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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.

To describe our institutional experience with MVP™ micro vascular plug systems for the treatment of pulmonary arteriovenous malformations (PAVMs). We performed a retrospective medical record review of 52 patients with 119 PAVMs treated exclusively with MVP™ systems (69 procedures/153 MVP™ systems) between July 2014 and July 2018. All patients had PAVMs with feeding artery diameters ≥ 2 mm. MVP™ systems were deployed according to physician preference. We collected patient demographic information; procedural data (including size of feeding artery, size and number of embolics used per PAVM, fluoroscopy time, contrast administration), technical success rates, complications, and persistence. Persistence was assessed using computed tomography angiography (CTA) performed 1–3 months and 3–5 years after embolization per clinical protocol. All procedures were technically successful without major complications. Mean feeding artery diameter was 3.3 ± 1.2 mm. Mean fluoroscopy time per procedure and contrast volume administered per procedure were 35 ± 16 min and 217 ± 101 mL, respectively. A mean of 1.3 ± 0.8 MVP™ systems was used per PAVM. There were no instances of persistence during a mean follow-up time of 328 ± 258 days (range 26 to 914 days). For PAVMs with feeding artery diameters of 2 to 7.9 mm (mean 3.3 ± 1.2 mm), MVP™ systems are safe and effective given their high technical success rates and lack of persistence. Further prospective work will be required to elucidate the advantages and disadvantages of these MVP™ systems for PAVM embolization. Level III.

Vascular anomalies can occur anywhere in the body, and the majority present in the pediatric population. Accurate classification is essential for proper clinical evaluation, particularly because multidisciplinary care is often required. The International Society for the Study of Vascular Anomalies classification offers a comprehensive classification for all subspecialties. In this review article, we present a magnetic resonance imaging protocol with exemplary cases of the most common types of vascular anomalies in the pediatric trunk and extremities using the current International Society for the Study of Vascular Anomalies classification.

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.

OBJECTIVES/HYPOTHESIS To assess clinical outcomes of patients with airway venous malformations treated with percutaneous sclerotherapy (VMPS). We highlight the role of foamed bleomycin as a less inflammatory sclerosant and the importance of collaboration between interventional radiology and otolaryngology-head and neck surgery (OHNS). STUDY DESIGN Retrospective, consecutive, single-center series. METHODS Sixteen airway VMPS treatment sessions were performed (5 patients). Endoscopic needle guidance was performed by OHNS. Sclerosants included ethanol and foamed bleomycin. The following data were tabulated for each patient: hospital length of stay (LOS), clinical response, and the presence/absence of airway swelling requiring prolonged intubation. Univariate analysis was performed. P < 0.05 was considered significant. RESULTS Thirty-one percent (5 of 16) of treatments required endoscopic guidance. Eighty-seven percent (7 of 8) of airway VMs treated with ethanol caused significant airway swelling compared to 0% (0 of 8) of airway VMs treated with bleomycin (P = 0.0004). The LOS was significantly greater for ethanol (5 ± 0.3 days) than for bleomycin (1 day, P = 0.001). Ninety-four percent (15 of 16) of airway VM treatments had at least a partial clinical response. There was no significant difference in clinical response between ethanol and bleomycin (P = 0.30). CONCLUSION Endoscopic and image-guided needle placement may be necessary to treat deep airway VMs. Bleomycin may cause less significant airway swelling than ethanol. This may reduce hospital LOS and prolonged intubation. Our results should be interpreted with caution because this is a very small retrospective study. Additional investigation is needed to establish safety and efficacy of foamed bleomycin. LEVEL OF EVIDENCE 4. Laryngoscope, 126:2726-2732, 2016.

In children with biliary atresia, hepatoportoenterostomy (HP) is recommended to improve bile flow. Biliary strictures are known potential complications after HP, which can again impair bile flow often leading to biliary cirrhosis and liver transplantation. In patients who are status post HP and have biliary strictures, nonsurgical therapeutic options such as endoscopic dilation can pose technical difficulties due to altered anatomy. Percutaneous transhepatic cholangiography with cholangioplasty is a valuable tool for obstructive cholangiopathies, but to our knowledge this has not been previously demonstrated to be successful in patients with multiple intrahepatic biliary strictures status post HP. Herein, we present 3 patients status post HP who presented with multiple intrahepatic biliary strictures and underwent successful percutaneous transhepatic cholangiography with cholangioplasty.

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.

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

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.

Purpose To investigate pulmonary venous anomaly as a cause of pulmonary arteriovenous malformation (PAVM) misdiagnosis. Materials and methods We reviewed adult patients within a 7.5-year period with CT scans initially diagnosed with PAVM and subsequent conventional pulmonary angiograms. Results Pulmonary arteriography showed no PAVM on arterial phase for 10 out of 99 patients, comprising the misdiagnosed group. Four misdiagnosed patients had pulmonary venous anomalies and six had nodular lesions on CT. Conclusion Pulmonary venous anomalies are vascular mimics of PAVMs that may lead to misdiagnosis. Optimal CT technique and careful imaging review are necessary to minimize inappropriate invasive angiography.

PurposePulmonary arteriovenous malformations (pAVMs) are major contributors to morbidity and mortality in hereditary hemorrhagic telangiectasia (HHT). Mutations in ENG and ACVRL1 underlie the vast majority of clinically diagnosed cases. The aims of this study were to characterize and compare the clinical and morphologic features of pAVMs between these two genotype groups.MethodsSixty-six patients with HHT and affected family members were included. Genotype, phenotypic data, and imaging were obtained from medical records. Morphologic features of pAVMs were analyzed using computed tomography angiography. HHT symptoms, pAVM imaging characteristics, frequency of procedural intervention, and HHT severity scores were compared between ENG and ACVRL1 genotype groups.ResultsENG mutation carriers were more likely than ACVRL1 mutation carriers to have pAVMs (P 0.001) or multiple lesions (P = 0.03), and to undergo procedural intervention (P = 0.02). Additionally, pAVMs in ENG carriers were more likely to exhibit bilateral lung involvement and growth over time, although this did not reach statistical significance. The HHT severity score was significantly higher in ENG than in ACVRL1 (P = 0.02).ConclusionThe propensity and multiplicity of ENG-associated pAVMs may contribute to the higher disease severity in this genotype, as reflected by the HHT severity score and the frequency of interventional procedures.

Classification of vascular anomalies (VAs) is challenging due to overlapping clinical symptoms, confusing terminology in the literature and unfamiliarity with this complex entity. It is important to recognize that VAs include two distinct entities, vascular tumours (VTs) and vascular malformations (VaMs). In this article, we describe SE Mitchell Vascular Anomalies Flow Chart (SEMVAFC), which arises from a multidisciplinary approach that incorporates clinical symptoms, physical examination and magnetic resonance imaging (MRI) findings to establish International Society for the Study of Vascular Anomalies (ISSVA)-based classification of the VAs. SEMVAFC provides a clear visual pathway for physicians to accurately diagnose Vas, which is important as treatment, management, and prognosis differ between VTs and VaMs.

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 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.

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.

Cystic fibrosis (CF) is a recessive genetic disease characterized by dehydration of the airway surface liquid and impaired mucociliary clearance. As a result, individuals with the disease have difficulty clearing pathogens from the lung and experience chronic pulmonary infections and inflammation. There may be intermittent pulmonary exacerbations or acute worsening of infection and obstruction, which require more intensive therapies. Hemoptysis and pneumothorax are complications commonly reported in patients with cystic fibrosis.This document presents the CF Foundation's Pulmonary Therapies Committee recommendations for the treatment of hemoptysis and pneumothorax.The committee recognized that insufficient data exist to develop evidence-based recommendations and so used the Delphi technique to formalize an expert panel's consensus process and develop explicit care recommendations.The expert panel completed the survey twice, allowing refinement of recommendations. Numeric responses to the questions were summarized and applied to a priori definitions to determine levels of consensus. Recommendations were then developed to practical treatment questions based upon the median scores and the degree of consensus.These recommendations for the management of the patient with CF with hemoptysis and pneumothorax are designed for general use in most individuals but should be adapted to meet specific needs as determined by the individuals, their families, and their health care providers. It is hoped that the guidelines provided in this manuscript will facilitate the appropriate application of these treatments to improve and extend the lives of all individuals with cystic fibrosis.

Objectives/Hypothesis: Hereditary hemorrhagic telangiectasia (HHT)-related epistaxis leads to alterations in social functioning and quality of life. Although more than 95% experience epistaxis, there is considerable variability of severity. Because no standardized method exists to measure epistaxis severity, the purpose of this study was to determine factors associated with patient-reported severity to develop a severity score. Study Design: Prospective, survey-based study. Methods: HHT care providers and a focus group of patients were interviewed to determine epistaxis-associated factors. From this, an electronic survey was developed and administered to patients with HHT. Descriptive analyses were performed with calculations of means and medians for continuous and proportions for categorical variables. Multiple ordinal logistic and linear regression models were developed to determine risk factors for epistaxis severity. Results: Nine hundred respondents from 21 countries were included. Eight hundred fifty-five (95%) subjects reported epistaxis. The mean (standard deviation) age was 52.1 (13.9) years, and 61.4% were female. Independently associated risk factors for self-reported epistaxis severity included epistaxis frequency (odds ratio [OR] 1.57), duration (OR 2.17), intensity (OR 2.45), need for transfusion (OR 2.74), anemia (OR 1.44), and aggressiveness of treatment required (OR 1.53, P < .001 for all). Conclusions: Risk factors for increasing epistaxis severity in patients with HHT include frequency, duration, and intensity of episodes; invasiveness of prior therapy required to stop epistaxis; anemia; and the need for blood transfusion. From these factors, an epistaxis severity score will be presented. Laryngoscope, 2010

Littoral cell angioma (LCA) is a rare primary splenic vascular tumor originating from littoral cells lining the splenic red pulp sinuses. LCAs are rarely seen in children. We present the US, CT, and MRI findings including diffusion-weighted imaging (DWI) in a 2-year-old boy with histologically proven LCA. Previous studies on liver lesions have shown that DWI allows differentiation of vascular tumors from primary neoplasms and metastatic disease. The current case indicates that increased ADC values within the splenic lesions suggest a vascular etiology, which might help narrow the differential diagnosis.

Background Although iliac vein thrombus is uncommon in adolescents, it can present with pelvic inflammatory disease (PID) symptoms.

Lymphatic malformations of the femur are rare benign conditions, with few published reports in the literature. Herein is presented an ambulatory 19-year-old male subject with a painful lymphatic malformation of the distal femur characterized by a serpiginous intraosseous and extraosseous extent. Laboratory and physical examination were unremarkable except for a right knee joint effusion and patellar grind. The malformation was sclerosed twice with doxycycline, resulting in patient-reported decreased associated pain from an intensity of 9 of 10 at presentation to 2 of 10 at 9-month follow-up.

To examine the value of magnetic resonance (MR) imaging for predicting the occurrence of skin burns in patients with venous malformations who undergo percutaneous alcohol embolization was the objective of the study. Pre-procedural MR imaging at 1.5 T from 40 patients with venous malformations who had undergone percutaneous alcohol embolization was retrospectively reviewed by two observers for these features: anatomic location, definition (well-defined or ill-defined), and the presence of skin, subcutaneous tissue, muscle, tendon, bone, joint, and deep venous system involvement. One observer recorded the length of skin involvement and volume of the malformation. Univariate and multivariate analysis tests were used to determine whether an association between the occurrence of skin burns and MR imaging features existed. The anatomic locations of the venous malformations were the lower extremity (20 out of 40), upper extremity (11 out of 40), trunk (four out of 40), head/neck (three out of 40) and pelvis (two out of 40). Of the 40 subjects, 15% (six out of 40) experienced skin burns. There was a significant association between the absence of muscle involvement (p = 0.0198) as well as the length of skin involvement (p = 0.027), with the occurrence of skin burns. Malformation size and all other features were not significantly associated with skin burns. Skin burns in patients with venous malformations treated with alcohol embolization are associated with the length of skin involvement and with the absence of deeper tissue involvement, as depicted on MR imaging.

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.Retrospective cross-sectional study in patients with a diagnosis of HHT using Curacao criteria or genetic testing.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.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, P0.001). 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.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.4. Laryngoscope, 126:1029-1032, 2016.

Aim To evaluate the relative accuracy of contrast-enhanced time-resolved angiography with interleaved stochastic trajectories versus conventional contrast-enhanced magnetic resonance imaging (MRI) following International Society for the Study of Vascular Anomalies updated 2014-based classification of soft-tissue vascular anomalies in the head and neck in children. Materials and methods Time-resolved angiography with interleaved stochastic trajectories versus conventional contrast-enhanced MRI of children with diagnosis of soft-tissue vascular anomalies in the head and neck referred for MRI between 2008 and 2014 were retrospectively reviewed. Forty-seven children (0–18 years) were evaluated. Two paediatric neuroradiologists evaluated time-resolved MRA and conventional MRI in two different sessions (30 days apart). Blood-pool endovascular MRI contrast agent gadofosveset trisodium was used. Results The present cohort had the following diagnoses: infantile haemangioma ( n= 6), venous malformation (VM; n= 23), lymphatic malformation (LM; n= 16), arteriovenous malformation (AVM; n= 2). Time-resolved MRA alone accurately classified 38/47 (81%) and conventional MRI 42/47 (89%), respectively. Although time-resolved MRA alone is slightly superior to conventional MRI alone for diagnosis of infantile haemangioma, conventional MRI is slightly better for diagnosis of venous and LMs. Neither time-resolved MRA nor conventional MRI was sufficient for accurate diagnosis of AVM in this cohort. Conventional MRI combined with time-resolved MRA accurately classified 44/47 cases (94%). Conclusion Time-resolved MRA using gadofosveset trisodium can accurately classify soft-tissue vascular anomalies in the head and neck in children. The addition of time-resolved MRA to existing conventional MRI protocols provides haemodynamic information, assisting the diagnosis of vascular anomalies in the paediatric population at one-third of the dose of other MRI contrast agents.

Injury to the renal vasculature is an uncommon occurrence in the setting of blunt abdominal trauma. The authors report the case of a 2-year-old girl who developed occlusion from an intimal flap in a renal artery (grade IV renal injury) after blunt abdominal trauma that was treated with a stent placement. The stent procedure was technically successful in establishing renal artery flow. However, several months after the procedure, the patient developed stenosis within the stent and ultimately required a nephrectomy. To the authors' knowledge, this is the first report of percutaneous management of an acute intimal flap in a renal artery in a 2-year-old.

Purpose To assess the safety and quality of life in adult patients undergoing cecostomy tube placement. Materials and Methods Percutaneous cecostomy was performed in 23 adults (10 men and 13 women) with neurogenic bowel for whom noninvasive therapeutic approaches for chronic refractory constipation or fecal incontinence had failed. Mean patient age was 41 years (range, 19–74 y). A retrospective, standardized questionnaire evaluated satisfaction and quality of life before and after cecostomy. Results All 23 cecostomy procedures were technically successful with no intraprocedural complications. At a mean follow-up of 42 months (range, 1–160 mo), there was one (5%) major complication, a pericecal abscess. One or more minor complications in 11 of 23 (48%) patients included leaking around the tube (5 of 23; 22%) and partial or complete dislodgment of the tube (3 of 23; 13%). In all cases, the cecostomy tube was exchanged successfully. Satisfaction scores improved from a mean of 2.2 points (range, 0–6 points; median, 1.5) to 7.6 points (range, 4–10 points; median, 8). The percentage of patients using laxative softeners decreased from 74% to 40%, and patients requiring assistance decreased from 52% to 35% after cecostomy placement. Conclusions Percutaneous cecostomy is a safe procedure for the management of adult patients. Patients are able to achieve greater independence in their activities of daily living and are highly satisfied with the outcomes.

A gastro-jejunal (G-J) feeding tube is a safe and useful temporizing method of providing enteral access in children. Although G-J tubes are often used to obviate the need for a surgical jejunostomy, their long-term use is often associated with mechanical failure.To review the clinically effective durability of G-J feeding tubes in providing enteral access in children.We performed a retrospective review of 102 patients at the Johns Hopkins Children's Center from 1994-2003 whose underlying diagnosis necessitated the need for postpyloric enteral access.Long-term follow-up was obtained in 85 (48 M; 37 F) patients with a median (range) age of 2.0 (0.1-18.0) yr. The most common indication for G-J tube placement was gastroesophageal reflux with aspiration in 51 patients and feeding intolerance and vomiting in 19 patients. The mean (range) number of tube replacements was 2.2 (1-14) over a median (range) duration of follow-up of 39 (2-474) days. The indication for G-J tube replacement included: tube displacement (58), a clogged tube (41), and a cracked tube or ruptured balloon (35). In 52 cases, the cause for G-J tube replacement was undetermined.G-J feeding tubes are associated with the frequent need for tube maintenance and replacement and may not be the most feasible clinical option in providing long-term (1 month) enteral access in children intolerant to gastrostomy tube feeds. Future studies are needed to develop innovative percutaneous jejunostomy tube placement techniques that facilitate long-term enteral access.

Refractory ascites is a serious complication of advanced cirrhosis with a 1-year transplant-free survival of 20–50%. The aim of our study was to investigate the short- and long-term effects of transjugular intrahepatic portosystemic shunt (TIPS) in the management of refractory ascites. In all 65 patients (39 M, 26 F; Child B 55%, Child C 45%, mean MELD score 14.8 ± 6.6) with liver disease (alcoholic 40%, cryptogenic 20%, HCV 14%, others 26%) and refractory ascites were included in this study. Forty-eight (74%) patients had no signs of hepatic encephalopathy (HE), 16 (24%) had mild and 1 (2%) had moderate HE before TIPS; 28 (43%) had mild (>1.2 and 2.4 mg/dl) renal dysfunction. Mean follow-up was 55.5 ± 70.2 weeks. Treatment success, defined as complete response, partial response, and no response, and survival was determined at 3 weeks, and 3, 6, 12, 24, and 36 months after TIPS. TIPS was successful in all patients. Mean portal venous pressure gradient improved significantly after TIPS (24 ± 8 to 10 ± 4). During follow-up, 40 (58%) patients died and 17 (27%) patients had liver transplantation (OLT); 20 (31%) patients had 38 shunt revisions due to lack of initial response or recurrence of ascites. The response was assessed in patients who were alive, without OLT, at each time point. Complete response was seen in 10%, 23%, 17%, 11%, 22% and 33%; partial response was seen in 46%, 46%, 40%, 44%, 28%, and 8%; and no response was seen in 44%, 31%, 43%, 41%, 39%, and 50% at 3 weeks, and 3, 6, 12, 24, and 36 months respectively. There were no pre-TIPS variables that could predict the response at 3 weeks, 3 months, or 6 months. Mild HE was seen in 8 (12%) patients and severe HE was seen in 16 (25%) immediately after TIPS. The mortality at 3 weeks, and 3, 6, 12, 24, and 36 months was 26%, 38%, 46%, 51%, 57%, and 58%, respectively. Three-week (P = 0.01) and 3-month (P = 0.04) mortality was higher in Child C patients compared to Child B. However, there were no independent predictors of survival on multivariate analysis at 3 or 6 months. Child-Pugh score 3 weeks after TIPS was a strong predictor of mortality. In conclusion, in patients with refractory ascites, TIPS was associated with a high mortality and morbidity. The response and the mortality were both unpredictable on the basis of pretransplant variables.

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.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.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 (ESS4), 285 (47.2%) reported moderate epistaxis (≥4 ESS7), 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 (p0.001, p0.001).The ESS is a major determinant of HR-QoL and should be considered as a measurement of treatment efficacy in HHT-related epistaxis.

Pulmonary arteriovenous malformations (PAVMs) are congenital abnormal communications between the pulmonary artery and pulmonary vein, forming a fight-to-left shunt. Patients present with symptoms of hypoxemia, paradoxical emboli, and/or, less commonly, rupture of the PAVM and hematemesis. They can occur sporadically or in association with heredity hemorrhagic telangectasia (HHT) [1]. Embolotherapy is currently the treatment of choice. Investigators have shown a significant improvement in PaO 2 after embolization with very few complications [1-3]. It is critical that the angioarchitecture of the PAVM be elucidated prior to embolization. Historically, this was done exclusively by angiography. With the advent of helical computed tomography, a noninvasive mapping technique has emerged. The angioarchitecture of PAVMs can be accurately depicted by analysis of both the axial and 3-D reconstructed images 14, 5]. The following is a pictorial review of PAVMs, with emphasis on the complex type, and the benefits of using 3-D computed tomography (CT) volume rendering for diagnosis, angioarchitecture characterization, and preembolization planning [6].

OBJECTIVE: This study was designed to evaluate the total costs associated with repair of laparoscopic cholecystectomy (LC)-related bile duct injuries. SUMMARY BACKGROUND DATA: The popularity of LC with both patients and surgeons is such that this procedure now exceeds open cholecystectomy by a ratio of approximately 4 to 10:1. However, costs associated with LC-related injuries, particularly regarding treatment patterns, have up to now not been explored fully. METHODS: The complete hospital and interventional radiology (IR) billing records for 49 patients who have completed treatment for laparoscopic cholecystectomy-related bile duct injuries were divided into 8 categories. These records were totaled for comparison of costs between patient groups that experienced different injuries and treatment patterns. RESULTS: Patients with LC-related bile duct injuries were billed a mean of $51,411 for all care related to repair of their bile duct injury. Patients incurred an average of 32 days of inpatient hospitalization and 10 outpatient care days. Postoperative treatment included long-term chronic biliary intubation averaging 378 days. Two patients (4%) died as a result of their LC-related complications. Patients with bile duct injuries that were recognized immediately at the time of the initial surgery ultimately experienced a total cost for their repair and hospitalization of 43% to 83% less than for patients in whom recognition of the injury was delayed (p < 0.019 to 0.070). In addition, the total hospitalization and outpatient care days was reduced by as much as 76% with early recognition of an iatrogenic injury. CONCLUSIONS: Repair of cholecystectomy-related bile duct injuries can run 4.5 to 26.0 times the cost of the uncomplicated procedure and carries a significant mortality rate. Intraoperative recognition of such an injury with immediate conversion to an open procedure for definitive repair can result in significant cost savings and relates directly to a decreased morbidity, mortality, length of hospitalization, and number of outpatient care days.

We report the outcomes of a small series of patients with refractory chylous ascites following urologic surgery treated with lymphangiography +/- sclerotherapy.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.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.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.

Evaluate retrospectively the long-term primary patency of directional atherectomy (DA) in the femoropopliteal arteries.DA was used alone in 59 patients (47%) or in combination with predilatation to allow passage of the device (43%) or after thrombolysis (10%) to treat 127 (93%) excentric atherosclerotic stenoses and nine (7%) occlusions of the femoropopliteal arteries. Forty-eight patients were followed by telephone interview, scheduled outpatient visits, color-flow Doppler evaluation, and angiography for 1-36 months (mean 16.9 months).Technical success (reduction of the stenosis or occlusion to less than 30% luminal diameter) was achieved in 110 lesions (80.3%) during 48 procedures in 37 patients. Mean luminal diameter was increased 54% with a concomitant increase in mean ankle/brachial indices of 0.33. According to Kaplan-Meier survival curves, patency at 12 and 24 months was 88% and 75%, respectively. When patients who retained patency but developed restenosis were excluded, the probability of patency at 12, 24, and 36 months was 76%, 58%, and 32%, respectively. Major and minor complications occurred in 15 (21.4%) procedures each for a total complication rate of 42.8%.Based on our results, DA is an effective method for percutaneous treatment of atherosclerotic disease involving the femoropopliteal arteries. It has similar patency but a relatively high complication rate compared with PTA.

Purpose The authors summarize their 11-year experience with percutaneous varieocele occlusion at the Johns Hopkins Hospital. Patients and Methods Data were obtained from the patients’ medical records and from a mailed questionnaire. Most of the data analysis is based on the 182 patients who responded to the questionnaire. Results Most of the occlusions were performed for infertility. The mean length of time couples had been attempting to conceive was approximately 44 months. Occlusion was technically successful in 95.7% of cases. Patients were followed up for a mean period of 59 months. Success is difficult to define because many patients and/or their wives received additional infertility treatment. Fifty-seven percent of all couples and 60% of a subgroup of couples who received no other treatment eventually conceived. Conclusion Percutaneous occlusion is a well-established treatment for varicoceles. Pregnancy rates and recurrence rates are comparable to those following surgical varicocelectomy. It is unlikely that resultant pregnancies occur from random chance alone.

Purpose The authors evaluated the technical success and immediate and long-term results of percutaneous varicocele embolotherapy in the adolescent population. Patients and Methods Fifty-nine adolescent patients were referred for outpatient spermatic venography and possible varicocele embolotherapy. Embolization was attempted with use of detachable balloons, coils, "sandwiched" dextrose, or a combination of these techniques. Data regarding follow-up were obtained through telephone inteviews or mailed questionnaires. Results The technical success rate for spermatic vein occlusion was 90%. Follow-up, obtained in 79% of the patients, ranged from 6 months to 8.75 years (mean, 4 years). Thirty-nine of 42 patients (93%) reported disappearance ( n = 31) or only a slight, asymptomatic residual varicocele ( n = 8). Three patients reported a recurrence of their varicocele. Complications occurred in three of 59 cases (5%), none had any long-term sequelae. In six cases, embolization was not feasible because of multiple collateral vessels or venous spasm. Conclusions Given the convenience of performing the procedure on an outpatient basis, the rapid recovery time, and long-term success and complication rates comparable to those with surgical ligation, we believe spermatic venography and percutaneous embolization is the treatment modality of choice for adolescent varicocele.

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.