Jeffrey Pollak

Liver Disease in Patients with Hereditary Hemorrhagic Telangiectasia

BACKGROUND Hereditary hemorrhagic telangiectasia, or Rendu-Osler-Weber disease, is an autosomal dominant disorder characterized by angiodysplastic lesions (telangiectases and arteriovenous malformations) that affect many organs. Liver involvement in patients with this disease has not been fully characterized. METHODS We studied the clinical findings and results of hemodynamic, angiographic, and imaging studies in 19 patients with hereditary hemorrhagic telangiectasia and symptomatic liver involvement. RESULTS We evaluated 14 women and 5 men who ranged in age from 34 to 74 years. All but one of the patients had a hyperdynamic circulation (cardiac index, 4.2 to 7.3 liters per minute per square meter of body-surface area). In eight patients, the clinical findings were consistent with the presence of high-output heart failure. The cardiac index and pulmonary-capillary wedge pressure were elevated in the six patients in whom these measurements were performed. After a median period of 24 months, the condition of three of the eight patients had improved, four were in stable condition with medical therapy, and one had died. Six patients had manifestations of portal hypertension such as ascites or variceal bleeding. The hepatic sinusoidal pressure was elevated in the four patients in whom it was measured. After a median period of 19 months, the condition of two of the six patients had improved, and the other four had died. Five patients had manifestations of biliary disease, such as an elevated alkaline phosphatase level and abnormalities on bile duct imaging. After a median period of 30 months, the condition of two of the five had improved, the condition of one was unchanged, heart failure had developed in one, and one had died after an unsuccessful attempt at liver transplantation. CONCLUSIONS In patients with hereditary hemorrhagic telangiectasia and symptomatic liver-involvement, the typical clinical presentations include high-output heart failure, portal hypertension, and biliary disease.

Pulmonary arteriovenous malformations: diagnosis and transcatheter embolotherapy.

The recent long-term studies from England, France, and the Netherlands, as well as our own, indicate that transcatheter embolotherapy is definitive treatment for PAVM. More recently, Puskas et al have questioned transcatheter embolotherapy as a primary treatment for patients with PAVM (4,56). Their opinion was based on two recurrences among five patients treated with transcatheter embolotherapy. It is not clear why one of the late recurrences in the series by Puskas et al happened, and the other recurrence could have been dut to early deflation of the balloon. Nevertheless, we believe that the collective experience in the larger series reporting on transcatheter embolotherapy of PAVM supports the use of embolotherapy as a primary modality of treatment. Because many patients have bilateral pulmonary malformations and many pulmonary malformations will grow with time, repeated surgical intervention is not ideal therapy. The recurrence rate of 8% reported by Remy et al using coils, and 2% reported by Pollak et al using balloons and coils supports our contention that transcatheter embolotherapy is durable and should be the initial treatment. Also, recurrences are easily retreated by transcatheter embolotherapy with durable results (54). We favor detachable balloons over coils for occluding PAVMs because immediate cross-sectional occlusion of the segmental artery is obtained in a position that preserves the most normal branches. The necessity for repeated introduction of coils, when using the coil method, contributes to longer procedure times with an increased risk of air introduction and, in our experience, a greater risk of postprocedure pleurisy. At the same time, we appreciate that approximately 70% of PAVMs can be occluded equally well with balloons or coils. We also believe that coils have unique advantages over balloons in specific anatomic situations including oversized arteries (where coils are the only option) and for occlusion of the aneurysm of a PAVM. As with all forms of embolotherapy, the interventionalist is best served by having more than one option of treatment, which for PAVM includes both balloons and coils. In summary, PAVMs are effectively managed by means of transcatheter embolotherapy. This therapy has been demonstrated to be safe and durable. Careful technique with modifications depending on the angioarchitecture of the PAVM is required. Patients with PAVMs require follow-up at 1 month and 1 year. While observations documenting serial growth of small PAVMs are somewhat limited, there is published evidence to support their growth with time (35,36). Because of these reports and our unpublished observations, we believe that patients with treated PAVM need long-term follow-up every 5 years to detect growth of small PAVMs that will ultimately reach a size where they may cause paradoxical embolization and stroke (1).

Embolotherapy of large pulmonary arteriovenous malformations: long-term results.

BACKGROUND The purpose of this study was to document the long-term results of transcatheter embolotherapy of large pulmonary arteriovenous malformations (PAVMs). METHODS From a data base of 221 consecutive patients with PAVMs treated by embolotherapy between 1978 and 1995, 45 patients with 52 PAVMs, supplied by feeding arteries 8 mm in diameter or larger, were selected for a retrospective investigation. RESULTS Of 45 patients with 52 large PAVMs, 38 patients (84%) with 44 PAVMs (85%) were cured by the first embolotherapy (mean follow-up, 4.7 years). Acute periprocedural complications included self-limited pleurisy (31%), angina secondary to air embolus (2%), and paradoxical embolization of a device during deployment (4%). None of these events led to short- or long-term sequelae. Seven patients (16%) had persistence of the PAVM attributable to either recanalization (n = 4) or interim accessory artery growth (n = 3). Two of these patients presented with ischemic stroke several years after the initial treatment. Persistent PAVMs (n = 8) were retreated successfully by a second procedure (n = 7), or a third procedure (n = 1) (mean follow-up, 5.9 and 5.3 years, respectively). CONCLUSIONS Embolotherapy of large PAVMs results in permanent occlusion in an overwhelming majority of patients. Continued patency due to recanalization or accessory artery growth is easily detected and treated.

Pulmonary arteriovenous malformations Cerebral ischemia and neurologic manifestations

Background: There is an increasingly recognized association between pulmonary arteriovenous malformations (PAVM) and cerebral ischemia, frequently attributed to paradoxical embolization. PAVM occur in 20 to 30% of the hereditary hemorrhagic telangiectasia (HHT) population. Objective: To evaluate the risk determinants for cerebral ischemia and neurologic manifestations in patients with PAVM. Methods: A retrospective cross-sectional study was performed on consecutive patients admitted between 1988 and 1992 for treatment of PAVM. The number of PAVM, feeding artery (FA) diameters, and aneurysmal sizes were determined by pulmonary angiography. Patients were categorized as having single or multiple PAVM with an FA diameter of ≥3 mm. History, examination, and cerebral imaging studies were used to determine the prevalence of neurologic manifestations. Patients were defined as having cerebral paradoxical embolization if there was radiologic evidence of cortical infarction. Results: There were 75 cases: 26 single PAVM and 49 multiple PAVM. Cortical infarction was present in 14% of patients with single PAVM. Patients with multiple PAVM had a greater prevalence of any infarction (OR 3.2; 95% CI, 1.2 to 9.44, p = 0.030), cortical infarctions (OR 2.3; 95% CI, 0.58 to 9.2, p = 0.230), subcortical infarctions (OR 2.1; 95% CI, 0.58 to 7.95, p = 0.249), abscesses (OR 2.3; 95% CI, 0.46 to 11.94; p = 0.295), and seizures (OR 6.4, 95% CI 0.77 to 53.2, p = 0.054). Patients with multiple PAVM had markedly greater odds of having any clinical or radiologic evidence of cerebral ischemic involvement (OR 4.5; 95% CI, 1.47 to 14; p = 0.008). Conclusion: There is a strong association between single PAVM and various neurologic manifestations. The prevalence is greater for patients with multiple PAVM, suggesting increased predisposition for paradoxical embolization with a greater number of malformations.

Clinical and anatomic outcomes after embolotherapy of pulmonary arteriovenous malformations

PURPOSE To assess long-term clinical and imaging results of technically successful pulmonary arteriovenous malformation (AVM) embolization. MATERIALS AND METHODS One hundred fifty-five patients with pulmonary AVMs underwent embolization during a period of 3 years. Recommended follow-up included clinical assessment, helical computed tomography, and physiologic evaluation within 1 year and then every 5 years. RESULTS Hereditary hemorrhagic telangiectasia was present in 148 patients (95%). Four hundred fifteen pulmonary AVMs were occluded during 205 procedures. Clinical follow-up was available in all patients over 3-7 years and imaging follow-up was available in 144 patients (393 lesions) over 1-7 years (mean, 2.9 y). Problems related to pulmonary AVMs occurred in 35 patients (23%) at 42 time points: 22 patients with 23 symptomatic events and 17 patients with 19 asymptomatic events. Symptoms resulted from growth of nonembolized pulmonary AVMs (n = 19), residual embolized pulmonary AVMs (n = 5), or both (n = 2). Symptoms consisted of respiratory manifestations (n = 13), cerebral ischemia (n = 4), brain abscess (n = 5), hemoptysis (n = 3), and seizure (n = 1). Imaging showed pulmonary AVM involution in 97% of embolized lesions and 11 residual lesions (2.8%) in 10 patients (6.9%). These were caused by recanalization (n = 7), presence of an accessory feeding artery (n = 1), pulmonary collateral vessels (n = 1), and bronchial collateral vessels (n = 2). CT detected 10 of the 11 residual lesions. Imaging detected 97 previously small pulmonary AVMs that had enlarged to a significant size in 28 patients (18%), 15 of whom were symptomatic and 13 of whom were asymptomatic. CONCLUSIONS Clinical and anatomic evaluation after pulmonary AVM embolization is important to detect persistent or reperfused lesions and enlarging lesions, with the latter more common. Patients with persistent, reperfused, or enlarging lesions often have symptoms, but a significant minority of patients are asymptomatic. More frequent assessment may improve detection before the onset of symptoms.

The Use of Cyanoacrylate Adhesives in Peripheral Embolization

Although liquid adhesives or glue have been used as embolic agents for nearly three decades, experience with them outside of neurointerventional indications is generally limited. Cyanoacrylates are the main liquid adhesives used in the vascular system and have an important role in managing vascular abnormalities, especially arteriovenous malformations. Vascular occlusion results as these agents polymerize on exposure to the ions in blood. A description of the properties, biologic interactions, techniques of use, and indications for acrylic embolization in the peripheral circulation is especially pertinent at this time because of the recent approval of n-butyl cyanoacrylate by the United States Food and Drug Administration.

Pelvic embolization for intractable postpartum hemorrhage: long-term follow-up and implications for fertility

OBJECTIVE To determine the long-term sequelae of pelvic embolization for postpartum hemorrhage and to study the effect on fertility and menses. METHODS Twenty-eight consecutive patients who underwent pelvic embolization for postpartum hemorrhage between the years 1977 and 2002 were included in the study. Chart review and telephone interviews were conducted to gather data regarding the type of delivery, causative factors of the bleeding, preembolization treatments, total blood loss, length of time between delivery and embolization, complications, long-term side effects, and subsequent pregnancies. RESULTS The average (± standard deviation) time to follow-up was 11.7 ± 6.9 years. The most common causes of hemorrhage were vaginal/cervical laceration, placenta accreta, and placenta previa. In only one case was the embolization unsuccessful, during which there was an accidental perforation of an internal iliac artery resulting in a retroperitoneal hematoma and subsequent total abdominal hysterectomy. All of the interviewed patients that desired to get pregnant after embolization were able to do so. Six patients reported a total of six uncomplicated pregnancies and deliveries in the years after their embolization. Of the remaining patients interviewed, none made subsequent attempts to get pregnant. The most commonly reported long-term side effects were transient buttock numbness (n = 2) and urinary frequency (n = 2). In no patients were the side effects severe enough to seek further medical attention. CONCLUSION Pelvic arterial embolization is a safe and effective procedure and offers patients a fertility-preserving alternative to hysterectomy for treatment of intractable postpartum hemorrhage.

MRI characteristics and classification of peripheral vascular malformations and tumors

Vascular malformations and tumors comprise a broad spectrum of lesions that can cause significant morbidity and even mortality in children and adults. Classification of vascular malformations into high flow and low flow has significant impact on management since the main treatment of the former is transarterial embolization and the later percutaneous sclerotherapy. Magnetic resonance imaging (MRI) is a noninvasive effective tool for imaging and classification of vascular malformations based on the presence of lobulated masses, signal voids, and hemodynamic flow characteristics. MRI also provides details about anatomic extent of the lesion, proximity to vital structures, and involvement of multiple tissue planes. The prototype of vascular tumors is infantile hemangioma with its typical involution after a proliferative phase during infancy. Hemangioma appears as a distinct intensely enhancing soft tissue mass with enlarged feeding arteries and draining veins. Less common vascular tumors include congenital hemangioma, kaposiform hemangioendothilioma, angiolipoma, angiosarcoma, and hemangiopericytoma.

Initial experience with venous stents in exertional axillary-subclavian vein thrombosis ☆ ☆☆ ★

PURPOSE Exertional thrombosis of the axillary and subclavian veins, also known as Paget-Schrötter syndrome, has been increasingly recognized in recent years as a cause of long-term morbidity. Recent aggressive approaches to treating Paget-Schrötter syndrome have suggested the association of early failure with residual subclavian vein stenosis. As a result, the use of endoluminal stents has been proposed as an aid to venous percutaneous transluminal angioplasty for this disorder. METHODS This report outlines the therapy of 11 consecutive patients with Paget-Schrötter syndrome who were treated at our institution between October, 1992, and December, 1995. Stents were placed when percutaneous transluminal angioplasty was unsuccessful at achieving an adequate residual lumen. RESULTS Stents were placed after initial thrombolysis in six patients and in late follow-up in two patients. Of the six patients who had stents placed at initial thrombolysis, first-rib resection was eventually performed in four. In two patients first-rib resection was not performed, and stent fracture occurred in both. Late patency was achieved in the stents of six of the eight patients. CONCLUSIONS Trials to evaluate stents as an adjunct to conventional therapy seem warranted. The use of stents alone without first-rib resection, however, appears to be associated with stent fracture.

Long-Term Outcome of Embolotherapy and Surgery for High-Flow Extremity Arteriovenous Malformations

PURPOSE To assess the long-term efficacy of embolotherapy in combination with surgery for management of symptomatic high-flow arteriovenous malformations (HFAVMs) of the lower and upper extremities. MATERIALS AND METHODS Twenty consecutive patients with symptomatic high-flow lower extremity AVMs (LE-AVMs; n = 9) and upper extremity AVMs (UE-AVMs; n = 11) were treated from 1982 to 1999. All nine patients with LE-AVM had pain and seven had ulceration of the skin. All 11 patients with UE-AVM had debilitating pain, seven had weakness of the affected hand, and two had bony erosion. Embolization of the nidus beneath the site of maximum pain or ulceration was performed percutaneously from the femoral artery through coaxially placed microcatheters (n = 18) or surgical cutdown (n = 2). Cyanoacrylate (isobutyl or n-butyl) diluted with iophendylate or ethiodized oil was used in 19 of 20 patients. RESULTS Follow-up was completed in eight of nine patients with LE-AVM (mean, 8.6 y) and nine of 11 patients with UE-AVM (mean, 7.4 y) after treatment. One patient with localized LE-AVM was functioning well 13 years after embolotherapy and another was functioning well 16 years after undergoing three embolotherapy procedures and two skin grafts. Five of nine patients with LE-AVM required below-the-knee (n = 4) or above-the-knee (n = 1) amputation 1-6 years after technically and clinically successful embolotherapy. All three trifurcation arteries were diffusely involved in HFAVM in patients requiring amputation. Healing of the two amputation sites, involved by AVM at the knee, was excellent after preoperative geniculate artery embolotherapy. All 11 patients with UE-AVM experienced marked symptomatic improvement; seven after embolotherapy alone and the other four after resection of AVM. One complication of digital spasm was reversed by administration of nerve blocks. CONCLUSIONS LE-AVM with diffuse involvement of all three trifurcation arteries ultimately required amputation because of recurrence of symptoms after technically and clinically successful embolotherapy. Cyanoacrylate embolotherapy alone or in combination with surgical resection of the AVM provided excellent long-term palliation in patients with UE-AVM.