Vascular malformations are aberrant proliferations of blood vessels that may present at birth or develop subsequently. Capillary malformations (CMs) represent the most common category of vascular malformations. They generally present as localized discoloration, including port-wine stains (PWSs) and telangiectasia. Certain PWSs, especially those affecting the V2 dermatome, may give rise to pyogenic granulomas or other epithelial or mesenchymal hamartomas.

We present a case of a 46-year-old male with a 5-year history of a solitary, progressively enlarging firm papule on an erythematous patch or “birthmark” on his left cheek. Despite infrequent manipulation, there were no reports of bleeding or any associated symptoms, including pain, pruritus, and thrills. The patient sought consultation for cosmetic reasons and wanted to have the lesion removed. Hence, an excision biopsy revealed a well-defined proliferation of mature thick and thin-walled vessels, lined by a single layer of endothelial cells, surrounded by dysmorphic and irregularly arranged vessels consistent with arteriovenous malformation with capillary malformation.

Capillary malformation (CM) is the most common form of vascular malformation. Fifty percent of port-wine stains (PWSs), which is the most common (CM) in children, are in the face area innervated by the second branch of the trigeminal nerve. Arteriovenous malformations manifest in the advanced stages of PWSs and, alongside pyogenic granuloma, represent underreported histological changes inside mature PWSs, especially in the face area innervated by the second branch of the trigeminal nerve (V2).

A girl, aged 15 years, was admitted due to sudden convulsion once and multiple pulmonary nodules on lung CT. Acrocyanosis or acropachy/toe deformity was not observed. Laboratory examinations showed an increase in hemoglobin (162 g/L) and a reduction in arterial partial pressure of oxygen (61.5 mm Hg). Lung CT showed irregular slightly high-density nodules in the middle lobe of the right lung, and contrast-enhanced CT scan showed obvious enhancement with thick vascular shadow locally. An investigation of medical history revealed that the girl's mother had a history of epistaxis and resection of pulmonary mass and the girl presented with tongue telangiectasia. The girl was diagnosed with hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformation. she was given interventional embolization therapy. Transcutaneous oxygen saturation reached 98% without oxygen inhalation on the day after surgery. Pulmonary angiography at 3 months after surgery showed the recurrence of pulmonary vascular malformation, and embolization of pulmonary arterial fistula was not performed since the guide wire could not enter the branch artery. There was still a need for long-term follow-up.
Adolescent ; Arteriovenous Fistula ; Arteriovenous Malformations ; Female ; Humans ; Multiple Pulmonary Nodules ; Neoplasm Recurrence, Local ; Pulmonary Artery/diagnostic imaging* ; Seizures

Arteriovenous malformations (AVM) are vascular disorders with a mixture of arterial, venous and small capillary-like channels with fistulous connections. Uterine arteriovenous malformations are rare cause of abnormal uterine bleeding with only a few reported cases. They may arise from pregnancy, miscarriage, previous cesarean section or other uterine surgery and gestational trophoblastic disease. Diagnosis can be made through angiography or doppler ultrasonography. Traditionally, uterine AVMs are treated with hysterectomy but with the advances in technology, minimally invasive conservative approaches such as radiologic arterial embolization or laparoscopic uterine artery ligation have become available. We present a case of a 29-year-old, G2P1 (1011) who had a three- month history of heavy, intermittent vaginal bleeding from uterine arteriovenous malformation after a miscarriage. Laparoscopic bilateral uterine artery occlusion, offered a minimally invasive treatment with high symptomatic effectiveness.
Uterine Artery ; Arteriovenous Malformations ; Uterine Diseases ; Ligation ; Uterine Hemorrhage

Arteriovenous Fistula/diagnostic imaging* ; Central Nervous System Vascular Malformations/diagnostic imaging* ; Embolization, Therapeutic ; Humans

Uterine arteriovenous vascular malformation (UAVM) is a disease that causes excessive bleeding. The symptoms do not subside without proper treatment and this can lead to life-threatening situations. The correct diagnosis of UAVM can be complicated if the patient's uterus did not completely discharge everything during abortion (in broader terms, retaining remnants of the products of conception). In this case, Doppler ultrasonography and computed tomography angiography with 3-dimensional rendering were used to analyze the cause of bleeding and provide proper treatment of this patient. Then, uterine artery embolization, dilatation, and curettage were performed safely and successfully. The patient no longer had symptoms of vaginal spotting during the planned follow up care. UAVM is uncommon; however, if reproductive-age women show repeated abnormal vaginal bleeding after dilatation and curettage, a diagnosis of UAVM must be considered based on the medical history and examination.
Angiography ; Arteriovenous Malformations ; Curettage ; Diagnosis ; Dilatation and Curettage ; Dilatation ; Female ; Follow-Up Studies ; Hemorrhage ; Humans ; Metrorrhagia ; Ultrasonography ; Ultrasonography, Doppler ; Uterine Artery ; Uterine Artery Embolization ; Uterine Hemorrhage ; Uterus ; Vascular Malformations

OBJECTIVE: To evaluate the safety and efficacy of the coil-protected technique for liquid embolization in neurovascular malformations. MATERIALS AND METHODS: Twenty-two patients who underwent coil-protected liquid embolization for symptomatic cranial (n = 13) and spinal (n = 9) arteriovenous fistula (AVF) or arteriovenous malformations (AVMs) were identified. A total of 36 target feeder vessels were embolized with N-butyl cyanoacrylate and/or Onyx (Medtronic). This technique was used to promote delivery of a sufficient amount of liquid embolic agent into the target shunt or nidus in cases where tortuous feeding arteries preclude a microcatheter wedging techniqu and/or to prevent reflux of the liquid embolic agent in cases with a short safety margin. The procedure was considered technically successful if the target lesion was sufficiently filled with liquid embolic agent without unintentional reflux. Angiographic and clinical outcomes were retrospectively evaluated. RESULTS: Technical success was achieved for all 36 target feeders. Post-embolization angiographies revealed complete occlusion in 16 patients and near-complete and partial occlusion in three patients each. There were no treatment-related complications. Of the six patients who showed near-complete or partial occlusion, five received additional treatments: two received stereotactic radiosurgery for cerebral AVM, two underwent surgical removal of cerebral AVM, and one underwent additional embolization by direct puncture for a mandibular AVM. Finally, all patients showed complete (n = 19) or near-complete (n = 3) occlusion of the target AVF or AVM on follow-up angiographies. The presenting neurological symptoms improved completely in 15 patients (68.2%) and partially in seven patients (31.8%). CONCLUSION: The coil-protected technique is a safe and effective method for liquid embolization, especially in patients with various neurovascular shunts or malformations who could not be successfully treated with conventional techniques.
Angiography ; Arteries ; Arteriovenous Fistula ; Arteriovenous Malformations ; Cyanoacrylates ; Follow-Up Studies ; Humans ; Methods ; Punctures ; Radiosurgery ; Retrospective Studies

PURPOSE: Hereditary hemorrhagic telangiectasia (HHT), a rare genetic vascular disorder, has been rarely reported in South Korea. We investigated the current prevalence and presenting patterns of genetically confirmed HHT in South Korea. MATERIALS AND METHODS: We defined HHT patients as those with proven mutations on known HHT-related genes (ENG, ACVRL1, SMAD4, and GDF2) or those fulfilling 3 or 4 of the Curaçao criteria. A computerized systematic search was performed in PubMed and KoreaMed using the following search term: (“hereditary hemorrhagic telangiectasia” AND “Korea”) OR (“Osler-Weber-Rendu” AND “Korea”). We also collected government health insurance data. HHT genetic testing results were collected from three tertiary hospitals in which the genetic tests were performed. We integrated patient data by analyzing each case to obtain the prevalence and presenting pattern of HHT in South Korea. RESULTS: We extracted 90 cases from 52 relevant articles from PubMed and KoreaMed. An additional 22 cases were identified from the three Korean tertiary hospitals after excluding seven cases that overlapped with those in the published articles. Finally, 112 HHT patients were identified (41 males and 71 females, aged 4–82 years [mean±standard deviation, 45.3±20.6 years]). The prevalence of HHT in South Korea is about 1 in 500,000, with an almost equal prevalence among men and women. Forty-nine patients underwent genetic testing, of whom 28 had HHT1 (ENG mutation) and 19 had HHT2 (ACVRL1 mutation); the other two patients were negative for ENG, ACVRL1, and SMAD4 mutations. CONCLUSION: The prevalence of HHT is underestimated in Korea. The rate of phenotypic presentation seems to be similar to that found worldwide. Korean health insurance coverage is limited to representative genetic analysis to detect ENG and ACVRL1 mutations. Further genetic analyses to detect HHT3, HHT4, and other forms of HHT should be implemented.
Arteriovenous Fistula ; Arteriovenous Malformations ; Diagnosis ; Epistaxis ; Female ; Genetic Testing ; Hemorrhage ; Humans ; Insurance, Health ; Korea ; Male ; Prevalence ; Telangiectasia, Hereditary Hemorrhagic ; Tertiary Care Centers

A 58-year-old male patient with severe claudication due to thrombosis of the left ilio-femoro-popliteal artery aneurysm. He also had a venous stasis ulcer with a history of multiple embolotherapy of arteriovenous malformation. Duplex sonography revealed reflux and varicose veins of the left great saphenous vein (GSV). A sequential bypass surgery was performed that consisted of excision of the left external iliac and common femoral artery aneurysm, external iliac to deep femoral interposition with an expanded polytetrafluoroethylene graft, and femoro-posterior tibial artery bypass with the reversed left GSV. Symptoms of claudication were alleviated and the chronic ulcer was healed in time. To our knowledge, this is the first report of successful bypass in a patient with arterial aneurysm, arteriovenous malformation, and venous insufficiency that can be diagnosed as an atypical case of Parkes Weber syndrome. Long-term follow-up is needed to define the fate of aneurysms and varicose vein graft.
Aneurysm ; Arteries ; Arteriovenous Fistula ; Arteriovenous Malformations ; Embolization, Therapeutic ; Femoral Artery ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Polytetrafluoroethylene ; Popliteal Artery ; Saphenous Vein ; Sturge-Weber Syndrome ; Thrombosis ; Tibial Arteries ; Transplants ; Ulcer ; Varicose Ulcer ; Varicose Veins ; Venous Insufficiency

With the field of neuroradiological procedures consistently expanding, breaking the border between the medical and surgical treatment, anesthetic involvement in those procedures is also increasing, which underscores the importance of related anesthesia management. The objective of this study is to review the closing or open endovascular procedures for intracranial aneurysm, arteriovenous malformation, acute ischemic stroke, and carotid stenosis and related anesthetic implications.
Anesthesia ; Arteriovenous Malformations ; Carotid Stenosis ; Endovascular Procedures ; Intracranial Aneurysm ; Stroke

Vascular anomalies comprise a heterogeneous group of disorders characterized by abnormal growth or development of blood vessels. Diagnosis of vascular anomalies is often challenging due to the large variety of conditions, which exhibit phenotypic heterogeneity and a wide range of symptomology and severity. Accurate diagnosis is crucial for appropriate evaluation and management, often requiring multidisciplinary specialists. Recent interdisciplinary collaboration has led to collaborative studies and their outcomes are being prospectively evaluated. While there is still a role for surgical intervention in various vascular anomalies, discoveries of pharmacologic agents effective in treating vascular anomalies have broadened our medical therapeutic options. This paper focuses on vascular anomaly issues often seen by the pediatricians and reviews the clinical pearls on infantile hemangiomas, lymphatic malformations, venous malformations, and arteriovenous malformations.
Arteriovenous Malformations ; Blood Vessels ; Cooperative Behavior ; Diagnosis ; Hemangioma ; Population Characteristics ; Prospective Studies ; Specialization