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Spinal vascular malformations include various entities such as spinal arteriovenous malformations (AVM) and represent a rare disease. Patients may present with acute or progressive sensorimotor deficits and/or nonspecific symptoms such as hypoesthesia or paresthesia, diffuse back and muscle pain, or feelings of weakness. When spinal arteriovenous malformation is suspected, magnetic resonance imaging (MRI) is the first diagnostic step to rule out other differential diagnoses, followed by a selective spinal angiography. Therapeutic options include endovascular therapy by embolization and/or surgical resection. If spinal AVM is treated properly, a significant reduction in symptoms can be achieved.Spinale arteriovenöse Malformationen gehören zur Gruppe der spinalen Gefäßmissbildungen und stellen ein seltenes und bisweilen unterdiagnostiziertes Krankheitsbild dar. Klinisch präsentieren sich Patienten mit akut oder schleichend einsetzenden sensomotorischen Defiziten und/oder unspezifischen Symptomen wie Hypästhesie/Parästhesie, diffusen Rücken- und Muskelschmerzen oder Schwächegefühl. Zum Ausschluss anderer Differenzialdiagnosen empfiehlt sich zunächst eine spinale Magnetresonanztomographie (MRT), die bei Erhärtung bzw. Bestätigung des Verdachts durch eine selektive spinale Angiographie ergänzt werden sollte. Therapeutisch bietet sich eine endovaskuläre Therapie mittels Embolisation und/oder eine offen-chirurgische (Teil‑)Resektion an. Bei rechtzeitiger Therapie kann eine deutliche Beschwerderegredienz erreicht werden.

Vascular malformations are congenital anomalies of the vascular system that often affect the head and neck region. Vascular malformations are typically present at birth and proportionately grow with the patient. In contrast to vascular tumors, they are composed of dysplastic vessels rather than proliferating cells. The most commonly used classification is that of the International Society for the Study of Vascular Anomalies (ISSVA). According to this classification, simple malformations include venous, capillary, lymphatic, arteriovenous malformations, and arteriovenous fistulas. Symptoms depend on the affected anatomical structures and can range from swelling to life-threatening bleeding, airway obstruction or blindness. Management options for vascular malformations include conservative approaches, surgical intervention, sclerotherapy and embolisation as determined by type of malformation, location and associated complications. This article provides an overview of the major types of vascular malformations that affect the head and neck.

An Unusual Hemisphere Syndrome Abstract. A patient with known hereditary hemorrhagic telangiectasia presents with transient right arm weakness and dizziness. A transient ischemic attack is diagnosed on clinical and risk factors. In the course of the disease, the patient suffers a convulsive event and fever occurs twice without a clear focus on the infection. The initial skull MRI shows a centroparietal lesion on the left with signs of accompanying edema. Due to this unusual concomitant edema, a neoplastic event must also be considered for differential diagnosis. However, the further examinations show no evidence of neoplasia, but the course MRI of the skull shows the image of septic embolisms with a brain abscess. After neurosurgical remediation and appropriate antibiotic treatment, the clinical course is pleasing. The cause of the septic embolisms was a pulmonary arteriovenous malformation due to hereditary hemorrhagic telangiectasia, which could be coiled without complications.Zusammenfassung. Ein Patient mit bekannter hereditärer hämorrhagischer Telangiektasie stellt sich aufgrund einer transienten rechtsseitigen Armschwäche und Schwindel vor. Aufgrund der Klinik und der Risikofaktoren wird die Diagnose einer transienten ischämischen Attacke gestellt. Im Verlauf erleidet der Patient ein Krampfereignis und fiebert zweimalig auf, ohne dass sich ein klarer Infektfokus zeigt. Die initiale Schädel-MRT zeigt eine zentroparietale Läsion links mit Zeichen eines Begleitödems. Aufgrund dieses ungewöhnlichen Begleitödems muss differenzialdiagnostisch auch an ein neoplastisches Geschehen gedacht werden. Die weiteren Untersuchungen zeigen jedoch keine Hinweise auf eine Neoplasie, die Verlaufs-MRT des Schädels dann jedoch das Bild septischer Embolien mit einem Hirnabszess. Nach neurochirurgischer Sanierung und entsprechender antibiotischer Behandlung zeigt sich ein erfreulicher klinischer Verlauf. Als Ursache der septischen Embolien fand sich eine pulmonale arteriovenöse Malformation im Rahmen der hereditären hämorrhagischen Teleangiektasie, die komplikationsfrei gecoilt werden konnte.

Foam sclerotherapy is well established in the treatment of varicose veins of different sizes.A literature review was performed to determine which specific indications for foam sclerotherapy exist.Treatment of venous leg ulcers, varices that might rupture, varicose veins in geriatric patients, pudendal veins and seroma could be determined as specific indications for foam sclerotherapy.In some indications, foam sclerotherapy is the treatment option of first choice. It is an effective and safe treatment option.

Clinical history, physical examination, evolution and imaging findings (Colour Doppler sonography, MRI if available) are of pivotal importance in the diagnostic pathway of an infantile vascular anomaly. Histopathology with specific stains and markers is contributive in difficult cases. Differentiation between vascular tumors (hemangioma) and vascular malformations is now well known and integrated into the ISSVA classification. We report here a 6-months-old boy, who presented with a localized cutaneous and expansive vascular birthmark in the left cheek and developed bleedings at the age of 18 months. Diagnostic features of a hemangioma were not evident, and the final diagnosis of a venous malformation was confirmed by histopathology.

Venous malformations are the prototype low-flow malformations in the head and neck region. Arteriovenous malformations (AVM) represent the main high-flow malformations. In recent years it has been possible to significantly optimize the therapeutic options for venous malformations. In addition to conventional surgery, laser treatment and sclerotherapy have become established techniques and the importance of embolization with new alcohol-based materials is increasing. AVM are progressive and destructive diseases. Therapy of choice is usually a combined treatment comprising embolization and surgical removal of the arteriovenous nidus. This curative approach is usually possible if diagnosis is made at an early stage. Incomplete embolization or sole ligation of the arterial supply causes progression. There is a clear need for improved therapeutic methods and pharmacotherapeutic approaches.

Congenital venous malformations (VM) at the hand are rare. VM consist of dysplastic venous vessels without progressive cellular proliferation. The therapy for VM is considerably different from that for vascular tumours (e. g., haemangiomas). Treatment options for vascular malformations are antithrombotic medication, local compression, resection of the VM, and obliteration of the lumina by percutaneous sclerosation. Here, the percutaneous sclerosation for the treatment of VM with sodium tetradecyl sulfate has been illustrated and discussed on the basis of 2 case reports.

We present the case report on a 66-year-old male patient who appeared in our clinic with recurrent epistaxis. Anterior rhinoscopy during acute bleeding yielded no information on the origin of hemorrhage. On renewed epistaxis, angiography was performed on which an arteriovenous malformation in the sphenoid sinus could be identified as the cause of epistaxis. Embolization of afferent vessels successfully reduced the malformation and stopped the bleeding. In cases of recurrent epistaxis, especially in combination with an unremarkable anterior rhinoscopy, angiography is recommended for the detection of life-threatening vessel malformations.

The understanding of hereditary vascular anomalies was hampered for a long time by unclear und unspecific terminology. Today, the classification of the International Society for the Study of Vascular Anomalies (ISSVA) differentiates between vascular tumours (mostly infantile haemangioma) with active endothelial proliferation and regression and vascular malformations (VM), which are defects of the vascular morphogenesis and are distinguished in predominantly venous, arterial, capillary, lymphatic, arteriovenous or combined VM. Symptoms are pain, swelling and restricted movement, accompanied by skin signs like dys-plastic veins and capillary VM (naevus flammeus). Thrombophlebitis and chronic venous insufficiency are related to venous VM. Arteriovenous VM are progressive and can cause ischaemic necroses, in rare cases even a high-output cardiac fail-ure. Lymphatic VM lead to localised swelling, in the long run often to recurrent erysipelas and lymphorroea. Primary imaging is provided by -ul-trasound including flow measurements. Mor-phol-ogy and organ involvement is best delineated by magnetic resonance imaging. Phlebography is used to image deep venous system anomalies and is always accompanied by varicography of the dysplastic parts of the venous VM. Digital subtraction angiography is performed to demon-strate the flow pattern in feeding arteries, the nidus and the drainage veins of arteriovenous VM. Besides size and localisation the prognosis of the patients is determined by the pressure (the high-er the pressure, the poorer the prognosis) and the flow rate (the higher the flow rate, the poorer the prognosis) in the VM. Diagnosis and treatment of these rare diseases are best performed in special-ised, interdisciplinary centres.

The treatment of congenital, vascular malformations is a challenge for physicians and patients. Although different therapeutic options have been described to date, their individual relevance has still to be defined. Â METHODS: We performed a retrospective study of 61 patients with a venous malformation (VM, mean age 22 years), who were referred to our depart-ment during the last 5 years. The size of the VM was larger than 5 cm in 41 patients (66 %). The lower extremities were involved in 45 cases (73 %). The most frequent clinical manifestations were recurrent swelling (80 %), pain (63 %), varicosis (60 %) and thrombophlebitis (39 %). MR angiography with venous sequences was always performed before treatment. Depending on the localisation and the extension of the VM, different techniques of embolisation were selected: foam sclerotherapy or application of synthetic glue by direct punction, coiling of pelvic veins or arterial embolisation with glue.42 patients (69 %) underwent a procedure because of the complaints or the extension of the VM. An embolisation was performed in 25 patients with 65 interventional sessions. The most frequent technique was foam sclerotherapy (45 ×), followed by glue injection (13 ×), pelvic -venous coiling (6 ×) and arterial embolisation with glue (1 ×). Fifteen patients (60 %) reported a very good and 8 patients a marked improvement (32 %). In two cases there was no change of the complaints. The postinterventional complications were severe pain (n = 3) and skin/fat necrosis at the toe of one patient.The embolisation of venous malformations is an effective therapeutic tool. Different techniques can be used to address specific localisations and morphological patterns. The com-plication rate is very low when a step-by-step -approach is used, so that a repeat intervention is feasible. However, a more specific documentation of the post-interventional changes of the VM is needed before a general recommendation can be given.

Beschrieben wird der Fall einer 27-jahrigen Patientin, die sich aufgrund einer zufallig festgestellten, asymptomatischen, retinalen Gefasveranderung in unserer Klinik vorstellte. Aufgrund der typischen funduskopisch erkennbaren Veranderungen wurde von uns die Diagnose einer arteriovenosen Malformation gestellt. Die weitere Diagnostik erbrachte keinen Anhalt fur das Vorliegen einer zerebralen oder fazialen Beteiligung.

Congenital malformations of the lung and diaphragm are a challenge in paediatric surgery. Depending on the malformation they show a broad spectrum of symptoms and a varying age at manifestation. Thus there are many diagnostic and therapeutic options, which require a good knowledge of the pathology. The treatment of these complex cases should lead to early referral to paediatric centres containing an interdisciplinary team with neonatologists, paediatric pulmonologists and cardiologists, ENT surgeons, anaesthesiologists, radiologists and paediatric surgeons. Some malformations are diagnosed prenatally and need intrauterine interventions. Decisive is the early diagnosis and treatment of these malformations. Nowadays the surgical therapy of neonates and infants with malformations of the lung and diaphragm is enriched by a number of endoscopic and endoluminal techniques, which are discussed critically in this article.

The differential diagnosis of vascular malformations of the upper aero-digestive tract may represent a diagnostic challenge and become a hindrance for therapy planning. The terminus "extracranial vascular anomalies" of the Head and Neck integrates hemangiomas and vascular malformations. The differential diagnosis contains benign and malign neoplasms, naevi, pigmentations and purpura.

Report on a 34-year-old woman with an arteriovenous malformation (AVM) in the area of the right hippocampus. On the basis of an interdisciplinary indication for step-by-step embolization of the AVM, the first partial embolization with Onyx occurred successfully and without complications. 10 days later, the second partial embolization also turned out well, but during retraction of the microcatheter an exit of Onyx happened into the guiding catheter in spite of continuous aspiration. From there the embolic material was carried off to the carotid T during the control angiography. Despite several efforts of retraction and fragmentation of the dislocated Onyx, finally infarctions with hemiparesis and aphasia resulted in the areas of the terminal branches of the left middle cerebral artery and the left pericallosal artery. However, the expert opinion could not ascertain a malpractice in the treatment of the AVM. Therefore, a suit was not filed against the interventional neuroradiologist.

A representative compilation of data on the incidence and prevalence of benign cutaneous vascular neoplasms and malformations in childhood has been made.A review of the literature has been made and basic data from an own epidemiological survey in the province of Tyrol (Austria) in 6-year-old children are evaluated.For capillary malformations with spontaneous regression (salmon patches), the reported numbers usually vary between 20 and 30% of the newborns, while true, persisting capillary malformations (port-wine stains) can be found in about 1%. Strawberry angiomas are found in about 3% of mature newborns but in up to 12.5% of preterm children. Complex vascular malformations or severe cases of vascular tumours are very rare.All in all, vascular lesions are very common findings in childhood, but in most cases harmless and transient in nature. The number of lesions that may require adequate but usually uncomplicated treatment amounts to about one percent of children (strawberry angiomas and port-wine stains). Complex and/or life-threatening severe vascular tumours and malformations that represent a considerable therapeutic challenge are extremely rare events.

The congenital retinocephalic facial vascular malformation syndrome is characterized by unilateral, nonhereditary retinal and cerebral arteriovenous malformations (AVMs) and is occasionally associated with orbital vascular changes. Typical signs are facial and oral mucosal vascular changes, rarely with changes of the maxilla or mandible. An AVM causes high blood flow because of direct connection (shunting) of major vessels without interposition of capillaries. Ocular complications include retinal and vitreous hemorrhages, edema, venous occlusion (risk of rubeosis iridis and secondary glaucoma). Neuroophthalmological changes comprise optic atrophy, papilledema, proptosis, pupillary changes, hemianopia, gaze paresis, nystagmus, cranial nerve palsies, strabismus, and amblyopia. Neurological complications include headache, subarachnoid hemorrhage, convulsions, cerebral hemorrhages, increased intracranial pressure, hydrocephalus, and stroke with hemiparesis. Threatening oral hemorrhages or epistaxis may rarely occur.

Vascular malformations are inborn errors of vasculogenesis in localised regions of the body. They are present at birth and grow proportionally with the child without ever showing any tendency to regress. This fact distinguishes them clearly from haemangiomas, which represent vascular tumours with high proliferation during the first year of life followed by a slow involution thereafter. The strategy for the treatment of vascular malformations differs substantially from the one for haemangiomas. According to the type of vascular malformation, the treatment may consist in laser therapy, sclerotherapy, selective embolisation, and/or surgical resection. Whereas systemic medication may save the life of children with life-threatening haemangiomas, such treatment would have no significant effect in children with vascular malformations. The aim of the surgical treatment is to perform a complete resection of the malformation in order to prevent its recurrence. However, since vascular malformations often have an infiltrative growth, frequently only subtotal resections can be performed to avoid unacceptable functional or cosmetic disfigurement of the body. Generally, an optimal management of children with vascular malformations includes a multidisciplinary approach with critical consideration of all potential treatment procedures.

We report the case of a 35-year-old woman who has been admitted to our emergency room because of the sudden onset of a left-sided hemiparesis. The physical examination showed disseminated teleangiectases on the upper and lower lip, on the mucosal surface of the tongue and on the skin. The cerebral CT scan presented a right-sided fronto-parietal lesion, more likely an abscess, confirmed on surgical removal. A meticulous family history showed the presence of similar clinical features among several family members, raising the certainty about the presence of the Rendu-Osler-Weber disease. This case is highly instructive in emphasizing the value of taking a careful medical history and how doing it is extremely important in our daily practice.

Haemangiomas represent the most common type of benign vascular tumours in childhood. A proliferative phase of unknown duration and extent is followed by an involutional period, which passes into regression in approximately 70 % of all cases. A multitude of different treatment options, such as corticosteroids, cryotherapy, laser, sclerotherapy, radiotherapy and surgical methods have been published. Due to the high rate of spontaneous regression, many authors advise not to undertake any treatment. The dilemma of this "wait and see" approach constitutes those cases in which sudden and pronounced growth is not followed by complete regression with possible severe aesthetic and functional impairment. To avoid this dilemma, a specific algorithm for the treatment of haemangiomas was instituted at our department, based essentially on early laser treatment when relevant growth is present.More than 2000 patients with haemangiomas as well as vascular malformations have been treated at our department in the last 16 years. An algorithm for treatment of these disorders is presented.All therapeutic procedures, especially direct neodymium-YAG laser treatment with either contact cooling or intralesional, is described and postinterventional results are presented.Early laser treatment of fast growing haemangiomas prevents uncontrolled proliferation of these childhood tumours and, in our experience, represents a definite improvement of long-term results when compared to the "wait and see" method.

The treatment of acute cerebrovascular disease plays a prominent role in clinical routine. However, prophylactic therapy of cerebrovascular disease including endovascular options is becoming more and more important. With particular regard to endovascular therapies, this summary deals with the natural course of cerebrovascular diseases and different treatment strategies for asymptomatic intracranial aneurysms, cerebral arteriovenous malformations, and extracranial carotid artery stenoses.

Vascular embolizations are complex and sophisticated procedures and can be a powerful alternative or useful adjunct to surgery in many clinical situations. Hemorrhage, vascular malformations, and tumors are the main indications for embolization procedures. Establishing the correct indication for intervention as well as the proper embolic agent and the most suitable catheterization technique requires advanced knowledge in interventional radiology, and an interdisciplinary approach is a prerequisite. A broad spectrum of microcatheters and embolization materials is available for these therapies. The desired level of occlusion and the individual vascular territory determine the choice of mechanical devices, particles, or liquid substances. Sclerosing agents and thrombin are used in special situations such as varicoceles and pseudoaneurysms.

The aim of this study was to determine the frequency of extracardial and particularly pulmonal right-to-left-shunts (RLS) in patients with acute cerebral ischemia of unknown origin.We investigated 52 patients under 60 years of age with cerebral ischemic stroke of unknown origin using transesophageal echocardiography (TEE) with special attention to the late phase of ultrasound contrast medium in the left atrium. Additionally we performed contrast transcranial doppler sonography (TCD).In the TEE, 25 patients (48%) showed cardial RLS. In seven patients with normal TEE (13.5%) we found evidence of RLS with contrast TCD (spontaneous RLS n=4, RLS after valsalva n=3). In none of these seven patients did we find late-phase contrast in the left atrium.The constellation of RLS detection in contrast TCD but not in TEE was frequently found. Particularly in spontaneous RLS, this constellation raises the suspicion of a pulmonary shunt. However our extended TEE protocol did not give additional evidence for a pulmonary location of the shunt.

The Abernethy malformation is a rare congenital portosystemic shunt in which the blood directly drains into the systemic vein bypassing the liver either through a complete (type 1) or a partial shunt (type 2). The diagnosis is most frequently established primarily with ultrasound. CT and MRI are used for further classification of the shunt and assessment of accompanying liver tumors and malformations. There is a wide spectrum of therapeutic options ranging from noninvasive conservative treatment to liver transplantation. The main prognostic factors are the occurrence of concomitant hepatic neoplasms and hepatic encephalopathy. We report two cases diagnosed with a type 1 shunt, hepatic encephalopathy, and associated liver tumors who underwent successful liver transplantation after having considered all therapeutic options.

The Klippel-Trenaunay-syndrom (KTS) is a congenital angiodysplasia of venous vessels characterized by three main symptoms: cutanous vascular naevi, hyperthropy of a limb and varicosis or venous malformations. The coincidence of KTS and arterial malformations such as renal artery aneurysm has been described twice in the literature. We report the case study of a 40-years-old male patient with KTS and aneurysms of a renal artery, the splenic artery, the superior mesenteric artery and of a popliteal artery and popliteal vein. After documenting the diagnostic and therapeutic course we describe this case as it relates to the clinical literature. In the differential diagnosis of KTS two different syndroms have to be pointed out: the Servelle-Martorell-syndrom and the Frederick Parkes Weber-syndrom.

We report on the case of a 31-year old female presenting a massive bleeding during extraction of left lower wisdom tooth, which could be managed by local compression. In the following transfemoral angiography an extensive arteriovenous malformation (avm) in the pterygomandibular space with osseous infiltration was diagnosed and embolised during the same session. After the acute stage the malformation could be controlled by repeated embolisation, the wound being closed by use of a transposition flap.Although arteriovenous malformations of the head and neck are rare, they can manifest with dramatic bleeding complications during surgical interventions. A thrill on palpation and auscultation may also refer to the existence of such a vascular lesion. An av-malformation may be stimulated by hormonal changes during puberty or pregnancy as well as by local trauma. Colour coded duplex sonography, magnetic resonance imaging and transfemoral angiography are suitable diagnostic tools. The treatment of choice is superselective embolisation followed by surgical resection of the vascular nidus, which should be performed by under an interdisciplinary approach.

Patients with arterio-venous fistula of the splenic hilum seek medical assistance because of abdominal discomfort and symptoms of portal hypertension. We report on a 43 year old female who was diagnosed with an arterio-venous aneurysm after suffering from dyspnoea, tachycardia and extrasystolies for several years. After uncomplicated distal splenopancreatectomy the patient was discharged free of symptoms.

The successful treatment of vascular anomalies depends on profound knowledge of the biologic behavior of vascular lesions and their correct classification. On the base of the clinical course Mulliken and Glowacki developed a biologic classification that was accepted as official classification by the ISSVA (International Society for the Study of Vascular Anomalies). Based on an extended literature research, this manuscript will give an overview of different internationally accepted treatment concepts. Even if a wait-and-see strategy can be recommended in many cases of uneventful hemangiomas in infants the proliferative growth of such lesions requires an adequate treatment indication. Vascular malformations that persist lifelong require treatment in the majority of the cases, especially when clinical symptoms occur. Based on individual parameters such as the diameter, location or growth behavior, different therapeutic options as cryotherapy, corticosteroids, laser therapy, sclerotherapy, surgical intervention and/or embolisation can be performed successfully. None of those treatment concepts, however, represents the only treatment method of choice.