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BackgroundPort-Wine Birthmarks (PWB) are congenital capillary malformations requiring multiple treatments. Optical coherence tomography (OCT), a noninvasive imaging technique, characterizes vessels in cutaneous vascular lesions, including PWBs.ObjectiveTo assess variability in blood vessel characteristics within and between individual PWBs.MethodsOCT was used to measure blood vessel density (%) and modal vessel diameter (micrometers) at increments of 0.05 mm from the skin surface to a depth of 0.50 mm at several adjacent spots of single PWBs in this cross-sectional study. Average ratios of vessel density and diameter in affected to control skin were obtained for each PWB by averaging data for all spots within a lesion. Statistical analysis was performed with a linear mixed effects model using SPSS software (IBM Corporation).ResultsThere was great variability in vessel density and diameter within and between PWBs. Depths where average ratios of vessel density were consistently greater in affected to control skin were shallow, between 0.15 mm and 0.2 mm deep from the skin surface.LimitationsSmall sample size and device's inability to measure diameters smaller than 20 micrometers.ConclusionThere is variability in vessel density and diameter within and between PWBs. Individualized treatment planning guided by OCT mapping should be studied further.

This article provides a comprehensive review of literature in the field of vascular anomalies from 2023 to 2024, focusing on innovative research findings. It covers new mechanisms, applications of new materials, novel diagnostic and classification concepts, and therapeutic advancements. Arranged according to the ISSVA classification and the "Guidelines for the Diagnosis and Treatment of Hemangiomas and Vascular Malformations (2024)" by the Group of Vascular Anomalies, Chinese Society of Plastic Surgery, Chinese Medical Association, the article introduces the diagnosis and treatment updates for each classified disease. It particularly emphasizes cutting-edge advancements in basic research, preclinical studies, genetics, and targeted therapies. The article presents a series of promising new discoveries and conclusions, aiming to stimulate clinical interest and encourage a deeper commitment to mechanistic research. [ABSTRACT FROM AUTHOR]

To assess the clinical efficacy and adverse reactions of 595nm PDL for treating pediatric PWS and to identify factors influencing treatment outcomes. Methods: This retrospective study included 974 pediatric patients with PWS treated at Beijing Children's Hospital from 2003 to 2021. Inclusion criteria required patients to be under 18, with solitary PWS and Fitzpatrick skin types II–IV. Treatment efficacy was evaluated using standardized photographs taken before and after treatment, with outcomes categorized by Achauer's clearance criteria. Ridit analysis assessed the impact of variables such as gender, age, lesion location, and treatment frequency on outcomes. Results: The overall efficacy rate was 65.3%, higher in females (69.7%) than males (59.7%). Adolescents had the highest efficacy (100%) due to better compliance. Limb lesions responded best (87.5%), followed by neck (75.0%), trunk (66.7%), and head/face (63.0%). Smaller lesions (≤ 3 cm²) achieved 76.8% efficacy. Efficacy rose with sessions, reaching 84.0% after three. The mandibular branch (V3) had the highest trigeminal efficacy (68.5%). Adverse reactions (4.31%) included pigmentation changes (2.87%), hypopigmentation (1.03%), and minimal scarring (0.41%). Conclusion: The efficacy of 595nm PDL for treating PWS in children is influenced by gender, age, lesion location, size, and the number of treatment sessions. PDL is an effective treatment for pediatric PWS with minimal adverse reactions. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Klippel–Trénaunay syndrome can present with atypical manifestations such as a bleeding vulvar hemangioma. This case report, the first documented in Uganda, highlights the need for awareness of such presentations and underscores the importance of continuous follow‐up in female patients to manage potential complications throughout adolescence and pregnancy. Klippel–Trénaunay syndrome (KTS) is a rare congenital disorder marked by bone and soft tissue hypertrophy, port‐wine stains, and varicosities. Cases involving genital hemangiomas are rare. This report highlights a 5‐year‐old girl in Uganda with typical KTS features, including hypertrophy and port‐wine stains, along with a bleeding vulvar hemangioma, emphasizing its uncommon presentation and potential complications. Treatment involved compression bandaging and timolol 0.2% solution. This case underscores the importance of awareness of atypical manifestations of hemangiomas with KTS and continuous follow‐up for female patients, especially through adolescence and pregnancy, due to potential complications such as prepubertal per vaginal bleeding, menorrhagia, and post‐partum bleeding. [ABSTRACT FROM AUTHOR]

Key Clinical Message: This report presents a 14‐year‐old male with seizures and facial port‐wine stains, who upon further evaluation was found to have SWS. Early diagnosis and consistent treatment of Sturge–Weber syndrome in children are essential to prevent seizures and improve quality of life. Anti‐seizure medications play a crucial role in preventing and controlling seizures. [ABSTRACT FROM AUTHOR]

Port wine stain (PWS) is a congenital vascular malformation that commonly occurs on the face and neck. Currently, the main treatments for port wine stain are pulsed dye laser (PDL) and photodynamic therapy (PDT). However, the efficacy evaluation of PWS mostly relies on the subjective judgement of clinicians, and it is difficult to accurately respond to many small changes after treatment. Therefore, some non-invasive and efficient efficacy assessment methods are also needed. With the continuous development of technology, there are currently many visualisation instruments to evaluate PWS, including dermoscopy, VISIA-CR™ system, reflectance confocal microscopy (RCM), high-frequency ultrasound (HFUS), optical coherence tomography (OCT), Photoacoustic imaging (PAI), laser speckle imaging (LSI) and laser Doppler imaging (LDI). Among them, there are simple and low-cost technologies such as dermoscopy and the VISIA-CR™ system, but they may not be able to observe the deeper structures of PWS. At this time, combining techniques such as HFUS and OCT to increase penetration depth is crucial to evaluate PWS. In the future, the combination of these different technologies could help overcome the limitations of a single technology. This article provides a systematic overview of non-invasive methods for evaluating treatment efficacy in port wine stains and summarises their advantages and disadvantages. [ABSTRACT FROM AUTHOR]

Awab H Saad,1,* Saeed Mohammed Omar,2,* Abeir Abbas Elgilli,2,* Ibtehal Abdalwahid Abdalmjeed Omer,2,* Morouj Hussein Jalaleldeen2,* 1Department of Internal Medicine, University of Medical Sciences and Technology, Khartoum, Khartoum State, Sudan; 2Department of Internal Medicine, University of Gadarif, Gadarif, Gadarif State, Sudan*These authors contributed equally to this workCorrespondence: Awab H Saad, Tel +249961162550 ; +966535911050, Email awabhashim95@gmail.comBackground: Sturge-Weber syndrome (SWS) is a complex rare genetic neuro-cutaneous disorder characterized by the presence of a port-wine stain, ophthalmic and intracranial angiomatosis leading to seizures, ocular, and oral abnormalities.Case Presentation: We report a 39-year-old, non-diabetic, non-hypertensive female refugee who presented initially with heart failure due to anemia for which she received blood transfusions. Later on admission, she developed multiple focal to bilateral seizures, severe irritability, aphasia, and right-sided hemiplegia, leading to admission to the ICU. A repeat medical history and examination revealed a faint left-sided ophthalmic port-wine stain that was initially unnoticed and a remote history of unprovoked seizures 20 years ago. Imaging revealed parietal calcifications and confirmed the diagnosis of SWS. Thus, a multidisciplinary approach was taken to fully understand the patient’s diagnosis and determine a treatment strategy, involving consultations with the neurology, ophthalmology, otolaryngology, and physiotherapy departments. Successful seizure control was achieved by administering IV phenytoin for 3 days and the up-titrating of oral carbamazepine to 1g daily through a nasogastric tube. Unfortunately, due to the unavailability of personnel or resources, other important assessments for patients with SWS, such as advanced neuroimaging, psychiatric, plastic and neuro-surgery evaluations, as well as dentistry reviews, could not be conducted.Conclusion: This case highlights the rare occurrence of adult-onset seizures in an undiagnosed SWS and their re-emergence after almost two decades without anti-seizure medications. It also highlights the importance of a comprehensive history and clinical examination, as this patient’s diagnosis of SWS could have been missed if she had not experienced seizures on admission. Our study also demonstrates the challenges associated with managing such a complex condition in settings with limited resources.Keywords: genetic, angioma, seizure, glaucoma, port-wine stain

The Koebner phenomenon (KP), also known as the isomorphic response, describes the process by which new lesions that are clinically and histologically identical to a patient's existing skin disease develop following trauma. Many skin diseases exhibit this characteristic, with variations that include possible, questionable, and pseudo-Koebner reactions, with the latter category occurring due to infectious agents seeding at a trauma site. Laser application, a type of controlled skin injury used for improving cutaneous lesions and skin rejuvenation, is also considered a form of trauma. This raises the question of whether controlled thermal injury can be regarded as a type of mechanical trauma capable of producing Koebner-related reactions. We conducted a literature review of cases or studies to identify laser-induced dermatoses that correspond to Koebner-related or pathergy reaction categories. As a whole, we identified nine case reports on true KPs, two cases on possible KPs, seventeen cases on laser-induced questionable KPs comprising cases of vasculitis, eczema or Meyerson reactions, and eruptive squamous atypia cases (ESA) as well as two pseudo-Koebner cases involving wart occurrences at laser application sites. Laser-induced Koebner reactions highlight several aspects of the KP. Firstly, the type of mechanical damage influences disease promotion, as different lasers are associated with different KPs. For example, hair removal lasers are linked with true and questionable KPs such as vasculitis while resurfacing lasers were found to be more connected with ESA occurrence. Secondly, the laser target is significant, with vascular laser application for port-wine stains tending to result in eczematous reactions, while hair follicle destruction can frequently lead to true KPs. Thirdly, the number of sessions matters; true KPs and eruptive squamous atypia questionable KPs typically appear after one to two sessions, whereas eczematous reactions require more sessions (at least four). Additionally, skin phototype is crucial, with darker phototypes showing a higher KP frequency as laser treatment for hypertrichosis relies on melanin absorption in the hair bulge or bulb for follicle destruction, as chromophore competes with the abundant melanin in the epidermis. Further research with larger-scale studies into trauma-specific Koebner reactions is vital for refining treatment protocols, minimizing post-laser adverse effects, and improving dermatological care outcomes. [ABSTRACT FROM AUTHOR]

Key Clinical Message Klippel–Trénaunay syndrome can present with atypical manifestations such as a bleeding vulvar hemangioma. This case report, the first documented in Uganda, highlights the need for awareness of such presentations and underscores the importance of continuous follow‐up in female patients to manage potential complications throughout adolescence and pregnancy. Abstract Klippel–Trénaunay syndrome (KTS) is a rare congenital disorder marked by bone and soft tissue hypertrophy, port‐wine stains, and varicosities. Cases involving genital hemangiomas are rare. This report highlights a 5‐year‐old girl in Uganda with typical KTS features, including hypertrophy and port‐wine stains, along with a bleeding vulvar hemangioma, emphasizing its uncommon presentation and potential complications. Treatment involved compression bandaging and timolol 0.2% solution. This case underscores the importance of awareness of atypical manifestations of hemangiomas with KTS and continuous follow‐up for female patients, especially through adolescence and pregnancy, due to potential complications such as prepubertal per vaginal bleeding, menorrhagia, and post‐partum bleeding.

Key Clinical Message This report presents a 14‐year‐old male with seizures and facial port‐wine stains, who upon further evaluation was found to have SWS. Early diagnosis and consistent treatment of Sturge–Weber syndrome in children are essential to prevent seizures and improve quality of life. Anti‐seizure medications play a crucial role in preventing and controlling seizures.

Background and objectivesPort wine birthmark, also known as port wine stain (PWS) is a skin discoloration characterized by red/purple patches caused by vascular malformation. PWS is typically treated by using lasers to destroy abnormal blood vessels. The laser heating facilitates selective photothermolysis of the vessels and attenuates quickly in the tissue due to high optical scattering. Therefore, residual abnormal capillaries deep in the tissue survive and often lead to the resurgence of PWS. Ultrasound (US) has also been proposed to treat PWS, however, it is nonselective with respect to the vasculature but penetrates deeper into the tissue. We aim to study the feasibility of a hybrid PWS treatment modality combining the advantages of both modalities.Materials and methodsIn this manuscript, we propose a photoacoustic (PA) guided US focusing methodology for PWS treatment which combines the optical contrast-based selectivity with US penetration to focus the US energy onto the vasculature. The PA signals collected by the transducers, when time-reversed, amplified, and transmitted, converge onto the PWS, thus minimally affecting the neighboring tissue. We performed two- and three-dimensional simulations that mimic realistic transducers and medium properties in this proof of concept study.ResultsThe time-reversed PA signals when transmitted from the transducers converged onto the vasculature, as expected, thus reducing the heating of the neighboring tissue. We observed that while the US focus is indeed affected due to experimental factors such as limited-view, large detector separation and finite detection bandwidth, and so forth, the US did focus completely or partially onto the vasculature demonstrating the feasibility of the proposed methodology.ConclusionThe results demonstrate the potential of the proposed methodology for PWS treatment. This treatment method can destroy the deeper capillaries while minimally heating the neighboring tissue, thus reducing the chances of the resurgence of PWS and as well as cosmetic scarring.

Sturge-Weber syndrome (SWS) is a neurocutaneous disorder that classically presents with a triad of vascular anomalies affecting the skin, eyes, and brain. Previously, the trigeminal nerve distribution of a port-wine birthmark (PWB) of the face was used to identify risk of SWS. However, recent evidence has demonstrated that PWBs are vascular, not neurologic, in embryologic origin, and facial PWBs at highest risk for the brain involvement of SWS involve the forehead location. Furthermore, a PWB involving the upper or lower eyelid carries a risk of glaucoma, which requires lifelong monitoring. The gold standard of treatment for PWB is the pulsed dye laser, which has many advantages when started as early as possible in infancy. In this review, we discuss the locations of facial PWBs at risk for neurologic and ophthalmologic complications, the differential diagnosis of facial vascular birthmarks, recommendations for patient referral(s) when needed, and the advantages of early laser therapy when desired for the PWB. We also provide additional resources for pediatricians to support patients and their families.

Sánchez-Espino LF, Ivars M, Antoñanzas J, Baselga E. Appl Clin Genet. 2023;16:63–81. It has come to our attention that there is an error in the paper title on page 63, the title “Sturge-Weber Syndrome: A Review of Pathophysiology, Genetics, Clinical Features, and Current Management Approache” should read “Sturge-Weber Syndrome: A Review of Pathophysiology, Genetics, Clinical Features, and Current Management Approach”.

Photocoagulation of blood vessels offers unambiguous advantages to current radiofrequency approaches considering the high specificity of blood absorption at available laser wavelengths (e.g., 532 nm and 1.064 µm). Successful treatment of pediatric vascular lesions, such as port-wine stains requiring microvascular hemostasis, has been documented. Although laser treatments have been successful in smaller diameter blood vessels, photocoagulation of larger sized vessels is less effective. The hypothesis for this study is that a primary limitation in laser coagulation of large diameter blood vessels (500-1000 µm) originates from shear stress gradients associated with higher flow velocities along with temperature-dependent viscosity changes. Laser (1.07 µm) coagulation of blood vessels was tested in the chicken chorio-allantoic membrane (CAM). A finite element model is developed that includes hypothetical limitations in laser coagulation during irradiation. A protocol to specify laser dosimetry is derived from OCT imaging and angiography observations as well as finite element model results. Laser dosimetry is applied in the CAM model to test the experimental hypothesis that blood shear stress and flow velocity are important parameters for laser coagulation and hemostasis of large diameter blood vessels (500-1000 µm). Our experimental results suggest that shear stress and flow velocity are fundamental in the coagulation of large diameter blood vessels (500-1000 µm). Laser dosimetry is proposed and demonstrated for successful coagulation and hemostasis of large diameter CAM blood vessels.