Congenital Arteriovenous Malformation Research Articles

Parkes weber syndrome involving right lower limb: a case report.

Vascular malformations (VMs) are developmental abnormalities of the vascular system. Malformations may involve any segment of the vascular tree: arteries, capillaries, veins or lymphatics. High-flow arteriovenous malformations (AVMs) are associated with shunting of large amounts of arterial blood into the venous system; these lesions can have dynamic and dramatic hemodynamic manifestations, such as massive arteriolisation with gross venous engorgement, organomegaly of concerned anatomical region and high-output cardiac failure. Patients with Parkes Weber syndrome have clinically significant micro- or macrofistulous arteriovenous shunts, affecting usually one extremity. The patient has dilated, frequently visible pulsatile varicose veins and other visible signs of AV shunting. The abnormality is sporadic; it is likely a somatic mutation. There is frequent intraosseous involvement. The presence of high AV shunts differentiates Parkes Weber syndrome from Klippel-Trenaunay syndrome which is a clinical triad of capillary malformation, soft tissue and bone hypertrophy and venous and lymphatic malformations. The mutations in RASA 1 gene leads to Parkes Weber syndrome characterised by the presence of multiple, small (1-2cm in diameter) capillary malformations mostly localized on the face and limbs. We report a case of congenital AVM of the lower limb causing cardiac decompensation. The patient was safely and successfully treated by performing a knee disarticulation.

P04.08: Cariomegaly as a prompt in diagnosing vein of Galen malformation

Vein of Galen malformation is extremely rare congenital arterio-venous malformation. It could present prenatally as cardiomegaly, signs of congestive cardiac failure,hydrops,macrocephaly etc. Routine cranial ultrasound screening planes could potentially miss the finding unless targeted sonogram with color flow map is performed. We present one such case. A 29 year old primigravida was referred to our unit with cardiomegaly of unknown etiology detected at routine anomaly scan at 19 weeks of gestation. There was no significant medical or family history. No history suggestive of parvo virus infection/exposure. Her blood group was A Rh Positive and no history of any abortion/prior blood transfusion. On detailed anomaly screen, there was global cardiomegaly without any structural cardiac abnormalities. Bracheo-cephalic vein was dilated and so are the neck veins. Routine cranial screening planes were normal. With a high suspicion of volume overload probably by A-V malformation, targeted neurosonogram was performed to detect a large venous confluence measuring 4.1 x 3.9 cm. On three-dimensional angio-sonography using 3 D CFM we could demonstrate the presence of large Vein of Galen malformation with multiple feeding vessels with high flow velocity. In view of such findings, poor prognostication was explained to couple. Follow up scan performed a week later and there was increasing cardiomegaly with pericardial effusion. Couple decided to terminate the pregnancy and fetus was expelled 2 days later. Fetopsy confirmed our finding. Finding of a global cardiomegaly should prompt thorough anatomical evaluation. Routine cranial planes would potentially miss Vein of Galen malformation unless suspected. Use of color flow map would help in the diagnosis and 3 D anigosonography could assist in thorough evaluation and prognostication.

Malformación arteriovenosa pudenda con trombosis espontánea secundaria de cuerpo cavernoso. Reporte de caso

Congenital arteriovenous malformations of the pelvic and perineal system are rare conditions, with few reported cases, making it difficult to standardize the diagnosis and management. Given the above, the case is presented of a young adult patient with no history of trauma, who presented with a spontaneous perineal hematoma, associated with local pain in penis, irritative low urinary tract symptoms. He also had erectile dysfunction and penile curvature, which was diagnosed by MRI of the pelvis, and arteriography and definitively managed with selective embolization in the Fundación Cardiovascular, Colombia. The diagnostic and therapeutic approach was closely related to the data found in the world literature. Further studies are needed to establish the effectiveness of embolization as definitive management of this condition.Although arteriovenous malformations involving the pelvic and perineal system are uncommon, it must be taken into account in the differential diagnosis of patients in the emergency department with perineal hematoma with no history of trauma or recent sexual intercourse.© 2013 Sociedad Colombiana de Urología. Published by Elsevier España, S.L. All rights reserved.

Interventional treatment of arterio-venous malformations

BackgroundDue to their hemodynamic effects and tendency to progress, the majority of congenital arterio-venous malformations (AVM) require treatment. AVM are classified according to clinical severity (Schobinger classification, stages I–V) and angiographic appearance (types I–IV).TreatmentEndovascular embolization is the treatment of choice. However, because complications such as necrosis and neuropathy occur in up to 15% of cases, treatment is challenging and is dependent on the angiographic appearance. Critical for treatment success is elimination of the so-called nidus, which is the location of the short circuit connection between the artery and the often extended aneurysm-like drainage vein. Embolization of only the feeding artery without the actual nidus or the incomplete elimination of drainage veins should be avoided. Absolute 96 % alcohol in addition to coiling of the nidus has been established as the most effective technique.ConclusionUnlike other embolic agents, alcohol leads to a definitive destruction of the vessel wall. Recanalization and recurrence are excluded due to adequate elimination of the nidus.

ABSTRACT 321

Background and aims: Vein of Galen Malformation (VGAM) is a rare condition. VGAM is hallmarked by multiple arterio-venous shunts from choroidal vessels into median prosencephalic vein of Markoswki. Arterial-venous shunt at choroidal vessels leads to overload of venous system and high cardiac output failure. When untreated, this malformation has poor outcome leading to death. Aims: We present two newborn cases of VGAM who presented with signs of cardiomegaly and a normally structured heart. Methods: Case I: One-day old newborn who presented with tonic-clonic seizures and cardiac failure (cardiomegaly and hepatomegaly). Initial cranial ultrasound scan (CUSS) revealed arterio-venous malformation (AVM) confirmed in Head CT with areas of cerebral infarction. Patient underwent endovascular embolisation which was complicated with diffuse subarachnoid hemorrhage. Patient developed fixed pupils, extensive brain injury and died. Case II: Four-day old baby who presented with poor feeding and signs of cardiac failure (cardiomegaly and refractory metabolic acidosis). Routine CUSS revealed a midline AVM suggesting VGAM. Patient underwent endovascular embolisation complicated by pulmonary embolism and extensive hypoxic brain injury leading to death. Results: We had differentialdiagnosis of sepsis, congenital heart defect, AVM and metabolic disorder. Echocardiographyshowed normally structured heart. Case I had neurological symptoms but Case IIhad normal neurological examination and AVM was found in a routine CUSS. Both cases confirmed the poor prognosis of this congenital condition. Conclusions: We recommend routine CUSS in newborns with unclear diagnosis of cardiac failure. Complete neurological examination (including auscultation of the fontanel) and CUSS as a non-invasive screening method should be performed in all cardiac patients.

Malformação arteriovenosa da artéria hipogástrica — A propósito de um caso clínico

Congenital arteriovenous malformations (AVMs) can occur in any vascular territory but their pelvic location is rare in male patients, and presents a difficult therapeutic challenge.The authors present a case report of a 72 year old man, who presented with symptoms of dysuria, hematuria, and pelvic discomfort. The study by computed tomography (CT) detected the presence of a pelvic AVM with feeding vessels from the left hypogastric artery. The patient underwent transcatheter selective embolization and, due to recurrent symptoms, percutaneous procedure was repeated one year after. Patient has been followed anually and remains asymptomatic and the CT demonstrated reduction in AVM size.In this report, we will discuss the therapeutic approach to congenital pelvic malformations.

Congenital renal arteriovenous malformation presenting with gross hematuria after a routine jog: a case report

IntroductionCongenital renal arteriovenous malformations are abnormal communications between the intrarenal venous and arterial systems. An unusual cause of massive gross hematuria and an even rarer cause of hemodynamically significant anemia, its presentation remains variable from incidental imaging findings to severe hypertension and congestive heart failure.Case presentationWe present a case of a 44-year-old Chinese man with no personal or familial history of bleeding diasthesis that presented with gross hematuria leading to clot retention after routine physical activity.ConclusionsWe have presented this case in an effort to highlight the possibility of this entity as a cause of acute upper urinary tract hemorrhage and the need for a computed tomography angiogram to clinch the diagnosis.

Large congenital renal arteriovenous malformation treated with interlock coil embolization

Renal vascular malformation is an abnormal communication between the intrarenal arterial and venous system. These malformations include arteriovenous malformation (AVM) and arteriovenous fistula (AVF).1 Renal AVM is a congenital developmental abnormality in which the renal artery and renal vein communicate through a network of abnormal vessels. By contrast, renal AVF is a direct communication between an artery and a vein. The etiology of congenital AVM is unknown, while the cause of acquired renal AVF is usually found to be percutaneous renal biopsy, other procedures, and incidental renal trauma. A 57-year-old woman visited our hospital with intermittent left flank discomfort of several years’ duration. The symptom had worsened with position change and had become particularly bothersome during the preceding few months. She had no past medical history including trauma or procedures. On physical examination, a continuous bruit was audible over the left flank area. Urinalysis and blood tests showed normal findings. A contrast-enhanced computed tomography scan of the abdomen demonstrated aneurysmally dilated enhancing vessels with attenuation similar to the arteries in the left kidney (Panel A). Digital subtraction arteriography showed a feeding artery to a large AVM with abnormal dilation of the nidus and early opacification of venous flow (Panel B1). In this patient, the large AVM was treated with interlock coils (Panel B2). After super-selective catheterization, coil-embolization of all feeders was performed successfully (Panel B3). Magnetic resonance imaging (MRI) before treatment presented aneurysmally dilated vessels with high flow and suggested an underlying vascular malformation (Panel C1). A followup MRI taken 6 months post-embolization showed successful occlusion of the AVM with coils (Panel C2). In this case, AVM was managed with deployment of coils into the vascular malformation, which promoted thrombosis.2 Complications can arise from the rapid shunting of the coils into the venous circulation, leading to emboli entering the pulmonary circulation or the left side of the heart if interarterial communication exists. Blocking the venous output of the AVM can help in preventing this complication. It is also important for the angiographer to be familiar with retrieval techniques if such a complication arises.3 Images in Vascular Medicine

Giant Congenital Intercostal Arteriovenous Malformation With Extensive Involvement of Chest Wall and Ribs: Surgical Experience

Intercostal arteriovenous malformations (AVMs) are rare lesions. Review of literature shows that most reported cases are secondary to trauma or iatrogenic in origin. Congenital intercostal AVMs are extremely rare. We believe that only 1 case report of congenital intercostal arteriovenous malformation has been reported previously in the literature. We present an exceedingly rare case of giant congenital intercostal AVM in a young patient diagnosed on contrast-enhanced computed tomography of the thorax and treated by surgical resection of the involved chest wall and ribs with reconstruction of the surgical defect.

Complicated Congenital Gluteal Arteriovenous Malformation With Hemorrhage in Pregnancy

Extracranial congenital arteriovenous malformations (AVMs) are rare clinical entities that can be progressive in nature. The influence of pregnancy on lesion progression has been discussed in the past. This report presents an unusual case of 23-year-old primigravida woman who presented at 36 weeks' gestation with complicated necrotic ulceration and hemorrhage of the right gluteal region. A hyperpigmented mark with varicosities was initially noted at birth, but during pregnancy it showed remarkable progression and was first identified as an AVM. After hemorrhage control and induced delivery, the lesion was successfully treated with several embolizations. Complete wound healing was achieved, but because of partial recurrence at 3 years, repeat embolization was performed, with satisfactory clinical improvement and residual 25% arteriovenous shunting on transarterial lung perfusion scintigraphy study. AVM complications during pregnancy are uncommon, and this case supports the prior opinion that pregnancy can stimulate lesion progression. Especially in undiagnosed and previously untreated cases, this can lead to life-threatening complications for the mother and fetus. Long-term lesion management usually requires combined endovascular and surgical treatment.

Abnormal Vessel of the Ear Associated with Congenital Arteriovenous Malformation in the Postauricular Region

Variations in the branching pattern of the common, external, and internal carotid arteries can present as arteriovenous malformations, and their basis can be explained embryologically. Our case was a rare variation presenting as a congenital, very gradually increasing bluish painless swelling at the region of the left lobule of the ear arising from an abnormal vessel (from the postauricular artery) which was explored under general anesthesia through a postauricular curved incision. The abnormal vessel and other feeding vessels were ligated and a sclerosing agent injected. Anomalies of pharyngeal arch arteries like our case can be found resulting from the persistence of channels that normally disappear, and prior knowledge of these anomalies is essential before surgeries like mastoidectomy to prevent alarming hemorrhage.

The Role of Transarterial Embolization in the Management of Hematuria Secondary to Congenital Renal Arteriovenous Malformations

Objective: To evaluate the efficacy and safety of transarterial embolization (TAE) in the management of hematuria secondary to congenital renal arteriovenous malformations (AVM). Patients and Methods: Between May 2007 and February 2012, 6 patients with congenital AVM treated with TAE were analyzed retrospectively, followed by a brief review of TAE in the treatment of congenital AVM. Clinical records with respect to general conditions, location, embolic materials, complications and overall outcome were collected from the original hospital charts and outpatient medical records. Results: Three patients with AVM were confirmed by contrast-enhanced CT scans, and the other 3 patients were detected by renal angiography. TAE was performed with steel coils in 2 patients and n-butyl-2-cyanoacrylate (NBCA) in 4 patients. After a mean follow-up of 22 months, no serious adverse effects were observed in all patients. There were no complaints of hematuria at the end of the follow-up period. Conclusion: For unexplained massive hematuria, congenital renal AVM needs to be considered as a differential diagnosis. Selective renal angiography and embolization should be recommended as the first choice to treat massive hematuria secondary to congenital renal AVM.

Giant congenital malformation of the perirectal plexus in computed tomography imaging – case report.

SummaryBackground:Congenital arteriovenous malformation (AVM) in the pelvic area is uncommon in males.Case Report:The described case is of a giant lesion of this type that caused recurrent hemorrhaging in the lower part of the gastrointestinal tract. Preliminary diagnosis of vascular pathology was made on the basis of an endoscopic examination that revealed numerous pulsating protuberances of the rectal wall, in which blood flow was identified by means of transrectal ultrasonography. Complementing the diagnostics with a CT revealed a considerable extent of malformation, as well as its morphology and anatomical relations with the surrounding tissues.Results:Following a two-year follow-up period, the malformation did not progress or demonstrate any intensification of clinical symptoms, therefore the patient continues to undergo conservative treatment.

Adequate time of initiation of continuous infusion of nitroglycerin for controlling pulmonary arterial pressure during ethanol embolotherapy of congenital arteriovenous malformation of the extremities

Adequate time of initiation of continuous infusion of nitroglycerin for controlling pulmonary arterial pressure during ethanol embolotherapy of congenital arteriovenous malformation of the extremities

Pelvic congenital arteriovenous malformation diagnosed by transrectal ultrasonography: A case report

Arteriovenous malformations (AVMs) of the pelvis are relatively rare and difficult to treat because of multiple and extensive feeding vessels. We report the case of a 69-year-old male with pelvic congenital AVM that was detected during tests for dysuria. He visited our hospital complaining of voiding difficulty. Digital rectal examination revealed a pulsating mass that was palpable on the right side of the prostate. Transrectal ultrasonography showed multiple hypoechoic lesions adjacent to the prostate and colour Doppler ultrasonography revealed the flow regions corresponded to the hypoechoic lesions. Computed tomography demonstrated large-to-small tubular vessels adjacent to the prostate, while pelvic angiography showed many small feeder arteries extending mainly from the right internal iliac artery. He was diagnosed as having pelvic congenital AVM. Uroflowmetry revealed slight voiding difficulty without residual urine. The patient decided against treatment and requested we monitor his clinical course.

Laparoscopic Management of Uterine Arteriovenous Malformation Via Occlusion of Internal Iliac Arteries

Uterine arteriovenous malformation (AVM) can be congenital or acquired. When acquired (e.g., fistula), it results from abnormal arteriovenous communication between one or more uterine arteries and a myometrial and/or endometrial venous plexus, without the interposition of a vascular nidus. Arteriovenous malformation is composed of a tortuous net of fragile low-resistant arteriovenous shunts. Other arteries can be involved in fistulas, including ovarian arteries or those from the round ligaments of the uterus, in particular in congenital AVMs, which develop from failure in embryologic differentiation that leads to multiple abnormal vascular connections. In these cases, extension to pelvic vessels other than uterine arteries is frequent. Acquired AVMs often result in trauma to the uterus such as dilation and curettage in 85% of cases, gestational trophoblastic disease, or endometrial carcinoma.

OP20.08: Congenital uterine arteriovenous malformation: a case report and literature review

OP20.08: Congenital uterine arteriovenous malformation: a case report and literature review

Limitations of fetal ultrasonography and magnetic resonance imaging in prenatal diagnosis of congenital cerebral arteriovenous malformations with hemorrhagic onset

A fetus at 30 gestational weeks was observed on fetal ultrasonography to have a dilated right lateral ventricle. After delivery, the entity was diagnosed as a prenatal intracerebral hemorrhage (ICH) due to a ruptured arteriovenous malformation (AVM). Ultrasonography and MRI examinations performed before birth indicated a cerebral aneurysm in the territory of the right middle cerebral artery. However, digital subtraction angiography revealed an intracystic hemorrhage due to a ruptured cerebral AVM. Arteriovenous malformations in children are rare, difficult to diagnose, and result in permanent sequelae after delayed treatment. Patient prognosis depends on early and accurate diagnosis and intervention. Outcomes can be improved if an AVM in a child is detected at the onset of ICH for young infants in the prenatal or early postnatal periods. Early AVM diagnosis is limited to fetal ultrasonography and MRI, and special consideration through invasive examination including neonatal digital subtraction angiography is urged unless a correct and clear diagnosis is made at an early stage. Prenatal ICH due to an AVM is rare. The authors discuss their observations and findings.

Pelvic congenital arteriovenous malformation diagnosed by transrectal ultrasonography: A case report

Arteriovenous malformations (AVMs) of the pelvis are relatively rare and difficult to treat because of multiple and extensive feeding vessels. We report the case of a 69-year-old male with pelvic congenital AVM that was detected during tests for dysuria. He visited our hospital complaining of voiding difficulty. Digital rectal examination revealed a pulsating mass that was palpable on the right side of the prostate. Transrectal ultrasonography showed multiple hypoechoic lesions adjacent to the prostate and colour Doppler ultrasonography revealed the flow regions corresponded to the hypoechoic lesions. Computed tomography demonstrated large-to-small tubular vessels adjacent to the prostate, while pelvic angiography showed many small feeder arteries extending mainly from the right internal iliac artery. He was diagnosed as having pelvic congenital AVM. Uroflowmetry revealed slight voiding difficulty without residual urine. The patient decided against treatment and requested we monitor his clinical course.

Renal Hücreli Karsinoma Benzeyen Konjenital Bir Renal Arteriyovenöz Malformasyon

Renal arteriovenous malformation is a rare condition. It is usually asymptomatic. When symptomatic, it presents with hematuria, flank pain, a mass on renal imaging and abnormal radiologic findings. It can mimic renal cell carcinoma. The diagnosis and treatment of renal arteriovenous malformations are different from those of renal cell carcinoma. We described a renal arteriovenous malformation mimicking renal cell carcinoma. A 35-year old man was admitted to the hospital with a history of right flank pain. Computerized tomography scan of the patient revealed a right renal mass, enhancing on contrast imaging. The patient underwent right simple nephrectomy. Pathologic examination of the nephrectomy specimen revealed a congenital cystic arteriovenous malformation. We suggest that it is essential to make a differential diagnosis of any lesion mimicking renal cell carcinoma.