The induction of VEC apoptosis may be an action mechanism of ALA-PDT in the treatment of PWS.