Intraoperative Neuromonitoring during Peripheral Arteriovenous Malformation Embolization.

PURPOSE: To evaluate whether intraoperative neuromonitoring (IONM), including pre-embolization lidocaine injection challenge ("provocative testing") is associated with reduced risk of irreversible nerve injury during embolization of peripheral arteriovenous malformations (AVMs). MATERIALS AND METHODS: Medical records of patients with peripheral AVMs who underwent embolotherapy with IONM with provocative testing between 2012 and 2021 were reviewed retrospectively. Data collected included patient demographic characteristics, AVM location and size, embolic agent used, IONM signal changes after lidocaine and embolic agent injections, postprocedural adverse events, and clinical outcomes. Decisions regarding whether embolization would proceed at specific locations were based on IONM findings after the lidocaine challenge and as embolization proceeded. RESULTS: A cohort of 17 patients (mean age, 27 years ± 19; 5 women) who underwent 59 image-guided embolization procedures with adequate IONM data was identified. No permanent neurologic deficits occurred. Transient neurologic deficits were observed in 3 patients (4 sessions), comprising skin numbness (2 patients), extremity weakness (1 patient), and extremity weakness and numbness (1 patient). All neurologic deficits resolved by postoperative day 4 without additional treatment. CONCLUSIONS: IONM, including provocative testing, during AVM embolization may minimize potential nerve injury.

Postembolization Persistence of Pulmonary Arteriovenous Malformations: A Retrospective Comparison of Coils and Amplatzer and Micro Vascular Plugs Using Propensity Score Weighting.

BACKGROUND. Endovascular embolization of pulmonary arteriovenous malformations (PAVMs) was historically performed with embolic coils. The Amplatzer Vascular Plug device (AVP) was introduced for this purpose in 2007 and the Micro Vascular Plug device (MVP) in 2013. OBJECTIVE. The purpose of this study was to compare coils, AVPs, and MVPs in terms of risk of persistence after PAVM embolization by use of propensity score weighting to account for biases in device selection. METHODS. This retrospective study included 112 patients (78 women and girls, 34 men and boys; mean age, 45 years) who underwent embolization of 393 PAVMs with a single device type (coil, MVP, or AVP) from January 2003 to January 2020. Persistence was defined as less than 70% reduction in PAVM sac size or contrast enhancement of the sac on follow-up pulmonary CTA. A Cox proportional hazards regression model was used to assess associations between embolic device selection and PAVM persistence. Inverse propensity score weighting was used to account for differences in embolic device selection based on patient and PAVM characteristics. RESULTS. The median postembolization follow-up period was 1.5 years (IQR, 0.3-5.6 years). Persistence was found in 10% (41/393) of PAVMs, including 16% (34/207) of those treated with coils, 8% (7/88) of those treated with AVPs, and 0% (0/98) of those treated with MVPs. Variables associated with embolization device (p < .25) were age, sex, pediatric versus adult status, smoking status, PAVM complexity, PAVM laterality, number of feeding arteries, and feeding artery diameter. The Cox regression model incorporated inverse propensity score weighting to account for the differences between treatment groups in these variables and incorporated feeding artery diameter because of imbalance remaining after weighting. With coils as the referent, MVPs had a hazard ratio for persistence of less than 0.01 (95% CI, < 0.01 to < 0.01; p < .001), and AVPs had a hazard ratio of 0.37 (95% CI, 0.16-0.90; p = .03). CONCLUSION. The risk of persistence after PAVM embolization was significantly lower for MVPs alone than for coils or AVPs alone. In addition, the risk of persistence was lower for AVPs than for coils. CLINICAL IMPACT. The findings support the clinical use of MVPs as the preferred device for PAVM embolization over coils and polytetrafluoroethylene-covered plugs.

Fostering research in pediatric interventional radiology: needs assessment and suggestions for support.

BACKGROUND: Due to the rarity of pediatric diseases, collaborative research is the key to maximizing the impact of research studies. A research needs assessment survey was created to support initiatives to foster pediatric interventional radiology research. OBJECTIVE: To assess the status of pediatric interventional radiology research, identify perceived barriers, obtain community input on areas of research/education/support, and create metrics for evaluating changes/responses to programmatic initiatives. MATERIALS AND METHODS: A survey link was sent to approximately 275 members of the Society for Pediatric Interventional Radiology (SPIR) between May and October 2020. Data was collected using a web-based interface. Data collected included practice setting, clinical role, research experience, research barriers, and suggestions for future initiatives. RESULTS: Fifty-nine surveys were analyzed with a staff physician survey response rate of 28% (56/198). A wide range of practice sizes from 15 countries were represented. Respondents were predominantly staff physicians (95%; 56/59) with an average of 11 years (range: 1-25 years) of clinical experience working at academic or freestanding children's hospitals. A total of 100% (59/59) had research experience, and 70% (41/58) had published research with a mean of 30 peer-reviewed publications (range: 1-200). For job security, 56% (33/59) of respondents were expected or required to publish, but only 19% (11/58) had research support staff, and 42% (25/59) had protected research time, but of those, 36% (9/25) got the time "sometimes or never." Lack of support staff, established collaborative processes, and education were identified as top barriers to performing research. CONCLUSIONS: The needs assessment survey demonstrated active research output despite several identified barriers. There is a widespread interest within the pediatric interventional radiology community for collaborative research.

Hyperpigmentation after Foamed Bleomycin Sclerotherapy for Vascular Malformations.

The present report documents 6 patients who developed distinctive hyperpigmented skin lesions after bleomycin sclerotherapy for vascular malformations of the face, neck, and extremities. The patients ranged in age from 2 to 65 years and included both black and white and male and female patients. The bleomycin treatment dose varied from 15 to 45 U, with 5 of the 6 patients receiving foamed bleomycin. The hyperpigmented lesions were near the patient's vascular anomaly and attributable to postprocedural cutaneous pressure (eg, electrocardiographic [ECG] leads or tape). Hyperpigmentation faded slowly over time but was visible up to 3 years after the procedure.

Bleomycin Foam Treatment of Venous Malformations: A Promising Agent for Effective Treatment with Minimal Swelling.

PURPOSE: To report clinical and radiographic outcomes of patients with venous malformations (VMs) treated with bleomycin foam. MATERIALS AND METHODS: Twenty patients (age, 2-68 y) presented with symptoms of swelling (n = 19; 95%), pain (n = 14; 70%), and bleeding (n = 4; 20%). Lesions were located in the head and neck in 17 patients (85%), extremities in two (10%), and mediastinum in one (5%). Twenty-seven embolizations were performed, with a mean of 1.7 ± 1.0 treatments per patient (range, 1-4). An average of 0.45 ± 0.4 U/kg of bleomycin foam was used per procedure, with a range of 0.1-2.3 U/kg. RESULTS: All procedures were technically successful with no intraprocedural complications. Mean follow-up was 66 days ± 80, with a range of 4-403 days. Postprocedure complications were minor in 6 of 27 procedures (22%) and major in 2 of 27 procedures (7%). All 20 patients (100%) reported improvement in their symptoms after a single treatment session. Postprocedural magnetic resonance (MR) imaging demonstrated volume reduction of treated lesions in 13 of 14 patients (93%), with a mean lesion volume reduction of 66% ± 21. Enhancement on MR imaging after treatment was decreased in 11 of 14 patients (79%), increased in two (14%), and stable in one (7%). T2 signal intensity on MR imaging after treatment was decreased in 12 of 14 patients (86%) and stable in two (14%). CONCLUSIONS: The use of bleomycin foam for the percutaneous treatment of VMs is safe and effective. Foaming bleomycin may be used to address the dose limitations of the liquid.

MR imaging characteristics of soft tissue vascular anomalies in children.

Accurate classification of soft tissue vascular anomalies is critical since treatment options and morbidity differ significantly for the various groups of vascular anomalies (VA). A classification system introduced by Mulliken and Glowacki in 1982 explained the biology of VA, thus resulting in improved management and communication between different disciplines taking care of children with soft tissue vascular anomalies. This classification has been updated by the International Society for the Study of Vascular Anomalies in 1996 and forms the basis for the current nomenclature. Although the majority of vascular anomalies can be accurately classified by their clinical history and a physical exam, imaging is necessary to determine the full anatomical extent of the anomaly, to follow up treatment response, to confirm diagnosis, and to provide correct classification in challenging cases when necessary. This article will review the key magnetic resonance imaging (MRI) features of most common soft tissue vascular anomalies in children. MRI and dynamic contrast-enhanced magnetic resonance angiography are crucial for the evaluation of vascular anomalies in children because of lack of radiation, high soft tissue resolution, and the capability of dynamic contrast-enhanced images to reflect the hemodynamics of the anomalies.

Lymphangiography with sclerotherapy: a novel therapy for refractory chylous ascites.

INTRODUCTION: We report the outcomes of a small series of patients with refractory chylous ascites following urologic surgery treated with lymphangiography +/- sclerotherapy. MATERIALS AND METHODS: Retrospective review revealed three patients who underwent lymphangiography for prolonged lymphatic leak following urological surgery. Contrast material is injected slowly into a lymphatic vessel on the dorsum of the foot and serial imaging is used to capture the location and degree of lymphatic leak in order to guide definitive treatment. Demographic and clinical details were collected and are reported. RESULTS: Three patients were identified from 2005-2008 (one following donor nephrectomy and two following retroperitoneal lymph node dissection). All patients presented with abdominal distension within 30 days of surgery. Traditional conservative measures failed in all patients. Lymphangiography localized all leaks (renal hilum, paraspinal, and retrocaval). One patient elected for successful surgical repair after localization. The remaining two patients resolved immediately following lymphangiography; one of these patients underwent percutaneous doxycycline sclerosis. With over 1 year of follow up there have been no recurrences or long term sequelae. CONCLUSIONS: Lymphangiography is a valuable management option for the rare patient with chylous ascites refractory to conservative therapy. Prompt resolution of prolonged chylous ascites following lymphangiography should encourage its use in such difficult cases.

Subtotal splenic embolization is a safe and effective treatment for isolated splenic vascular tumors associated with consumptive coagulopathy.

Consumptive coagulopathy is a known complication of large vascular tumors. We describe 2 episodes of consumptive coagulopathy in young children, which were secondary to isolated splenic vascular tumors. One child was successfully treated by subtotal embolization of the spleen, whereas the second child required splenectomy after an initial embolization improved--but did not fully control--his consumptive coagulopathy.

Update on pediatric extracranial vascular anomalies of the head and neck.

PURPOSE: Vascular anomalies most frequently present at birth or in early childhood, and the craniofacial region is the most common site of involvement. A long history of misleading nomenclature born of confusion about the presentation and natural history of various vascular anomalies has made appropriate diagnosis difficult. The present article emphasizes the importance of clarity of nomenclature for proper diagnosis, both clinically and radiographically, to guide appropriate therapy. In addition, updates on clinical concepts, imaging, and treatment strategies will be discussed. Pediatric vascular anomalies can be divided into two broad categories: vascular tumors and vascular malformations. This biologic classification is based on differences in natural history, cellular turnover, and histology. An updated classification was introduced in 1996 by the International Society for the Study of Vascular Anomalies (ISSVA) to include infantile hemangioma variants, other benign vascular tumors, and combined lesions. Widespread confusion propagated throughout the literature and in clinical practice stems from the continued improper use of many of the terms used to describe vascular tumors and malformations ignoring their pathophysiology. This leads to errors in diagnosis and the dissemination of misinformation to patients and clinicians. Certain terms should be abandoned for more appropriate terms. The clinical presentation usually identifies what general type of vascular anomaly is present, either vascular tumor or vascular malformation. Imaging provides crucial information about the initial diagnosis and aids in follow-up. CONCLUSIONS: Adoption and use of uniform nomenclature in the ISSVA classification system is the first vital step in correct diagnosis and treatment of often complicated vascular tumors and vascular malformations. A multidisciplinary team approach is necessary to provide optimal care for patients, and the necessity for specialists in all areas to communicate using standardized terminology cannot be overemphasized.

Outcomes of Venous Malformation Sclerotherapy: A Review of Study Methodology and Long-Term Results.

It is very important that patients seeking sclerotherapy for the treatment of venous malformations are aware of the expected course of the therapy. They should be thoroughly counseled about the complications, the need for multiple sessions of therapy, and also about the expected clinical outcome. The aim of this review is to discuss the long-term outcomes of sclerotherapy for the treatment of venous malformation. Many studies have discussed their individual center's experiences and short-midterm results, but there is a relative paucity of data on long-term outcomes. We have reviewed the literature and also shared our experience of a large cohort of patients ( n = 116) with a relatively longer follow-up period of more than 1 year. Venous malformations are very complex lesions and their treatment is quite variable depending on its extent and complexity. As a result, outcome studies vary considerably in the choice of sclerosant, study methodology, outcome assessment (clinical vs. imaging), and grading scales. This review also highlights this extreme heterogeneity in the literature of the sclerotherapy outcome and summarizes a few national and international studies for comparison.

Preoperative Epoxy Embolization Facilitates the Safe and Effective Resection of Venous Malformations in the Hand and Forearm.

BACKGROUND: The standard of care for treatment of low-flow venous malformations (VMs) is percutaneous sclerotherapy. These lesions are seldom surgically resected, especially if the malformation is in an anatomically difficult location. Percutaneous sclerotherapy is safe and effective. However, the drawbacks to sclerotherapy are the need for repeated treatments and risks of skin ulceration, deep venous thrombosis, scarring/contractures, and nerve damage. Surgical resection can be difficult because of intraoperative bleeding, intraoperative lesional decompression, and difficulty in localization. METHODS: We describe our initial experience with 11 patients who underwent surgical resection of VMs located in the hand and forearm after preembolization of 27 total sites using n-butyl-cyanoacrylate or ethylene vinyl alcohol copolymer. RESULTS: Of the 11 patients treated, 5 had focal VMs, 3 had multifocal VMs, and 3 had diffuse VMs throughout the affected extremity. Four of the 5 patients with focal VMs were followed for at least 1 year, and no further treatment was required. All 3 of the patients with diffuse VMs have required ongoing treatment. No major functional impairments were reported, and there were no major procedure-related complications. CONCLUSIONS: Overall, embolization of the malformation before surgical resection facilitated localization, demarcation, and removal of the lesion.

Incorporating physician assistants and physician extenders in the contemporary interventional oncology practice.

Interventional radiology (IR) has been for the last few years undergoing a transformation from a service oriented to a clinically oriented specialty. With increasing oncologic procedures and patient volume, the balance between quality clinical care, and the time constraints on the busy interventionalist pull in opposing forces. The need for greater clinical support staff in the IR practice is unquestionable. Physician Assistants (and other Physician Extenders) have been in the medical field since the 1960s with intensive clinical training, capabilities of providing patient care and ability to generate revenue income more than justifies their place in the IR. The contemporary model of a clinical orientated service within IR for cancer patients undergoing interventional oncology procedures should include Physician Extenders as a vital part of the team allowing delivery of high-quality patient care.

The treatment of venous malformations with percutaneous sclerotherapy at a single academic medical center.

PURPOSE: We report a retrospective analysis of venous malformation patients treated with percutaneous sclerotherapy, describing their clinical manifestations, therapeutic outcomes and procedural complications. MATERIALS AND METHODS: We reviewed our Vascular Anomalies database for all patients who underwent percutaneous sclerotherapy for venous malformation between January 2005 and July 2011 and retrieved 186 patients, out of which 116 were included in the final analysis. The majority of patients were treated using 100% alcohol (72%) and the rest were treated with <100% alcohol, Sodium Tetradecyl Sulfate or combination of these therapies. The most common location was the lower extremity in 67 patients (58%), followed by the head and neck in 27 (23%) and the upper extremity in 11 (9%). Retrospective review of medical records was performed. Outcomes were classified on an improvement scale based on clinical therapeutic effects. RESULTS: Two-hundred and forty-five sclerotherapy procedures were performed in 116 patients, of which 52 patients (45%) underwent a single procedure, 32 (28%) had two procedures and 32 (28%) underwent ≥3 procedures. Median follow-up period from the last procedure was 2.5 months (interquartile range of 2.0 to 6.75 months). Significant improvement was seen in 37 patients (32%), moderate improvement in 31 (27%), mild improvement in 20 (17%), no improvement in 21 (18%) and worse than before in 7 (6%) patients. Major post-procedural complications were nerve injuries in 6 patients (5%), deep vein thrombosis in 5 (4%), muscle contracture in 2 (2%), infection in 3 (3%), skin necrosis in 4 (3%) and other complications in 3 (3%). CONCLUSION: Our study demonstrated that 76% of our patients with venous malformation had some level of improvement in symptoms with majority (72%) undergoing only one or two percutaneous sclerotherapy procedure/s. Although major complications occurred in 20% of the patients, majority (74%) of the complications either resolved spontaneously or were successfully treated.

The minimal important difference of the epistaxis severity score in hereditary hemorrhagic telangiectasia.

OBJECTIVES/HYPOTHESIS: Hereditary hemorrhagic telangiectasia (HHT) is a disease of abnormal angiogenesis, causing epistaxis in over 96% of patients. The Epistaxis Severity Score (ESS) was developed as a standardized measurement of nasal symptoms among HHT patients. The minimal important difference (MID) of a disease index estimates the smallest change that a patient and clinician would identify as important. This study aims to establish the MID of the ESS in a diverse population of HHT patients. STUDY DESIGN: Retrospective cross-sectional study in patients with a diagnosis of HHT using Curacao criteria or genetic testing. METHODS: The ESS questionnaire and Medical Outcomes Study 36-Item Short Form (SF-36) were administered to participants recruited through the HHT Foundation Web site. Demographics and relevant medical histories were collected from all participants. An anchor-based method using a change of 5 in the Physical Component Summary (PCS) of the SF-36 and a distributional method were used to estimate the MID. RESULTS: A total of 604 subjects were recruited between April and August 2008. All participants reported epistaxis. An increasing ESS in the study cohort showed a significant negative correlation to the PCS (r = -0.43, P < 0.001). The MID was determined to be 0.41 via the anchor-based approach and 1.01 via the distribution-based approach, giving a mean MID of 0.71. CONCLUSION: Using both the anchor-based and distribution-based approaches, the estimated MID for the ESS in HHT is 0.71. Further implications include key metrics to help guide treatment responses in clinical care and essential information to calculate power and sample size for future clinical trials. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:1029-1032, 2016.

Pancreaticoduodenectomy: role of interventional radiologists in managing patients and complications.

Although the mortality rate after pancreaticoduodenectomy has decreased, the morbidity rate remains high. Major morbidity is often managed with the aid of interventional radiologists. The objective of this study was to evaluate the cooperative roles of interventional radiologists and pancreatic surgeons in complex pancreatic surgery, specifically pancreaticoduodenectomy. Our pancreaticoduodenectomy database was reviewed for all patients undergoing pancreaticoduodenectomy between January 1, 1995 and December 31, 2000. The interventional radiologic procedures for each patient were evaluated. A total of 1061 patients underwent pancreaticoduodenectomy. The overall mortality and morbidity rates were 2.3% and 35%, respectively. Five hundred ninety patients (56%) had no interventional radiologic procedures, whereas 471 patients (44%) had interventional radiologic procedures. Of those, 342 (32%) had preoperative biliary drainage (PBD) and 129 (12%) required postoperative interventional radiologic procedures. Percutaneous aspiration/catheter drainage was required in 84 patients for intra-abdominal abscess, biloma, or lymphocele, with 24 requiring two or more abscess drains. Thirty-nine patients underwent postoperative PBD for bile leaks due to anastomotic disruption, undrained biliary segments, or T-tube/bile stent dislodgment. Eighteen patients had hemobilia/gastrointestinal bleeding treated by angiography with embolization. The reoperation rate for the entire cohort of 1061 patients was 4.1% (n = 43). Nineteen of the 129 patients (15%) requiring postoperative radiologic intervention required reoperation. Although 4 of 18 patients who required embolization for bleeding subsequently required surgical intervention for the same reason, only 4 of 84 patients undergoing abscess drainage later required operation for anastomotic disruption or unsuccessful percutaneous drainage. As would be expected, the patients who required postoperative radiologic intervention (n = 129) had a higher incidence of postoperative complications including pancreatic fistula (20% vs. 6%, P < 0.01), bile leakage (22% vs. 1%, P < 0.01), and wound infection (16% vs. 8%, P < 0.01). With the complications in these 129 patients, the postoperative mortality rate was only 6.2% compared to 1.7% in patients who did not require radiologic intervention (n = 932, P < 0.01). The median postoperative length of stay was 15 days in those patients requiring postoperative radiologic intervention, 10 days in those not requiring intervention (P < 0.01; postoperative interventional radiology vs. no postoperative interventional radiology), and 29.5 days for patients needing reoperation. Interventional radiologists play a critical role in the management of some patients undergoing pancreaticoduodenectomy. Although complications such as anastomotic leaks, abscess formation, and bleeding can result in increased mortality and a longer hospital stay, the skills of the interventional radiology team provide expert management of some life-threatening complications, thus avoiding reoperation, speeding recovery times, and minimizing morbidity.

The effects of epistaxis on health-related quality of life in patients with hereditary hemorrhagic telangiectasia.

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease mainly characterized by epistaxis in more than 96% of patients. Recently, a validated questionnaire known as the HHT Epistaxis Severity Score (ESS) was developed. However, little is known about the relationship between epistaxis and quality of life. We hypothesize that epistaxis severity is a major factor predicting health-related quality of life (HR-QoL) in HHT patients. METHODS: This is a cross-sectional study. The ESS questionnaire and Medical Outcomes Study 36-item short form (SF-36) were administered to subjects through an Internet survey. All participants had a definitive diagnosis of HHT through Curaçao criteria or genetic testing. Demographic information, genetics, and extensive histories were also collected. Descriptive analyses were performed with calculations of means and standard deviations (SDs) for continuous variables and proportions for categorical variables. Linear regressions were then performed to assess the association between HR-QoL and ESS. RESULTS: A total of 604 subjects participated between April and August 2008. All patients reported epistaxis, 285 (47.2%) had telangiectasias, and 545 (90.2%) had a family history of HHT; 167 (27.6%) patients had mild epistaxis (ESS <4), 285 (47.2%) reported moderate epistaxis (≥4 ESS <7), and 152 (25.2%) reported severe epistaxis (ESS ≥7). Patients with severe epistaxis had lower scores for both the Physical Component Summary (PCS) and the Mental Component Summary (MCS) of HR-QoL when compared to those with mild epistaxis (p < 0.001, p < 0.001). CONCLUSION: The ESS is a major determinant of HR-QoL and should be considered as a measurement of treatment efficacy in HHT-related epistaxis.

Delayed diagnosis of iliac vein thrombus in a sexually-active adolescent with Klippel-Trénaunay syndrome.

BACKGROUND: Although iliac vein thrombus is uncommon in adolescents, it can present with pelvic inflammatory disease (PID) symptoms. CASE: A 19-year-old sexually active female with Klippel-Trénaunay syndrome (KTS) presented with fever, abdominal and lower extremity pain. Physical findings included cervical motion tenderness and left lower extremity swelling and erythema. The patient was admitted for PID and cellulitis. Despite antimicrobial treatment her pain continued. Neisseriae gonorrhea and Chlamydia trachomatis cultures were negative. Abdominal and pelvic computed tomography scans revealed a left internal iliac vein thrombus. Anticoagulation therapy was initiated; her pain improved. On hospital day 7 she developed pulmonary emboli. SUMMARY AND CONCLUSION: In sexually active adolescents with known risk factors for thromboembolism such as KTS, symptoms and signs considered characteristic for PID can be present in association with an iliac vein thrombus.