-
Photodiagnosis and Photodynamic Therapy Feb 2024Hematoporphyrin monomethyl ether mediated photodynamic therapy (HMME-PDT) has emerged as an alternative approach for port-wine stain (PWS), which was primarily treated...
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 5 mg/kg. Lesion areas were irradiated with 532-nm light for 20-25 min. Efficacy was assessed according to fading of lesions and graded as excellent (≥90 %), good (60 %-89 %), fair (20 %-59 %), or poor (<20 %). Adverse events were recorded. Clinical data were analyzed including gender, age, lesion sub-type, lesion location and number of treatments.
RESULTS
Overall, 72.4 % of patients achieved an effective response, with 27.6% showing excellent efficacy, 24.8 % showing good efficacy and 20.1 % showing fair efficacy. Only 27.6 % showed poor efficacy. Patients under the age of 18 obtained a better efficacy than adults. Lesions in face showed a better therapeutic outcome than those in neck or trunk and extremities. A more effective response was seen in pink type compared with nodular thickening type. Multiple HMME-PDT treatments could improve the clinical response. Lesion location, lesion sub-type, number of treatments were independent influential factors on efficacy. Adverse events included edema, blister, crust, hypopigmentation, hyperpigmentation, pain, itch and burning sensation. No severe systemic side events were observed.
CONCLUSIONS
HMME-PDT was effective for treating PWS and was safe and well-tolerated by patients. It is worth further investigation in efficacy and safety involving more patients from medical institutions in different regions in China. The optimal treatment parameters and treatment protocols are still being explored in the clinical treatment for PWS.
Topics: Adult; Humans; Photochemotherapy; Port-Wine Stain; Photosensitizing Agents; Hematoporphyrins; Treatment Outcome
PubMed: 38097121
DOI: 10.1016/j.pdpdt.2023.103933 -
Pediatric Dermatology 2024Capillary malformation-arteriovenous malformation is a rare autosomal dominant disorder associated with EPHB4 loss-of-function mutations. We report the...
Capillary malformation-arteriovenous malformation is a rare autosomal dominant disorder associated with EPHB4 loss-of-function mutations. We report the unique presentation of a 6-year-old girl with multiple capillary malformations in a unilateral segmental distribution affecting the right hemiface, right upper chest, and right arm associated with overgrowth. Targeted next-generation sequencing on a tissue sample revealed a novel heterozygotic variant in the EPHB4 gene (NM_004444.5 (EPHB4): c.715T>A, p.[Cys239Ser]). This case highlights a distinct presentation of CM-AVM type 2 and showcases a new variant in EPHB4 not previously reported in the literature.
Topics: Female; Humans; Child; p120 GTPase Activating Protein; Port-Wine Stain; Arteriovenous Malformations; Capillaries; Mutation
PubMed: 38092051
DOI: 10.1111/pde.15493 -
Current Medical Imaging 2024Klippel-Trenaunay syndrome (KTS) is a rare congenital disease that mainly involves blood vessels and is characterized by the presence of capillary malformations (port...
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.
CONCLUSION
KTS is a rare and potentially multisystem disease requiring multidisciplinary management for which imaging examination is an important auxiliary diagnostic method. Various complications may occur during its development, so regular follow-up is required to prevent serious accidents.
Topics: Male; Humans; Adult; Klippel-Trenaunay-Weber Syndrome; Hemangioma, Cavernous; Diagnostic Imaging
PubMed: 38087454
DOI: 10.2174/0115734056251193231016042812 -
JAAD Case Reports Jan 2024
PubMed: 38073866
DOI: 10.1016/j.jdcr.2023.10.008 -
Oman Journal of Ophthalmology 2023We present the case of a pregnant young woman with symptomatic diffuse choroidal hemangioma (CH), diagnosed with Sturge-Weber syndrome (SWS). Three months postpartum,...
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. On ultrasound, there was diffuse thickening of the choroid with a focus superior and nasal to the disc with active vascularization - a diagnosis of diffuse CH was made. Magnetic resonance imaging (MRI) and MR venography studies of the brain and orbit revealed engorged orbital vasculature, and the diagnosis of SWS was made. Three months after Cesarean section with birth of a healthy baby boy, VA returned to 6/9 with full resorption of subretinal fluid. On fundoscopic exam, there were no retinal or vein occlutions but there were tortuous blood vessels with A-V crossing. This is a rare case of newly diagnosed SWS with symptomatic diffuse CH in pregnancy. Postpartum, there was full resolution of the subretinal fluid. A watchful waiting approach can be considered in such cases.
PubMed: 38059083
DOI: 10.4103/ojo.ojo_28_23 -
Orthodontics & Craniofacial Research Jun 2024This 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...
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.
Topics: Humans; Retrospective Studies; Child; Male; Dentition, Mixed; Female; Port-Wine Stain; Radiography, Panoramic; Tooth Abnormalities; Odontogenesis; Tooth Eruption; Adolescent
PubMed: 38058228
DOI: 10.1111/ocr.12734 -
Pyogenic Granuloma After Treatment of Port-Wine Stain With Hemoporfin-Mediated Photodynamic Therapy.Dermatologic Surgery : Official... Mar 2024
Topics: Humans; Port-Wine Stain; Granuloma, Pyogenic; Hematoporphyrins; Photochemotherapy; Hemangioma, Capillary
PubMed: 38048227
DOI: 10.1097/DSS.0000000000004049 -
Indian Journal of Plastic Surgery :... Oct 2023Laser technology has significantly improved giving better results, which in turn has led to an increase in the indications for laser therapy. Vascular anomalies comprise...
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. Therefore, in most of the vascular lesions, lasers can bring improvement of a varying degree, while for superficial hemangiomas and port wine stain (PWS) laser treatment is now the standard of care. However, even though there is increase in the use of lasers in clinical practice, many surgeons are still unaware of its versatility and they are unsure about its safety. This article provides a brief overview of laser and intense pulsed light (IPL) technology, and describes the key principles in using these energy sources in vascular malformations. Readers are also familiarized with possible adverse effects and measures to prevent and treat them.
PubMed: 38026771
DOI: 10.1055/s-0043-1775871 -
JID Innovations : Skin Science From... Nov 2023Photo-mediated ultrasound therapy (PUT) is a cavitation-based, highly selective antivascular technique. In this study, the effectiveness and safety of PUT on cutaneous...
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 0.707 J/cm fluence laser pulses were applied concurrently with ultrasound bursts (n = 7, < .01). The effectiveness of removing blood vessels in the skin at depth up to 1 mm was further assessed by H&E-stained histology at multiple time points, which included days 1, 3, 7, 14, and 21 after treatment. Additional immunohistochemical analyses with CD31, caspase-3, and Masson's trichrome stains were performed on day 3 after treatment. The results show that the PUT-induced therapeutic effect was confined and specific to blood vessels only, whereas unwanted collateral damage in other skin tissues such as collagen was avoided. The findings from this study demonstrate that PUT can efficiently and safely remove hypervascular dermal capillaries using laser fluence at a level that is orders of magnitude smaller than that used in conventional laser treatment of vascular lesions, thus offering a safer alternative technique for clinical management of cutaneous vascular malformations.
PubMed: 38024557
DOI: 10.1016/j.xjidi.2023.100237 -
Pediatric Dermatology 2024A 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...
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.
Topics: Male; Child; Humans; Mutation; Capillaries; Port-Wine Stain; Vascular Malformations; Abnormalities, Multiple; Proto-Oncogene Proteins p21(ras)
PubMed: 38018302
DOI: 10.1111/pde.15458