Pulmonary thromboendarterectomy in the setting of a mediastinal venous malformation with a congenitally absent left subclavian vein.

Venous malformations have static venous lakes that predispose to spontaneous venous thrombosis within the malformation due to its low-flow static state. Thrombi of varying sizes can then embolize continually into the pulmonary arterial circulation, and occlude and narrow elastic pulmonary arteries causing chronic thromboembolic pulmonary hypertension (CTEPH). Pulmonary thromboendarterectomy (PTE) is potentially curative in CTEPH, but has not been previously reported in the setting of mediastinal and chest wall venous malformations. We report the case of a 21-year-old female with such a large malformation treated successfully with PTE. The patient underwent complete endovascular reconstruction of her subclavian vein system from the axillary vein to the innominate vein stump with covered stent grafts to exclude the malformations from causing recurrent pulmonary emboli. This was followed by embolization of the malformation to allow for the surgical approach. The series of events in this case serves as a novel approach in managing such rare patients.

Percutaneous embolization of arteriovenous malformations at the plantar aspect of the foot.

Peripheral arteriovenous malformations (AVM) remain most challenging among various congenital vascular malformations to be treated. Here we present three illustrative patients with Yakes type IIIb and type IV AVM at the plantar aspect of the foot who were successfully treated by minimally invasive embolization. The value of the Yakes AVM classification system to guide the therapeutic decision making by directing specific therapeutic procedures to specific AVM types defined by their angioarchitecture is demonstrated. Direct percutaneous AVM puncture with coiling of aneurysmal outflow vein and subsequent ethanol embolization is shown. Finally, the report illustrates that several AVM types can coexist.

Venous malformations: clinical diagnosis and treatment.

Venous malformation (VM) is the most common type of congenital vascular malformation (CVM). They are present at birth and are often symptomatic, causing morbidity and pain. VMs can be challenging to diagnose and are often confused with hemangioma in terminology as well as with imaging. An accurate clinical history and cross-sectional imaging are critical for diagnosis and for devising management. This manuscript will review imaging approaches to diagnosing VMs and current treatment strategies.

Ethanol embolization of arteriovenous malformations: interim results.

PURPOSE: To assess retrospectively the interim results and the complications of ethanol embolization treatment of arteriovenous malformations (AVMs). MATERIALS AND METHODS: Institutional review board approval was obtained for a retrospective review of patient medical and imaging records. Informed consent was not required by the institutional review board. Written consent for the procedure was obtained from all patients after a discussion about the advantages and risks of the procedure. After a general anesthetic was administered, 40 patients (16 male, 24 female; age range, 9-53 years) with inoperable AVMs in the body and extremities underwent staged ethanol embolizations (range, 1-24; median, 3). Pulmonary artery pressure and arterial blood pressure were monitored as ethanol was injected. Ethanol embolizations (50%-100% ethanol mixed with nonionic contrast material) were performed by using transcatheter and/or direct puncture techniques. Ten patients underwent additional coil deployment during ethanol embolization. Clinical follow-up (range, 2-48 months; mean, 14.6 months; median, 12 months) was performed in all patients, and results from imaging follow-up (range, 0-48 months; mean, 8.4 months; median, 6 months) were available from the last treatment session in 28 patients. Therapeutic outcomes were established by evaluating the clinical outcome of symptoms and signs, as well as the degree of devascularization at follow-up angiography. RESULTS: One hundred seventy-five ethanol embolizations were performed in 40 patients. Sixteen (40%) of 40 patients were cured, 11 (28%) had partial remission, seven (18%) had no remission, and one (2%) experienced aggravation. Treatment failed in five patients (12%). Ethanol embolization was considered effective (cure, 16 patients; partial remission, 11 patients) in 27 patients (68%). Eleven patients will need further treatment sessions for residual AVMs. Twenty-one patients (52%) experienced complications. Twenty-seven minor complications (skin and transient peripheral nerve injuries) (27 [15%] of 175 procedures) occurred in 18 (45%) of 40 patients. All minor complications were healed with wound dressing and observation. Five major complications (five [3%] of 175 procedures) occurred in five (12%) of 40 patients, and four patients recovered completely. CONCLUSION: Ethanol embolization has the potential for cure in the management of AVMs of the body and extremities but with acceptable risk of minor and major complications.

Management of arteriovenous malformations: a multidisciplinary approach.

BACKGROUND: Management of arteriovenous malformations (AVMs) remains challenging because of their unpredictable behavior and high recurrence rate. A multidisciplinary approach based on a new classification scheme and improved diagnostic techniques may improve their management. The purpose of this study was to review our experience with combined embolotherapy, sclerotherapy (embolo/sclerotherapy), and surgical procedures to manage AVMs. METHODS: A total of 797 patients with congenital vascular malformations (January 1995 through December 2001) was investigated with noninvasive studies. Once an AVM was diagnosed, all underwent angiographic confirmation as a roadmap for treatment. Embolo/sclerotherapy and surgical procedures were instituted by the multidisciplinary team with periodic follow-up per protocol. Seventy-six patients with AVMs were reviewed retrospectively to assess the diagnosis and management by a multidisciplinary approach. RESULTS: Seventy-six (9.5% of all CVM) patients had AVMs, mostly infiltrating, extratruncular form (61/76). Embolo/sclerotherapy with various combinations of absolute ethanol, N-butyl cyanoacrylate (NBCA), contour particles, and coils were used in 48 patients. Sixteen patients with surgically accessible localized lesions completed preoperative embolism and sclerotherapy through 24 sessions, with subsequent surgical excision with minimal morbidity. Interim results were excellent, with no evidence of recurrence in all 16 patients with a mean follow-up of 24 months. Thirty-two patients with surgically inaccessible lesions (infiltrating) were treated with embolism and sclerotherapy alone. There were nine failures in a total of 171 sessions. Interim results with a mean of 19 months' follow-up of embolism and sclerotherapy alone were excellent in the majority (25/32) and good to fair among the rest (7/32). However, 31 complications, mostly minor (27/31), occurred in 30 sessions. Four major complications occurred, including facial nerve palsy, pulmonary embolism, deep vein thrombosis, and massive necrosis of an ear cartilage. CONCLUSIONS: Diagnosis and management of AVMs by a multidisciplinary approach that integrates surgical therapy with embolism and sclerotherapy appears to improve the results and management with limited morbidity and no recurrence during early follow-up.

Endovascular management of high-flow arteriovenous malformations.

This article describes the various methods of endovascular management of high-flow arteriovenous malformations. Concepts of patient management and various therapies for vascular malformations are discussed. Nontraumatic acquired arteriovenous malformations, arteriovenous fistulae, and endovascular treatment of arteriovenous malfromations are described.