How to Treat Peripheral Arteriovenous Malformations

Arteriovenous malformations (AVMs) are direct communications between primitive reticular networks of dysplastic vessels that have failed to mature into capillary vessels. Based on angiographic findings, peripheral AVMs can be classified into six types: type I, type IIa, type IIb, type IIc, type IIIa, and type IIIb. Treatment strategies vary with the types. Type I is treated by embolizing the fistula between the artery and the vein with coils. Type II (IIa, IIb, and IIc) AVM is treated as follows:first, reduce the blood flow velocity in the venous segment of the AVM with coils; second, perform ethanol embolotherapy of the residual shunts. Type IIIa is treated by transarterial catheterization of the feeding arteries and injection of diluted ethanol. Type IIIb is treated by transarterial or direct puncture approaches. A high concentration of ethanol is injected through the transarterial catheter or direct puncture needle. When the fistula is large, coil insertion is required to reduce the amount of ethanol. Type I and type II AVMs showed the best clinical results; type IIIb showed a satisfactory response rate. However, type IIIa showed the poorest response rate, either alone or in combination with other types. Clinical success can be achieved by using different treatment strategies for different angiographic AVM types.

Transvaginal Direct Puncture and Ethanol Sclerotherapy for Cervicovaginal Venous Malformations: A Case Report and Literature Review

Cervicovaginal venous malformations are extremely rare. Sclerotherapy is proven to be effective for superficial venous malformations but not for venous malformations in the lower genital tract of female. A 52-year-old female presented with intermittent vaginal bleeding. The amount of vaginal bleeding gradually increased over 3 months. Contrast-enhanced pelvis CT showed several phleboliths and dilated vessels, but pelvic angiography showed no early draining veins, nidus, or feeding artery. We performed transvaginal direct puncture and ethanol sclerotherapy rather than surgical treatment because she wanted to preserve the uterus. After four sessions of sclerotherapy, she had significantly decreased vaginal bleeding without complications. Here, we report the first case of cervicovaginal venous malformations successfully treated with transvaginal direct puncture and ethanol sclerotherapy.

How to Treat Peripheral Arteriovenous Malformations

Arteriovenous malformations (AVMs) are direct communications between primitive reticular networks of dysplastic vessels that have failed to mature into capillary vessels. Based on angiographic findings, peripheral AVMs can be classified into six types: type I, type IIa, type IIb, type IIc, type IIIa, and type IIIb. Treatment strategies vary with the types. Type I is treated by embolizing the fistula between the artery and the vein with coils. Type II (IIa, IIb, and IIc) AVM is treated as follows:first, reduce the blood flow velocity in the venous segment of the AVM with coils; second, perform ethanol embolotherapy of the residual shunts. Type IIIa is treated by transarterial catheterization of the feeding arteries and injection of diluted ethanol. Type IIIb is treated by transarterial or direct puncture approaches. A high concentration of ethanol is injected through the transarterial catheter or direct puncture needle. When the fistula is large, coil insertion is required to reduce the amount of ethanol. Type I and type II AVMs showed the best clinical results; type IIIb showed a satisfactory response rate. However, type IIIa showed the poorest response rate, either alone or in combination with other types. Clinical success can be achieved by using different treatment strategies for different angiographic AVM types.

Transvaginal Direct Puncture and Ethanol Sclerotherapy for Cervicovaginal Venous Malformations: A Case Report and Literature Review

Cervicovaginal venous malformations are extremely rare. Sclerotherapy is proven to be effective for superficial venous malformations but not for venous malformations in the lower genital tract of female. A 52-year-old female presented with intermittent vaginal bleeding. The amount of vaginal bleeding gradually increased over 3 months. Contrast-enhanced pelvis CT showed several phleboliths and dilated vessels, but pelvic angiography showed no early draining veins, nidus, or feeding artery. We performed transvaginal direct puncture and ethanol sclerotherapy rather than surgical treatment because she wanted to preserve the uterus. After four sessions of sclerotherapy, she had significantly decreased vaginal bleeding without complications. Here, we report the first case of cervicovaginal venous malformations successfully treated with transvaginal direct puncture and ethanol sclerotherapy.

Effects of percutaneous angioplasty on kidney function and blood pressure in patients with atherosclerotic renal artery stenosis

BACKGROUND: Previous randomized controlled trials of revascularization for atherosclerotic renal artery stenosis (ARAS) were not successful. We investigated the effects of percutaneous transluminal angioplasty with stent insertion (PTA/S) on kidney function and blood pressure (BP) control in patients with ARAS. METHODS: From 2000 to 2017, 47 subjects who underwent PTA/S for ARAS were identified. A high-risk group was defined, composed of patients having one or more of the following clinical presentations: pulmonary edema, refractory hypertension, and rapid deterioration of kidney function. Subjects who met the criteria of ‘kidney function improvement’ or ‘hypertension improvement’ after PTA/S were classified as responders. RESULTS: Twenty-one (44.7%) subjects were classified into the high-risk group. Two subjects (8.0%) in the low-risk group (n = 25) and 5 subjects (27.8%) in the high-risk group (n = 18) showed improvement in kidney function after PTA/S (P = 0.110). In patients with rapid decline of kidney function, estimated glomerular filtration rate improved from 28 (interquartile range [IQR], 10–45) mL/min/1.73 m² to 41 (IQR, 16–67) mL/min/1.73 m² at 4 months after PTA/S, although the difference was not significant (P = 0.084). Regarding BP control, 9 (36.0%) and 14 (77.8%) subjects showed improvement after PTA/S in the low- (n = 25) and high-risk (n = 18) groups, respectively (P = 0.007). In patients with refractory hypertension, the systolic BP dropped from 157 (IQR, 150–164) mmHg to 140 (IQR, 131–148) mmHg at 4 months after PTA/S (P = 0.005). Twenty-five subjects were defined as responders and comprised a significant proportion of the high-risk group (P = 0.004). CONCLUSION: PTA/S might improve BP control and kidney function in patients with ARAS presenting with high-risk clinical features. The optimal application of PTA/S should be based on individual assessment of the clinical significance of renal artery stenosis.

Long Term Outcomes of Femorofemoral Crossover Bypass Grafts

PURPOSE: Femorofemoral crossover bypass (FCB) is a good procedure for patients with unilateral iliac artery disease. There are many articles about the results of FCB, but most of them were limited to 5 years follow-up. The purpose of our study was to analysis the results of FCB with a 10-year follow-up period. MATERIALS AND METHODS: Between January 1995 and December 2010, 133 patients were operated in Samsung Medical Center (median follow-up: 58.8 months). We retrospectively analysed patient characteristics, the preoperative treatment, the operative procedure, and material used. RESULTS: The indications for FCB were claudication in 110 and critical limb ischemia in 23 patients. Three patients were died due to myocardiac infarction, intracranial hemorrhage, and acute respiratory failure within 30 days after surgery. The one-year primary and secondary patency rates were 89% and 97%, the 5-year primary and secondary patency rates were 70% and 85%, and the 10-year primary and secondary patency rates were 31% and 67%. The 5-year and 10-year limb salvage rates were 97% and 95%, respectively. CONCLUSION: Our long term analysis suggests that FCB might be a valuable alternative treatment modality in patients with unilateral iliac artery disease.

Portal Vein Stenting for Delayed Jejunal Varix Bleeding Associated with Portal Venous Occlusion after Hepatobiliary and Pancreatic Surgery

OBJECTIVE: The study aimed to describe portal stenting for postoperative portal occlusion with delayed (≥ 3 months) variceal bleeding in the afferent jejunal loop. MATERIALS AND METHODS: Eleven consecutive patients (age range, 2–79 years; eight men and three women) who underwent portal stenting between April 2009 and December 2015 were included in the study. Preoperative medical history and the postoperative clinical course were reviewed. Characteristics of portal occlusion and details of procedures were also investigated. Technical success, treatment efficacy (defined as disappearance of jejunal varix on follow-up CT), and clinical success were analyzed. Primary stent patency rate was plotted using the Kaplan-Meier method. RESULTS: All patients underwent hepatobiliary-pancreatic cancer surgery except two children with liver transplantation for biliary atresia. Portal occlusion was caused by benign postoperative change (n = 6) and local tumor recurrence (n = 5). Variceal bleeding occurred at 27 months (4 to 72 months) and portal stenting was performed at 37 months (4 to 121 months), on average, postoperatively. Technical success, treatment efficacy, and clinical success rates were 90.9, 100, and 81.8%, respectively. The primary patency rate of portal stent was 88.9% during the mean follow-up period of 9 months. Neither procedure-related complication nor mortality occurred. CONCLUSION: Interventional portal stenting is an effective treatment for delayed jejunal variceal bleeding due to portal occlusion after hepatobiliary-pancreatic surgery.

Usefulness of intraopertive ultrasonography during directional atherectomy using SilverHawk/TurboHawk system

PURPOSE: Directional atherectomy (DA) was introduced for the management of infrainguinal arterial stenosis or occlusive lesions. The procedure success rate in the DEFINITIVE LE study was determined using radiologic imaging. The aim of our study was to determine the usefulness of intraoperative ultrasonography (USG) during DA for evaluating the early results of this procedure. METHODS: Patients who underwent DA from January to December 2014 were reviewed retrospectively. Twenty lesions from 14 patients with femoral artery stenosis (>70% stenosis) with short segment occlusive lesions (<2 cm in length) were treated. Among 20 lesions, 3 were treated with the TurboHawk system with a protective device due to lesion calcification. The percentage of stenosis during and after DA was determined with USG. RESULTS: Median follow-up was 5.1 months, and the procedural success rate (<30% stenosis at the end of the procedure) was 100% on angiography, but only 30% on intraoperative USG. On USG, median residual stenosis was 40% (range, 28%–42%) at the end of DA, 40% (range, 30%–55%) at 1 month, 55% (range, 35%–85%) at 6 months, and 64% (range, 60%–100%) at 1 year. There was one dissection, but no cases of perforation, pseudoaneurysm, or thrombosis. Primary patency, which was defined as a peak systolic velocity ratio ≤3.5 with no reintervention at 6 months, was found in 18 lesions (90%), and 11 of 14 patients (78.6%) were free of ischemic symptoms such as claudication at 6 months. CONCLUSION: Our results demonstrated that DA with intraoperative USG is an effective treatment option for short segment occlusive lesions of the femoral artery.

Renal artery stenosis presenting with nephrotic-range proteinuria: a case report

Renal artery stenosis (RAS) is commonly presented with hypertension and chronic kidney disease. We report a rare case of RAS occurring in a 78-year-old man who presented with nephrotic-range proteinuria. Renal biopsy on the left side was performed, and results showed mesangiopathic glomerulonephritis, which was not compatible with the cause of nephrotic-range proteinuria. Proteinuria was decreased by angiotensin receptor blocker, but azotemia was aggravated. Therefore, angiotensin receptor blocker was discontinued inevitably and thorough evaluation for the possibility of RAS was performed. Computed tomography angiography revealed significant RAS on the left side and a renal artery stent was inserted. After stenting, aortic dissection developed and progressed despite tight control of blood pressure. After inserting another stent graft through the true lumen of the left renal artery, the patient's renal function and proteinuria improved markedly.

Experience of direct percutaneous sac injection in type II endoleak using cone beam computed tomography

Cone beam CT, usually used in dental area, could easily obtain 3-dimensional images using cone beam shaped ionized radiation. Cone beam CT is very useful for direct percutaneous sac injection (DPSI) which needs very precise measurement to avoid puncture of inferior vena cava or vessel around sac or stent graft. Here we describe two cases of DPSI using cone beam CT. In case 1, a 79-year-old male had widening of preexisted type II endoleak after endovascular aneurysm repair (EVAR). However, transarterial embolization failed due to tortuous collateral branches of lumbar arteries. In case 2, a 72-year-old female had symptomatic sac enlargement by type II endoleak after EVAR. However, there was no route to approach the lumbar arteries. Therefore, we performed DPSI assisted by cone beam CT in cases 1, 2. Six-month CT follow-up revealed no sign of sac enlargement by type II endoleak.

A Phase I Study of Human Cord Blood-Derived Mesenchymal Stem Cell Therapy in Patients with Peripheral Arterial Occlusive Disease

BACKGROUND AND OBJECTIVES: Half of patients with critical limb ischemia (CLI) are ineligible for revascularization at diagnosis. The aim of this study was to assess the safety and feasibility of intramuscular human umbilical cord blood-derived mesenchymal stem cell (hUCB-MSC) therapy in patients with CLI due to atherosclerosis obliterans (ASO) or thromboangiitis obliterans (TAO). METHODS AND RESULTS: A total of eight patients (all male, median age 52 years, range 31~77) with CLI were enrolled in this phase I trial. All patients were considered ineligible for further revascularization to improve CLI. We injected 1x10(7) hUCB-MSCs per single dose intramuscularly into the affected limb. The primary end points of safety were occurrence of adverse events (procedure-related complication, allergic reaction to hUCB-MSCs, graft-versus-host disease, cardiovascular and cerebrovascular events) and improvement of symptoms/clinical parameters (healing of foot ulcer, ankle-brachial index, and pain-free walking distance). Angiogenesis was measured with conventional angiography and scored by an independent reviewer. There were four adverse events in three patients. One patient, developed whole body urticaria after injection on treatment day, which disappeared after one day of antihistamine treatment. The other adverse events included diarrhea, oral ulceration, and elevation of serum creatinine level; all conditions improved without treatment. Abnormal results of laboratory parameters were not detected in any patients. Three of four ulcerations (75%) healed completely. Angiographic scores increased in three of eight patients. CONCLUSIONS: This phase I study demonstrates that intramuscular hUCB-MSC injection is a safe and well tolerated treatment for patients with end-stage CLI due to ASO and TAO.

Ten year outcomes after bypass surgery in aortoiliac occlusive disease / 대한외과학회지

PURPOSE: Most outcome studies of bypass surgery are limited to five years of follow-up. However, as human life expectancy has increased, analyses of more long-term outcomes are needed. The aim of this study is to evaluate 10-year outcomes of anatomical bypasses in aortoiliac occlusive disease. METHODS: From 1996 to 2009, 92 patients (82 males and 10 females) underwent aortic anatomical bypasses to treat aortoiliac occlusive disease at Samsung Medical Center. The patients were reviewed retrospectively. Kaplan-Meier survival analyses were performed using PASW ver. 18.0 (IBM Co). RESULTS: A total of 72 patients (78.3%) underwent aorto-femoral bypasses (uni- or bi-femoral), 15 patients (16.3%) underwent aorto-iliac bypasses (uni- or bi-iliac), and 5 patients (5.4%) underwent aorto-iliac and aorto-femoral bypasses. The overall primary patency rates of the 92 patients were 86.2% over 5 years and 77.6% over 10 years. The 10-year limb salvage rate and overall survival rate were 97.7% and 91.7%, respectively. CONCLUSION: The overall patency rates of bypass graft and limb salvage rates decreased as time passed. The analysis of results after bypass surgery to treat arterial occlusive disease will be needed to extend for 10 years of follow-up.

A hybrid operation in a patient with complex right subclavian artery aneurysm / 대한외과학회지

We report a hybrid surgery including endovascular aneurysm repair and debranching procedures to treat a patient with a complex right subclavian artery aneurysm. The patient was a 70-year-old woman who presented with dry cough and hoarseness. The aneurysm was characterized by the absence of a proximal neck, and involvement of the origin of the right vertebral artery. She underwent carotid-vertebral artery bypass, stent graft from the innomiate artery to the common carotid artery and carotid-axillary artery bypass. Great saphenous vein was used for the carotid-vertebral artery bypass and 7 mm reinforced polytetrafluoroethylene graft was used for the carotid-axillary artery bypass. The postoperative course was uneventful.

Natural History of Type II Endoleaks after Endovascular Aneurysm Repair in Abdominal Aortic Aneurysm / 대한혈관외과학회지

PURPOSE: Endovascular aneurysm repair (EVAR) for the treatment of abdominal aortic aneurysm (AAA) has shown excellent early outcomes. However, long-term durability continues to be questioned because of endoleaks. The optimal management of type II endoleaks remains controversial because little is known about their long-term natural history. The purpose of this study was to evaluate the natural history of type II endoleaks and to study factors associated with persistent type II endoleaks. METHODS: On a retrospective basis, we analyzed 98 patients who underwent EVAR for incidence and outcome of type II endoleaks. Patients with type II endoleaks were evaluated using computed tomography angiography or Duplex scans at one, 6 and 12 months after their EVAR, and annually thereafter to evaluate both the persistence of the endoleak and the size of the aneurysm sac. RESULTS: Type II endoleaks were detected during the follow-up periods in 38 patients (39.8%), who underwent EVAR (mean follow-up: 23.7 months). Spontaneous sealing of type II endoleaks by 6 months after EVAR occurred in 15 patients (39.4%), meaning that there were 23 patients (60.6%) whose leaks were ongoing for more than 6 months. Four patients were treated using embolization because of an enlarging aneurysm sac. CONCLUSION: Most type II endoleaks are transient and do not require intervention. However, particularly persistent endoleaks could lead to aneurysm enlargement and to delayed aortic rupture. We did not find a significant difference in any number of preoperative patient factors between patients with transient, persistent, or no type II endoleaks. Further studies based on independent data sets are needed to validate these results.

The Prevalence of Abdominal Cystic Lesion in Patients with Vascular Disease

PURPOSE: This study evaluated the prevalence of cystic disease in the intra-abdominal organs in a normal population and a group of patients with vascular disease. METHODS: We reviewed the medical records and computed tomography scans of the vascular disease group and the control group. Vascular disease group (n=586) were patients who suffered with abdominal aortic aneurysm (AAA), aorto-iliac occlusive disease (AOD) and peripheral arterial occlusive disease (PAOD) and who were treated in our institute. The control group (n=424) were patients who visited the health promotion center in our institute during the same period. RESULTS: The prevalence of abdominal cysts in the control group and the vascular disease group was 41.7% and 65.2%, respectively. The vascular disease group showed a higher prevalence of abdominal cystic lesion for all the specific vascular diseases. However, when the two groups were stratified according to age, the vascular disease group displayed no significant difference in the prevalence of abdominal cyst compared to that of the control group. CONCLUSION: Abdominal cystic lesions are common in patients with vascular disease, including AAA, AOD, and PAOD. But this high prevalence of abdominal cystic lesions seems to be associated with the advanced age of vascular disease patients. Further molecular-based research is needed to clarify the pathogenetic relationship between vascular disease and abdominal cystic lesions.

Indications and Short-term Results of Open Surgical Repair of Abdominal Aortic Aneurysm in an Endovascular Era

PURPOSE: To assess the role of OR in treatment of AAA patients, we reviewed the indications and our current results of OR of AAA. METHODS: We retrospectively investigated the database of 366 patients (mean age, 68.3+/-8.7 years, male 86%) who underwent open surgical (n=291, 80%) or endovascular treatments (n=75, 20%) of AAA in a tertiary referral center between Sep. 2003 to Aug. 2010. Treatment-related morbidities and mortality rates within 30 days were investigated according to the indications for treatment, clinical features, anatomic location, and underlying causes of AAA. RESULTS: According to the location of AAA, we treated 343 (94%) infrarenal, 17 (5%) juxtarenal and 6 (2%) suprarenal AAAs. Underlying causes of AAA were degenerative (90%), infected (3%), inflammatory (3%), Marfan's syndrome (2%), and vasculitis (0.5%). Clinically, 338 (92%) were non-ruptured and 28 (8%) were ruptured AAAs. 75% of patients were treated with OR after the inception of reimbursement of aortic device while 25% of patients were treated with EVARs. The operative mortality rates of OR was 0.4% in patients with non-ruptured infrarenal AAA, 0% in patients with non-ruptured juxta- and suprarenal AAA and 21.4% in ruptured AAA patients. CONCLUSION: In an era of endovascular treatment of AAA, we have experienced excellent surgical results after OR in patients with non-ruptured AAA with various clinical features. Though EVAR is rapidly replacing OR in treatment of infrarenal AAAs, OR has its own role in treatment of AAA patients with unfavorable conditions for EVAR. The role of OR should not be underestimated.

Tips for Delayed Open Conversion in Patients with a Type III Endoleak after Endovascular Aortic Aneurysm Repair

Type III endoleak is one of the causes of secondary intervention after endovascular aortic aneurysm repair (EVAR). We report two cases of a late type III endoleak with a review of the literature. One case had a disconnected iliac limb and the other case was due to a defect of the main body fabric at the bifurcation level. Both cases were successfully treated by open conversion. Before open conversion, it should be determined how to get proximal aortic control (suprarenal vs. infrarenal and cross-clamping vs. balloon inflation). When the suprarenal aortic control is required, to reduce suprarenal clamping time, it is not necessary to remove the endograft completely. Partial endograft removal and a graft-to-graft anastomosis is an alternative, if it is well incorporated and not infected. Making a plan for delayed open conversion should be individualized according to the type and status of the endograft and the vascular anatomy.

Clinical Features and Mutations in the ENG, ACVRL1, and SMAD4 genes in Korean Patients with Hereditary Hemorrhagic Telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is an inherited disorder that is characterized by abnormal communication between the arteries and veins in the skin, mucosa, and various organs. HHT has been reported to show significant phenotypic variability and genetic heterogeneity with wide ethnic and geographic variations. Although mutations in the endoglin (ENG) and activin A receptor type II-like 1 (ACVRL1) genes have been known to cause HHT for more than 10 yr, little is known about the clinical features or genetic background of Korean patients with HHT. In addition, mutations in mothers against decapentaplegic homolog 4 (SMAD4) are also seen in patients with the combined syndrome of juvenile polyposis and HHT. This study examined five Korean patients with the typical manifestations of HHT such as frequent epistaxis and pulmonary arteriovenous malformations. Direct sequencing of the ENG and ACVRL1 genes revealed one known mutation, ENG c.277C>T, in one patient and two novel mutations, ENG c.992-1G>C and ACVRL1 c.81dupT in two patients, respectively. The remaining two patients with negative results were screened for SMAD4 mutations as well as gross deletions of ENG and ACVRL1 using multiple ligation-dependent probe amplification, but none was detected. Despite the small number of patients investigated, we firstly report Korean patients with genetically confirmed HHT, and show the genetic and allelic heterogeneity underlying HHT.

Pulmonary Venous Malformation in a 4-Year-Old Boy: a Case Report

We report a case of a pulmonary venous malformation in a 4-year-old boy who presented with recurrent pneumonia. A radiograph revealed a right infrahilar mass and a hyperlucent right lung. Computed tomography (CT) demonstrated a mass containing intensely enhancing areas and multiple phleboliths located in the right lower lobe and encasing the right bronchus and right inferior pulmonary vein. Magnetic resonance imaging (MRI) precisely revealed the mass demarcation. A right lower lobectomy was performed and a pathological examination confirmed the diagnosis of a venous malformation. To the best of our knowledge, a venous malformation in pulmonary tissue has not been reported in the English literature. Herein, we report a case of a pulmonary venous malformation, with the radiograph, CT, MRI, and blood pool scan findings, along with its pathologic correlation.

Ethanol Embolotherapy of Pelvic Arteriovenous Malformations: an Initial Experience

OBJECTIVE: We retrospectively assessed the results of performing ethanol embolization for pelvis arteriovenous malformations (AVMs). MATERIALS AND METHODS: During the past 10 years, eight patients (8 females, age range: 27-52 years) with AVMs in the pelvic wall (n = 3) and uterus (n = 5) underwent staged ethanol embolizations (range: 1-5, mean: 2.5) under general anesthesia. Ethanol embolization was performed by the use of the transcatheter and/or direct puncture techniques. Clinical follow-up was performed for all of the patients, and imaging follow-up was available for seven patients. The therapeutic outcomes were established by evaluating the clinical outcome of the signs and symptoms, as well as the degree of devascularization observed on post-procedural angiography. RESULTS: During the 20 sessions of ethanol embolization, the solitary transarterial approach was used 14 times, the transvenous approach was used three times and direct puncture was used once. For two patients, the transarterial and transvenous or direct puncture approaches were used together in one session. For four patients, ethanol and coils were used as embolic agents, and n-butyl cyanoacrylate (NBCA) and ethanol were used in one patient. Seven (88%) of eight patients were cured of their AVMs and one patient (12%) displayed improvement. Major complications were seen in two patients (25%). CONCLUSION: Ethanol embolization is effective for the treatment of pelvic arteriovenous malformations, though there is a chance of a major complication.