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1.
Circ Res ; 135(1): 159-173, 2024 Jun 21.
Artigo em Inglês | MEDLINE | ID: mdl-38900856

RESUMO

Over the past several centuries, the integration of contemporary medical techniques and innovative technologies, like genetic sequencing, have played a pivotal role in enhancing our comprehension of congenital vascular and lymphatic disorders. Nonetheless, the uncommon and complex characteristics of these disorders, especially considering their formation during the intrauterine stage, present significant obstacles in diagnosis and treatment. Here, we review the intricacies of these congenital abnormalities, offering an in-depth examination of key diagnostic approaches, genetic factors, and therapeutic methods.


Assuntos
Doenças Linfáticas , Humanos , Doenças Linfáticas/terapia , Doenças Linfáticas/genética , Doenças Vasculares/congênito , Doenças Vasculares/genética , Doenças Vasculares/terapia , Doenças Vasculares/diagnóstico , Animais , Malformações Vasculares/genética , Malformações Vasculares/terapia , Vasos Linfáticos/anormalidades , Predisposição Genética para Doença
2.
Mil Med Res ; 11(1): 39, 2024 Jun 20.
Artigo em Inglês | MEDLINE | ID: mdl-38902798

RESUMO

BACKGROUND: Despite the efficacy of absolute ethanol (EtOH), its radiolucency introduces several risks in interventional therapy for treating vascular malformations. This study aims to develop a novel radiopaque ethanol injection (REI) to address this issue. METHODS: Iopromide is mixed with ethanol to achieve radiopacity and improve the physicochemical properties of the solution. Overall, 82 male New Zealand white rabbits are selected for in vivo radiopacity testing, peripheral vein sclerosis [animals were divided into the following 5 groups (n = 6): negative control (NC, saline, 0.250 ml/kg), positive control (EtOH, 0.250 ml/kg), low-dose REI (L-D REI, 0.125 ml/kg), moderate-dose REI (M-D REI, 0.250 ml/kg), and high-dose REI (H-D REI 0.375 ml/kg)], pharmacokinetic analyses (the blood sample was harvested before injection, 5 min, 10 min, 20 min, 40 min, 1 h, 2 h, 4 h, and 8 h after injection in peripheral vein sclerosis experiment), peripheral artery embolization [animals were divided into the following 5 groups (n = 3): NC (saline, 0.250 ml/kg), positive control (EtOH, 0.250 ml/kg), L-D REI (0.125 ml/kg), M-D REI (0.250 ml/kg), and H-D REI (0.375 ml/kg)], kidney transcatheter arterial embolization [animals were divided into the following 4 groups (n = 3): positive control (EtOH, 0.250 ml/kg), L-D REI (0.125 ml/kg), M-D REI (0.250 ml/kg), and H-D REI (0.375 ml/kg); each healthy kidney was injected with saline as negative control], and biosafety evaluations [animals were divided into the following 5 groups (n = 3): NC (0.250 ml/kg), high-dose EtOH (0.375 ml/kg), L-D REI (0.125 ml/kg), M-D REI (0.250 ml/kg), and H-D REI (0.375 ml/kg)]. Then, a prospective cohort study involving 6 patients with peripheral venous malformations (VMs) is performed to explore the clinical safety and effectiveness of REI. From Jun 1, 2023 to August 31, 2023, 6 patients [age: (33.3 ± 17.2) years] with lingual VMs received sclerotherapy of REI and 2-month follow-up. Adverse events and serious adverse events were evaluated, whereas the efficacy of REI was determined by both the traceability of the REI under DSA throughout the entire injection and the therapeutic effect 2 months after a single injection. RESULTS: The REI contains 81.4% ethanol (v/v) and 111.3 mg/ml iodine, which can be traced throughout the injection in the animals and patients. The REI also exerts a similar effect as EtOH on peripheral venous sclerosis, peripheral arterial embolization, and renal embolization. Furthermore, the REI can be metabolized at a similar rate compared to EtOH and Ultravist® and did not cause injury to the animals' heart, liver, spleen, lungs, kidneys and brain. No REI-related adverse effects have occurred during sclerotherapy of VMs, and 4/6 patients (66.7%) have achieved complete response at follow-up. CONCLUSION: In conclusion, REI is safe, exerts therapeutic effects, and compensates for the radiolucency of EtOH in treating VMs. TRIAL REGISTRATION: The clinical trial was registered as No. ChiCTR2300071751 on May 24 2023.


Assuntos
Etanol , Malformações Vasculares , Animais , Coelhos , Etanol/uso terapêutico , Etanol/farmacologia , Masculino , Malformações Vasculares/terapia , Malformações Vasculares/tratamento farmacológico , Humanos , Meios de Contraste/farmacocinética , Meios de Contraste/farmacologia , Meios de Contraste/uso terapêutico , Iohexol/análogos & derivados
3.
Laryngorhinootologie ; 103(S 01): S125-S147, 2024 May.
Artigo em Inglês, Alemão | MEDLINE | ID: mdl-38697145

RESUMO

Vascular anomalies in the head and neck area are usually rare diseases and pose a particular diagnostic and therapeutic challenge. They are divided into vascular tumours and vascular malformations. A distinction is made between benign tumours, such as infantile haemangioma, and rare malignant tumours, such as angiosarcoma. Vascular malformations are categorised as simple malformations, mixed malformations, large vessel anomalies and those associated with other anomalies. Treatment is interdisciplinary and various modalities are available. These include clinical observation, sclerotherapy, embolisation, ablative and coagulating procedures, surgical resection and systemic drug therapy. Treatment is challenging, as vascular anomalies in the head and neck region practically always affect function and aesthetics. A better understanding of the genetic and molecular biological basis of vascular anomalies has recently led to clinical research into targeted drug therapies. This article provides an up-to-date overview of the diagnosis, clinic and treatment of vascular anomalies in the head and neck region.


Assuntos
Pescoço , Malformações Vasculares , Humanos , Terapia Combinada , Embolização Terapêutica , Cabeça/irrigação sanguínea , Neoplasias de Cabeça e Pescoço/terapia , Neoplasias de Cabeça e Pescoço/diagnóstico , Hemangioma/terapia , Hemangioma/diagnóstico , Comunicação Interdisciplinar , Colaboração Intersetorial , Pescoço/irrigação sanguínea , Equipe de Assistência ao Paciente , Escleroterapia , Malformações Vasculares/terapia , Malformações Vasculares/diagnóstico
4.
J Med Vasc ; 49(2): 103-111, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38697706

RESUMO

Vascular malformations can present with a variety of symptoms and an unpredictable course with the occurrence of wounds. Ulcerations in patients with vascular malformations are fortunately rare. Although few data exist, complications may involve a variety of mechanistic or hemodynamic factors. A rigorous etiological and vascular assessment is therefore essential. In view of the paucity of recommendations, the Wound and Healing Group of the French Society of Vascular Medicine, based on the literature on the subject, presents a number of suggestions for the diagnosis and management of wounds associated with vascular malformations.


Assuntos
Malformações Vasculares , Cicatrização , Humanos , Doença Crônica , Malformações Vasculares/complicações , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/terapia
5.
Oral Maxillofac Surg Clin North Am ; 36(3): 355-368, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38632013

RESUMO

Craniomaxillofacial vascular anomalies encompass a diverse and complex set of pathologies that may have a profound impact on pediatric patients. They are subdivided into vascular tumors and vascular malformations depending on biological properties, clinical course, and distribution patterns. Given the complexity and potential for leading to significant functional morbidity and esthetic concerns, a multidisciplinary approach is generally necessary to optimize patient outcomes. This article reviews the etiology, clinical course, diagnosis, and current management practices related to vascular anomalies in the head and neck.


Assuntos
Cabeça , Pescoço , Malformações Vasculares , Humanos , Criança , Malformações Vasculares/terapia , Malformações Vasculares/diagnóstico , Pescoço/irrigação sanguínea , Cabeça/irrigação sanguínea , Neoplasias de Cabeça e Pescoço
6.
Pediatr Ann ; 53(4): e129-e137, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38574074

RESUMO

Vascular anomalies represent a diverse group of disorders of abnormal vascular development or proliferation. Vascular anomalies are classified as vascular tumors and vascular malformations. Significant advances have been made in the understanding of the pathogenesis, natural history, and genetics of vascular anomalies, allowing for improvements in management including targeted molecular therapies. Infantile hemangiomas are the most common vascular tumor of childhood and follow a distinct natural history of proliferation and involution. Although benign, infantile hemangiomas can be associated with important complications. The use of beta-blockers has revolutionized the management of infantile hemangiomas. Other vascular tumors include pyogenic granulomas, congenital hemangiomas, and kaposiform hemangioendotheliomas, among others. Vascular malformations are categorized based on the type of involved vessel, including capillary malformations, venous malformations, lymphatic malformations, arteriovenous malformations, and mixed vascular malformations. Expert multidisciplinary management of vascular anomalies is critical to optimize outcomes in these patients. [Pediatr Ann. 2024;53(4):e129-e137.].


Assuntos
Hemangioendotelioma , Hemangioma , Síndrome de Kasabach-Merritt , Malformações Vasculares , Neoplasias Vasculares , Humanos , Neoplasias Vasculares/diagnóstico , Neoplasias Vasculares/terapia , Malformações Vasculares/diagnóstico , Malformações Vasculares/terapia , Malformações Vasculares/patologia , Hemangioma/diagnóstico , Hemangioma/terapia , Hemangioma/patologia
7.
Pediatr Blood Cancer ; 71(5): e30921, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38439088

RESUMO

The diagnosis of vascular anomalies remains challenging due to significant clinical heterogeneity and uncertain etiology. Evaluation using biopsy and/or genetic testing for somatic variants is invasive, expensive, and prone to sampling error. There is great need for noninvasive and easily measured blood laboratory biomarkers that can aid not only in diagnosis, but also management of treatments for vascular anomalies. Angiopoietin-2, a circulating blood angiogenic factor, is highly elevated in patients with kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon and kaposiform lymphangiomatosis. Here, we describe our clinical experience using serum angiopoietin-2 as a biomarker for diagnosis and monitoring response to treatment.


Assuntos
Angiopoietina-2 , Malformações Vasculares , Humanos , Angiopoietina-2/sangue , Biomarcadores/sangue , Hemangioendotelioma/sangue , Hemangioendotelioma/diagnóstico , Hemangioendotelioma/terapia , Síndrome de Kasabach-Merritt/sangue , Síndrome de Kasabach-Merritt/diagnóstico , Síndrome de Kasabach-Merritt/terapia , Malformações Vasculares/sangue , Malformações Vasculares/diagnóstico , Malformações Vasculares/terapia
8.
J Nanobiotechnology ; 22(1): 120, 2024 Mar 18.
Artigo em Inglês | MEDLINE | ID: mdl-38500178

RESUMO

Nanotechnology has demonstrated immense potential in various fields, especially in biomedical field. Among these domains, the development of nanotechnology for diagnosing and treating vascular anomalies has garnered significant attention. Vascular anomalies refer to structural and functional anomalies within the vascular system, which can result in conditions such as vascular malformations and tumors. These anomalies can significantly impact the quality of life of patients and pose significant health concerns. Nanoscale contrast agents have been developed for targeted imaging of blood vessels, enabling more precise identification and characterization of vascular anomalies. These contrast agents can be designed to bind specifically to abnormal blood vessels, providing healthcare professionals with a clearer view of the affected areas. More importantly, nanotechnology also offers promising solutions for targeted therapeutic interventions. Nanoparticles can be engineered to deliver drugs directly to the site of vascular anomalies, maximizing therapeutic effects while minimizing side effects on healthy tissues. Meanwhile, by incorporating functional components into nanoparticles, such as photosensitizers, nanotechnology enables innovative treatment modalities such as photothermal therapy and photodynamic therapy. This review focuses on the applications and potential of nanotechnology in the imaging and therapy of vascular anomalies, as well as discusses the present challenges and future directions.


Assuntos
Nanopartículas , Nanoestruturas , Neoplasias , Malformações Vasculares , Humanos , Meios de Contraste , Qualidade de Vida , Nanotecnologia , Nanoestruturas/uso terapêutico , Neoplasias/diagnóstico por imagem , Neoplasias/tratamento farmacológico , Nanopartículas/uso terapêutico , Nanopartículas/química , Malformações Vasculares/diagnóstico , Malformações Vasculares/terapia , Nanomedicina/métodos
9.
Eur J Radiol ; 174: 111397, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38452733

RESUMO

PURPOSE: To investigate quantitative changes in MRI signal intensity (SI) and lesion volume that indicate treatment response and correlate these changes with clinical outcomes after percutaneous sclerotherapy (PS) of extremity venous malformations (VMs). METHODS: VMs were segmented manually on pre- and post-treatment T2-weighted MRI using 3D Slicer to assess changes in lesion volume and SI. Clinical outcomes were scored on a 7-point Likert scale according to patient perception of symptom improvement; treatment response (success or failure) was determined accordingly. RESULTS: Eighty-one patients with VMs underwent 125 PS sessions. Treatment success occurred in 77 patients (95 %). Mean (±SD) changes were -7.9 ± 24 cm3 in lesion volume and -123 ± 162 in SI (both, P <.001). Mean reduction in lesion volume was greater in the success group (-9.4 ± 24 cm3) than in the failure group (21 ± 20 cm3) (P =.006). Overall, lesion volume correlated with treatment response (ρ = -0.3, P =.004). On subgroup analysis, volume change correlated with clinical outcomes in children (ρ = -0.3, P =.03), in sodium tetradecyl sulfate-treated lesions (ρ = -0.5, P =.02), and in foot lesions (ρ = -0.6, P =.04). SI change correlated with clinical outcomes in VMs treated in 1 PS session (ρ = -0.3, P =.01) and in bleomycin-treated lesions (ρ = -0.4, P =.04). CONCLUSIONS: Change in lesion volume is a reliable indicator of treatment response. Lesion volume and SI correlate with clinical outcomes in specific subgroups.


Assuntos
Escleroterapia , Malformações Vasculares , Criança , Humanos , Soluções Esclerosantes/uso terapêutico , Estudos Retrospectivos , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/terapia , Veias , Resultado do Tratamento
10.
Medicine (Baltimore) ; 103(7): e37225, 2024 Feb 16.
Artigo em Inglês | MEDLINE | ID: mdl-38363925

RESUMO

Fibro-adipose vascular anomaly (FAVA) is a rare and complex vascular malformation associated with persistent pain, limb contracture, and even restriction of activity. However, the pathophysiology of FAVA remains unclear. Although FAVA is a benign vascular malformation, it is highly misdiagnosed and often thus undergoing repeated surgical resection and interventional sclerotherapy, resulting in worsening of symptoms and irreversible dysfunction. Therefore, aggressive diagnosis and treatment are essential. There are several different treatment options for FAVA, including surgical resection, sclerotherapy, cryoablation, drug therapy, and physical therapy. This article reviews the clinical manifestations, pathological features, pathogenesis, and treatment methods of FAVA.


Assuntos
Fibromialgia , Doenças Vasculares , Malformações Vasculares , Humanos , Resultado do Tratamento , Malformações Vasculares/terapia , Malformações Vasculares/cirurgia , Doenças Vasculares/complicações , Fibromialgia/complicações , Dor/etiologia , Obesidade/complicações , Escleroterapia/métodos
11.
J Cosmet Dermatol ; 23(6): 2215-2219, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38389293

RESUMO

BACKGROUND: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare disease with a wide range of manifestations. KTWS is characterized by a clinical triad of varicosities of the extremities, cutaneous vascular malformations, and hypertrophy of soft tissues and long bones. The diagnosis is made clinically supplemented with magnetic resonance imaging and computed tomography. AIM: Hereby we aim to highlight the significance of the possible life-threatening first-time presentations associated with the GI system in previously undiagnosed KTWS patients. PATIENT: We report the case of a 47-year-old male with KTWS, who presented with various symptoms such as rectorrhagia since childhood, digestive problems and abnormal lateral vascular malformations of the left buttock which extended all the way to the leg, vascular malformations of the left fourth and fifth toes as well as soft tissue swelling of the left foot. There was no evidence of other clinical presentations. The patient was hospitalized with severe rectorrhagia and a hemoglobin level of 3/9. Physical examination revealed a blood pressure of 85/55 and pulse rate of 115. Ruptured aneurysm of the superior mesenteric artery was found on angiography and subsequently treated with embolization. Dermatologic evaluation showed pitting edema of the left leg and foot and multiple vascular lesions. Thus a diagnosis of KTWS was established. Pulsed dye laser therapy and compression bandage was performed for the patient. The patient's follow-up was done 3 months after discharge for which the patient was again consulted by a dermatologist and gastroenterologist. Lymphedema of the left leg had improved to a great extend so treatment with compression bandage was continued. Colonoscopy was repeated for the patient to evaluate and control possible active sources of bleeding, due to potential life-threating complications. RESULTS: According to previous findings, there have been few case reports of KTWS presenting with gastrointestinal manifestations, fewer of which have covered acute life-threatening bleedings associated with this system.


Assuntos
Síndrome de Klippel-Trenaunay-Weber , Humanos , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Síndrome de Klippel-Trenaunay-Weber/complicações , Síndrome de Klippel-Trenaunay-Weber/terapia , Masculino , Pessoa de Meia-Idade , Doenças Raras , Malformações Vasculares/terapia , Malformações Vasculares/diagnóstico , Malformações Vasculares/complicações
12.
BMJ Case Rep ; 17(2)2024 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-38359957

RESUMO

A young male patient presented with an incidental finding of a large supraglottic vascular lesion. The lesion was initially noted during intubation 4 years ago. Although originally listed for elective excision, there was a significant delay and at the time of surgery, the lesion proved too large to remove and a significant threat to the patient's airway. An emergency tracheostomy was performed, followed by two consecutive treatments with sclerotherapy agents to reduce the size of the lesion. It was then successfully excised using a Thunderbeat ultrasound and bipolar dissection and cautery device.


Assuntos
Escleroterapia , Malformações Vasculares , Humanos , Masculino , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/terapia
13.
Pediatr Radiol ; 54(2): 347-356, 2024 02.
Artigo em Inglês | MEDLINE | ID: mdl-38191809

RESUMO

Pediatric intracranial arteriovenous shunts are rare vascular malformations that can be diagnosed prenatally or postnatally, as an incidental finding or due to complications. We propose a review of cerebral vascular malformations in newborns and infants with special emphasis on neurosonography and Doppler ultrasound as the first diagnostic method. Sonography can thus contribute in the planning of further studies that are always necessary, and in post-therapy follow-up.


Assuntos
Veias Cerebrais , Malformações Vasculares , Lactente , Criança , Humanos , Recém-Nascido , Seguimentos , Ultrassonografia , Veias Cerebrais/diagnóstico por imagem , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/terapia , Ultrassonografia Doppler
14.
Clin Hemorheol Microcirc ; 86(1-2): 121-131, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37638426

RESUMO

AIM: To evaluate the usefulness of handheld ultrasound in comparison with high-end ultrasound for lesion evaluation before and after sclerotherapy in pediatric patients with venous malformations (VMs). MATERIAL AND METHODS: 10 pediatric patients prior to and after sclerotherapy were scanned by an experienced examiner using handheld ultrasound (Vscan AirTM) and high-end ultrasound (LOGIQ E9/E10) as reference. Patients with associated venous thromboses and intralesional aneurysms had been excluded. Results were interpreted independently by two readers in consensus. RESULTS: 10 patients (4-17 years; 10.0±4.32 years; female n = 6, male n = 4) with 10 VMs (4 of the head and neck region, 4 of the upper and 2 of the lower extremities) were examined. 7 phleboliths were detected. The average rating score achieved by the high-end device never was less than 4, by Vscan AirTM never less than 3. An exception was the assessment of AV fistulas. In comparison with the evaluation of variables examined, we found a significant difference between the high-end scanner and the handheld device regarding the achieved image quality. CONCLUSION: Vscan AirTM ultrasound device allows new possibilities for procedure planning and post-procedural control of pediatric patients with VMs.


Assuntos
Malformações Vasculares , Humanos , Criança , Masculino , Feminino , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/terapia , Ultrassonografia , Escleroterapia/métodos , Veias/diagnóstico por imagem , Pescoço , Resultado do Tratamento , Estudos Retrospectivos
15.
J Vasc Interv Radiol ; 35(1): 127-136.e1, 2024 01.
Artigo em Inglês | MEDLINE | ID: mdl-37704038

RESUMO

PURPOSE: To retrospectively evaluate sclerotherapy using consecutive polidocanol and bleomycin foam (CPBF) for large untreated venous malformations (VMs) and/or those resistant to prior treatment. MATERIALS AND METHODS: This retrospective study included all patients treated with CPBF for untreated VMs larger than 10 mL and/or refractory to treatment between May 2016 and October 2019. Baseline and follow-up VM volumes were measured on fat-suppressed T2-weighted magnetic resonance (MR) imaging. Outcome was evaluated on postprocedural MR imaging volumetry and by a retrospective survey assessing clinical response and adverse events. Imaging response was considered good for volume reduction from 50% to 70% and excellent for volume reduction ≥70%. Symptoms and quality-of-life (QoL) scores were compared before and after CPBF sclerotherapy. RESULTS: Forty-five patients (mean age, 16 years; range, 1-63 years; 25 males) with 57 VMs were analyzed and treated by 80 sclerotherapy. Sixty percent (27 of 45) of patients had undergone prior treatment for VM. Median VM volume was 36.7 mL (interquartile range, 84 mL) on pretherapy MR imaging. Good and excellent results after the last sclerotherapy were achieved in 36% (16 of 45) and 29% (13 of 45) of patients, respectively, corresponding to a decrease of >50% in 60% (34 of 57) of VMs. QoL score increased by at least 3 points, regardless of initial symptoms. Most patients did not desire additional sclerotherapy owing to near complete symptomatic relief, even for patients who did not achieve a good response. Swelling, pain, and motor impairment scores significantly improved after CPBF. Adverse events included fever (44%, 15 of 34) and nausea/vomiting (29%, 10 of 34). CONCLUSIONS: CPBF sclerotherapy represents an effective therapy for large and/or refractory VMs with minimal adverse events.


Assuntos
Escleroterapia , Malformações Vasculares , Masculino , Humanos , Adolescente , Escleroterapia/efeitos adversos , Escleroterapia/métodos , Polidocanol , Estudos Retrospectivos , Soluções Esclerosantes , Bleomicina/efeitos adversos , Qualidade de Vida , Veias/anormalidades , Imageamento por Ressonância Magnética , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/terapia , Resultado do Tratamento
16.
Phlebology ; 39(1): 44-48, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37859422

RESUMO

BACKGROUND: Venous malformations (VMs) are the most common vascular malformations, which can be classified as focal, multifocal, or diffuse types. But the risk of focal venous malformations with muscle involvement is not well defined. METHODS: This is a single-center, retrospective review of patients treated for focal VMs between February 2021 and February 2022. RESULTS: We assessed 26 patients focal VMs with 47 lesions; 18 (69%) were unifocal, 3 (12%) were dual-focal, and 5 (19%) were multifocal type VMs, and 29 (62%) were intramuscular VMs. The lower limbs intramuscular VMs had a significantly elevated risk of focal VMs (relative risk [RR],1.7; 95% confidence interval [CI], 1.148-2.394). CONCLUSION: Intramuscular involvement of the body should be considered in focal VMs. The lower limbs intramuscular VMs had a significantly elevated risk of focal VMs.


Assuntos
Malformações Vasculares , Veias , Criança , Humanos , Veias/anormalidades , Malformações Vasculares/terapia , Risco , Músculos/patologia , Estudos Retrospectivos
17.
Rofo ; 196(2): 186-194, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37922942

RESUMO

PURPOSE: Analysis of clinical and diagnostic findings in rare urogenital and perineal vascular malformations only occurring in 2-3 % of vascular anomalies with regard to clinical symptoms and treatment decisions. MATERIALS AND METHODS: All 25 out of 537 patients presenting with congenital urogenital and perineal vascular malformations at our institution from 2014 to 2021 were included. Vascular anomaly classification, anatomical location, clinical symptoms at presentation, diagnostic imaging, and pain intensity were retrospectively assessed from the patient record and therapy management was evaluated. RESULTS: In total, 25 patients (10 females (40 %), 15 males (60 %)), aged 6 to 77 years were included. Diagnoses were: 10 (40 %) venous malformations (VMs), 5 (20 %) lymphatic malformations (LMs) and 10 (40 %) arteriovenous malformations (AVMs). Malformation manifestations were: 12 (32 %) lesser pelvis, 12 (32 %) external genitalia, and 13 (34 %) perineal/gluteal region. One AVM was located in the kidney. The leading clinical symptom was pain. The mean intensity was 6.0/10 for LM, 5.7/10 for VM, and 4.5/10 for AVM. Further major symptoms included physical impairment, local swelling, and skin discoloration. Bleeding complications or sexual dysfunction were rare findings. Patients with VM reported significantly more symptoms than patients with AVM (p = 0.0129). In 13 patients (52 %) minimally invasive therapy was indicated: 10 (77 %) sclerotherapies and 3 (23 %) transcatheter embolization procedures. Complete symptomatic remission was achieved in 9 (69 %) patients, partial response in 3 (23 %) patients, and 1 patient showed no clinical response to therapy. Follow-up appointments without the need for immediate minimally invasive therapy were significantly more common in patients with AVMs than in patients with VMs (p = 0.0198). CONCLUSION: To create a higher awareness of congenital urogenital and perineal vascular malformations. Awareness of this rare condition avoids misdiagnosis. Therapy decisions should be symptom-oriented. Emergency intervention is rarely required, even in fast-flow vascular malformations. KEY POINTS: · Venous malformations cause more symptoms with higher pain intensity than arteriovenous malformations.. · Diagnosis and adequate treatment can be hampered by a lack of awareness of the clinical presentation.. · Bleeding complications are rare, even in high-flow vascular malformations.. · Pain and physical impairment are the most commonly observed symptoms in these patients..


Assuntos
Malformações Arteriovenosas , Malformações Vasculares , Masculino , Feminino , Humanos , Estudos Retrospectivos , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/terapia , Malformações Arteriovenosas/diagnóstico por imagem , Malformações Arteriovenosas/terapia , Diagnóstico por Imagem , Dor , Resultado do Tratamento
18.
J Vasc Surg Venous Lymphat Disord ; 12(2): 101698, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37890587

RESUMO

OBJECTIVE: The objective of this study was to retrospectively evaluate the effectiveness of polidocanol in managing pain, swelling, functional limiting and cosmetic disorders in patients with venous malformations (VMs). METHODS: This retrospective study included patients who underwent sclerotherapy with polidocanol for VMs between 2020 and 2022. Patient records, imaging findings, and evaluation questionnaires used in the preprocedure and follow-up phases were reviewed. After sclerotherapy, patients were followed up at 1, 2, 3, and 6 months. During these visits, the previously used 11-point verbal numerical rating scale (from 0 [no pain] to 10 [worst pain thinkable]) was used to evaluate the severity of symptoms such as pain, swelling, cosmetic discomfort, and functional limitation, and patients were asked to report the number of days per week they experienced these symptoms owing to the VM. RESULTS: A total of 194 sclerotherapy procedures (mean, 1.6 ± 0.3 procedures) in 84 patients (55 female and 29 male patients; mean age, 22.45 ± 11.83 years) were conducted. The majority of these malformations (81%, or 68 patients) were located in the extremities. We found a significant decrease in pain, swelling, functional limitation, cosmetic appearance, and number of painful days between all time points, except for the comparison between months 3 and 6 (P < .001) CONCLUSIONS: Polidocanol sclerotherapy is a safe and effective treatment for VMs that significantly decreases patient complaints and has a very low complication rate. Particularly, following patients at short intervals and administering additional sclerotherapy sessions when necessary will significantly increase patient satisfaction.


Assuntos
Polietilenoglicóis , Escleroterapia , Malformações Vasculares , Humanos , Masculino , Feminino , Criança , Adolescente , Adulto Jovem , Adulto , Polidocanol/efeitos adversos , Escleroterapia/efeitos adversos , Escleroterapia/métodos , Estudos Retrospectivos , Soluções Esclerosantes/efeitos adversos , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/terapia , Malformações Vasculares/complicações , Resultado do Tratamento , Dor/etiologia
19.
J Vasc Surg Venous Lymphat Disord ; 12(2): 101697, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37890588

RESUMO

OBJECTIVE: This study aims to investigate the difference in safety and efficacy between two treatments for venous malformations (VMs), electrochemotherapy combined with polidocanol foam (ECP) and bleomycin polidocanol foam (BPF), providing alternative therapies for VMs. METHODS: We conducted a retrospective review of 152 patients with VMs treated with ECP and BPF. Pre- and post-treatment magnetic resonance images (MRIs) were collected, and clinical follow-up assessments were performed. Imaging results were used to calculate lesion volume changes. Clinical outcomes included changes in pain and improvements in perceived swelling. Patients were followed up at 1 week and 6 months after surgery. All emerging complications were documented in detail. RESULTS: Of the 152 patients, 87 (57.2%) received BPF treatment, and 65 (42.8%) received ECP treatment. The most common location of VMs was the lower extremities (92/152; 60.2%), and the most common symptom was pain (108/152; 71.1%). Forty-three patients had previously undergone therapy in the BPF group (43/87; 49.4%), whereas 30 patients had received prior treatment in the ECP group (30/65; 46.2%). The study found that the percentage of lesion volume reduction in the BPF group was not significantly different from that in the ECP group (75.00% ± 17.85% vs 74.69% ± 8.48%; P = .899). ECP was more effective when the initial lesion volume was greater than 30 mL (67.66% ± 12.34% vs 73.47% ± 8.00%; P = .048). Patients treated with BPF had significantly less posttreatment pain than those treated with ECP, in different baseline lesion size. In the overall sample, pain relief was significantly higher in the BPF group than in the ECP group (4.21 ± 1.19 vs 3.57 ± 0.76; P = .002). However, there was no difference in pain relief between the two groups for the treatment of initially large VMs (4.20 ± 0.94 vs 3.70 ± 0.87; P = .113). The ECP group was significantly more likely to develop hyperpigmentation (5/87; 5.75% vs 11/65; 16.92%; P = .026) and swelling (9/87; 10.34% vs 16/65; 24.62%; P = .019) 1 week after surgery than the BPF group. CONCLUSIONS: Our study demonstrates that both BPF and ECP are effective treatments for VMs, with BPF being a safer option. ECP is a better choice for patients with the initial lesion volume greater than 30 mL, but it is more likely to lead to early swelling and hyperpigmentation.


Assuntos
Eletroquimioterapia , Hiperpigmentação , Polietilenoglicóis , Malformações Vasculares , Humanos , Polidocanol/efeitos adversos , Soluções Esclerosantes , Bleomicina/efeitos adversos , Escleroterapia/efeitos adversos , Escleroterapia/métodos , Eletroquimioterapia/efeitos adversos , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/terapia , Malformações Vasculares/complicações , Resultado do Tratamento , Dor/etiologia , Estudos Retrospectivos , Hiperpigmentação/etiologia
20.
Acta Radiol ; 65(1): 145-151, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37743550

RESUMO

BACKGROUND: Vascular malformations are rare diseases that should be treated in dedicated vascular anomaly centers (VAC). There is only a small amount of data on the diagnostic and therapeutic handling of these patients, before they are referred to a VAC. PURPOSE: To demonstrate the disease-specific patient characteristics in a German VAC, which are required to determine diagnostic and therapeutic steps. MATERIAL AND METHODS: In a retrospective study, all patients who were treated in the VAC from April 2014 until August 2021 were identified. In total, 593 patients were included in this study. RESULTS: Almost all patients had previously consulted a physician (591/593, 99.7%). A mean of two different physicians had been consulted (range 0-10). Patients with more complex, syndromal vascular malformations had significantly more previous appointments (P = 0.0018). In only 44% (261/593) of patients, the referral diagnosis was made correctly. Most patients had been previously treated for their vascular anomaly: pharmacotherapy (n = 130; 21.9%), compression garments (n = 141; 23.8%), surgical resection (n = 80; 17.3%) and sclerotherapy (n = 68; 11.5%). Fifty-two patients who had been falsely diagnosed had also received therapy prior to their referral to the VAC (8.8%). Most patients received an ultrasound examination in the VAC (n = 464; 78.2%). Most frequently, compression therapy was prescribed (n = 256; 43.2%), followed by sclerotherapy (n = 175, 29.5%) and pharmacotherapy (n = 55; 9.3%). CONCLUSION: Patients suffering from vascular anomalies often go through a complicated scheduling with numerous outpatient appointments and have a high risk of misdiagnosis and mistreatment prolonging the medical condition. Therefore, patients with vascular anomalies should be treated in a dedicated vascular anomaly center.


Assuntos
Malformações Vasculares , Humanos , Estudos Retrospectivos , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/terapia , Escleroterapia , Ultrassonografia
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