Port wine stains

Sturge-Weber syndrome (SWS) is a rare congenital disorder associated with systemic vascular malformations characterized by port-wine stains, epilepsy, and glaucoma. Patients with SWS can develop stroke-like symptoms such as hemiparesis. We report a case of a 63-year old woman with SWS who developed left-sided hemiparesis and was finally diagnosed with myelin-oligodendrocyte glycoprotein (MOG) antibody-positive encephalitis. Brain magnetic resonance imaging (MRI) revealed right-dominant bilateral leptomeningeal enhancement, thickened dura mater, and a cerebellar lesion...

UNLABELLED: Sturge-Weber syndrome is a rare congenital neurocutaneous syndrome with an incidence of 1 in 50000 characterised by facial capillary malformation and vascular anomalies in the brain and eye. We present the case of a five-year-old child diagnosed with Sturge-Weber syndrome. The patient exhibited high-grade fever, headaches, and generalized tonic-clonic seizures. The history revealed a port-wine stain on the face and a history of seizures from the age of four months. Diagnostic imaging confirmed the presence of leptomeningeal vascular malformation, calcification in the brain, and abnormal electroencephalogram patterns, establishing the diagnosis of Sturge-Weber syndrome...

Sturge-Weber Syndrome (SWS) is a rare congenital developmental disorder that arises from the abnormal persistence of the embryonic vascular plexus. The syndrome encompasses hamartomatous malformations that can impact the skin, eyes, and nervous system. The broad array of clinical manifestations and potentially life-threatening complications underscores the crucial and imperative need for an accurate diagnosis. An effective treatment strategy for SWS patients involves a multidisciplinary approach. Dental procedures in these individuals pose challenges due to the potential risk for substantial bleeding during both intra- and postoperative phases...

A 17-year-old boy with typical neurodevelopment presented with 3 months of lower-limb weakness without a history of trauma. Physical examination, plain radiography and a T2-weighted MRI revealed a port-wine stain over the lumbar region, significant scoliosis below the cervical segment, and a multifocal flow void sign (steal phenomenon) from epidural space to dorso-lumbar spine, respectively (Figure 1). CT angiography identified an enhancing dorsal vascular mass with involvement of the spinal canal including destruction of the vertebral body determined to be extensive arteriovenous shunts on digital subtraction angiography (DSA) (Figure 2)...

Klippel-Trenaunay syndrome (KTS) is characterized by port-wine stains, mixed vascular malformations, and soft tissue and bone hypertrophy. Klippel-Trenaunay syndrome is occasionally complicated by chyluria, for which there is no effective treatment currently. We report a case of KTS complicated by intractable chyluria and hematuria due to a lymphatic-ureteral fistula. The patient was successfully treated with multiple lymphaticovenular anastomoses (LVAs).A 66-year-old woman with an enlarged left lower extremity since childhood was diagnosed with KTS...

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Pulsed dye laser (PDL) is the most commonly used method for port-wine stain (PWS); however, no studies have reported the safety of PDL. This review aimed to collect and summarize complications reported in relevant literature, assess complication rates in treating PWS with PDL, and explore the relevant influencing factors. A systematic review and meta-analysis were conducted to search for related studies in PubMed, Embase, and the Cochrane Library until August 2022. Two reviewers independently evaluated the risk of bias of included studies...

BACKGROUNDS: Hematoporphyrin monomethyl ether mediated photodynamic therapy (HMME-PDT) has emerged as an alternative approach for port-wine stain (PWS), which was primarily treated with pulsed dye laser (PDL). This study was aimed to evaluate the efficacy and safety of HMME-PDT for PWS and to explore influential factors on the efficacy. METHODS: A total of 254 patients were enrolled. Patients received an intravenous injection of HMME at 5mg/kg. Lesion areas were irradiated with 532-nm light for 20-25min...

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a rare congenital disease that mainly involves blood vessels and is characterized by the presence of capillary malformations (port wine stains), varicose veins, soft tissue and/or bone hypertrophy. CASE PRESENTATION: We report a 28-year-old man who was diagnosed 20 years ago with Klippel-Trenaunay syndrome. Approximately 3 years ago, he found enlarged masses on both upper extremities and a new dark red mass that was pathologically diagnosed as cavernous haemangioma appeared on the right index finger...

No abstract text is available yet for this article.

We present the case of a pregnant young woman with symptomatic diffuse choroidal hemangioma (CH), diagnosed with Sturge-Weber syndrome (SWS). Three months postpartum, there was full spontaneous resolution of the subretinal fluid and improvement in the visual acuity (VA). A 29-year-old, 31-week pregnant female with a coagulation disorder and enoxaparin (clexane) treatment, complained of a left visual disturbance of 2 weeks duration. On examination, the right eye was normal; left eye VA was 6/12 (20/40) with a left eyelid port-wine stain and on funduscopic examination there was a serous retinal detachment...

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...

No abstract text is available yet for this article.

Laser technology has significantly improved giving better results, which in turn has led to an increase in the indications for laser therapy. Vascular anomalies comprise vascular tumors and malformations. They are classified according to the type of vessels involved including arteries, capillaries, postcapillary venules, veins, lymphatic vessels, and a combination of two or more of these. Laser needs a chromophore to get absorbed and act. Hemoglobin, both oxy and deoxy, is one of the naturally occurring chromophore that is abundant in vascular lesions...

Photo-mediated ultrasound therapy (PUT) is a cavitation-based, highly selective antivascular technique. In this study, the effectiveness and safety of PUT on cutaneous vascular malformation was examined through in vivo experiments in a clinically relevant chicken wattle model, whose microanatomy is similar to that of port-wine stain and other hypervascular dermal diseases in humans. Assessed by optical coherence tomography angiography, the blood vessel density in the chicken wattle decreased by 73.23% after one session of PUT treatment in which 0...

A 6-year-old boy with multiple capillary malformations of the port-wine birthmark (PWB) type on the right leg since birth presented with a varicose vein and segmental overgrowth of the affected leg. Genetic testing on affected skin confirmed the presence of a somatic novel pathogenic HRAS 30 bp in-frame duplication/insertion in the switch II domain. This case illustrates the phenotypic overlap of different genotypes and shows that somatic HRAS pathogenic variants, especially in-frame duplications/insertions, must be added to the list of the underlying causes in capillary malformations...

Capillary malformations (port-wine stains) are congenital skin lesions that are characterized by dilated capillaries and post-capillary venules. Capillary malformations are caused by altered functioning of the vascular endothelium. Somatic genetic mutations have predominantly been identified in the endothelial cells of capillary malformations, providing an opportunity for the development of targeted therapies. However, there is currently limited in-depth mechanistic insight in the pathophysiology and a lack of pre-clinical research approaches...

OBJECTIVES: Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder that is characterized by a segmental dermatomal facial port-wine stain birthmark and is frequently accompanied by ipsilateral brain and eye abnormalities. We present a case of a patient with SWS who exhibited hypogonadotropic hypogonadism, growth hormone (GH) deficiency, and central hypothyroidism at the age of 20 despite the absence of radiographic findings in the pituitary and hypothalamus...

Klippel-Trenaunay syndrome (KTS) is a very rare vascular malformation syndrome also referred to as a capillary-lymphatic-venous malformation with unknown aetiology. The aim of our paper is to highlight interesting images, regarding a rare case of foetal Klippel-Trenaunay syndrome diagnosed prenatally in our department and confirmed postnatally with a favourable evolution during the gestation and neonatal periods. This case was diagnosed at 26 weeks gestation and characterised through ultrasound by the presence of superficial multiple cystic structures of different sizes spreading over the left leg with hemihypertrophy and reduced mobility...

Vascular lesions impact up to 5% of children and range in clinical impact from minor cutaneous aberrations to large masses impacting both form and function. Vascular lesions may be characterized as tumors or malformations. Establishing a clear diagnosis is imperative to understanding the natural history of a vascular lesion and developing a treatment plan. Medical, surgical, intralesional, and laser therapy are all effective and indicated on a case-by-case basis. There are a number of important surgical considerations for operative management of these lesions...