Vascular Arteriovenous Malformations Research Articles

The Role of Manual Compression in Doppler Ultrasound Mapping of Arteriovenous Vascular Malformations in the Upper and Lower Extremities

Doppler ultrasonography (US) is an important noninvasive technique for diagnosing and assessing arteriovenous malformation (AVM). US, magnetic resonance, computed tomography, and angiograms make it possible to locate and describe these malformations, in particular the nidus. The AVM nidus shows as a high-density tangle of tortuous and dilated vessels (arteries and veins) caused by abnormal communications (AV shunts) between these vessels. Locating these AV shunts precisely within the nidus is difficult. A new manual compression technique used during US exploration allows us to accurately differentiate afferent/efferent vessels from the AV shunts located in the nidus.

СОСТОЯНИЕ СВЕРТЫВАЮЩЕЙ СИСТЕМЫ КРОВИ У ДЕТЕЙ ПРИ СОСУДИСТЫХ МАЛЬФОРМАЦИЯХ И ВОСПАЛИТЕЛЬНЫХ ЗАБОЛЕВАНИЯХ ЧЕЛЮСТНО-ЛИЦЕВОЙ ОБЛАСТИ

The article presents the results of a study of hemostasis in children with vascular malformations and inflammatory diseases of the maxillofacial region using standard and integrated methods for assessing hemostasis. Objective of the research: to assess the state of the hemostatic system in children with vascular malformations and inflammatory diseases of the maxillofacial region using standart and integrated methods. Materials and methods: on the basis of St. Vladimir's Children's Clinical Hospital in 2015–2019. А prospective open controlled comparative nonrandomized selective study of coagulological status was performed using standard coagulological tests and integral methods for assessing hemostasis (thromboelastography – TEG, thrombodynamics – TD) in 105 patients with lymphatic, lymphovenous, venous, arteriovenous vascular malformations of the head and neck (age 6 [5; 9] years), 47 children with infectious and inflammatory diseases of the maxillofacial area (age 10 [4; 17] years). The control group included 37 children (age 9 [5; 13] years). Results: according to the data, in most patients with vascular malformations, the coagulogram did not reveal pronounced shifts in hemostasis. TEG indices demonstrated hypercoagulability in vascular malformations: the onset time of clot R formation was 11,20 [8,72; 15,75] min (the norm is 9–27 min); clot K formation time was reduced to 3,90 [2,30; 6,02] min (the norm is 2–9 min). According to TD data, the process of fibrin clot growth in patients with vascular malformations accelerated: the clot V growth rate was 32,80 [28,70; 40,20] μm/min (norm 20–29 μm/min), an increase in the rate was observed mainly due to the acceleration of the clot growth initiation. The formation of spontaneous clots was noted in 32% of cases. In the group of infectious and inflammatory diseases of the maxillofacial area, the hypercoagulation was most pronounced: fibrinogen level significantly increased compared with the control group (3,21 [2,69; 4,72] g/l vs 2,69 [2,37; 3,17 ] g/l; p=0,0025). The soluble fibrin monomer complex (SFMC) indicator statistically significantly increased to 5,50 [0,00; 6,00] mg% (1,50 [0,00; 5,00] mg% in the control group, p=0,006). TEG showed a decrease in R (11,10 [8,82; 14,32] min vs 15,2 [11,8; 20,6] min; p=0,001) and K (3,65 [2,45; 5,10] min vs 6,70 [4,20; 8,25] min; p=0,0001), statistically significant compared to the control group. The alpha angle increased to 45,70 [36,20; 56,02] (30,8 [23,25; 41,15] in the control group, p=0,0001); maximum amplitude (MA) and clot density (G) increased in comparison with the control: 56,6±8,68 mm vs 48,10±6,20 mm; p=0,0001 and 6,50 [4,80; 8,00] Kd/sc vs 4,60 [3,60; 5,30] Kd/sc; p=0,0001. The TD indices in patients in this group also reflected a hypercoagulable state: the rate of V clot formation increased to 38,60 [33,00; 51,40] μm/min (30,45 [27,60; 33,30] μm/min in the control group, p<0,0001); the initial rate of clot formation also increased – 63,50 [59,10; 68,62] μm/min (58,74 [56,57; 64,20] μm/min in the control group, p=0,0055); the size (CS) and density (D) of the clot increased – 1425,5 [1265,7; 1505,2] μm (1251,00 [1168,82; 1322,50] μm in the control group, p=0,0029) and 25 362,50 [21 922,00; 29 210,50] conv. units (22 883,00 [20 807,25; 24 658,64] conv. units in the control group, p=0,0208). Spontaneous clots were formed in 50% of cases. Conclusion: integral tests are a sensitive method for assessing the state of hemostasis in patients with vascular malformations and infectious and inflammatory diseases. The revealed hypercoagulable shifts in the form of clot growth rate acceleration work as the basis for a thorough dynamic assessment of the hemostasis system in children with vascular malformations and infectious-inflammatory diseases in order to prevent thrombotic complications.

Особливості діагностики та комплексного лікування артеріовенозних форм вроджених судинних мальформацій кінцівок

Мета. Поліпшення результатів лікування хворих з артеріовенозними формами вроджених судинних мальформацій (АВФ ВСМ) кінцівок на основі розроблення диференційованих алгоритмів діагностики та лікування.
 Матеріали і методи. Робота базується на даних комплексного клінічного дослідження та результатах хірургічного лікування 155 пацієнтів з АВФ ВСМ кінцівок. Строки спостереження становили від 1 міс до 10 років. Чоловіків було 65 (42%), жінок - 90 (58%), число чоловіків і число жінок співвідносилися як 1: 1,4. Середній вік пацієнтів становив (25,1 ± 10,4) року. У віці до 18 років (дитяча вікова група) було 53 (34%) хворих. Клінічний матеріал аналізували в залежності від клініко-анатомічних форм захворювання, які були розподілені на 11 груп на основі робочої класифікаційної схеми «VASC + Т».
 Результати. Розроблення класифікаційної схеми вроджених судинних мальформацій уможливило поліпшення формування плану діагностики та етапного лікування пацієнтів у вигляді алгоритмів у залежності від клініко-анатомічної форми захворювання. Застосування етапного комплексного підходу з використанням емболізаційних, ендовенозних та хірургічних методик дало змогу уникнути розвитку великих післяопераційних ускладнень.
 Висновки. Впровадженням розробленої тактики досягнутo задовільних віддалених результатів лікування у 136 (94,4%) пацієнтів.

Cerebral Arteriovenous Malformations

A significant improvement of central nervous system arteriovenous vascular malformations (AVM) outcome has been observed due to the advances in all modalities of diagnosis and treatment. The authors report the advances in diagnosis and integrated global treatment of AVM`s.

Spinal Arteriovenous Vascular Malformations in Patients with Neural Tube Defects.

Neural tube defects, such as tethered cord, intradural lipoma, or myelomeningocele may coexist with spinal vascular malformations. The coexistence of these 2 rare entities is suggestive of a causal relationship between them, which may lead to further understanding of their pathogenesis. We present a series of 6 patients with epidural spinal arteriovenous fistulas associated with neural tube defects. We retrieved cases of spinal vascular malformations associated with neural tube defects seen at our institution. The clinical presentation, MR imaging/MRA and angiographic imaging, treatment outcomes, and long-term neurologic outcomes were analyzed. Descriptive statistical analyses are reported. Six patients with epidural arteriovenous fistulas and neural tube defects were included in this study. The mean age at presentation was 42 years, and the most common presenting symptoms were lower extremity weakness followed by sensory disturbances and bladder/bowel dysfunction. In most cases (5/6), the fistulas were located at the sacral level. All cases were fed by the lateral sacral artery (6/6). Four patients had prior spine surgery, but the fistula was in the operative bed in 2 cases. All fistulas were extradural with secondary intradural venous drainage. Five patients underwent transarterial embolization with Onyx, and 1 patient had a treatment-related complication. It is conceivable that there is a pathophysiologic link between neural tube defects and development of spinal vascular malformations. Delayed neurologic deterioration or high conus signal in a patient with a neural tube defect should suggest the possibility of such an association.

Oral Dokuları Etkileyen Arteriovenöz Vasküler Malformasyon Vakalarında Dental Tedavi Yaklaşımı

Large arteriovenous vascular malformations (AVM) of the jaws are relatively rare and potentially life-threatening lesions. When these lesions are not suspected, extraction of teeth or other surgery procedures can be fatal in dentistry. Gingival bleeding seems to be a symptom common to most documented cases. Despite their benign histology, deep lesions can produce serious systemic signs and symptoms due to extensive arteriovenous shunting and soft tissue hypertrophy. Mandibular AVM shows a wide variety of signs and symptoms, such as dental mobility, otalgia, secondary pain due to thrombosis, facial asymmetry, and cosmetic distress. A clinical case report was presented in this study. A 24-year-old girl, seen for gingival bleeding during oral hygiene and facial asymmetry, was found to have a high-flow AVM located around the right mandible. In dental and clinical examination, although she had good oral hygiene, common gingival bleeding was observed. At the mandibular rigth molar region, non-hemorrhagic, gingival granulomatous swelling was noted. Scaling and root planning and polishing was performed to the teeth carefully. The patient was called maintenance phases. During 2 year, no recurrence of the lesion has been seen. Dentists must always consider the possibility and be able to recognize the clinical signs of AVMs in order to propose proper treatment. Because, AVMs of the maxillofacial region give rise to dental emergencies and may cause disfigurement, morbidity, even death.

3:54 PMAbstract No. 332 - Ethylene vinyl alcohol copolymer in the treatment of high flow arteriovenous vascular malformations: long term results and histology

3:54 PMAbstract No. 332 - Ethylene vinyl alcohol copolymer in the treatment of high flow arteriovenous vascular malformations: long term results and histology

Uterine arteriovenous vascular malformation masked by partial molar pregnancy: diagnostic challenge and subsequent embolic treatment.

Uterine arteriovenous vascular malformations (UAVM) are uncommon and usually present in women of reproductive age. When these occur in a pregnant patient or in the immediate postpartum period, clinical presentation may overlap with several other entities such as retained products of conception (RPOC), postpartum endometritis, as well as gestational trophoblastic disease (GTD) [1]. We present a case of a UAVM that presented with life-threatening vaginal bleeding several months after dilatation and curettage (D&C) of a molar pregnancy. The UAVM was diagnosed with ultrasound (US) and magnetic resonance imaging (MRI), and subsequently treated with transarterial embolization (TAE).

Uterine arteriovenous malformation

Uterine arteriovenous vascular malformations (UAVM) are uncommon vascular diseases, occurring during reproductive age. Patients affected by UAMVs usually present with recurrent pregnancy loss or menorrhagia. Initial evaluation of UAVMs is made with ultrasonography (US) and US-Doppler. Magnetic resonance is used when a UAMV is suspected at US. Treatment can be surgical (hysterectomy or surgical removal of AVM), or with selective uterine arterial embolization. We report a case of UAMV, from its clinical signs to diagnostic confirmation and subsequent treatment.

Prise en charge des malformations vasculaires cérébrales découvertes en période ante- ou néonatale

Three types of brain arteriovenous vascular malformations can be found during the neonatal period, according to their anatomical location. Vein of Galen malformations are the most common. The others are pial arteriovenous malformations or dural arteriovenous malformations, which include dural sinus malformations. They can be asymptomatic, but most often they are associated with different symptoms, related to their angioarchitecture or their effect on the brain. High-flow arteriovenous malformations can thus be responsible for heart failure. Local or regional venous hyperpressure exposes the patient to subacute or chronic brain lesions, or to hydrovenous disorders such as hydrocephalus. Some types of venous reflux can expose patients to brain hemorrhage. The treatment chosen for these vascular malformations and their consequences is transarterial or transvenous embolization, depending on the angioarchitecture and type of lesion. The schedule for the treatment will be determined according to the malformation type and its local or general effects on the brain. The aim of this article is to present the recommendations of the French National Referral Center for neurovascular malformations in children, in order to help clinicians and radiologists treat these patients during pre- or neonatal period.

Extravascular Injection of Sclerotic Agents does not Affect Vessels in the Rat: Experimental Implications for Percutaneous Sclerotherapy of Arteriovenous Malformations

ObjectivesSclerotherapy is useful for the treatment of arteriovenous vascular malformations. However, intravascular administration of sclerotic agents into small arteriovenous niduses is often difficult. Extravascular administration of sclerotic agents causes reduction of vascular flow on Doppler echo during clinical sclerotherapy. Therefore, we aimed to investigate whether the extravascular injection of sclerotic agents affects tiny vessels. DesignAnimal study. MaterialsThe effect of extravascular injection of sclerotic agents on vessels was investigated using rat femoral and superficial inferior epigastric vessels. MethodsAfter surgical exposure of vessels, absolute ethanol, 5% ethanolamine oleate and 3% polidocanol were injected into perivascular surrounding tissues, and their effect on vessels was evaluated after 14 days using histology and coloured silicone rubber injection. ResultsThe integrity of the vascular lumen, endothelial cells and vascular patency were not affected by injection of sclerotic agents. ConclusionsAttenuation of vascular flow of an arteriovenous shunt after extravascular injection of sclerotic agents is transient and/or trivial and does not cause disruption of vessels. Therefore, sclerotic agents should be delivered to obtain sufficient destruction of arteriovenous malformation lesions and blood flow.

Pulmonalvaskuläre Beteiligung bei Morbus Osler

In hereditary haemorrhagic teleangiectasia (HHT) can be accompanied by pulmonary arteriovenous vascular malformations (PAVM). Pulmonary hypertension (PH) is regarded as a rare pulmonary manifestation. We non-invasively assessed the pulmonary circulation in 20 patients with HHT using standard resting echocardiography including contrast studies. In 14 patients a mutation in the endoglin gene was present. The other 6 patients carried a mutation in the Alk-1 gene. We identified 4 patients with manifest PH, among them 2 patients (both with endoglin mutations) with concurrent thromboembolism, and 2 patients (both with Alk-1 mutations) with hepatic manifestations of HHT. Two patients required specific pulmonary vasoactive therapy with sildenafil and bosentan, respectively. Another patient received embolisation therapy for hypercirculatory PH due to hepatic arteriovenous malformations. Pulmonary arteriovenous malformations were found in 8 patients (7 with endoglin, and 1 with Alk-1 mutations), among them were 2 patients with PH. Patients with HHT should undergo echocardiographic screening for PAVM as well as PH. When PH is detected, other conditions such as hepatic or thromboembolic diseases should be considered, regardless of the underlying genetic defect.

Two vascular arteriovenous malformations with left-to-right shunting and right-heart failure in a single patient

Arteriovenous malformations may lead to right-heart failure in cases of hemodynamically significant left-to-right shunting. Here, we report the case of a 37-year-old female who presented with congestive heart failure related to an isolated anomalous connection of the left pulmonary vein to the left brachiocephalic vein (partial anomalous pulmonary venous connection). After successful reconnection to the left appendage and clinical improvement, the patient once again developed progressive signs of heart failure. Several arteriovenous malformations were identified in the liver as the underlying cause of the patient's high-output heart failure, and the patient was retrospectively diagnosed with hereditary hemorrhagic telangiectasia. Target embolization led to right-ventricular remodeling, and persistent clinical improvement. To our knowledge, this is the first report of two rare AV-malformations with left-to-right shunting and progressive right-heart failure in a single individual.

Characterization of a distinct syndrome that associates complex truncal overgrowth, vascular, and acral anomalies: a descriptive study of 18 cases of CLOVES syndrome

Overgrowth syndromes with complex vascular anomalies pose a challenge for diagnosis and management. The purpose of this descriptive study is to present a cohort of patients with congenital lipomatous overgrowth, vascular malformations, and epidermal nevi syndrome, a distinct clinical phenotype characterized by a complex truncal-lipomatous mass, vascular, acral, and other anomalies. This cohort was ascertained following review of patient data entered into the Vascular Anomalies Center database of the Children's Hospital, Boston over a period of 7 years. Clinical data, imaging findings, and the photographic archive were reviewed. The search identified 18 unrelated patients with a distinct phenotype. Variable portions of the truncal masses observed in these patients were composed of a lymphatic malformation. Capillary malformations and high-flow lesions were commonly encountered. The lipomatous mass infiltrated the adjacent anatomic spaces and was associated with capillary, lymphatic, venous, and arteriovenous vascular malformations. Paraspinal-intraspinal extension was associated with morbid sequelae. Acral deformities included large, wide feet and hands, macrodactyly, and a wide sandal gap. Scoliosis and other musculoskeletal, neurologic, renal, and cutaneous malformations were also encountered. The uniform and highly characteristic features of the truncal lipomatous mass, in combination with vascular, acral, and other anomalies, provide evidence of a distinct nosologic and clinical entity. Morbid sequelae of the truncal involvement in this condition can be deforming and disabling; hence, prompt diagnosis and multidisciplinary care are necessary.

Thalidomide: a treatment option for bleeding GI angiodysplasias in dialysed patients.

Thalidomide: a treatment option for bleeding GI angiodysplasias in dialysed patients.

Embolization of High-Flow Craniofacial Vascular Malformations with Onyx

Various techniques and materials have been used for the endovascular treatment of craniofacial high-flow arteriovenous vascular malformations, because their rarity precludes standardization of their treatment. The aim of this retrospective review is to assess Onyx as the primary embolic agent in the treatment of these vascular malformations. Six patients with arteriovenous fistulas and 3 with arteriovenous malformations (AVMs) of the head and neck region were treated with intra-arterial (IA)/direct percutaneous injections of Onyx. Adjunctive maneuvers used during embolization included external compression of the arterial feeders or venous outflow (6 patients), balloon assist (4 patients), and direct embolization of the draining vein remote to the fistula site (1 patient). n-butyl-2-cyanoacrylate (n-BCA) was used in addition to Onyx for rapid induction of thrombosis in a large venous pouch (1 patient) and for cost containment purposes (1 patient). Four patients were treated surgically after the embolization. There were no neurologic complications secondary to the embolization procedure. The arteriovenous shunt was eliminated in all of the fistulous lesions and 2 of the 3 AVMs. The embolization was incomplete in 1 patient with a large AVM who declined further endovascular or surgical procedures. Untoward events included 2 instances of catheter entrapment (of 9 IA injections), blackish skin discoloration necessitating surgical revision in 1 patient, and difficulty of balloon deflation/wire withdrawal during a balloon-assisted embolization. Onyx appears to be a safe and effective liquid embolic agent for use in the treatment of craniofacial high-flow vascular malformations with distinct advantages and disadvantages compared with n-BCA.

Arteriovenous vascular malformations confined to the hand: An algorithm of management based on a new classification

Arteriovenous vascular malformations confined to the hand: An algorithm of management based on a new classification

Arteriovenous vascular malformations confined to the hand: an algorithm of management based on a new classification

Management of arteriovenous malformations confined to the hand is diverse and ranges from conservative measures to amputation. This paper is a retrospective review of 13 patients with arteriovenous malformations of the hand who were managed using an algorithm of treatment which considered the clinical presentation and the anatomical structure of the lesion.

Hemangioma Versus Vascular Malformation: Presence of Nerve Bundle Is a Diagnostic Clue for Vascular Malformation

Arteriovenous vascular malformations and hemangiomas are benign vascular lesions that are difficult to distinguish from one another clinically. Also, they may be confused with each other at histopathology. Therefore, histochemical stains for the presence of an artery are frequently used to distinguish between the two. Because it is clinically relevant to differentiate between arteriovenous vascular malformations and hemangiomas, this study was carried out to explore additional diagnostic clues that may help in the diagnosis and differentiation of these lesions. A total of 167 cases of benign extracranial vascular lesions were retrieved from the anatomic pathology file of our institution. These comprised 66 cases diagnosed as arteriovenous vascular malformations and 101 cases previously diagnosed as hemangiomas. The hematoxylin-eosin-stained glass slides were reviewed, Movat pentichrome histochemical stain was used to identify elastic vessels (arteries/arterioles), and S100 immunostain was used to identify nerves within these vascular lesions. For immunohistochemistry, the avidin-biotin detection method was used. With Movat stain, the presence of thick-walled elastic arteries was detected in 12 of the 101 cases previously diagnosed as hemangiomas, and these cases were therefore reclassified as vascular malformations. Using the same criterion, 2 of the 66 cases originally diagnosed as arteriovenous vascular malformations were reclassified as hemangiomas because they lacked arterial structures. Thus, with this strict criterion, we ended up with 91 cases of hemangiomas and 76 cases of arteriovenous vascular malformations. Intralesional nerves were identified in 91% (69/76) of cases of arteriovenous vascular malformations, including all the 12 arteriovenous vascular malformations previously diagnosed as hemangiomas. In contrast, no intralesional nerve was detected in any of the 91 hemangiomas. These results show that nerve bundles are consistently present in vascular malformations and absent in hemangiomas and so can be used as a diagnostic clue to differentiate between these lesions. Also, in addition to describing a previously unreported component of vascular malformations, these data further confirm the hamartomatous nature of these lesions.

The angiosomes of the head and neck: anatomic study and clinical applications.

The angiosome concept was introduced over a decade ago by Taylor and Palmer, whereby the body was considered to be composed anatomically of multiple three-dimensional composite blocks of tissue supplied by particular source arteries. Since then, detailed studies of the forearm and leg have been examined by Taylor and his coworkers. This study focuses on another region--the head and neck. Six fresh head and neck cadaver specimens were examined after infusion with a radio-opaque lead oxide mixture and correlated with over 24 previous body studies. The vascular anatomy of the skin, superficial musculoaponeurotic system (SMAS), muscles, brain, dura, and bone was examined. Each layer was painstakingly removed, photographed, labeled, and mapped to the respective arteries and veins. A radiologic subtraction technique was used to allow successive layers to be compared. This information was then scanned into a computer, analyzed, color coded, and labeled, thereby producing a three-dimensional study of the head and neck region to identify the respective angiosomes. As in previous detailed examinations of the leg and forearm, the angiosomes were found to be connected usually within tissues, such as muscle, skin, specialized organs or glands, rather than between the tissues. The muscles usually had vessels of two or more angiosomes supplying them and fell into three major groups based on the similarity of their arterial supply. In some areas, the midline anastomoses were rich, especially in the integument of the scalp, forehead, and lips. In other regions, the midline vascular connections were poor, especially in the tongue and palate. No fewer than 13 angiosomes of the head and neck, supplied by the branches of the external carotid, internal carotid, and subclavian arteries, have been defined, mapping their three-dimensional territories in the skin, the deep soft tissues, and the bones. Although most angiosomes spanned between skin and bone, three territories, those of the vertebral, lingual, and ascending pharyngeal vessels, were confined to the deep tissues without cutaneous representation. Finally, this study provides additional data for the surgeon to help plan safer incisions and better reconstructive flap procedures. It also gives information that may help explain the etiology and treatment of head and neck arteriovenous vascular malformations.