Port-wine Stains Research Articles

Sporadic hypertrophic and nodular port-wine stain: a study of 27 cases with emphasis on histological features and novel mutation type

AimsTo investigate the clinicopathological features and molecular characteristics of sporadic hypertrophic and nodular port-wine stains (PWS).MethodsWe analysed the clinicopathological and molecular characteristics of 27 sporadic hypertrophic and nodular PWS retrieved...

Open reduction and plate fixation of fractures of both bones in the forearm in Klippel-Trenaunay-Weber syndrome: a case report

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare disease characterized by vascular malformations, port-wine staining, and soft tissue and bone hypertrophy. Lesions occur in the lower extremities in 95% of cases, whereas only 5% occur in the upper extremities. Several case reports have described the treatment of fractures in the lower extremities in patients with KTWS. However, the risk of massive bleeding, bone deformity, and poor bone quality can lead to suboptimal surgical outcomes. No reports describing the treatment of forearm shaft fractures in KTWS could be found in the English-language literature. Intramedullary nailing is difficult due to the deformed bone and the risk of nonunion. Open reduction and internal fixation (ORIF) should be considered with caution in KTWS patients due to the risks of intraoperative bleeding and wound problems. The authors report that satisfactory results were obtained with ORIF after preoperative vascular embolization in a 60-year-old KTWS patient with fractures in both bones of the forearm.

A rare case of a 5-year-old girl with Klippel-Trénaunay syndrome and a bleeding focal vulvar hemangioma in Uganda.

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.

AESTHETIC SURGICAL APPROACH FOR STURGE-WEBER SYNDROME AND FACIAL ARTERIOVENOUS TUMORS

Introduction: Sturge-Weber Syndrome (SWS) is a rare, congenital neurovascular condition associated with facial cutaneous capillary or venous malformations. The manifestations are intrinsically related to vascular malformations, resulting in neurological symptoms, soft tissue abnormalities, and restricted bone growth. Methodology: This is a case report of a single patient diagnosed with SWS, following the guidelines established by Resolution 466/12 of the National Health Council of Brazil (CNS). Results and Discussion: A Vascular Surgery approach with embolization was performed prior to the Plastic Surgery intervention. It is important to note that this particular patient did not present the common neurological manifestations. Additionally, port-wine stains manifested as giant hemangiomas on the face. Conclusion: A multidisciplinary team is essential for patients with this condition, as it can facilitate surgical resection when vascular studies are performed prior to surgery. Moreover, a multidisciplinary approach is crucial due to the varied clinical presentation and chronic conditions that require treatment.

Intracerebral hemorrhage in Sturge Weber Syndrome: A case report

Background. Encephalotrigeminal angiomatosis also called Sturge Weber Syndrome (SWS) is neurocutaneous abnormality with angioma affecting the leptomeninges and the face skin, commonly in the eyes and maxillary distribution of trigeminal nerve. The characteristic of SWS is dilated face skin veins, and referred to nevus flammeus and port wine stain. Case reports. We report a case of Sturge Weber Syndrome in a 41 years old male with uncontrolled hypertension and smoking, left-sided weakness, and left-sided facial port wine stain. Conclusions. Neurological examination was suggestive of SWS and further radiological examination confirmed the diagnosis.

Optical clearing agent combined with hemoporfin-mediated photodynamic therapy for improved efficacy of port-wine stain patients: A pilot study.

Optical clearing agent combined with hemoporfin-mediated photodynamic therapy for improved efficacy of port-wine stain patients: A pilot study.

Sturge weber syndrome: A case report.

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.

Sturge-Weber Syndrome: a case report

Sturge-Weber syndrome is a rare congenital, non-familial sporadic condition also known as encephalotrigeminal angiomatosis, is a phakomatoses consisting of neurological, skin and ocular manifestations. Symptoms reported varies in severity and age of onset but it is commonly detected in infancy. The Ophthalmic feature of this disease consists of glaucoma and is also associated with vascular manifestation of the conjunctiva, epi-sclera, choroid and retina. In the present case, a 19-year-old male presented with a port wine stain on the right side of the face, leptomeningeal angioma, ocular hypertension, temporal hemianopia of the left eye and choroidal hemangioma with macular scar of the right eye, seizure and weakness of the left side of the body. The purpose of presenting the case is to illustrate both the characteristic presentation and to underline the importance of its diagnosis in the clinical ophthalmological practice. BIRDEM Med J 2024; 14(3): 166-169

Potential efficacy of fractional CO<sub>2</sub> laser in treating port wine stain birthmarks with hypertrophy

Potential efficacy of fractional CO<sub>2</sub> laser in treating port wine stain birthmarks with hypertrophy

54402 Investigating a Possible Association of Hearing Impairment with Port-Wine Stains and Sturge-Weber Syndrome

54402 Investigating a Possible Association of Hearing Impairment with Port-Wine Stains and Sturge-Weber Syndrome

52542 Evaluation of Private and Medicaid Insurance Coverage for Port-Wine Stain Treatment with Pulsed Dye Laser

52542 Evaluation of Private and Medicaid Insurance Coverage for Port-Wine Stain Treatment with Pulsed Dye Laser

Maternal history of angioma is associated with infantile hemangioma and port-wine stain in children: a population-based, cohort study of mother-child pairs from the United Kingdom.

The two most prevalent childhood vascular abnormalities are infantile hemangioma (IH) and port-wine stain (PWS). They become apparent shortly after birth but have distinct pathophysiology and clinical manifestations. The goal of this study was to determine if mother's history of angioma or PWS is associated with these vascular abnormalities. We evaluated an UK anonymized electronic medical records database with medical records that were linked between children and their mothers. Cox proportional hazards models were used to evaluate the association between maternal factors and the time of onset of either IH or PWS in children. Between 2004 and 2021, 639,085 children were linked to their mom's medical data with a total of 4,270,773 person-years of follow up. Children born to mothers with an angioma as compared to a mother without an angioma were more than 60% more likely to have an IH (HR: 1.64 [1.07, 2.52]). Children born to mothers with a PWS as compared to children born to mothers without a PWS were nearly 20 times more likely to have a PWS (18.95 [4.71,76.26]). Mothers with angiomas were not more likely to have children with PWS and mothers with PWS were not more likely to have children with IH. The effect estimates were minimally changed after adjustment. We demonstrated that children born to mothers with angiomas or PWS were at increased risk of IH or PWS, respectively.

A feasible way to explore real blood vessels thermal responses to laser irradiation by combing optical clearing and the reflectance spectra measurements: animal experiment study.

Laser therapy has been widely used to treat port-wine stains (PWS) and other cutaneous vascular lesions via selective photothermolysis. Animal models are a valuable tool for investigating thermal responses beneath the skin. However, in previous animal experiments, such as the dorsal skin chamber model, one side of the skin was removed, resulting in the loss of mechanical support for the target blood vessel. In this study, the optical clearing technique was applied to the dorsal skin, allowing direct observation of real thermal responses within the tissue without removing the covering skin. The target blood vessels were irradiated with a pulsed 1064nm Nd: YAG laser. The corresponding thermal responses were recorded using a CCD camera. Additionally, variations in skin reflectance spectra were measured before and after laser irradiation. Due to the optical clearing and reflectance spectra measurement, vessel responses such as contraction, reperfusion, and full occlusion were correlated with specific variation patterns in reflectance spectral signals.

Evaluation of Private and Medicaid Insurance Coverage for Port-Wine Stain Treatment with Pulsed Dye Laser

Evaluation of Private and Medicaid Insurance Coverage for Port-Wine Stain Treatment with Pulsed Dye Laser

Laser-Induced Koebner-Related Skin Reactions: A Clinical Overview.

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.

Relationship Between Dermoscopic Vascular Pattern and Laser Efficacy in Children with Port Wine Stains

Relationship Between Dermoscopic Vascular Pattern and Laser Efficacy in Children with Port Wine Stains

Topical sirolimus in dermatology: a systematic review.

Topical sirolimus is increasingly utilised off-license to manage various dermatological conditions whilst avoiding typical adverse effects associated with systemic sirolimus. However, widespread use is limited by a highly heterogeneous evidence base of mixed quality. to evaluate the current evidence base for the indications, efficacy and safety profile for topical sirolimus in dermatology. A literature search was conducted from 2005 to July 4th, 2023, of English language studies, with the following databases consulted: MEDLINE, PubMed, Embase, CENTRAL and EBSCO. Key words included 'topical', 'rapamycin', 'sirolimus' and 'dermatology'. Data on drug efficacy, concentration, side effects, co-interventions and follow up were extracted. The search identified 202 studies; 71 studies met the inclusion criteria. Efficacy of topical sirolimus was demonstrated in facial angiofibromas (799 patients) compared to placebo across multiple randomised controlled trials with a predominant concentration of 0.1%. Evidence was mixed for sirolimus use in port-wine stains (61 patients), with evidence of effectiveness in combined sirolimus and pulsed-dye laser. Multiple case reports demonstrated clinical improvement with topical sirolimus use in cutaneous vascular abnormalities (33 patients) at a higher concentration of 1%. Other applications of topical sirolimus were predominantly case reports demonstrating generally favourable outcomes. Topical sirolimus was generally well tolerated - most reported adverse effects were localised irritation and pruritus. Ointment-based preparations and once-daily dosing appeared to confer a better side effect profile. Most high-quality data pertain to the efficacy of topical sirolimus in treating facial angiofibromas in tuberous sclerosis. Outcomes are generally promising in other indications and good tolerability, but data quality is mixed.

Under Pressure: A Case of Developmental Glaucoma and Congenital Glaucoma in Sturge–Weber Syndrome

Abstract Sturge–Weber syndrome (SWS), also known as encephalo-trigeminal angiomatosis, is a sporadic, congenital neurocutaneous disorder. It is characterized by capillary malformation affecting the brain, meninges, eyes, and skin. The ophthalmic, maxillary, and mandibular distributions of the trigeminal nerve are involved. Eye involvement with vascular malformation produces glaucoma and can lead to vision loss. Here, we present a rare case of SWS with port-wine stain, painful vision loss in left eye, and open-angle glaucoma in the other eye of the same patient. The painful blind eye was treated with cyclocryotherapy with successful control of intraocular pressure.

A Cautionary Tale of Vascular Abnormalities in Klippel-Trenaunay Syndrome: A Case Report

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder characterized by a port-wine stain birthmark, venous anomalies, and soft-tissue hypertrophy. Diagnosis of KTS is made based on two of the three characteristic diagnostic features. Klippel-Trenaunay syndrome is unilateral and more commonly affects the lower extremities. This case report describes a unique patient who lacked a patent deep system in the affected limb, making the traditional treatment of varicosities a dangerous outcome for this patient.

Clinical use of lasers and energy-based devices in selected skin diseases

Introduction and purpose In recent years, there has been notable progress in the role of light-emitting technologies, including lasers and other energy-based devices, across various medical disciplines. Dermatology stands out as one of the prominent fields where laser treatments have gained significant popularity. In the clinical setting, a variety of laser types are utilized. A fundamental aspect of ensuring the safe use of laser devices involves comprehending their mechanisms at the tissue level. This knowledge enables practitioners to attain the desired outcomes effectively while minimizing the risk of complications. Lasers have demonstrated effectiveness in treating cutaneous vascular conditions because of their capacity to selectively target intravascular oxyhemoglobin. Rosacea, juvenile hemangioma, and port wine stains are among the vascular skin conditions for which laser therapy has shown notable success. Laser therapy is employed in treating hypertrophic scars, keloids, and acne scars because it facilitates the restructuring of the skin's architecture at the scar site, with the goal of minimizing its size and visibility. Laser therapy stands as the gold standard for removing tattoos. Materials and methods The methodology for conducting literature search involved utilizing medical subject headings terms to explore PubMed. Search terms included: “lasers”, “intense pulsed light”, “dermatology”, “rosacea”, “acne”, “scars”, “tattoo removal”. Conclusions Laser dermatological treatments are gaining popularity worldwide, driving new innovations and clinical applications. This review highlights the significant role and diverse clinical applications of lasers and intense pulsed light in dermatological practice. Improving the quality of skin in patients suffering from skin diseases can significantly increase their quality of life.