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BACKGROUND: Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome. Only a few studies have reported the treatment of PPV, including a case using photodynamic therapy (PDT) to treat PPV-associated port-wine stains (PWS). OBJECTIVE: To investigating the efficacy and adverse effects of hemoporfin-PDT in PPV-associated PWS. METHODS: The efficacy and adverse effects in patients with PPV who underwent two sessions of hemoporfin-PDT from January 2019 to December 2022 were retrospectively analyzed. RESULTS: Twenty patients were included (13 females, 7 males, age range: 2-31 years; mean: 8.20 ± 8.92 years). Two, nine, seven, and two patients had PPV types Ia, IIa, IIb, and IIIa, respectively. After two treatments, the visual evaluation indicated the color of the PWS in 4, 5, 6, and 5 patients showed poor, fair, good, and excellent improvements, respectively. The combined good and excellent improvement rates in patients with PWS and pigmentary nevus overlapping in the same treatment area and in patients with PWS in the treatment areas only were 33.3% versus 87.5%, respectively, and were significantly different (p = 0.02). Minor side effects, such as edema, scabbing, hyperpigmentation, and blistering, were observed in some patients after PDT. CONCLUSION: Hemoporfin-PDT is an effective treatment for PPV-associated PWS. Patients with PWS and pigmentary nevus overlapping in the same treatment area showed poorer efficacy than patients with PWS in the treatment areas only.
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Hematoporfirinas , Síndromes Neurocutâneas , Fotoquimioterapia , Mancha Vinho do Porto , Humanos , Mancha Vinho do Porto/tratamento farmacológico , Feminino , Masculino , Fotoquimioterapia/efeitos adversos , Fotoquimioterapia/métodos , Criança , Adolescente , Estudos Retrospectivos , Adulto , Pré-Escolar , Adulto Jovem , Síndromes Neurocutâneas/tratamento farmacológico , Síndromes Neurocutâneas/diagnóstico , Hematoporfirinas/administração & dosagem , Hematoporfirinas/efeitos adversos , Hematoporfirinas/uso terapêutico , Resultado do Tratamento , Fármacos Fotossensibilizantes/administração & dosagem , Fármacos Fotossensibilizantes/efeitos adversosRESUMO
Background: Hematoporphyrin monomethyl ether (HMME) is a promising photosensitizer for photodynamic therapy (PDT) and has found wide application in the treatment of port-wine stains (PWS). Objective: This study aims to observe and analyze the clinical efficacy and safety of HMME-PDT in the treatment of PWS patients. It also aims to evaluate the usefulness of color Doppler flow imaging (CDFI), an ultrasound technique for detecting blood flow in skin lesions, in assessing clinical efficacy. Methods: Thirty-three patients with PWS underwent HMME-PDT at our dermatology outpatient clinic between January 2019 and March 2020. Data on patient demographics, lesion location, lesion type (pink, purple, nodular thickening), treatment frequency, and pre- and post-treatment images were collected and retrospectively analyzed. CDFI was performed on three patients. Results: All patients received intravenous HMME and underwent irradiation with 532 nm green LED light. Of these, 5 patients received 1 session of HMME-PDT, 14 received 2 sessions, 9 received 3 sessions and the remaining 5 patients received more than 3 sessions. Of the 33 patients, 9 were cured (27.27%), 10 showed improvement (30.30%), 11 experienced a reduction in symptoms (33.33%), and 3 showed no significant improvement (9.09%). Most patients reported local pain and oedema, and no systemic adverse effects were observed. Clinical efficacy correlated with lesion type and total number of treatment sessions. CDFI appears to be an excellent technique for assessing clinical efficacy. Conclusion: HMME-PDT is a safe and effective method for the treatment of PWS. CDFI examination appears to be a promising assessment tool. However, further validation with larger sample sizes is warranted.
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SIGNIFICANCE: Vascular-targeted photodynamic therapy (V-PDT) is a clinically approved therapeutic approach for treating vascular-related diseases, such as port-wine stains (PWS). For accurate treatment, varying light irradiance is required for different lesions due to the irregularity of vascular size, shape and degree of disease, which commonly alters during different stages of V-PDT. This makes quantitative analysis of the treatment efficiency urgently needed. APPROACH: Lesion images pre- and post- V-PDT treatment of patients with PWS were used to construct a quantitative method to evaluate the differences among lesions. Image analysis techniques were applied to evaluate the V-PDT efficiency for PWS by determining the Euclidean distances and two-dimensional correlation coefficients. RESULTS: According to the image analysis, V-PDT with good treatment efficiency resulted in a larger Euclidean distance and a smaller correlation coefficient compared with the case having lower V-PDT efficiency. CONCLUSIONS: A new method to quantify the Euclidean distances and correlation coefficients has been proposed, which is promising for the quantitative analysis of V-PDT efficiency for PWS.
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Fotoquimioterapia , Fármacos Fotossensibilizantes , Mancha Vinho do Porto , Mancha Vinho do Porto/tratamento farmacológico , Fotoquimioterapia/métodos , Humanos , Fármacos Fotossensibilizantes/uso terapêutico , Feminino , Masculino , Adulto , Ácido Aminolevulínico/uso terapêutico , Criança , AdolescenteRESUMO
BACKGROUND: Port wine stains (PWS) are vascular malformations, and photodynamic therapy (PDT) is a promising treatment. Emerging drug delivery methods employ nanoparticles (NPs) to enhance drug permeability and retention in diseased blood vessels and improve drug bioavailability. (-) -epigallocatechin-3-gallate glycine (EGCG) has anti-angiogenetic effects and boosts photodynamic therapy. Chlorin e6 (Ce6) is capable of efficiently producing singlet oxygen, rendering it a very promising photosensitizer for utilization in nanomedicine. MATERIAL AND METHODS: EGCG-Ce6-NPs were synthesized and characterized using various techniques. The photodynamic effects of EGCG-Ce6-NPs on endothelial cells were evaluated. The compatibility and toxicity of the nanoparticle was tested using the CCK-8 assay. The intracellular uptake of the nanoparticle was observed using an inverted fluorescence microscope, and the intracellular fluorescence intensity was detected using flow cytometry. The ROS generation and apoptosis induced by EGCG-Ce6-NPs was observed using confocal laser scanning microscopy and flow cytometry respectively. RESULTS: EGCG-Ce6-NPs exhibited stability, spherical shape of uniform size while reducing the particle diameter, low polydisperse profile and retaining the ability to effectively generate singlet oxygen. These characteristics suggest promising potential for enhancing drug permeability and retention. Additionally, EGCG-Ce6-NPs demonstrated good compatibility with endothelial cells and enhanced intracellular uptake of Ce6. Furthermore, EGCG-Ce6-NPs increased activation efficiency, induced significant toxicity, more reactive oxygen species, and a higher rate of late apoptosis after laser irradiation. CONCLUSION: This in vitro study showed the potentials EGCG-Ce6-NPs for the destruction of endothelial cells in vasculature.
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Catequina , Clorofilídeos , Nanopartículas , Fotoquimioterapia , Fármacos Fotossensibilizantes , Porfirinas , Fármacos Fotossensibilizantes/farmacologia , Fármacos Fotossensibilizantes/farmacocinética , Fotoquimioterapia/métodos , Nanopartículas/química , Catequina/análogos & derivados , Catequina/farmacologia , Catequina/farmacocinética , Catequina/química , Humanos , Porfirinas/farmacologia , Porfirinas/farmacocinética , Células Endoteliais/efeitos dos fármacos , Polifenóis/farmacologia , Apoptose/efeitos dos fármacos , Oxigênio Singlete/metabolismo , Sobrevivência Celular/efeitos dos fármacosRESUMO
Objective: Port-wine stains are a kind of dermatological disease of congenital capillary malformation. Based on the biological characteristics of port-wine stains and the advantages of microneedle transdermal administration, we intend to construct a nanodrug co-loaded with rapamycin (RPM), an anti-angiogenesis drug, and photochlor (HPPH), a photosensitizer, and integrate the nanodrug with dissolvable microneedles (MN) to achieve anti-angiogenesis and photodynamic combination therapy for port-wine stains. Methods: First, RPM and HPPH co-loaded nanoparticles (RPM-HPPH NP) were prepared by the emulsification solvent-volatilization method, and its ability to generate reactive oxygen species (ROS) was investigated under 660 nm laser irradiation. Mouse hemangioendothelioma endothelial cells (EOMA) were used as the subjects of the study. The cellular uptake behaviors were examined by fluorescence microscopy and flow cytometry. The cytotoxicity effects of RPM-HPPH NP with or without 660 nm laser irradiation on EOMA cells were examined by MTT assays (with free RPM serving as the control). Then, hyaluronic acid (HA) dissolvable microneedles loaded with RPM-HPPH NP (RPM-HPPH NP@HA MN) were obtained by compounding the nanodrug with HA dissolvable microneedle system through the molding method. The morphological characteristics and mechanical properties of RPM-HPPH NP@HA MN were investigated by scanning electron microscope and electronic universal testing machine. The penetration ability of RPM-HPPH NP@HA MN on the skin of nude mice was evaluated by trypan blue staining and H&E staining experiment. Results: The RPM-HPPH NP prepared in the study had a particle size of 150 nm and generated large amounts of ROS under laser irradiation. At the cellular level, RPM-HPPH NP was taken up by EOMA cells in a time-dependent manner. The cytotoxicity of RPM-HPPH NP was higher than that of free RPM with or without laser irradiation. Under laser irradiation, RPM-HPPH NP exhibited stronger cytotoxic effects and the difference was statistically significant (P<0.05). The height of the needle tip of RPM-HPPH NP@HA MN was 600 µm and the mechanical property of a single needle was 0.75048 N. Trypan blue staining and HE staining showed that pressing on the microneedles could produce pores on the skin surface and penetration of the stratum corneum. Conclusion: RPM-HPPH NP@HA MN can deliver RPM-HPPH NP percutaneously to the lesion tissue and realize the synergistic treatment of port-wine stains with anti-angiogenic therapy and photodynamic therapy, providing a new strategy for the construction of nanodrug-loaded microneedle delivery system and the clinical treatment of port-wine stains.
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Nanopartículas , Agulhas , Mancha Vinho do Porto , Sirolimo , Animais , Camundongos , Nanopartículas/química , Mancha Vinho do Porto/tratamento farmacológico , Sirolimo/administração & dosagem , Fármacos Fotossensibilizantes/administração & dosagem , Administração Cutânea , Fotoquimioterapia/métodos , Espécies Reativas de Oxigênio/metabolismo , Células Endoteliais/efeitos dos fármacos , Sistemas de Liberação de Medicamentos , Inibidores da Angiogênese/administração & dosagem , Hemangioendotelioma/tratamento farmacológicoRESUMO
OBJECTIVE: This study aims to evaluate the potential impact of port wine stains on the development of permanent teeth in mixed dentition and provide insights for managing tooth abnormalities in patients with port wine stains. MATERIALS AND METHODS: A retrospective analysis was conducted on 21 patients with mixed dentition and unilateral maxillary port wine stains. Two researchers concurrently utilized Nolla Analysis to assess the developmental stage of bilateral maxillary and permanent mandibular teeth based on panoramic radiographs. The cumulative developmental values of upper and lower permanent teeth on both sides were calculated, and a comparison was made between the developmental stages of the upper and lower jaws. RESULTS: Port wine stains can influence the maturity of permanent upper teeth, within the unilateral maxillary port wine stains range, with an early developmental completion observed on the affected side compared to the unaffected side. While the developmental stages of the lower teeth on both sides showed similarities. CONCLUSIONS: Port wine stains can accelerate the maturity of teeth on the affected side, leading to alterations in the order of tooth eruption and subsequent abnormal occlusion in children with mixed dentition. These findings provide a basis for developing appropriate management strategies for addressing tooth abnormalities in patients with port wine stains.
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Capillary malformations (CM) (port-wine stains) are congenital skin lesions that are characterized by dilated capillaries and postcapillary venules. CMs are caused by altered functioning of the vascular endothelium. Somatic genetic mutations have predominantly been identified in the endothelial cells of CMs, providing an opportunity for the development of targeted therapies. However, there is currently limited in-depth mechanistic insight into the pathophysiology and a lack of preclinical research approaches. In a monocenter exploratory study of 17 adult patients with CMs, we found somatic sequence variants in the GNAQ (p.R183Q, p.R183G, or p.Q209R) or GNA11 (p.R183C) genes. We applied an endothelial-selective cell isolation protocol to culture primary endothelial cells from skin biopsies from these patients. We successfully expanded patient-derived cells in culture in 3 of the 17 cases while maintaining endothelial specificity as demonstrated by vascular endothelial-cadherin immunostainings. In addition, we tested the angiogenic capacity of endothelial cells from a patient with a GNAQ (p.R183G) sequence substitution. These proof-of-principle results reveal that primary cells isolated from CMs may represent a functional research model to investigate the role of endothelial somatic mutations in the etiology of CMs, but improved isolation and culture methodologies are urgently needed to advance the field.
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OBJECTIVE: To conduct a retrospective analysis of Hemoporfin photodynamic therapy (HMME-PDT) in the treatment of port-wine stains (PWS). METHOD: A retrospective analysis was conducted based on the clinical data from March 2017 to December 2022, so as to summarize the demographic characteristics, clinical efficacy and adverse reactions. The effectiveness of HMME-PDT was examined with respect to treatment times, age, gender, subtype, and location of PWS lesions. RESULT: The age of the 2952 cases ranged from 8 months to 56 years old (median, 2.8 years), with 1419 males (48.07 %), and 1533 females (51.93 %). There were 669 cases of pink type (22.66 %), 2184 cases of purplish red type (73.98 %), and 99 cases of nodular thickening type (3.35 %). The prevalence location was face (88.04 %), neck (14.94 %), limbs and trunk. 1602 cases (54.27 %) had never received treatment, 661 cases (22.39 %) had been treated by pulse dye laser (PDL), 229 cases (7.76 %) had previously been treated by PDT, 296 cases (10.03 %) had received both the modalities. The 2952 cases completed totally 7996 HMME-PDT times. Cure rate and effective rate increased continuously with the number of treatments. The pink type has the highest cure rate and effective rate, followed by the purplish red type and the last was the nodular thickening type. The therapeutic effects are considerably influenced by age, subtype, and treatment site (P < 0.05). However, there was no significant difference in the effectiveness of HMME-PDT between both genders. The local adverse reactions after the first treatment included edema (97.73 %), itching (82.62 %), purpura-like change (79.51 %), crusts (24.59 %), infection (4.07 %), scars (1.08 %), hyperpigmentation (0.61 %), and depigmentation (0.41 %). Nausea and vomiting occurred in 2 juveniles and 1 young adult (5, 6 and 22 years old respectively) immediately after treatment, and did not interfere with the administration of the treatment. Patients aged 21-30 were found to have a 3.4-fold higher likelihood of undergoing HMME-PDT under general anesthesia compared to those aged 15 or younger. There was no distinct systemic adverse reaction, such as allergic responses, cardiovascular effects, neurological symptoms, hematological abnormalities, respiratory symptoms, or musculoskeletal issues. CONCLUSION: HMME-PDT is preferred in treating PWS, with relatively high effective rate and cure rate, mild local reactions and no distinct systemic adverse reaction.
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Fotoquimioterapia , Mancha Vinho do Porto , Adulto Jovem , Humanos , Masculino , Feminino , Criança , Adolescente , Adulto , Fármacos Fotossensibilizantes/uso terapêutico , Mancha Vinho do Porto/tratamento farmacológico , Mancha Vinho do Porto/patologia , Fotoquimioterapia/métodos , Estudos Retrospectivos , Hematoporfirinas/uso terapêutico , Resultado do TratamentoRESUMO
Background: Port-wine stains (PWS) are predominantly located on the face and neck. Hence, PWS affect not only aesthetic appearance but may also cause psychological distress. However, very few studies have researched on the quality of life (QoL) in Chinese patients with PWS. Aim: To evaluate QoL in patients with PWS. Methods and Materials: Skindex-29 and Dermatology Life Quality Index (DLQI) were used to investigate the QoL of the patients with PWS and healthy controls. Results: In total, 198 adults with PWS and 200 healthy controls were included. The scores of symptomatic, emotional and functional dimensions of Skindex-29 in the observational group were significantly higher as compared with the control group (P<0.05). Female gender, lesion location and the treatment history (such as pulsed dye laser or photodynamic therapy) had a negative impact on the scores (emotional and functional dimensions) of the patients with PWS. The median DLQI score was 3 points in the observational group and 0 points in the control group (P<0.05), and QoL of patients who had a history of previous treatments was diminished. Conclusion: The QoL of patients with PWS is impaired. Patients' QoL is negatively influenced by female gender, lesion location and treatment history. The Skindex-29 scale seems to be more applicable to the study of QoL of patients with PWS.
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BACKGROUND: Port wine stains slowly grow thicker over time, nodules appear on the surface, and the color slowly deepens from pink to purple. Even after laser treatment, some port wine stains will recur and slowly grow, and the erythema appears again. The purpose of this study was to investigate the effectiveness of bleomycin in combination with triamcinolone acetonide in the treatment of inhibiting the proliferation and recurrence of port wine stains. RESULTS: Histopathological change: Before treatment, dense capillaries were distributed within the lesion, and blood fills the lumen. Lack of normal skin structure: After bleomycin and triamcinolone acetonide injection, the number of capillaries was significantly reduced, and fibrosis occurred in tissues. Changes in facial morphology: After treatment, the port wine stains became thinner, the asymmetry of the face was effectively improved, and the appearance have been significantly improved. After 5 years of follow-up, there were no recurrent cases. CONCLUSION: Bleomycin and triamcinolone acetonide injection can effectively inhibit the proliferation of port wine stains and prevent port wine stains from recurring after treatment.
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INTRODUCTION: Pulsed dye laser (PDL) is currently considered to be the first-line treatment for port-wine stains (PWSs) on the extremities despite its less than satisfactory therapeutic efficacy. Hemoporfin-mediated photodynamic therapy (HMME-PDT) is a vascular-targeted therapy that has rarely been used to treat PWSs on the extremities. Here, we evaluate the clinical efficacy and safety of HMME-PDT for the treatment of PWSs on the extremities. METHODS: Clinical data and dermoscopic images of PWSs on the extremities were obtained from 65 patients who underwent HMME-PDT between February 2019 and December 2022. The clinical efficacy of HMME-PDT was analyzed by comparing the pre- and post-treatment images. The safety of HMME-PDT was evaluated through observation during the treatment period and post-treatment follow-up. RESULTS: The efficacy rate of a single HMME-PDT session was 63.0% and that of two and three to six sessions was 86.7% and 91.3%, respectively. A positive correlation was found between therapeutic efficacy and the number of HMME-PDT sessions. The therapeutic efficacy of HMME-PDT was better on the proximal extremities than on other parts of the extremities (P = 0.038), and the efficacy of treating PWSs in each site was relatively improved with an increase of treatment time. The clinical efficacy of HMME-PDT differed across four PWS vascular patterns identified by dermoscopy (P = 0.019). However, there was no statistical difference in the therapeutic efficacy based on age, sex, type of PWS, and treatment history (P > 0.05), which may be partly attributed to the relatively small sample size or poor cooperation of infant patients. No obvious adverse reactions were observed during the follow-up period. CONCLUSIONS: HMME-PDT is a very safe and effective treatment for PWSs on the extremities. Multiple HMME-PDT treatments, lesions located in proximal limbs, and PWSs with type I and IV vascular patterns under dermoscopy were associated with higher efficacy of HMME-PDT. Dermoscopy may help predict the clinical efficacy of HMME-PDT. TRIAL REGISTRATION NO: 2020KJT085.
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BACKGROUND: Patients with capillary malformations (CMs) may undergo medical tattooing (MT) as an alternative to laser therapy. But little is known about treatment results and impact from the patients' perspective. OBJECTIVES: In this cross-sectional digital survey study, we evaluated the patient-reported outcomes of MT for CMs. METHODS: MT practices were identified via the Dutch Association of Skin Therapists and Google. These practices invited all their CM patients who had undergone MT between January 2011 and September 2021 to participate. Baseline and treatment characteristics, tattooing effectiveness, patient satisfaction with treatment outcomes, and complications were evaluated using a custom-made online survey. Quality of life was assessed with the Dermatology Life Quality Index (DLQI) questionnaire. Factors associated with treatment effectiveness and patient satisfaction were identified via bivariate analysis and ordinal logistic regression analysis. RESULTS: Most of the 89 respondents were female (69%). Almost all CMs were located on the face (90%) and mainly (dark) red (74%). Nearly all patients had undergone laser therapy (91%). Median number of tattooing sessions was 5 (IQR: 4.0-8.0). Thirty-seven percent of the patients perceived >75% color reduction. Younger patients were more likely to obtain lower treatment effectiveness (OR 0.44, 95% CI: 0.20-0.97). Most patients (83%) were satisfied with treatment results. Patients with lighter (OR 0.30, 95% CI: 0.13-0.72), non-facial (OR 0.15, 95% CI: 0.03-0.89), and hypertrophic CMs (OR 0.30, 95% CI: 0.11-0.82) were less likely to be satisfied with treatment outcomes. Patients with lighter skin types were more satisfied (OR 2.89, 95% CI: 1.23-6.80). Complications included transient pain (23%), bleeding (3.4%), hypertrophic scarring (1.1%), hypopigmentation (1.1%), and a halo around the tattoo (1.1%). CONCLUSION: MT seems a valid alternative treatment in addition to laser therapy for CMs, with mild complications. Most patients are (very) satisfied with treatment results, while color reduction is incomplete. Hence, it seems appropriate to decide together with patients whether or not to use MT as primary treatment or secondary to laser therapy.
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Capilares , Medidas de Resultados Relatados pelo Paciente , Tatuagem , Malformações Vasculares , Feminino , Humanos , Masculino , Estudos Transversais , Qualidade de Vida , Malformações Vasculares/cirurgia , Capilares/anormalidades , Capilares/cirurgiaRESUMO
Hemoporfin-mediated photodynamic therapy (HMME-PDT) is commonly used in the treatment of port-wine stains (PWS). However, the influential factors for the efficacy of the treatment are not well defined. This study intends to observe the influential factors for the efficacy of HMME-PDT in the treatment of port-wine stains (PWS). A total of 551 patients with PWS of head and neck was enrolled in this retrospective study. Further screening the patients of facial PWS, 484 patients were chosen. Patients were treated with HMME-PDT. All patients received 1~3 sessions of treatment with 2~3-month intervals. We photographed the lesions before each session and 2~3 months after the last session. Ages, sessions, lesion subtypes, and previous treatment history were related to the response of HMME-PDT (P =0.032, P<0.001, P=0.012, P=0.003 respectively). Treatment sessions were the independent factor correlated with efficacy after 3 sessions of treatment. Patients with no treatment history targeting PWS showed higher efficacy than those were treated with laser or other photodynamic treatment (P<0.05). The efficacy was higher by increasing the sessions of treatment. The efficacy was higher for lesion on maxillary prominence area and mandibular prominence area that on frontonasal prominence area and optic vesicle area (P<0.05). HMME-PDT is an effective in the treatment of PWS. Patients received no previous treatment for PWS, total treatment sessions and lesion on maxillary prominence area and mandibular prominence area are positive factors.
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Má Oclusão , Fotoquimioterapia , Mancha Vinho do Porto , Humanos , Fármacos Fotossensibilizantes/uso terapêutico , Mancha Vinho do Porto/tratamento farmacológico , Mancha Vinho do Porto/patologia , Estudos Retrospectivos , Fotoquimioterapia/efeitos adversos , Hematoporfirinas/farmacologia , Hematoporfirinas/uso terapêutico , Resultado do TratamentoRESUMO
BACKGROUND: Port-wine stains (PWS) are congenital low-flow vascular malformations of the skin. PWS tend to become thicker and darker with time. Laser therapy is the gold standard and the first-line therapy for treating PWS. However, some resistant PWS, or PWS that have tissue hypertrophy, do not respond to this therapy. Our aim is to evaluate the role of surgery in the treatment of PWS birthmarks. METHODS: A literature search was performed in PubMed, Scopus, Web of Science (WOS) and Google Scholar for all papers dealing with surgery for port-wine stains, from January 2010 to December 2020 using the search strings: (capillary vascular malformation OR port-wine stains OR Sturge Weber Syndrome OR sws OR pws) AND (surgical OR surgery). RESULTS: Ten articles were identified and used for analysis. They were almost all case series with a short follow up period and lacked an objective-systematic score of evaluation. CONCLUSIONS: Delay in treatment of port wine stains may result in soft tissue and bone hypertrophy or nodules with disfiguring or destructive characteristics. The correction of PWS-related facial asymmetry often requires bone surgery followed by soft tissue corrections to achieve a more harmonious, predictable result.
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Background: Hemoporfin-mediated photodynamic therapy (PDT) is an effective treatment for port-wine stains (PWS), and pain is the main adverse effect of this therapy. General anesthesia is commonly used for pain management during PDT, but the effect of general anesthetics on the subsequent treatment efficacy of PDT in PWS has not been reported. Objectives: To assess the use of general anesthesia combined with PDT compared with PDT alone in 207 PWS patients, and to provide further safety and efficacy data on this combined therapy. Methods: Propensity score matching (PSM) was used at a 2:1 ratio to create a general anesthetic group (n = 138) and a highly comparable nonanesthetic group (n = 69). The clinical outcomes were evaluated, and the treatment reactions and adverse effects were recorded after one treatment with PDT. Results: After matching, there was no significant difference in the demographic data of the patients in the two groups (p > 0.05), while the treatment efficacy was significantly higher in the general anesthetic group than in the nonanesthetic group (76.81 vs. 56.52%, p < 0.05). Moreover, logistic regression analysis confirmed that patients receiving general anesthesia showed an association with a good response to PDT (OR = 3.06; 95% CI, 1.57-6.00; p = 0.0011). Purpura lasted longer in the general anesthetic group, but the other treatment reactions and adverse effects were similar in the two groups (p > 0.05). No serious systemic adverse reactions were observed. Conclusion: We recommend this combined therapy, which is associated with painless, as a high efficacy treatment option for PWS patients, especially for patients with a poor response to multiple PDT alone treatments.
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Port-wine stains (PWS) are frequently refractory to laser treatments. The aim of this study is to evaluate the role of treatment interval time. From 1990, 216 patients underwent Pulsed Dye Laser sessions. The laser sessions were scheduled at a minimum interval of 4 weeks to a maximum of 48 weeks. Clinical outcomes were assessed 8 weeks after the last laser session. Better results were obtained with 8 weeks interval time between therapy session, and high efficacies were also found for intervals of 4, 6 and 10 weeks. For greater interval instead, the effectiveness is significantly lower.
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Terapia a Laser , Lasers de Corante , Mancha Vinho do Porto , Humanos , Lasers de Corante/uso terapêutico , Resultado do Tratamento , Mancha Vinho do Porto/radioterapia , Mancha Vinho do Porto/cirurgia , Terapia a Laser/métodosRESUMO
Background Sturge-Weber syndrome (SWS) is a rare condition associated with a GNAQ gene mutation, which affects neural crest cells. A pulsed dye laser (PDL) is a first-line therapy for SWS, but its outcomes are worse than those in patients with port-wine stains (PWS). Photodynamic therapy (PDT) is a promising therapeutic option for PWS. However, its use for PWS associated with SWS has rarely been studied. Aims To investigate the therapeutic and adverse effects of photodynamic therapy in treating SWS-associated PWS. Methods Patients with SWS and matched patients with large size facial PWS were included in this study. Both colorimetric assessment and visual evaluation were conducted to evaluate patients' responses to treatment. Results Colorimetric assessment (blanching rate) and visual evaluation (scores of colour improvement) showed that after two PDT treatments, the SWS and PWS groups had similar treatment responses (21.2% vs. 29.8%; 3.39 vs. 3.65; P = 0.18, P = 0.37). However, there was a significant difference in efficacy between patients with SWS with and without a treatment history (12.4 and 34.9%, respectively; P = 0.02), as well as between patients with lesions located on the central and lateral faces (18.5 and 36.8%, respectively; P = 0.01). Both the SWS and PWS groups experienced minor adverse effects, and the frequency of these effects was not significantly different between the two groups. Limitation The study was limited by a small sample size and the possibility of later onset of glaucoma. In addition, false-negative magnetic resonance imaging screening results for SWS could not be ruled out due to the young age of some participants. Conclusion Photodynamic therapy is a safe and effective therapeutic option for SWS-associated PWS. Patients without a treatment history, and lesions on the lateral face, responded well, demonstrating good efficacy.
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Background: Conventional treatments of port wine stain birthmarks often do not achieve the desired outcome in patients with hypertrophy. Potential reasons include deeper and larger blood vessels, abnormal arrangement of blood vessels, and darker or thicker epidermis. However, these factors may not significantly limit the efficacy of fractional carbon dioxide (CO2) laser. The aim of this case report was to examine the extended application of fractional CO2 laser in treating patients with hypertrophic port wine stain birthmarks. Methods: Two cases with hypertrophic port wine stain birthmarks treated with fractional CO2 laser for 5 years are described in this case report. Results: When compared with conventional treatment, both cases reported better outcomes, including a reduced risk of infection, pigmentation, and scarring, a decrease of clinical erythema, and much less pain. Conclusions: The findings demonstrate that fractional CO2 laser has the potential to be an effective modality for the treatment of patients with hypertrophic port wine stains.
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Hipertrofia , Lasers de Gás , Transtornos da Pigmentação , Mancha Vinho do Porto , Humanos , Dióxido de Carbono , Lasers de Gás/uso terapêutico , Mancha Vinho do Porto/radioterapia , Mancha Vinho do Porto/cirurgiaRESUMO
Background: Port wine stains (PWS) often cause cosmetic effects and psychological distress. Pulsed dye lasers (PDL) and photodynamic therapy (PDT) are the most commonly used treatments. PDL is still the "gold standard" of therapy to date. However, its shortcomings have become apparent as clinical applications have increased. PDT has been proven as an alternative to PDL. Patients with PWS still lack enough evidence about PDT to make informed treatment decisions. Objective: The purpose of this systematic review and meta-analysis was to assess the safety and effectiveness of PDT for PWS. Methods: The online datasets, comprising PubMed, Embase, Web of Science, and the Cochrane Library, were searched for meta-analysis-relevant publications. Two reviewers separately evaluated the risk of bias in each listed study. Grading of Recommendations Assessment, Development, and Evaluation (GRADE) was used to assess the treatment and safety outcomes. Results: Our search retrieved 740 hits and only 26 studies were finally included. Among the 26 studies included, 3 were randomized clinical trials, and 23 were prospective or retrospective cohort investigations. Based on a gathered assessment, the percentage of individuals achieving a 60% improvement was estimated to be 51.5% [95% confidence interval (CI): 38.7-64.1; I 2 = 83.8%] and a ≥75% improvement was 20.5% (95% CI: 14.5-26.5; I 2 = 78.2%) after 1-8.2 treatment sessions (GRADE score: very low). Due to the statistical diversity of the meta-analysis, a subgroup assessment was performed to determine the sources of diversity. The collected findings indicated that the impact of PDT on enhancing the medical effectiveness of PWS was significant in different treatment sessions, different types of ages, different locations of PWS, and different types of PWS. Pain and edema occurred in most patients. Hyperpigmentation was present in 7.9-34.1% of the patients in 17 studies. Photosensitive dermatitis, hypopigmentation, blister, and scar were infrequently reported, with 0-5.8% incidences. Conclusion: Photodynamic therapy is recommended as a safe and effective treatment for PWS based on the current evidence. However, our findings are based on poor-quality evidence. Therefore, comparative investigations of a large scale and high quality are necessary to support this conclusion.
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BACKGROUND AND OBJECTIVES: Port-wine stains are defined as congenital benign vascular lesions. The treatment of port-wine stains remains a challenge, worldwide. This study aimed to analyze the histological characteristics in different types of port-wine stains and provide guidance for clinical decision-making. METHODS AND MATERIALS: Biopsies were from the hospital from 2015 to 2021. H&E staining, Immunofluorescence staining, Masson's trichrome staining and Weigert staining were performed on the tissues. RESULTS: A total of 35 port-wine stains patients were included in the study of four distinct types, namely red port-wine stains (11 cases), purple port-wine stains (seven cases), hypertrophic port-wine stains (nine cases) and nodular port-wine stains (eight cases). The mean vessel diameter of the different types was 38.7 ± 5.9 µm, 93.5 ± 9.7 µm, 155.6 ± 21.8 µm and 155.6 ± 29.54 µm, respectively. Mean vessel depth was 396.4 ± 31 µm, 944.2 ± 105.4 µm, 2,971 ± 161.3 µm and 3,594 ± 364.6 µm, respectively. The vessels in red port-wine stains, purple port-wine stains and hypertrophic port-wine stains were mainly composed of capillary and venous malformations, whereas those in nodular port-wine stains were venous or arteriovenous malformations. LIMITATION: The main limitation of the current study was the small number of patients. CONCLUSION: As the disease progresses, vessel diameters become larger, the vessel wall becomes thicker and vessels were found in a greater depth. A treatment plan should be scientifically formulated keeping in mind the histological characteristics of port-wine stains.