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

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

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

Arteriovenous malformation (AVM) of the mandible is a rare vascular condition that can manifest as a wide range of symptoms and, on rare occasions, cause fatal hemorrhage. The symptoms of mandibular AVM can range from soft tissue swelling and tooth mobility to severe hemorrhage. The recognition of early symptoms is crucial for the prevention of a fatal hemorrhage and for the proper diagnosis and treatment of mandibular AVM. For emergency hemostasis of a ruptured mandibular AVM, manual compression with gauze, topical thrombin, absorbable hemostat, suturing the lesion, and replanting the extracted tooth is recommended. Multiple treatment options for mandibular AVM are available, such as arterial embolization, venous embolization, direct surgical closure, and bone resection. A combination of treatment options should be considered in complicated cases. We report a case of a 10-year-old girl with a previous history of telangiectasia on the right cheek presented with cardiac arrest resulting from massive bleeding immediately after a tooth extraction.
Arteriovenous Malformations ; Cheek ; Child ; Diagnosis ; Emergencies ; Female ; Heart Arrest ; Hemorrhage ; Hemostasis ; Humans ; Mandible ; Telangiectasis ; Thrombin ; Tooth ; Tooth Extraction ; Tooth Mobility

OBJECTIVES: Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of vascular malformations with an absence of capillaries between arteries and veins. One major manifestation site is the nasal mucous membrane where recurrent nosebleeds occur. Our clinical strategy to treat patients with HHT has the aim to reduce nasal bleeding long-term with minimal local and general side effects. METHODS: We describe staged diagnosis and therapy including individual medical treatments of 97 patients with HHT. The success of treatment is monitored with a systematic questionnaire. RESULTS: The neodymium-doped yttrium aluminium garnet (Nd:YAG) laser therapy remains standard treatment of choice with no major side effects despite the need for repeated treatment. In addition new treatment strategies like nasal occlusion, local drug therapy, and nasal septal splinting show initial success. CONCLUSION: Improvement of the quality of life of HHT patients can be achieved by a multimodal concept. Several new treatment strategies like nasal septal splinting and nasal occlusion successfully expand the range of established methods. Further studies have to prove the safety and long-term effectiveness of the described individual medical treatments.
Arteries ; Arteriovenous Malformations ; Bevacizumab ; Capillaries ; Diagnosis ; Drug Therapy ; Epistaxis ; Humans ; Laser Therapy ; Mucous Membrane ; Quality of Life ; Splints ; Telangiectasia, Hereditary Hemorrhagic* ; Vascular Malformations ; Veins ; Yttrium

Arteriovenous malformation (A-V malformation) is defined as an abnormal connection between arteries and veins that lead to A-V shunting with an intervening network of vessels. A-V malformation is a rare condition, and spontaneous regression is also rare. A-V malformation becomes symptomatic when the surrounding tissue and osseous structures are negatively affected. A-V malformation has a high recurrence rate and is relatively hard to treat. In this case, a huge mass with pulsatile and bruit on the medial plantar area were observed. With the diagnosis of A-V malformation in accordance with the results from ultrasonography, magnetic resonance imaging and computed tomography angiography, and mass excision with feeding vessel ligation through plantar midfoot approach was completed successfully.
Angiography ; Arteries ; Arteriovenous Malformations* ; Diagnosis ; Foot* ; Ligation ; Magnetic Resonance Imaging ; Recurrence ; Ultrasonography ; Veins

Clinicians must be able to recognize post-extraction complications and treat them in a timely manner; complications that may potentially be life-threatening require special attention. Although arteriovenous malformation (AVM) is a very rare disorder, it may induce life-threatening hemorrhage during surgical intervention in the pertinent site. The present article examines the diagnosis and treatment modalities of AVM based on the case of a patient who was diagnosed with AVM with continuous bleeding after tooth extraction and who was successfully treated.
Arteriovenous Malformations* ; Dental Care ; Diagnosis ; Hemorrhage* ; Humans ; Postoperative Hemorrhage ; Tooth Extraction* ; Tooth* ; Vascular Malformations

The incidence of uterine arteriovenous malformation (AVM) is rare. However, it is clinically significant in that it can cause life-threatening vaginal bleeding. We report a case of a large uterine AVM with positive serum beta-human chorionic gonadotropin. A presumptive diagnosis was made; a uterine AVM accompanied by, early pregnancy or retained product of conception. Because this uterine AVM was extensive, transcatheter arterial embolization of both uterine arteries and extra-uterine feeding arteries was performed. Three months after undergoing transcatheter arterial embolization, complete resolution of the uterine AVM was confirmed without major complication.
Arteries* ; Arteriovenous Malformations* ; Chorion* ; Chorionic Gonadotropin* ; Diagnosis ; Fertilization ; Incidence ; Pregnancy ; Uterine Artery* ; Uterine Hemorrhage

Pulmonary arteriovenous malformations are rare vascular anomalies of the lung, only a few cases of which have been diagnosed prenatally. The diagnostic clue for prenatal diagnosis was cardiomegaly with a particularly enlarged left atrium. All previous cases of pulmonary arteriovenous malformations diagnosed prenatally have been reported as an isolated anomaly or in association with simple heart defects. We here describe the first case of a pulmonary arteriovenous malformation with a complex heart defect that was diagnosed prenatally at 21.0 weeks of gestation and confirmed by postmortem autopsy.
Arteriovenous Malformations* ; Autopsy ; Cardiomegaly ; Heart Atria ; Heart Defects, Congenital ; Heart* ; Lung ; Pregnancy ; Prenatal Diagnosis

Intracranial dural arteriovenous fistula (dAVF) usually results in various problems in the brain. But it can be presented as a myelopathy, which may make early diagnosis and management to be difficult. We recently experienced a case of cervical myelopathy caused by intracranial dAVF. A 60-year-old man presented with a 3-year history of gait disturbance due to a progressive weakness of both legs. Neurological examination revealed spastic paraparesis (grade IV) and Babinski sign on both sides. Magnetic resonance imaging showed serpentine vascular signal voids at C2-T1 on T2-weighted image with increased signal intensity and swelling of spinal cord at C1-C4. We performed a brain computed tomography angiography and found intracranial dAVF with multiple arteriovenous shunts. Venous drainages were noted at tentorial veins and cervical perimedullary veins. After Onyx embolization, the patient showed gradual improvement in motor power and gait disturbance. The venous drainage pattern is a well-known prognostic factor of dAVF. In our case, the intracranial dAVF drained to spinal perimedullary vein, which seemed to result in the ischemic myelopathy. Although it is rare condition, it sometimes can cause serious complications. Therefore, we should keep in mind the possibility of intracranial dAVF when a patient presents myelopathy.
Angiography ; Arteriovenous Fistula ; Brain ; Central Nervous System Vascular Malformations* ; Drainage ; Early Diagnosis ; Gait ; Humans ; Ischemia ; Leg ; Magnetic Resonance Imaging ; Middle Aged ; Neurologic Examination ; Paraparesis, Spastic ; Reflex, Babinski ; Spinal Cord ; Spinal Cord Diseases* ; Spinal Cord Ischemia ; Veins