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Background A subclavian-superior vena cava arteriovenous fistula is usually acquired and secondary to trauma or operations, while congenital causes are very rare. A congenital arteriovenous fistula leads to congestive heart failure soon after birth and is typically diagnosed in early infancy. Case presentation We present an unusual case of a 21-year-old female suffering from new-onset heart failure at 20 years old who was diagnosed with a congenital arteriovenous fistula from the right subclavian artery to the superior vena cava (RSA-to-SVC) with stenosis at the proximal initial site of the fistula. The patient successfully underwent transcatheter occlusion for the fistula and had a significant improvement in symptoms at the 3-month follow-up. Conclusions An RSA-to-SVC fistula is a very rare congenital disorder that can lead to shunt-related heart failure. If there is an indication for closure, as with the patient presented, percutaneous device closure can be considered a reasonable option.

Congenital arteriovenous fistula involving the external carotid system is rare. This paper reports a case of congenital external carotid artery-external jugular vein arteriovenous fistula admitted to Xuanwu Hospital, and reviews the literature. The patient was a boy, 9 years old, with a history of pulsatile mass and thrill in the right neck since his birth. External carotid artery-external jugular vein fistula was confirmed by the digital subtraction angio-graphy. And coil embolization was done later. Postoperative immediate angiography confirmed the complete occlusion of the fistula, and partial branch of the external carotid artery can be seen. The abnormal clinical manifestation disappeared after the procedure without any complications. This case and relevant literatures remind us that congenital external carotid artery-external jugular vein arteriovenous fistula has its unique features, and it can be treated by coil embolization safely and effectively.

Thoracic aortocaval fistulae are rare entities where a direct shunt between thoracic arteries and systemic veins is seen. They can be traumatic or congenital in origin. Congenital thoracic aortocaval fistulae usually involve descending aorta and azygos, hemiazygos systems. Presenting symptoms range from continuous murmur to signs of congestive heart failure. In this case report, imaging findings of a 3-year-old girl referred for continuous murmur over the left sternal border are presented. Computed tomography angiography revealed multiple tortuous vessels along the descending aorta with a course toward the left brachiocephalic vein, and was suspicious for an aorta-venous fistula. Subsequent digital subtraction angiography for treatment planning showed a fistula originating at the level of the left 6th intercostal artery, with direct drainage into the left brachiocephalic vein without involvement of the azygos/hemiazygos system.

A case of congenital renal arteriovenous fistula (AVF) complicating pregnancy with gross hematuria was managed successfully by superselective embolization with metallic coils. The patient was in the first trimester of her pregnancy at 12 weeks of gestation. The AVF was detected by color Doppler sonography and confirmed by renal arteriography. Because of its easy accessibility and absence of irradiation, ultrasound is the first choice for pregnant patients. Color Doppler ultrasound is effective in diagnosing AVF, and it is also helpful in the long-term followup after treatment. The cirsoid-type renal congenital arteriovenous fistula has a characteristic sonographic appearance with a cluster of tubular anechoic structures in the kidney, which produce continuous turbulent high-velocity flow signals and a burr-like boundary flow spectrum. When the sonographic features are present, the diagnosis of renal AVF should be made, after which renal arteriography can be performed to confirm it. Selective embolization provided a safe and effective treatment with minimal damage to the parenchyma and without compromising renal function.

Congenital coronary arteriovenous fistula (CAVF) is a rare and its incidence during coronary angiogram is estimated about 0.1% to 0.2%. In 1908 Maude Abbott described the first CAVF. Majority of coronary artery fistulas are identified during echocardiography or coronary arteriography. They are usually small and clinically difficult to detect. Most of them resolve spontaneously and does not cause any symptoms. A large coronary fistula is usually 3 times bigger the size of a coronary artery. We report a case of congenital arteriovenous fistula arising from left circumflex artery communicating to one of the branches of right pulmonary artery, having normal coronaries. This 77-year-old female with positive stress myoview & multiple risk factors, presented with paroxysmal atrial fibrillation. Asymptomatic CAVF up to the 7th decade of life makes this case rare and unique.

Despite increased recognition of the contribution of venous outflow to intracranial pressure (ICP),1 there has been limited attention to venous hypertension as a cause of secondary intracranial hypertension, especially in children. Interventions for venous hypertension focus on addressing the underlying etiology, and can vary as widely as the underlying causes. We present a series of nine cases of pediatric venous hypertension treated at our institution, with an emphasis on the causes and successful treatment modalities. To understand the contribution of venous outflow to ICP, one needs to consider its contribution to intracranial contents. Intracranial blood volume as a whole measures 100–130 ml, compared to 75 ml of intracranial cerebrospinal fluid (CSF), which dominates ICP management. Moreover, 40% of the intracranial blood volume is found in the venous circulation, while only 15% is in the arterial circulation, another target in intracranial physiologic optimization. Additionally, if cerebral venous outflow fails to match arterial inflow, the resulting changes in intracranial contents can further predispose the patient to intracranial hypertension.1 Consideration of venous hypertension has been particularly limited in pediatrics. Although venous sinus thrombosis and post-traumatic occlusion have recently received dedicated attention in pediatrics,2 3 there have only been isolated reports of other causes of venous hypertension in children, such as venous sinus stenosis,4 extramural venous sinus obstruction,5 6 direct arteriovenous shunting as seen in dural arteriovenous fistulas,7 as well as jugular foramen stenosis in achondroplasia8 and craniosynostosis.9 We present a compendium of pediatric cases of venous hypertension treated at our institution, typically diagnosed clinically and on cerebral angiography. After obtaining IRB approval, we reviewed our database of pediatric imaging studies for evidence of venous hypertension, specifically looking for findings of venous outflow stenoses or occlusion, and the presence of ‘pseudophlebitic’ pattern with tortuous, ‘cork-screw’ cortical veins. We also reviewed available clinical measures of ICP, such as invasive monitoring, optical coherence tomography, opening pressure, and optic nerve sheath diameter. We reviewed cross-sectional imaging for ventriculomegaly, dilated or tortuous optic nerve sheaths, and the presence of empty sella. The different treatment modalities were assessed using chart review. The identified cases fall into two categories: cases of venous outflow impairment, with treatment focused on relief of obstruction; and cases of arteriovenous shunting, requiring embolization. Use of CSF diversion and medical management is also addressed. Causes of venous outflow impairment in our case series include: transverse sinus stenosis, cervical and jugular venous stenosis, compression of jugular veins at the jugular foramina in the setting of achondroplasia, jugular venous compression by the styloid process, and a restrictive small vessel venopathy. Cases of arteriovenous shunting include: direct arterio-venous fistula in the setting of hereditary hemorrhagic telangiectasia, congenital arteriovenous fistula presenting with an acquired Chiari I malformation, pial and retinal arteriovenous malformation presenting with subarachnoid hemorrhage in the setting of Wyburn-Mason syndrome, and a holohemispheric arteriovenous malformation and/or flow-related arteriopathy with extensive shunting. By reviewing these cases, and the relative success of interventions undertaken, we aim to present a guide to approaching the pediatric venous hypertension patient. Disclosures Z. Voronovich: None. A. Carlson: None. C. Taylor: None. R. Blakely: None. D. Sorte: None.

The authors report a case of iatrogenic brachial arteriovenous fistula (AVF) on the left arm in a 20-year-old man with a history of autosomal dominant polycystic kidney and failing kidney transplant. An attempt to create vascular access for hemodialysis by utilization of an existing iatrogenic brachial AVF was undertaken. The patient underwent surgical superficialization of a concomitant enlarged and deeply located vein. Four weeks after the procedure, the AVF was successfully cannulated for hemodialysis. In this case, iatrogenic/congenital AVF was successfully converted to vascular access for hemodialysis.

Arteriovenous fistulas (AVF) and true aneurysms are uncommon arterial vascular disorders of the breast. The etiology can be either acquired or congenital. Coexistence of a congenital AVF and true aneurysm of internal mammary artery (IMA) branches is a very rare condition. We present a case of congenital AVF and true aneurysm of the IMA in a woman, age 56 years. To the best of our knowledge, this is the first published case of the coexistence of a congenital AVF with a true aneurysm of the breast. The radiologic findings of these rare entities have been reviewed according to the literature.

This study aimed to investigate the treatment efficiency of interventional embolization therapy in puerile congenital deep femoral arteriovenous fistula. A retrospective analysis was conducted for 9 cases of congenital deep femoral arteriovenous fistulae treated in our department in the past 5 years. B-ultrasound examination indicated that all puerile patients suffered from deep femoral arteriovenous fistulae, which was confirmed by angiography examination. For all patients, endovascular interventional embolization therapy was conducted and angiography re-examination was implemented after 4 weeks. If there were residual orificium fistulae, the interventional embolization therapy was conducted again. In the 6 month to 2 year follow-up period, improvement of clinical symptoms was observed. Following interventional embolization, 9 cases of deep femoral arteriovenous fistulae were completely occluded and the clinical symptoms were improved. No relapses occurred. In addition, after three embolization treatments, the disease condition of one case was controlled well and the disease condition did not progress. Interventional embolization therapy has a number of advantages, including simple surgery and reliable treatment efficacy. Therefore, it is worthy of promotion and application in the clinic.

Naturally formed arteriovenous fistula (AVF) causing local vascular aneurysm dilatation in the forearm ulnar artery region is rare and is exceedingly uncommon in any age group. Presented is a case of AVF in the left ulnar artery of a 39-year-old man in whom there was no history of trauma; the deformity had been noted since childhood. The AVF had become tortuous and enlarged in size as the patient aged. As a result, aneurysm dilatation formed on the base of the AVF and that of the ulnar artery origin. Despite normal preoperative Allen test result and normal preoperative finger pressure measurement with ulnar artery occlusion, arterial duplex imaging showed that the radial artery was the dominant artery of the left arm; the AVF was resected and the base of the aneurismal dilatation, which was directly related to the ulnar artery, was repaired for the sake of the natural continuity of ulnar blood flow.

Wir berichten über eine 46-jährige Patientin mit einem seit 2 Jahren bestehenden, therapieresistenten Ulcus am rechten Fußrücken bei kongenitaler komplexer AV-Fistel. Bereits vor 12 Jahren hatte sich in der gleichen Lokalisation erstmals ein Ulcus entwickelt. Eine Coil-Embolisation führte trotz unvollständiger Okklusion der AV-Fistel zu einer Abheilung über einen Zeitraum von 10 Jahren. Aktuell imponierte um das Ulcus eine Hyperpigmentierung im Sinne eines klinischen Hinweises auf eine venöse Insuffizienz. Durch eine ausschließlich konservative Therapie der venösen Komponente der AV-Fistel konnte eine vollständige Abheilung des Ulcus erzielt werden. Wir diskutieren anhand dieses Fallberichtes die unterschiedlichen Aspekte in der Entstehung und Therapie eines Ulcus bei AV-Fistel im Rahmen eines Stewart-Bluefarb-Syndroms.

Angiodysplasia is a unifying concept and designation for all developmental vascular anomalies currently known in the medical literature as angiomatosis, arteriovenous malformation, congenital arteriovenous fistula, congenital hemangioma or lymphangiomatosis, telangiectasia, vascular hamartoma, and all those clinical disorders identified by eponyms, such as Klippel-Tranaunay syndrome and Rendu-Osler-Weber syndrome. A new classification is proposed to catalog the histomorphologic spectrum of angiodysplasia likely to be found in each individual case with the caveat that virtually all angiodysplastic lesions will have mixed histomorphology although one type may predominate. Examples of the most commonly encountered angiodysplastic lesions are described and illustrated in this review.

A 54-year-old man was admitted to hospital because of chronic right-sided heart failure. Echocardiography revealed dilatation of all chambers of the heart. Cardiac catheterization showed high-output heart failure due to left-to-right shunt caused by a congenital fistula between the right iliac artery and vein. The fistula was closed by percutaneous implantation of a covered stent. 15 months later, the patient denied any cardiac complaint. On echocardiography, the size of all chambers had almost normalized, cardiac catheterization proved a normal cardiac output. By angiography and oximetry, neither a residual nor a relapsing shunt were seen.

Objective: The aim of the present study was to consider the importance of medical information obtained via the Internet for difficult cases in hospitals, especially in those located in rural areas. We report here a case of congenital arteriovenous fistula (AVF) in the upper extremities. Patient: A 30-year-old lady was transported to our hospital by ambulance due to massive bleeding in her left hand. She was seen by our current cardiovascular surgery team for the first time, although she had been diagnosed with congenital AVF of the left arm 9 years previously. Because it was asymptomatic, she was followed up by observation. During 5 years of observation, symptoms such as cyanosis, pain, and refractory ulcers gradually developed. When she was 26 years old, she was referred to a university hospital in Akita, but surgery had already been judged to be impossible. When she was 30 years old, traumatic bleeding in her left hand and hemorrhagic shock led her to be taken to our hospital by ambulance. Using the Internet, we found an institution that had treated a large number of cases of AVF. After controlling the bleeding, we referred her to that institution. However, she could not be treated without an above-elbow amputation. Conclusion: Congenital AVF in the upper extremities is a rare vascular anomaly and has been generally accepted to be an extremely difficult disease to treat. Treatment should be started as early as possible before the presence of any symptoms. When a specialist is not available near the hospital, precise information must be found using the Internet and the patient should be referred without any delay.

Congenital arteriovenous fistula of the hand is uncommon. We report a case of unusual congenital arteriovenous fistula forming multiple fistulous communications between the arteries and veins in the right hand. Clinical findings were confirmed by color Doppler sonography and digital subtraction angiography.

SUMMARy Introduction Congenital renal arteriovenous fistulas (AVF) are rare, especially if they are associated with other developmental renal anomalies. Case Outline A 34­y ear­o ld female was hospitalized due to total painless hematuria and bladder tam­ ponade. Excretory urography revealed a horseshoe kidney with normal morphology of pyelocaliceal system and ureters. Aortography and selective renovasography detected a cluster­li ke vascular forma­ tion with multiple arteriovenous fistulas (AVF). Due to a large AVF gauge and poor flow of the efferent vein to the inferior vena cava, a surgical procedure of two renal artery segmentary branches ligation and division was performed. During the operative procedure, the presence of multiple superficial renal hemangiomas was detected. Conclusion Although selective arterial embolization represents the preferable treatment option, con­ ventional surgery remains favorable alternative in selected cases with large and complex AVF. k eywords: congenital arteriovenous fistula; horseshoe kidney; multiple hemangiomas; selective arte­ rial embolization; conventional surgery

An arteriovenous fistula is an aberrant communication between an artery and a vein. Here is a case in which risk factors including multiple fistulae, dangerous location, and high-flow blood occurred simultaneously. The patient is a 13-year-old boy diagnosed with bilateral arteriovenous fistulae between the jugular vein and carotid artery separately, with cardiac dilatation and pulmonary artery hypertension. We performed right arteriovenous fistulae resection, ligated the involved small vessels, and tightened the internal jugular vein. The tiny left arteriovenous fistulae were treated with transarterial embolism. The blood flow bruit and swelling of his neck completely disappeared, with clear improvement of his life quality, the pulmonary artery pressure has dropped from 54 mmHg to 39 mmHg. Surgery is one of the main therapies of arteriovenous fistula currently, we need to make a balance between good effect and low risk, aiming at early treatment to avoid serious complications.

Congenital arteriovenous fistula complicated by arteriovenous aneurysm is very rare. We report a case of a 23-year-old man diagnosed with an asymptomatic congenital aorto-azygous fistula complicated by an arteriovenous aneurysm. Video-assisted thoracoscopic surgery (VATS) was successfully performed to resect the abnormal feeding artery and ligate the draining azygos vein. There were no postoperative complications, and late follow-up showed a satisfactory outcome. Our experience indicates that VATS is a safe and appropriate option to treat this kind of vascular malformation.

This is a case of a 67-year-old woman with a congenital arteriovenous fistula of the right upper extremity arising from the right subclavian artery. It was treated successfully with catheter-directed embolization techniques. In reviewing the literature, a high incidence of right-sided congenital arteriovenous malformations of the proximal upper extremity was identified. This high incidence may be related to the complexity of the embryologic development of the right subclavian artery.

Congenital arteriovenous fistulas (AVF) without associated vascular malformations are uncommon. Only a very few cases of AVF have been reported in the neck. We describe our findings in a patient with AVF treated by a combined vascular and endovascular approach.

A 34-year-old man was seen at the hospital because of a pulsating mass in the left buttock. A pigmented pulsating mass inside the left buttock was palpable and vascular murmur was heard. Ultrasonography revealed a lesion extending from the skin surface of the buttock to the fascia of the musculus gluteus maximus, and gluteal arteriovenous fistula was diagnosed. Preoperative angiography showed that the superior and inferior gluteal arteries flowed into and the inferior gluteal vein flowed out of the fistula. The diagnosis of congenital gluteal arteriovenous fistula was established and a percutaneous arteriovenous fistulectomy was performed on the patient in April 1996. The postoperative course was uneventful and the fistula disappeared. Arteriovenous fistula is rare. It commonly occurs as a solitary and well localized form. We consider that surgery is actively indicated for the disease.

A 7-year-old girl with a right-sided congenital arteriovenous fistula in the neck was admitted with signs of cardial incompensation. Her fistula was fed from the right vertebral artery in antegrade as well as retrograde directions. A steal from the intracranial arteries was established. In addition, smaller feeding arteries from the neck were found. She was operated on with ligation of the right vertebral artery proximal to the fistula but the attempted ligation of the artery cranially to the fistula was unsuccessful. She was therefore embolized by the formation of a plug of platinum fiber coils in the upper right vertebral artery. Catheterization was performed from the left vertebral artery via the basilar artery. Persisting minor feeders to the fistula from cervical arteries were embolized in a second session. Finally, surgical extirpation of the fistula was performed together with the operative ligation of a crossover feeding artery from the left vertebral artery. Her heart size, heart rate and blood pressure were successively normalized.

A case of congestive heart failure in a neonate resulting from a congenital abdominal arteriovenous fistula is described; an aneurysm of the ductus arteriosus was also found in the patient. Abdominal aortography was performed and demonstrated dilated feeding arteries, including the hypogastric arteries, which communicated with a dilated umbilical vein. Ligation of the feeding arteries and the umbilical vein led to resolution of the heart failure. Transient liver dysfunction occurred, however. Symptoms improved dramatically upon removal of the ligature from the umbilical vein. Although there is one previous report of an arteriovenous fistula involving the umbilical vein, we know of no prior report of a congenital arteriovenous fistula in association with a ductus arteriosus aneurysm.

To demonstrate that airway obstruction may be the first manifestation of a congenital fistula, a female newborn is reported who presented with increasing stridor during her first 23 months of life. Magnetic resonance imaging and Doppler echocardiography revealed an enlarged innominate artery with turbulent flow. Angiography demonstrated an arteriovenous fistula between the right subclavian artery and right subclavian vein and an abnormal origin of the right internal thoracic artery. Bronchoscopy showed a pulsating compression of the middle section of the trachea. Closure and division of the fistula and aortotruncopexy were performed. The stridor disappeared, and there was marked relief of the tracheal obstruction, confirmed by bronchoscopy. It is concluded that, a search for enlarged vessels is necessary in cases of airway obstruction.

This paper describes a case of congenital arteriovenous fistula of the lower extremity caused by delivery.; A 55-year-old woman was seen at the hospital because of a pulsatile tumor in the region of the knee and hot sensation. She had the first child by painless labour at the age of 31 when she did not receve any injury to the extremities. Angiography revealed multiple arteriovenous fistulae in the left deep femoral artery, superficial femoral artery, and popliteal artery, and an angioma in the upper region of the left knee joint. Treatment included surgical ligation and excision for the communicating vessels and intraarterial coil embolization for the angioma. After the operation, symptoms disappeared and the angioma decreased in size. On the 38th hospital day the patient was discharged from the hospital. There has no sign of recurrence so far.

1. A case of arteniovenous fistula within the tibia of a girl is described. 2. Removal and replacement of a large part of the tibial diaphysis was followed by "re-formation" of the bone. 3. The literature of the condition is reviewed.

An asymptomatic young woman was found to have a continuous machinery murmur over the second right interspace near the sternum. Investigations showed an arteriovenous fistula involving the right internal thoracic artery and accompanying vein. Selective transcatheter embolization was successfully performed.

We report a 5-week-old boy with a congenital dural caroticocavernous fistula (CCF). He had gradually progressive proptosis, dilated conjunctival veins, chemosis, abducens nerve palsy and an objective bruit. Angiography of the right common carotid artery revealed fistulous communication in the cavernous sinus (CS) region. The CS was fed by the middle meningeal artery and drained through the superior ophthalmic vein and the superior petrosal vein. The child's symptoms and signs disappeared within a few weeks and did not recur over 11 months. At that time, however, angiography still showed a small communication between the right external carotid artery and the CS.

There is no more difficult lesion to manage than congenital arteriovenous fistula. The advanced lesions are extremely vascular and unless they lend themselves to total excision, prompt recurrence is the rule. For the same reason, embolization is not successful and as the major feeding vessels are occluded, access to the tumor becomes more and more limited. In order to obliterate the tumor, it must be destroyed at the microvascular level. So far, only ethanol has proved effective in this regard, and this agent must be used conservatively to avoid excessive destruction of normal tissue and systemic damage.

A 38-year-old woman presented with a large congenital arteriovenous fistula in her left hand with ischaemic atrophy of the thumb and index finger. Radical resection of the lesion and ray amputation of the index finger was done. To allow radical excision and reconstruction of the thumb and skin defect, we used an extended wrap-around flap. This flap is useful for primary reconstruction of the thumb following radical excision of a congenital arteriovenous fistula involving the thumb.

A 5-year-old boy with congenital fistula between the right vertebral artery and concomitant veins underwent a successful transcatheter fistula occlusion with a Gianturco coil. Cathet Cardiovasc Diagn 43:434–437, 1998. © 1998 Wiley-Liss, Inc.

Congenital arteriovenous fistulas between the thoracic arteries and the systemic veins are rare, and in clinical terms may mimic patency of the arterial duct. We present a neonate with a large arteriovenous fistula between the left sixth intercostal artery and the left brachiocephalic vein, to the best of our knowledge a unique site of drainage. To our knowledge, ours is also the earliest presentation and treatment of a thoracic arteriovenous fistula.

We present a unique case of renovascular hypertension due to combined renal artery disease in a 22-year-old woman. Renal angiography revealed renal artery stenosis with poststenotic dilatation and an aneurysm due to fibromuscular dysplasia in the left kidney, and a congenital arteriovenous fistula in the right kidney. The results of a captopril test and plasma renin sampling demonstrated that the renin-angiotensin-aldosterone system was stimulated in both kidneys, accounting for the hypertension in this patient. Almost all cases of renovascular hypertension are due to only one underlying renal artery disease. This is the first case of renovascular hypertension associated not only with renal artery stenosis and an aneurysm due to fibromuscular dysplasia, but also with a congenital arteriovenous fistula. (Hypertens Res 1995; 18: 255-257)

A patient with a congenital arteriovenous fistula between the middle meningeal artery and a cortical vein with a giant venous varix is described. The patient presented with an intracerebral hemorrhage that had been evacuated. Permanent cure of the fistula was achieved without morbidity by embolization with Ivalon particles.