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Journal of the European Academy of... Jul 2019Since the early '80s, the pulsed dye laser has been the standard treatment tool for non-invasive port wine stain (PWS) removal. In the last three decades, a considerable...
BACKGROUND
Since the early '80s, the pulsed dye laser has been the standard treatment tool for non-invasive port wine stain (PWS) removal. In the last three decades, a considerable amount of research has been conducted to improve clinical outcomes, given that a fraction of PWS patients proved recalcitrant to laser treatment. Whether this research actually led to increased therapeutic efficacy has not been systematically investigated.
OBJECTIVE
To analyse therapeutic efficacy in PWS patients globally from 1986 to date.
METHODS
PubMed was searched for all available PWS trials. Studies with a quartile percentage improvement scale were included, analysed and plotted chronologically. Treatment and patient characteristics were extracted. A mean clearance per study was calculated and plotted. A 5-study simple moving average was co-plotted to portray the trend in mean clearance over time. The data were separately analysed for multiple treatment sessions in previously untreated patients.
RESULTS
Sixty-five studies were included (24.3% of eligible studies) comprising 6207 PWS patients. Of all patients, 21% achieved 75-100% clearance. Although a few studies reported remarkably good outcomes in a subset of carefully selected patients, there was no upward trend over time in mean clearance.
CONCLUSION
The efficacy of PWS therapy has not improved in the past decades, despite numerous technical innovations and pharmacological interventions. With an unwavering patient demand for better outcomes, the need for development and implementation of novel therapeutic strategies to clear all PWS is as valid today as it was 30 years ago.
Topics: Humans; Laser Therapy; Lasers, Dye; Photochemotherapy; Port-Wine Stain; Treatment Outcome
PubMed: 30908756
DOI: 10.1111/jdv.15599 -
Journal of Clinical and Translational... Sep 2019Despite extensive efforts to optimize laser therapy, i.e., the current gold standard treatment, a majority of port wine stain (PWS) patients responds suboptimally to... (Review)
Review
UNLABELLED
Despite extensive efforts to optimize laser therapy, i.e., the current gold standard treatment, a majority of port wine stain (PWS) patients responds suboptimally to laser therapy. This paper describes the niceties of a novel PWS treatment modality termed site-specific pharmaco-laser therapy (SSPLT). In contrast to the classic approach of enhancing the extent of intravascular photocoagulation (the photothermal response), SSPLT focuses on optimization of post-irradiation thrombus formation (i.e., the hemodynamic response) by combining conventional laser therapy with the administration of thermosensitive drug delivery systems that encapsulate prothrombotic and antifibrinolytic drugs. The aim of SSPLT is to instill complete lumenal occlusion in target vessels, which has been linked to optimal PWS blanching.
RELEVANCE FOR PATIENTS
The current treatment options for PWS patients are limited in efficacy. Novel therapeutic modalities are needed to more effectively treat patients with recalcitrant PWSs. SSPLT is an experimental-stage treatment modality that could serve as an adjuvant to pulsed dye laser therapy for a selected group of patients whose PWS is ill-responsive to standard treatment. The expected clinical result of SSPLT is improved lesional blanching.
PubMed: 31579838
DOI: No ID Found -
Photodiagnosis and Photodynamic Therapy Sep 2022Hemoporfin-mediated photodynamic therapy (Hemoporfin-PDT) is a safe and effective treatment modality for port-wine stain (PWS). However, there is still no consensus...
BACKGROUND
Hemoporfin-mediated photodynamic therapy (Hemoporfin-PDT) is a safe and effective treatment modality for port-wine stain (PWS). However, there is still no consensus about the influential factors for the efficacy of the treatment. This study investigated the influential factors associated with the efficacy of Hemoporfin-PDT.
METHODS
We retrospectively analyzed 321 PWS patients who underwent Hemoporfin-PDT at our center from August 2017 to July 2021. The correlation between efficacy versus sex, age, location, type of PWS, treatment numbers, and the lesion size were analyzed.
RESULTS
The numbers of treatment sessions undertaken were associated with the response to therapy, and compared with patients who received one session, patients who received two or more sessions showed a better response (ORadj=2.46, 95%CI, 1.49-4.07; ORadj=6.01, 95%CI, 3.38-10.70, P<0.001). The effect on central face, peripheral face, and neck was superior to the extremity and trunk, respectively (P<0.001). The lesion size smaller than and equal to 25 cm² showed a better effect than those whose lesion size was larger than 64 cm² (ORadj=1.92, 95%CI, 1.03-3.57, P=0.040). However, other variables, including sex and age, were not associated with the efficacy of the treatment.
CONCLUSIONS
Hemoporfin-PDT is an effective and safe treatment for PWS. The number of treatments was a favorable factor for Hemoporfin-PDT, smaller lesion sizes showed a better effect than the larger one, and the location of extremity and trunk was a negative factor.
Topics: Hematoporphyrins; Humans; Photochemotherapy; Photosensitizing Agents; Port-Wine Stain; Retrospective Studies; Treatment Outcome
PubMed: 35840007
DOI: 10.1016/j.pdpdt.2022.103003 -
European Journal of Paediatric... May 2022Patients with Sturge-Weber syndrome type 1 (SWS1) have a port-wine birthmark (PWB) as cutaneous hallmark. Up to 35% of neonates with a high risk PWB develop SWS1....
BACKGROUND
Patients with Sturge-Weber syndrome type 1 (SWS1) have a port-wine birthmark (PWB) as cutaneous hallmark. Up to 35% of neonates with a high risk PWB develop SWS1. Clinical manifestations are severe and often progressive. Especially early onset seizures are associated with worse neurocognitive outcome. Identification of pre-symptomatic SWS1 patients is hampered because brain MRI in the first months of life does not always show the for SWS1 characteristic leptomeningeal capillary malformation (LMC).
OBJECTIVES
Identification of sensitive and specific MRI predictors for early SWS1 diagnosis.
METHODS
In this retrospective single centre study, we included 24 SWS1 patients and 20 controls. We studied specificity and sensitivity for SWS1 diagnosis of LMC and indirect MRI signs such as choroid plexus (CP) size and thickness, abnormal white matter signal, lobar cerebral atrophy, ischemia and cortical calcifications.
RESULTS
In SWS1 patients CP thickness and CP thickness ratio on non-contrast brain MRI was significantly increased. The optimal cut-off value of 5.6 mm on the affected side corresponded with a sensitivity of 91.7% and a specificity of 100% for confirmation of SWS1 diagnosis. In 21% of children aged ≤3 months with a later confirmed SWS1 diagnosis, LMC on initial MRI could not be discerned but CP thickness ≥5.6 mm on the affected side confirmed SWS1 diagnosis.
CONCLUSIONS
In this study, CP size ratio and thickness were found to be sensitive and specific signs additional to earlier described criteria to support SWS1 diagnosis in neonates and infants which need to be confirmed in other series.
Topics: Child; Early Diagnosis; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Port-Wine Stain; Retrospective Studies; Sturge-Weber Syndrome
PubMed: 35461064
DOI: 10.1016/j.ejpn.2022.04.002 -
Skin Health and Disease Feb 2024Port-wine stains (PWS) are capillary vascular anomalies that are often treated with pulsed-dye laser (PDL). Revascularization limits persistent clearance; however, the... (Review)
Review
Port-wine stains (PWS) are capillary vascular anomalies that are often treated with pulsed-dye laser (PDL). Revascularization limits persistent clearance; however, the anti-angiogenic effects of sirolimus (SIRO) may inhibit revascularization. This review aims to determine differences in PWS outcomes when treated with PDL monotherapy or in combination with SIRO. A systematic review was conducted using PubMed, Cochrane, and Embase databases. The following search terms were used: 'port wine stain PDL SIRO', 'port wine stain PDL', and 'port wine stain PDL and topical treatment' with (MeSH) and (Title/Abstract) limits. The search was limited to the English language and human-subject studies conducted between 1 January 2000 and 1 June 2023. Inclusion criteria included studies evaluating SIRO as an adjunct to PDL in patients with PWS. Data extraction and quality assessment were performed by two independent reviewers. A total of nine studies met the inclusion criteria, which included randomized controlled trials (3), case series (2), case reports (3), and a prospective intrapatient study (1), which represented a total of 58 patients. Five studies showed improvement of a measured post-treatment PDL parameter including shortening treatment time and less frequent dosing. A subset of studies (4/9) which did not demonstrate significant clinical improvements exhibited significant photographic evidence of improvement. Heterogeneity among the studies highlights the need for further research and standardization. While adjunctive SIRO shows promise, larger studies and comprehensive evaluation methods are required to establish conclusive safety and efficacy guidelines to shape clinical decision-making.
PubMed: 38312256
DOI: 10.1002/ski2.333 -
BMC Ophthalmology Dec 2020To evaluate the choroidal changes in Sturge-Weber syndrome (SWS) secondary glaucoma and non-glaucoma port-wine stain (PWS) patients by enhanced depth imaging optical...
Choroidal alterations of Sturge-Weber syndrome secondary glaucoma and non-glaucoma port-wine stain patients distinguished by enhanced depth imaging optical coherence tomography.
BACKGROUND
To evaluate the choroidal changes in Sturge-Weber syndrome (SWS) secondary glaucoma and non-glaucoma port-wine stain (PWS) patients by enhanced depth imaging optical coherence tomography (EDI-OCT).
METHODS
SWS and PWS patients who were over 3 years old and treated or screened at our ophthalmology department were included in the study. Baseline demographics, EDI-OCT and fundus photography data were collected from all patients.
RESULTS
Overall, 46 non-glaucoma PWS (NGPWS) patients and 35 SWS secondary glaucoma (SG) patients were included, with mean ages of 16.52 ± 13.63 and 13.94 ± 8.27 years, respectively (p > 0.05). Among these patients 2 exhibited bilateral PWS and unilateral glaucoma. Thus, the two eyes of each patient were divided into NGPWS and SG group, respectively. Twenty-one eyes had choroidal hemangiomas and 7 eyes had excessive thickening of the choroid without choroidal hemangiomas. Choroidal hemangiomas were only observed in ipsilateral eyes of SG patients. The choroidal thicknesses of the ipsilateral and fellow eyes of NGPWS patients were 358.10 ± 117.40 μm (45 eyes) and 288.20 ± 79.04 μm (41 eyes), respectively (p < 0.05). The choroidal thicknesses of the ipsilateral and fellow eyes of SG patients were 511.40 ± 242.10 μm (15 eyes) and 283.90 ± 92.27 μm (29 eyes), respectively (p < 0.05). Significant differences were found between the ipsilateral eyes of SWS and PWS patients (p < 0.05). Six of 13 eyes (46%) with choroidal hemangiomas exhibited post-operative posterior segment complications.
CONCLUSIONS
NGPWS and SG patients had a thicker choroid in the ipsilateral eye. The trend was even more pronounced in SG patients. Choroidal hemangiomas were only found in the ipsilateral eyes of SG. In addition, choroidal hemangioma was a risk factor for post-operative posterior segment complications in SWS patients.
Topics: Adolescent; Adult; Child; Child, Preschool; Choroid; Glaucoma; Humans; Port-Wine Stain; Sturge-Weber Syndrome; Tomography, Optical Coherence; Young Adult
PubMed: 33287757
DOI: 10.1186/s12886-020-01744-y -
The Journal of Dermatological Treatment Dec 2023Although pulsed dye laser (PDL) is the treatment of choice for port-wine stains (PWS), clinical resistance to PDL has been observed in 20-30% of cases. Several... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Although pulsed dye laser (PDL) is the treatment of choice for port-wine stains (PWS), clinical resistance to PDL has been observed in 20-30% of cases. Several alternative treatment modalities have been introduced; however, there is still a lack of definite recommendations regarding the optimal treatment for difficult-to-treat PWS.
OBJECTIVE
We aimed to systematically review and analyze the comparative effectiveness among treatments for problematic PWS.
METHODS & MATERIALS
We systematically searched for comparative studies assessing treatments for patients with difficult-to-treat PWS through relevant biomedical databases until August 2022. A Network Meta-Analysis (NMA) was conducted to estimate the odds ratio (OR) for all pairwise comparisons. The primary outcome is the improvement of lesions of more than 25%.
RESULTS
Of the 2498 studies identified, six treatments from five studies were available for NMA. Compared with 585 nm short-pulsed dye laser (SPDL), intense pulsed light (IPL) was the most effective in clearing lesions (OR 11.81, 95% CI 2.15 to 64.89, very low confidence rating), followed by 585 nm long-pulsed dye laser (LPDL) (OR 9.95, 95% CI 1.75 to 56.62, very low confidence rating). The 1064 nm NdYAG, 532 nm NdYAG, and LPDL >585 nm exhibited potential superiority over SPDL 585 nm, although statistical significance was not observed.
CONCLUSIONS
IPL and 585 nm LPDL are likely to be more effective than 585 nm SPDL for treating difficult-to-treat PWS. Well-designed clinical trials are warranted to confirm our findings.
Topics: Humans; Lasers, Dye; Low-Level Light Therapy; Network Meta-Analysis; Port-Wine Stain; Treatment Outcome
PubMed: 37424384
DOI: 10.1080/09546634.2023.2231582 -
Clinical Neurophysiology : Official... Oct 2021Port-wine birthmark (PWB) is a common occurrence in the newborn, and general pediatricians, dermatologists, and ophthalmologists are often called on to make an...
OBJECTIVE
Port-wine birthmark (PWB) is a common occurrence in the newborn, and general pediatricians, dermatologists, and ophthalmologists are often called on to make an assessment of risk for Sturge-Weber syndrome (SWS) due to workforce shortages in pediatric neurologists and MRI's low sensitivity for SWS brain involvement in infants. We therefore aimed to develop a quantitative EEG (qEEG) approach to safely screen young infants with PWB for SWS risk and optimal timing of diagnostic MRI.
METHODS
Forty-eight infants (prior to first birthday) underwent EEG recording. Signal processing methods compared voltage between left and right sides using a previously defined pipeline and diagnostic threshold. In this test sample, we compared sensitivity/specificity of the qEEG metric against MRI performed after the first birthday. We also used likelihood ratio testing to determine whether qEEG adds incremental information beyond topographical extent of PWB, another risk marker of brain involvement.
RESULTS
qEEG helped predict SWS risk in the first year of life (p = 0.031), with a sensitivity of 50% and a specificity of 81%. It added about 40% incremental information beyond PWB extent alone (p = 0.042).
CONCLUSION
qEEG adds information to risk prediction in infants with facial PWB.
SIGNIFICANCE
qEEG can be used to help determine whether to obtain an MRI in the first year of life. The data collected can assist in developing a predictive model risk calculator that incorporates both PWB extent and qEEG results, which can be validated and then employed in the community.
Topics: Cohort Studies; Electroencephalography; Female; Humans; Infant; Infant, Newborn; Male; Port-Wine Stain; Predictive Value of Tests; Prospective Studies; Sturge-Weber Syndrome
PubMed: 34454271
DOI: 10.1016/j.clinph.2021.06.030 -
Cureus Sep 2023As social media usage grows, more patients are turning to various platforms to gain and share medical information. One platform, TikTok, has become immensely popular,...
BACKGROUND
As social media usage grows, more patients are turning to various platforms to gain and share medical information. One platform, TikTok, has become immensely popular, with over one billion users. Despite its potential use as an educational tool, TikTok can be unreliable and misleading as a medical information source.
OBJECTIVE
We aim to discuss the information available on TikTok regarding laser treatment for port wine stains (PWS).
METHODS
Two independent reviewers analyzed 200 TikTok videos with the hashtags #portwinestainlaser or #portwinestaintreatment, examining the video creator's role (e.g., patient, parent, physician, or other), tone towards PWS and treatment options, and content (e.g., educational or non-educational, mention of any treatment risks).
RESULTS
Most videos were produced by non-medical professionals (83%), and only a small number discussed the potential adverse effects of PWS treatment options (15%).
CONCLUSION
While TikTok may have a role in educating patients about certain dermatologic conditions and treatment options, it is important to encourage patients to seek medical advice from a qualified medical professional before making any treatment decisions. Furthermore, the future of patient education may need to evolve to include social media platforms.
PubMed: 37842395
DOI: 10.7759/cureus.45119 -
Cureus Jan 2024Klippel-Trenaunay syndrome (KTS) is a rare genetic syndrome comprising an abnormal development of soft tissues and the lymphovascular system with bony overgrowth, venous...
Klippel-Trenaunay syndrome (KTS) is a rare genetic syndrome comprising an abnormal development of soft tissues and the lymphovascular system with bony overgrowth, venous malformation, and port wine stains. We present an interesting case of a three-year-old child brought to our hospital with a swollen limb and raised skin lesions associated with bleeding from minor trauma. Most of the clinical characteristics of KTS were seen in our patient, including arteriovenous, soft tissue, capillary, and lymphatic abnormalities. The diagnosis of KTS is based on clinical examinations and imaging investigations. He had gross hypertrophy of the left lower limb with measurable lengthening compared to the opposite limb. Ultrasonography of the left limb revealed soft tissue hypertrophy with abnormal venous communication. The management of KTS is mainly symptomatic and should be approached conservatively if the patient has functional limbs without edema, bleeding, ulceration, or pain.
PubMed: 38361716
DOI: 10.7759/cureus.52361