Baseline Quality of Life in Pediatric Patients With Low-flow Vascular Malformations.

OBJECTIVE: The aim of this study was to quantify and describe baseline patient and parent-proxy health-related quality of life scores in patients with low-flow vascular malformations at a single, tertiary-care vascular anomalies clinic. STUDY DESIGN: This is a retrospective study of data collected on patients with low-flow vascular malformations between the ages of 2 to 25 who were seen at a single, tertiary-care center vascular anomalies clinic. A total of 266 patients are included in this study. RESULTS: Patients with lymphatic malformations report decreased quality of life scores as compared with venous malformations in the emotional, psychological, school, and social domains. Patients with lower extremity malformation report decreased quality of life scores as compared with head/neck, trunk, upper extremity, and multifocal malformations; most notably in the physical domain. CONCLUSIONS: Treatment of low-flow vascular malformations should aim to improve patient quality of life. The use of standardized health-related quality of life measures in this study quantifies baseline quality of life scores among patients with low-flow vascular malformations.

Introduction to interventional oncology: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

Interventional radiology (IR) is a valuable resource when caring for pediatric solid tumor patients. As minimally invasive, image-guided procedures become more relied upon to answer challenging diagnostic questions and provide alternative therapeutic options, IR is poised to become a contributing member of the multidisciplinary oncology team. Improved imaging techniques allow for better visualization during biopsy procedures, transarterial locoregional treatments have the potential to deliver targeted cytotoxic therapy while limiting systemic side effects, and percutaneous thermal ablation can be used to treat chemo-resistant tumors of various solid organs. Additionally, interventional radiologists are able to perform routine, supportive procedures for oncology patients that include central venous access placement, lumbar punctures, and enteric feeding tube placements with high levels of technical success and excellent safety profiles.

Introduction to interventional radiology's role in palliative care for children with cancer: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

As palliative care continues to be an area of increasing emphasis in pediatric oncology programs, it is important to raise awareness about minimally invasive, image-guided procedures that can supplement more conventional palliative interventions, such as systemic analgesics, external beam radiation, and locoregional anesthesia. These procedures, when performed for appropriately selected patients, can often facilitate discharge from an inpatient facility, and help patients meet their end-of-life goals. This article specifically discusses three palliative procedures performed by interventional radiologists that can assist pediatric palliative care teams in: (a) percutaneous thermal ablation of painful bone metastases, (b) cryoneurolysis, and (c) tunneled drainage catheter placement for malignant pleural effusions and ascites.

ACR Appropriateness Criteria® Crohn Disease-Child.

Crohn disease is an inflammatory condition of the gastrointestinal tract with episodes of exacerbation and remission occurring in children, adolescents, and adults. Crohn disease diagnosis and treatment depend upon a combination of clinical, laboratory, endoscopic, histological, and imaging findings. Appropriate use of imaging provides critical information in the settings of diagnosis, assessment of acute symptoms, disease surveillance, and therapy monitoring. Four variants are discussed. The first variant discusses the initial imaging for suspected Crohn disease before established diagnosis. The second variant pertains to appropriateness of imaging modalities during suspected acute exacerbation. The third variant is a substantial discussion of recommendations related to disease surveillance and monitoring of Crohn disease. Finally, panel recommendations and discussion of perianal fistulizing disease imaging completes the document. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Percutaneous image-guided treatment of aneurysmal bone cysts: is there a superior treatment option?

BACKGROUND: Aneurysmal bone cysts (ABCs) are often treated with intralesional surgery (curettage) with or without adjuvant treatments. Side effects and conflicting results regarding recurrence rates do not suggest one clearly superior therapy. Percutaneous therapeutic options including sclerotherapy and thermal ablation have gained popularity as potential alternatives. OBJECTIVE: The purpose of this retrospective review is to report this institution's experience and results of various image-guided minimally invasive treatments in a single institution series of cases referred to interventional radiology by orthopedic surgery after surgical failure or in patients with anatomically challenging ABCs. MATERIALS AND METHODS: This study identified all patients ≤18 years old who received percutaneous therapy for an ABC, including cryoablation, doxycycline sclerotherapy, microwave ablation or a combination of these modalities. Procedural details, complications, imaging follow-up and clinical follow-up were analyzed. RESULTS: A total of 21 patients received 41 procedures, with major complications seen in 7.7% (3/39) of procedures involving cryoablation or doxycycline sclerotherapy. Patients receiving cryoablation required an average of 1.7 procedures (median: 1 procedure, range: 1-4 procedures) while patients receiving doxycycline sclerotherapy required an average of 3 procedures (median: 2 procedures, range: 1-6 procedures). Patients were followed clinically and with computed tomography or magnetic resonance imaging (average: 23.9 months, range: 3.9-68.3 months). Follow-up imaging demonstrated improvement in 17 (85%) patients. Clinically, 93.8% (15/16) of patients who presented with fracture or pain had markedly reduced or absent pain as well as no fractures. CONCLUSION: Percutaneous image-guided treatment of ABCs demonstrates a favorable efficacy and safety profile. Adding cryoablation may lead to fewer total procedures than using doxycycline sclerotherapy alone.

Coagulopathy and related complications following sclerotherapy of congenital venous malformations.

BACKGROUND: Congenital venous malformations (VMs) are low-flow vascular anomalies that can cause coagulation abnormalities. This phenomenon, referred to as localized intravascular coagulopathy (LIC), is characterized by elevated D-dimer, hypofibrinogenemia, and/or thrombocytopenia. Increased risk for LIC includes patients with an extensive VM, multifocal VM, and Klippel-Trenaunay and CLOVES syndromes. Peri-procedural anticoagulation has been given to prevent complications from LIC in patients undergoing surgical/interventional procedures; however, the rate of clinically relevant complications from sclerotherapy is largely unknown. The purpose of this study is to describe a single-institution's incidence of LIC in patients with VMs and coagulopathy-related complications following sclerotherapy. DESIGN/METHODS: Retrospective chart review of patients, 0-21 years of age, with VM who underwent sclerotherapy without peri-procedural anticoagulation and had coagulation profiles evaluated within 1 month prior to sclerotherapy. DATA COLLECTED: diagnosis type (high vs. low risk for LIC), coagulation profile (including PT/PTT, D-dimer, fibrinogen, and platelet count), sclerosant used, and post-procedure outcomes. Coagulopathy-related complications included clinically relevant bleeding, deep vein thrombosis (DVT), and pulmonary embolism (PE). RESULTS: N = 138 patients; 59.4% were female. The most common location of VM was the lower extremity (47.8%; 66/138). Of patients with high-risk VMs (29/138 [21%]), 11/29 (37.9%) had laboratory values consistent with LIC, whereas 5% (5/109) of low-risk VMs had LIC. In sum, 492 sclerotherapy procedures were performed with no complications of bleeding, DVT, or PE. CONCLUSION: Patients undergoing sclerotherapy for VM with abnormal coagulation profiles may not require peri-procedural low molecular weight heparin (LMWH). Further studies are needed to precisely define which patients would benefit from anticoagulation.

Percutaneous Core Needle Biopsy: Considerations in the Pediatric Patient.

Image-guided percutaneous biopsy is the cornerstone of solid tissue diagnosis. The ability to safely sample tissue in locations that previously required surgery or necessitated empiric therapy has allowed for more personalized treatment options, as well as more rapid development of novel therapeutics. In children, these same advantages are accompanied by a smaller margin for error and rapidly expanding indications. The intent of this review is to outline the role of image-guided biopsy in the management of childhood disease, how this role is changing, and the practical aspects of managing and performing pediatric biopsies.

Percutaneous cryoablation of chondroblastoma and osteoblastoma in pediatric patients.

BACKGROUND: To review the safety and efficacy of percutaneous cryoablation for the treatment of chondroblastoma and osteoblastoma in the pediatric and adolescent population. MATERIALS AND METHODS: A retrospective review from 2016 to 2020 was performed to evaluate clinical and imaging response to percutaneous cryoablation in 11 symptomatic patients with diagnosis of chondroblastoma and osteoblastoma treated from two pediatric hospitals with at least 12-month follow-up. Technical success (correct needle placement and potential full coverage of the tumor with the planned ablation zone) and clinical success (relief of the symptoms) were evaluated. The primary objective was to alleviate pain related to the lesion(s). Immediate and late complications were recorded. Patients were followed in clinic and with imaging studies such as MRI or CT for a minimum of 6 months. RESULTS: A total of 11 patients were included (mean 14 years, age range 9-17; male n = 8). Diagnoses were osteoblastoma (n = 4) and chondroblastoma (n = 7). Locations were proximal humerus (n = 1), femur condyle (n = 1), and proximal femur (n = 1) tibia (n = 3), acetabulum (n = 3), thoracic vertebra (n = 1) and lumbar vertebra (n = 1). Cryoablation was technically successful in all patients. Clinical success (cessation of pain) was achieved in all patients. No signs of recurrence were observed on imaging follow-up in any of the patients. One of the patients developed periprocedural right L2-L3 transient radiculopathy as major immediate complication. CONCLUSIONS: Percutaneous image-guided cryoablation can be considered potentially safe and effective treatment for chondroblastoma and osteoblastoma in children and adolescents.

Undiagnosed Metachromatic Leukodystrophy Presenting as Severe Gastrointestinal Bleeding and Cholestasis from Hemobilia.

Metachromatic leukodystrophy (MLD) is a neurodegenerative disorder caused by the accumulation of lipids called sulfatides throughout the nervous system. Sulfatides can also collect in other organs throughout the body including the gallbladder where they form polyps. Gallbladder polyps rarely have been found to bleed in patients with known MLD, presumably due to polyp shearing. Here we present a case of a child with autism presenting with severe gastrointestinal bleeding and direct hyperbilirubinemia, requiring significant resuscitation and biliary drain placement to tamponade ongoing bleeding. Subsequent neurologic and genetic investigation led to the diagnosis of MLD, with laparoscopic cholecystectomy revealing extensive, elongated gallbladder polyps. Clinicians who care for patients with MLD, including gastroenterologists who manage their progressive oropharyngeal dysphagia, should be aware of the risk for this life-threatening complication. Moreover, pediatric gastroenterologists and hepatologists should maintain a high index of suspicion for MLD in new patients presenting with developmental regression and gastrointestinal bleeding.

Simultaneous Presentation of Crohn's Disease and Takayasu Arteritis in a Teenage Patient.

A 14-year-old female with no significant medical history presented with hypertensive urgency, in the setting of 4 to 6 weeks of diarrhea, abdominal pain, headaches, anemia, weight loss, and high blood pressures. Her evaluation revealed signs of a systemic inflammatory process that was most suspicious for inflammatory bowel disease. However, when her hypertension was evaluated with a renal Doppler ultrasound, there were signs of narrowing, stenosis, and hypoplasia that led to a diagnostic angiogram of the abdominal aorta. Full body positron emission tomography scan revealed multiple areas of stenosis and aortic thickening with enhancement compatible with Takayasu arteritis. She received prednisone, methotrexate, and infliximab with marked improvement in her clinical symptoms and inflammatory markers.

Congenital Limb Overgrowth Syndromes Associated with Vascular Anomalies.

Congenital limb length discrepancy disorders are frequently associated with a variety of vascular anomalies and have unique genetic and phenotypic features. Many of these syndromes have been linked to sporadic somatic mosaicism involving mutations of the phosphoinositide 3-kinase (PI3K)/protein kinase B (AKT)/mammalian target of rapamycin (mTOR) pathway, which has an important role in tissue growth and angiogenesis. Radiologists who are aware of congenital limb length discrepancies can make specific diagnoses based on imaging findings. Although genetic confirmation is necessary for a definitive diagnosis, the radiologist serves as a central figure in the identification and treatment of these disorders. The clinical presentations, diagnostic and imaging workups, and treatment options available for patients with Klippel-Trenaunay syndrome, CLOVES (congenital lipomatous overgrowth, vascular anomalies, epidermal nevi, and scoliosis/spinal deformities) syndrome, fibroadipose vascular anomaly, phosphatase and tensin homolog mutation spectrum, Parkes-Weber syndrome, and Proteus syndrome are reviewed. ©RSNA, 2019.

Technical Feasibility and Clinical Effectiveness of Transjugular Intrahepatic Portosystemic Shunt Creation in Pediatric and Adolescent Patients.

PURPOSE: To examine the technical feasibility and clinical efficacy of transjugular intrahepatic portosystemic shunt (TIPS) creation in children and adolescents. MATERIALS AND METHODS: Retrospective review was performed of 59 patients (mean age 12.6 y [range, 1.5-20 y], mean weight 47.5 kg [range, 11.4-112.2 kg], mean Model for End-stage Liver Disease/Pediatric End-stage Liver Disease score 12.5 [range, 6-33]) who underwent 61 TIPS attempts at 3 tertiary children's hospitals from 2001 to 2017 for acute esophageal or gastroesophageal variceal bleeding, primary and secondary prevention of variceal bleeding, and refractory ascites. Pediatric liver disease etiologies included biliary atresia, cystic fibrosis, and ductal plate anomalies. Technical, hemodynamic, and clinical success and patency rates were reported at 1, 6, 12, and 24 months. Statistical analysis evaluated reasons for clinical failure. Kaplan-Meier analysis measured clinical success, patency, and transplant-free survival. RESULTS: Technical success was 93.4% (57/61) in 59 consecutive patients. Most common TIPS indications were treating and preventing esophageal and gastroesophageal variceal bleeding (57/59; 96.6%). Hemodynamic success was 94% (47/50). Clinical success was 80.7% (45/56). Two-year clinical success for acute variceal bleeding and ascites was 94.1% and 100%, respectively. Overall patency at 1, 6, 12, and 24 months was 98.0%, 97.8%, 94.3%, and 91.3%. Two-year transplant-free survival was 88.8%. Overall and major complication rates were 21.2% (13/61) and 8.2% (5/61), with 3 mortalities. Gradient reduction < 12 mm Hg correlated with clinical success (P < .01). CONCLUSIONS: TIPS creation in pediatric patients is technically feasible and clinically efficacious for treatment and prevention of esophageal and gastroesophageal variceal hemorrhage. High 2-year clinical success, patency, and survival rates should encourage providers to consider portosystemic shunts as a bridge to liver transplantation.

Cone-beam computed tomography guidance with navigational overlay for percutaneous lung nodule biopsy.

BACKGROUND: Cone-beam CT is increasingly used in pediatric interventional radiology procedures. However, the feasibility or safety of using this mode of imaging guidance for percutaneous lung nodule biopsy in children has not been assessed. OBJECTIVE: To retrospectively evaluate safety and diagnostic accuracy of percutaneous lung nodule biopsy in people treated at a pediatric hospital using cone-beam CT with navigational overlay. MATERIALS AND METHODS: Thirty-six consecutive patients from two large tertiary-care children's hospitals with lung nodules of 48 mm or smaller underwent percutaneous lung nodule biopsy using cone-beam CT with navigational overlay. We evaluated patient demographics, pre- and post-biopsy diagnoses, number of biopsy passes, complications, radiation exposure and technical success. RESULTS: Percutaneous lung nodule biopsy was performed for 37 nodules in 36 patients (23 males, 13 females, median age 15.5 years, range 8 months to 23 years). One patient underwent biopsy of two nodules at a single procedure. Median patient weight was 55 kg (range 8-97 kg). Pre-biopsy diagnoses included metastatic sarcoma or other solid tumor (n=11), leukemia/lymphoma (n=13), infection (n=10), chronic granulomatous disease (n=2) and post-transplant lymphoproliferative disorder (PTLD; n=1). Mean number of passes was 5 (range 2-15). Mean pre-procedure international normalized ratio (INR) was 1.1 and platelet count 193 × 109/L. Diagnostic specimens were obtained in 32 of 36 patients (89%). Thirteen complications were encountered in 12 patients (33% of cohort), including 9 pneumothoraces (4 requiring chest tubes); 1 hemothorax, which required a chest tube; and 3 self-limited pulmonary hemorrhages. Mean and median radiation doses were as follows: fluoroscopy time 3 min and 2.4 min, dose-area product (DAP) (recorded in 31 patients) 23,402 Gy·cm2 and 12,453 Gy·cm2, and air kerma 88 mGy and 58 mGy. CONCLUSION: Percutaneous lung nodule needle biopsy can be performed accurately using cone-beam CT with navigational overlay.

Thrombogenic superior vena cava syndrome from long-standing central venous access in a 5-year-old patient treated with balloon-expandable stents.

Thrombogenic superior vena cava syndrome is an uncommon, dangerous complication of long-standing central venous catheter use. The increased use of central venous catheters has resulted in more non-malignant cases of superior vena cava syndrome across all age groups. We present a 5-year-old male with superior vena cava syndrome associated with acute onset of severe upper extremity and facial swelling, dyspnea, and a right subclavian central venous catheter malfunction. The patient was ultimately treated with percutaneous stenting of the superior vena cava with balloon-expandable Palmaz stents following unsuccessful angioplasty, catheter-directed thrombolysis, and percutaneous thrombectomy. This case highlights a relatively uncommon complication in children from long-term central venous catheter access and describes an emerging, minimally-invasive therapeutic alternative that allows for preservation of age-appropriate superior vena cava luminal diameter as patients grow.

Role of interventional radiology in managing pediatric liver tumors : Part 2: percutaneous interventions.

Hepatoblastoma and hepatocellular carcinoma (HCC) are the most common pediatric liver malignancies, with hepatoblastoma occurring more commonly in younger children and HCC occurring more commonly in older children and adolescents. Although surgical resection (including transplant when necessary) and systemic chemotherapy have improved overall survival rate for hepatoblastoma to approximately 80% from 30%, a number of children with this tumor type are not eligible for operative treatment. In contradistinction, pediatric HCC continues to carry a dismal prognosis with an overall 5-year survival rate of 30%. The Paediatric Hepatic International Tumour Trial (PHITT) is an international trial aimed at evaluating both existing and emerging oncologic therapies for primary pediatric liver tumors. Interventional radiology offers a number of minimally invasive procedures that aid in diagnosis and therapy of pediatric liver tumors. For diagnosis, the PHITT biopsy guidelines emphasize and recommend percutaneous image-guided tumor biopsy. Additionally, both percutaneous and endovascular procedures provide therapeutic alternatives that have been, to this point, only minimally utilized in the pediatric population. Specifically, percutaneous ablation offers a number of cytotoxic technologies that can potentially eradicate disease or downstage children with unresectable disease. Percutaneous portal vein embolization is an additional minimally invasive procedure that might be useful to induce remnant liver hypertrophy prior to extended liver resection in the setting of a primary liver tumor. PHITT offers an opportunity to collect data from children treated with these emerging therapeutic options across the world. The purpose of this manuscript is to describe the potential role of minimally invasive percutaneous transhepatic procedures, as well as review the existing data largely stemming from the adult HCC experience.

Clinical Efficacy of Percutaneous Transhepatic Portal Vein Angioplasty for Late-onset Portal Vein Stenosis in Pediatric Liver Transplant Patients.

BACKGROUND: Stenosis of the portal vein (PV) is a rare complication after liver transplantation (LT) in pediatric patients, and it adversely affects outcomes. We reviewed the safety and efficacy of percutaneous transhepatic balloon angioplasty (PTBA) as a treatment for post-LT late-onset PV stenosis (PVS). METHODS: Three hundred eighteen patients between the ages of 0 and 21 years received an LT from 2001 to 2016 at this tertiary center. Twenty-one children were evaluated for PVS using percutaneous portal venography. RESULTS: Of the 21, 19 patients (7 female, 12 male) with a median age of 12 years (7-15 years) were diagnosed with PVS and received PTBA. Two patients were excluded: one did not have PVS, and one received shunt surgery. Median time between LT and PTBA was 83 months (interquartile range, 49-138). For patients in whom pressure could be accurately measured (n = 9), mean PV pressure gradient was 6.3 mm Hg (SD, 5.0) preprocedure and 0.9 mm Hg (SD, 1.2) postprocedure. Mean percentage improvement in gradient across the stenotic region was 86.2% (SD, 15.9%; P < 0.01). At 12-month postprocedure, there was a mean improvement (pre-PTBA vs post-PTBA means) in bilirubin by 28.2% (0.6 mg/dL vs 0.4 mg/dL, P = 0.07), aspartate aminotransferase by 31.2% (116.3 IU/L vs 28.1 IU/L, P = 0.04), alanine aminotransferase by 40.7% (140.3 IU/L vs 28.6 IU/L, P = 0.07), γ-glutamyltransferase by 29.0% (337.2 IU/L vs 38.0 IU/L, P = 0.06) and platelets by 62.1% (128.3 vs 191.1 × 10/L, P = 0.03). The PV patency was successfully maintained in 18 of 19 patients for a median of 16 months (interquartile range, 5-35). One patient received a successful repeat procedure for restenosis at 6 weeks. CONCLUSION: Angioplasty for PVS after pediatric LT is a safe and effective treatment with good patency and improved clinical outcomes. Longer follow-up studies are required.