Diagnosis and Treatment of Venous Malformations. Consensus Document of the International Union of Phlebology (IUP): updated 2013.

Venous malformations (VMs) are the most common vascular developmental anomalies (birth defects) . These defects are caused by developmental arrest of the venous system during various stages of embryogenesis. VMs remain a difficult diagnostic and therapeutic challenge due to the wide range of clinical presentations, unpredictable clinical course, erratic response to the treatment with high recurrence/ persistence rates, high morbidity following non-specific conventional treatment, and confusing terminology. The Consensus Panel reviewed the recent scientific literature up to the year 2013 to update a previous IUP Consensus (2009) on the same subject. ISSVA Classification with special merits for the differentiation between the congenital vascular malformation (CVM) and vascular tumors was reinforced with an additional review on syndrome-based classification. A "modified" Hamburg classification was adopted to emphasize the importance of extratruncular vs. truncular sub-types of VMs. This incorporated the embryological ongm, morphological differences, unique characteristics, prognosis and recurrence rates of VMs based on this embryological classification. The definition and classification of VMs were strengthened with the addition of angiographic data that determines the hemodynamic characteristics, the anatomical pattern of draining veins and hence the risk of complication following sclerotherapy. The hemolymphatic malformations, a combined condition incorporating LMs and other CVMs, were illustrated as a separate topic to differentiate from isolated VMs and to rectify the existing confusion with name-based eponyms such as Klippei-Trenaunay syndrome. Contemporary concepts on VMs were updated with new data including genetic findings linked to the etiology of CVMs and chronic cerebrospinal venous insufficiency. Besides, newly established information on coagulopathy including the role of D-Dimer was thoroughly reviewed to provide guidelines on investigations and anticoagulation therapy in the management of VMs. Congenital vascular bone syndrome resulting in angio-osteo-hyper/hypotrophy and (lateral) marginal vein was separately reviewed. Background data on arterio-venous malformations was included to differentiate this anomaly from syndromebased VMs. For the treatment, a new section on laser therapy and also a practical guideline for follow up assessment were added to strengthen the management principle of the multidisciplinary approach. All other therapeutic modalities were thoroughly updated to accommodate a changing concept through the years.

Guideline. Diagnosis and treatment of venous malformations. consensus document of the international union of phlebology (iup): updated-2013.

Venous malformations (VMs) are the most common vascular developmental anomalies (birth defects). These defects are caused by developmental arrest of the venous system during various stages of embryogenesis. VMs remain a difficult diagnostic and therapeutic challenge due to the wide range of clinical presentations, unpredictable clinical course, erratic response to the treatment with high recurrence/persistence rates, high morbidity following nonspecific conventional treatment, and confusing terminology. The Consensus Panel reviewed the recent scientific literature up to the year 2013 to update a previous IUP Consensus (2009) on the same subject. ISSVA Classification with special merits for the differentiation between the congenital vascular malformation (CVM) and vascular tumors was reinforced with an additional review on syndrome-based classification. A "modified" Hamburg classification was adopted to emphasize the importance of extratruncular vs. truncular subtypes of VMs. This incorporated the embryological origin, morphological differences, unique characteristics, prognosis and recurrence rates of VMs based on this embryological classification. The definition and classification of VMs were strengthened with the addition of angiographic data that determines the hemodynamic characteristics, the anatomical pattern of draining veins and hence the risk of complication following sclerotherapy. The hemolymphatic malformations, a combined condition incorporating LMs and other CVMs, were illustratedas a separate topic to differentiate from isolated VMs and to rectify the existing confusion with namebased eponyms such as Klippel-Trenaunay syndrome. Contemporary concepts on VMs were updated with new data including genetic findings linked to the etiology of CVMs and chronic cerebrospinal venous insufficiency. Besides, newly established information on coagulopathy including the role of D-Dimer was thoroughly reviewed to provide guidelines on investigations and anticoagulation therapy in the management of VMs. Congenital vascular bone syndrome resulting in angio-osteo-hyper/hypotrophy and (lateral) marginal vein was separately reviewed. Background data on arterio-venous malformations was included to differentiate this anomaly from syndrome-based VMs. For the treatment, a new section on laser therapy and also a practical guideline for follow up assessment were added to strengthen the management principle of the multidisciplinary approach. All other therapeutic modalities were thoroughly updated to accommodate a changing concept through the years.

Consensus Document of the International Union of Angiology (IUA)-2013. Current concept on the management of arterio-venous management.

Arterio-venous malformations (AVMs) are congenital vascular malformations (CVMs) that result from birth defects involving the vessels of both arterial and venous origins, resulting in direct communications between the different size vessels or a meshwork of primitive reticular networks of dysplastic minute vessels which have failed to mature to become 'capillary' vessels termed "nidus". These lesions are defined by shunting of high velocity, low resistance flow from the arterial vasculature into the venous system in a variety of fistulous conditions. A systematic classification system developed by various groups of experts (Hamburg classification, ISSVA classification, Schobinger classification, angiographic classification of AVMs,) has resulted in a better understanding of the biology and natural history of these lesions and improved management of CVMs and AVMs. The Hamburg classification, based on the embryological differentiation between extratruncular and truncular type of lesions, allows the determination of the potential of progression and recurrence of these lesions. The majority of all AVMs are extra-truncular lesions with persistent proliferative potential, whereas truncular AVM lesions are exceedingly rare. Regardless of the type, AV shunting may ultimately result in significant anatomical, pathophysiological and hemodynamic consequences. Therefore, despite their relative rarity (10-20% of all CVMs), AVMs remain the most challenging and potentially limb or life-threatening form of vascular anomalies. The initial diagnosis and assessment may be facilitated by non- to minimally invasive investigations such as duplex ultrasound, magnetic resonance imaging (MRI), MR angiography (MRA), computerized tomography (CT) and CT angiography (CTA). Arteriography remains the diagnostic gold standard, and is required for planning subsequent treatment. A multidisciplinary team approach should be utilized to integrate surgical and non-surgical interventions for optimum care. Currently available treatments are associated with significant risk of complications and morbidity. However, an early aggressive approach to elimiate the nidus (if present) may be undertaken if the benefits exceed the risks. Trans-arterial coil embolization or ligation of feeding arteries where the nidus is left intact, are incorrect approaches and may result in proliferation of the lesion. Furthermore, such procedures would prevent future endovascular access to the lesions via the arterial route. Surgically inaccessible, infiltrating, extra-truncular AVMs can be treated with endovascular therapy as an independent modality. Among various embolo-sclerotherapy agents, ethanol sclerotherapy produces the best long term outcomes with minimum recurrence. However, this procedure requires extensive training and sufficient experience to minimize complications and associated morbidity. For the surgically accessible lesions, surgical resection may be the treatment of choice with a chance of optimal control. Preoperative sclerotherapy or embolization may supplement the subsequent surgical excision by reducing the morbidity (e.g. operative bleeding) and defining the lesion borders. Such a combined approach may provide an excellent potential for a curative result. Conclusion. AVMs are high flow congenital vascular malformations that may occur in any part of the body. The clinical presentation depends on the extent and size of the lesion and can range from an asymptomatic birthmark to congestive heart failure. Detailed investigations including duplex ultrasound, MRI/MRA and CT/CTA are required to develop an appropriate treatment plan. Appropriate management is best achieved via a multi-disciplinary approach and interventions should be undertaken by appropriately trained physicians.

Remifentanil conscious sedation during regional anaesthesia for carotid endarterectomy: rationale and safety.

OBJECTIVES: to prospectively evaluate the safety and efficacy of remifentanil during regional anaesthesia for carotid endarterectomy. METHODS: twenty-eight consecutive patients underwent carotid endarterectomy with combined superficial and deep cervical plexus block supplemented with continuous intravenous 0.04 microg.kg(-1).min(-1)remifentanil infusion. Depth of sedation was monitored using the Observer's Assessment of Alertness/Sedation Scale (OAA/S). The degree of pain, discomfort and anxiety was self-assessed by the patients using a horizontal visual analogue scale. RESULTS: all patients experienced adequate comfort and analgesia. No local anaesthetic supplementation was necessary. No patient had a OAA/S score lower than 4 (with 5=awake/alert to 1=asleep). Respiratory depression did not occur. Selective shunting was required in four cases. No patient was converted to general anaesthesia. There were no permanent neurological deficits, cardiopulmonary complications or deaths. CONCLUSION: remifentanil as a supplement to regional anaesthesia for carotid endarterectomy, provides comfort and analgesia without hampering mental status evaluation.

Management of congenital venous malformations of the vulva.

OBJECTIVE: To discuss the differential diagnosis and the management of venous malformations of the vulva. METHODS: Five symptomatic patients were treated. The degree of pain and discomfort was self-assessed by using a horizontal visual analog scale before and after treatment. Preoperative evaluation included Doppler ultrasound scanning in all patients and magnetic resonance imaging (MRI) in one. All patients had direct-injection venography and sclerotherapy during the same session. Ethanol was used in two cases and polidocanol in three. Patients were followed-up by means of Doppler ultrasound scanning and office visits. RESULTS: All patients experienced marked swelling after the injection, and one developed cutaneous necrosis that healed within 2 weeks. Transient hemoglobinuria was observed in two cases. No early or late major complications occurred. At a mean follow-up of 23 months (range 5-43), all patients experienced complete relief from symptoms and currently have normal vulvar sensation. Four patients had complete ablation of the treated lesion. In one patient the procedure resulted in a significant, albeit incomplete, occlusion of the lesion, and no further treatment was deemed necessary. From a cosmetic standpoint, both patients and physicians considered the results successful. CONCLUSION: Vulvar venous malformations should be distinguished from vulvar varicosities, hematomas, soft-tissue neoplasms, and other vascular anomalies. Doppler ultrasound, MRI, and direct-injection venography are the most accurate diagnostic modalities. Sclerotherapy can successfully treat this condition. The procedure should be monitored with an imaging modality, preferably direct-injection venography with digital subtraction serial imaging.

Management of synchronous abdominal aortic aneurysm and renal carcinoma associated with duplication of the inferior vena cava.

The management of concomitant abdominal aortic aneurysm and intra-abdominal malignancies is still disputed; whether to treat the lesions simultaneously or as staged procedures being the main controversy. Abdominal aortic aneurysm associated with renal carcinoma is rare and combined aneurysm repair and nephrectomy appears to be the treatment of choice in selected patients. We report a case where the surgical management of simultaneously occurring abdominal aortic aneurysm and renal carcinoma was complicated by the presence of a duplicated inferior vena cava. The rationale for the treatment of this patient and the technical difficulties of the surgical procedure are discussed and a review of the literature to date is reported.

Sclerotherapy for venous malformations of the glans penis.

OBJECTIVES: To retrospectively evaluate the safety and efficacy of percutaneous sclerotherapy with polidocanol in 9 patients with venous malformations of the glans penis. Vascular malformations of the external genitalia can be aesthetically and functionally disabling. These lesions are rare and their treatment is still controversial. METHODS: Seven patients were asymptomatic, seeking advice for cosmetic reasons, and 2 patients had experienced several bleeding episodes during sexual intercourse. In all patients, Doppler ultrasound scanning was performed before treatment. All patients underwent direct intralesional injection of polidocanol. The concentration of the sclerosant varied from 1% to 3%, and the volume of the solution varied from 2 to 4 mL per injection. Four patients required multiple sclerotherapeutic sessions. RESULTS: All patients experienced marked swelling after the injection and three developed cutaneous blistering. Neither cutaneous necrosis nor early or late major complications occurred. At a mean follow-up of 35.1 months, 7 patients were cured and 2 had marked improvement. No patients presented with signs of recurrence, and the outcome was overall cosmetically successful. CONCLUSIONS: Sclerotherapy with polidocanol is a well-tolerated, safe, and effective treatment for venous malformations of the penis. In our opinion, it can be considered the treatment of choice.

Local delivery of human tissue kallikrein gene accelerates spontaneous angiogenesis in mouse model of hindlimb ischemia.

BACKGROUND: Human tissue kallikrein (HK) releases kinins from kininogen. We investigated whether adenovirus-mediated HK gene delivery is angiogenic in the context of ischemia. METHODS AND RESULTS: Hindlimb ischemia, caused by femoral artery excision, increased muscular capillary density (P:<0.001) and induced the expression of kinin B(1) receptor gene (P:<0.05). Pharmacological blockade of B(1) receptors blunted ischemia-induced angiogenesis (P:<0.01), whereas kinin B(2) receptor antagonism was ineffective. Intramuscular delivery of adenovirus containing the HK gene (Ad. CMV-cHK) enhanced the increase in capillary density caused by ischemia (969+/-32 versus 541+/-18 capillaries/mm(2) for control, P:<0.001), accelerated blood flow recovery (P:<0.01), and preserved energetic charge of ischemic muscle (P:<0.01). Chronic blockade of kinin B(1) or B(2) receptors prevented HK-induced angiogenesis. CONCLUSIONS: HK gene delivery enhances the native angiogenic response to ischemia. Angiogenesis gene therapy with HK might be applicable to peripheral occlusive vascular disease.

[The predictiveness of the duplex scan method in monitoring a venous femoro-distal bypass]. / Predittività della metodica duplex scan nel monitoraggio del bypass femoro-distale di vena.

The medium-term failure of femoro-distal revascularization using venous bypass is often preceded by the onset of intrinsic intraprosthetic lesions which, if not diagnosed, may develop and cause thrombosis in the venous prosthesis. The authors report their experience relating to the monitoring of these bypass operations using an echo-Doppler test which was found to be a sensitive and reliable method. In their opinion, frequent instrumental control may significantly reduce the number of occlusions in the venous prosthesis if a rapid surgical therapeutic approach is adopted towards those intraluminal lesions considered to represent a risk.

[Thromboendarterectomy and angioplasty of the carotid bifurcation. Technical notes]. / Tromboendoarteriectomia ed angioplastica della biforcazione carotidea. Note di tecnica.

Exceptional surgical reports of widespread atherosclerotic involvement of both internal and external carotid arteries required us to carry out an unusual surgical procedure. During a four years period an original carotid endarterectomy's technique was performed in four patients. We point on the importance of a correct external carotid endarterectomy on maintaining an effective intracranial arterial flow, especially when atherosclerotic lesions are contemporary present in the extra and intracranial internal carotid arteries.

[Right atrial angiosarcoma. report of a case treated successfully by surgery (author's transl)]. / Angiosarcoma dell'atrio destro. Aspetti clinici, ecocardiografici ed istologici in un caso operato con successo.

A 32 years old male presented with symptoms and signs of congestive heart failure. The echocardiographic and angiographic examinations demonstrated a large right atrial tumor prolapsing in the right ventricle through the tricuspid orifice. The patient underwent successful surgical resection of the tumor. Histologically in the lesion was diagnosed as angiosarcoma.

[Mitral insufficiency associated with interatrial defect (ostium secundum). Problems of diagnosis and surgical treatment]. / L'insufficienza mitralica associata a difetto interatriale (ostium secundum). Problemi di diagnosi e di terpaia chirurgica.

Of the 255 patients under observation of the AA. with ostium secundum interatrial defect who underwent surgery, 23 (9%) presented an associated mitral regurgitation. All patients had a hemodynamic test and a complete contrahistography, 8 patients polygraphic and 5 patients echocardiographic examinations. The clinical data alone, ecgraphic and Rx, supplied sufficient indications for a correct diagnosis in 87% of the cases. 22 patients underwent surgery: the septal defect was corrected in all patients, mitral regurgitation only in 9; in 5 by valve substitution, in 4 through conservative techniques. Only one patient died of cerebral coma. A report is made on the data relative to the clinical history, Rx, ecgraphic and hemodynamic examinations of the 23 patients under observation, the various anatomosurgical aspects of the valvular alterations, and the criteria adopted for the surgical correction of the mitral regurgitation.