Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies-expert opinion consensus.

OBJECTIVE: Complicated lymphatic anomalies (CLAs) are chronic, progressive, and debilitating conditions that share clinical features, yet key elements for optimal evaluation and management have not been established. We aimed to formulate expert opinion consensus-based guidelines for comprehensive evaluation of CLAs. STUDY DESIGN: Patient support groups dedicated to CLAs organized an international conference for vascular anomaly experts from 16 specialties to address the objective. Participants received a set of questions before the meeting and reviewed the literature. Data extracted from international lymphatic anomaly registries were presented and the group separated for panel discussions during the conference. The recommendations achieving consensus within the panel were presented to the entire audience. Open debate occurred until majority approval was achieved. RESULTS: The expert group was composed of 52 physicians who defined the clinical elements required to evaluate and diagnose a CLA. The radiology panel established the preferred anatomical and functional imaging methods for diagnosis and the elements required to be described during interpretation. Two medical panels compiled the metabolic and hematologic tests at diagnosis and also recommended functional studies. The surgical group recommended precautions for biopsy and the pathology panel provided biopsy specimen processing guidelines. CONCLUSIONS: Patients with CLAs require a comprehensive and targeted diagnostic plan for appropriate management, prevention of complications, and conservation of resources. As this population is managed by diverse medical and surgical specialties, we offer an expert multidisciplinary consensus-based opinion on the current literature and on data extracted from international lymphatic anomaly registries.

Recanalization of Chronic Portal Vein Occlusion in Pediatric Liver Transplant Patients.

PURPOSE: To evaluate technical and clinical success and report long-term outcomes of portal vein (PV) recanalization in pediatric orthotopic liver transplant (OLT) patients with chronic PV occlusion. MATERIALS AND METHODS: This is a retrospective review of 15 OLT patients (5 males) with chronic PV occlusion who underwent PV recanalization (33 procedures) between October 2011 and February 2018. Median age was 4.5 years (range, 1-16 years); median weight was 16.6 kg (range, 11.5-57.3 kg). Median time interval from OLT to first intervention was 3.25 years (range, 0.6-15.7 years). Clinical presentations included hypersplenism (n = 12), gastrointestinal bleeding (n = 9), and ascites (n = 3). One patient had incidental diagnosis of PV occlusion. Primary, primary-assisted, and secondary patency at 3, 6, 12, and 24 months were evaluated. RESULTS: Technically successful PV recanalization and reduction of PV pressure gradient to ≤ 5 mm Hg was performed in 13/15 patients (87%). Ten of 15 (67%) patients had successful recanalization with the first attempt. Clinical success, defined as improvement in signs and symptoms of portal hypertension, was achieved in 12/13 (92%) patients. Five of 33 (15%) major complications (Society of Interventional Radiology class C), including perisplenic hematoma (n = 2), hemoperitoneum (n = 2), and hepatic artery pseudo aneurysm (n = 1), were managed with pain medication and blood product replacement. Median follow-up was 22 months (range, 1-77 months). Median primary patency was 5 months. Primary patency at 3, 6, 12, and 24 months was 53.8%, 46.2%, 38.5%, and 30.8%, respectively. Primary-assisted patency was 84.6%, 76.9%, 53.8%, and 46.2%, respectively. Secondary patency was 92.3%, 84.6%, 53.8%, and 46.2%, respectively. CONCLUSIONS: PV recanalization is a safe and effective minimally invasive option in the management of chronic PV occlusion after pediatric OLT.

Case Report of Accelerated Idioventricular Rhythm in the Interventional Radiology Suite: A Sticky Situation.

Embolization is a well-established method of treatment of arteriovenous malformations. Most procedures are performed under general anesthesia due to the painful nature of some sclerosing agents, procedure duration, and need for immobilization to allow precise injection. Familiarity with the possible side effects of these agents is important for the anesthesiologist to facilitate timely and successful management of intra- and postprocedural complications. This case report describes an unusual presentation of systemic migration of glue-lipiodol mixture during embolization of a calf arteriovenous malformation in an adolescent that resulted in accelerated idioventricular rhythm and its successful management leading to complete resolution.

Temporal Evolution and Management of Fast Flow Vascular Anomalies in PTEN Hamartoma Tumor Syndrome.

Phosphatase and tensin homolog (PTEN) hamartoma tumor syndrome (PHTS) is characterized by formation of recurrent benign tumors described as PTEN hamartoma of soft tissue that may contain fast flow vascular anomalies (FFVA). The purpose of this study is to review the temporal evolution and management of FFVA in PHTS. A retrospective review of 22 patients (9 males), age 1 to 18 (median 9) years diagnosed with PHTS at a tertiary care pediatric hospital between October 2002 and August 2017 revealed 4 patients with FFVA. Imaging, management, and treatment complications were reviewed. During median follow-up of 8 (range: 4-13) years, ultrasound and magnetic resonance imaging performed for recurrent pain, showed progressive increase in the size of hamartomas and development of new FFVA in three-fourth patients. Medical management included pain medications, oral sirolimus, and physical and psychiatric therapy. Surgical excision of hamartoma ( n = 1) resulted in recurrence within 3 months. Between 4 and 24 (average 1.5/year) embolizations were performed per patient. Pain related to FFVA responded well to embolization. Pain secondary to PTEN hamartoma responded poorly to percutaneous sclerosant injection, but demonstrated improvement with sirolimus. There was no correlation between serum sirolimus levels and frequency/timing of recurrence of FFVA/hamartoma. Complications included sclerosant migration into digital arteries ( n = 1), subclavian vein stenosis due to glue migration ( n = 1), oral mucositis ( n = 4), and elevated triglycerides ( n = 4). Patients with PHTS present with recurrent pain requiring life-long management with a multi-disciplinary team. Pain due to FFVA responds to embolization, and pain due to hamartoma responds to sirolimus. This improves quality of life, but does not prevent disease progression.

Esophageal stents in children: Bridge to surgical repair.

Management of complex esophageal problems in children is challenging. We report our experience with the use of esophageal stents in three children with esophageal strictures, leaks, or airway-esophageal fistulae refractory to conventional treatment. The stent played a key role in allowing extubation of a child with a large tracheo-esophageal-pleural fistula and in the resolution of pulmonary infection in a child with esophago-bronchial fistula, both followed by surgery. In the third child, with stricture, stents were complicated with migration, esophageal erosion, and esophago-bronchial fistula. In our experience, esophageal stents were useful mainly as a bridge to definitive surgical repair.

Dynamic contrast enhanced magnetic resonance lymphangiography: Categorization of imaging findings and correlation with patient management.

OBJECTIVE: To review the technical aspects and categorize the imaging findings of dynamic contrast enhanced magnetic resonance lymphangiography (DCMRL) and correlate the findings with patient management options. MATERIALS AND METHODS: A retrospective review of patients who underwent DCMRL between June 2012 and August 2017 at a tertiary care paediatric hospital was performed. Twenty-five DCMRL studies were performed in 23 patients (9 males, 13 females, 1 ambiguous gender) with a median age of 4 years (range: 1 month-29 years). DCMRL imaging findings were reviewed, categorized and the impact on patient management was studied. RESULTS: DCMRL was technically successful in 23/25 (92%) studies. DCMRL findings were categorized based on the status of central conducting lymphatics (CCL) and alternate lymphatic pathways as follows: Type 1 - normal CCL with no alternate lymphatic pathways, Type 2 - partial (2a) or complete (2b) non-visualization of CCL with reflux of contrast into alternate pathways and Type 3 - normal CCL with additional filling of alternate pathways. Type 1 DCMRL patients (n = 5) were reassured and conservative management was continued, Type 2 patients (n = 10) had evidence of CCL obstruction hence thoracic duct ligation or embolization was avoided and other options such as lymphatic fluid diversion using Denver® shunt or lympho-venous anastomosis were used, and Type 3 patients (n = 8) were evaluated for elevated central venous pressure as a cause of lymphatic backflow in addition to Denver® shunt, lympho-venous anastomosis, thoracic duct ligation or embolization. CONCLUSION: DCMRL is an evolving imaging technique for understanding abnormalities of the central conducting lymphatics. Categorization of imaging findings may be helpful in guiding selection of management options.

Equivalent success and complication rates of tunneled common femoral venous catheter placed in the interventional suite vs. at patient bedside.

BACKGROUND: Femoral tunneled central line placement in the pediatric population offers an alternative means for intravenous (IV) access, but there is concern for higher complication and infection rates when placed at bedside. OBJECTIVE: To describe the complications and infection outcomes of primary femoral tunneled central venous catheter placement in the interventional radiology suite compared to the portable bedside location at a single tertiary pediatric institution. MATERIALS AND METHODS: We conducted a retrospective review comparing interventional radiology suites vs. bedside primary tunneled common femoral vein central line placement (January 2014 to December 2015). We identified 244 primary femoral placements in pediatric patients, ages 1 day to 18 years, using our electronic medical record and collected into a Research Electronic Data Capture. We compared categorical variables using the Fisher exact test. We compared continuous variables using the Wilcoxon rank test. RESULTS: In total, 2,375 pediatric patients received peripherally inserted and central lines; 244 of these were primary femoral tunneled central venous catheters (in 140 boys and 104 girls). In 140 children (mean age: 206 days), lines were inserted in the interventional radiology (IR) suite (technical success of 100%), with 14 (10.0%) complications including infection (n=7), malposition (n=2), bleeding (n=0), thrombosis (n=1) and line occlusion (n=4). The infection rate was 2.1 per 1,000 line days. In 104 children (mean age: 231 days), lines were placed at bedside (technical success 100%) with 14 (13.3%) complications including infection (n=3), malposition (n=5), bleeding (n=0), thrombosis (n=2) and line occlusion (n=4). The infection rate was 0.78 per 1,000 line days. The total line days were 7,109, of which 3,258 were in the IR suite and 3,851 in the bedside group. There was no statistical significance for complication rate (P=0.55) or infection rate (P=0.57) between bedside and interventional suite placements. CONCLUSION: In a cohort of children receiving primary femoral tunneled central venous catheters, the complication and infection rates in a bedside setting are not significantly increased compared to the lines placed in an IR suite. The perception of increased infection and complications from bedside-placed tunneled central venous catheters appears to be hyperbolized.

Trans-splenic Access for Portal Venous Interventions in Children: Do Benefits Outweigh Risks?

BACKGROUND: The primary concern of trans-splenic access for portal interventions is the risk of life-threatening intraperitoneal bleeding. OBJECTIVE: To review the clinical indications and efficacy and evaluate the risk factors for intraperitoneal bleeding during trans-splenic portal interventions in children. MATERIALS AND METHODS: A retrospective review of consecutive patients who underwent trans-splenic portal interventions at a tertiary care pediatric institution between March 2011 and April 2017 was performed. Forty-four procedures were performed in 30 children with a median age of 5 (0.3-18) years. Clinical indications, technical success, procedural success, and incidence of complications were recorded. Potential risk factors for intraperitoneal bleeding were evaluated using Wilcoxon rank and Fisher's exact tests. RESULTS: Trans-splenic access was 100% successful. In 35/44 (79%) procedures, the subsequent intervention was successful including recanalization of post-transplant portal vein occlusion in 10/13, embolization of bleeding Roux limb varices in 8/8, recanalization of chronic portal vein thrombosis in native liver in 7/13, splenoportography and manometry in 6/6, and occlusion of portosystemic shunts in 4/4 procedures. Intraperitoneal bleeding occurred during 12/44 (27%) procedures and was managed with analgesics, blood transfusion, and peritoneal drainage without the need for splenectomy or splenic artery embolization. Statistically significant correlation of bleeding was found with intraprocedural anticoagulation, but not with patient age, weight, platelet count, INR, ascites, splenic length, splenic venous pressure, vascular sheath size, or tract embolization technique. CONCLUSION: Trans-splenic access is a useful technique for successful pediatric portal interventions. Although it entails a substantial risk of intraperitoneal bleeding, this can be managed conservatively.

Innovative Use of Denver Shunt in a Child with Generalized Lymphatic Anomaly.

BACKGROUND: Denver® shunts have traditionally been used for palliation of refractory malignant and chylous peritoneal and pleural collections. We describe an innovative use of the Denver shunt in a child with generalized lymphatic anomaly. MATERIALS AND METHODS: Retrospective chart review of a 6-year-old girl with generalized lymphatic anomaly, who presented with refractory lymphorrhea from the labium majus, was performed. This was managed with innovative placement of the Denver shunt between a large abdominal wall cyst and the peritoneal cavity. RESULTS: There was progressive reduction in lymphorrhea with complete cessation at 11 months post shunt placement. At 33-month follow-up, the shunt remains patent with no further lymphorrhea. CONCLUSION: Denver shunts can be utilized for the creation of internal drainage pathways besides its traditional uses for draining peritoneal and pleural fluids.

Single-Incision Versus Conventional Technique for Tunneled Central Line Placement in Children.

PURPOSE: A single-incision technique for tunneled central venous access has been described. This study evaluates whether single-incision technique in children is comparable to the conventional method, with regard to procedure time, fluoroscopy time, and complication rate. MATERIALS AND METHODS: This is a retrospective review of 303 internal jugular vein tunneled central catheter placements whose age ranged from newborn to 17 years (median 1.7 years) by pediatric interventional radiologists from January 2014 through December 2015. 223 catheters were placed (181 patients) using the single-incision technique, and 80 catheters were placed (72 patients) using the conventional two-incision technique. Data were obtained from electronic medical records and PACS including procedure time, fluoroscopy time, and complication rates which were compared for both single-incision and conventional techniques. RESULTS: Technical success for the single-incision and conventional technique groups was 99.1 and 98.8%, respectively. Early complication rate was 12.1% for the single-incision technique and 17.5% for the conventional technique (p = 0.254). Overall complication rate was 26% (3.8/1000 line days) for the single-incision technique and 37.5% (4/1000 line days) for the conventional technique (p = 0.085). Median procedure time was 25 min for the single-incision technique and 26 min for the conventional technique (p = 0.427). Median fluoroscopy time was 1.7 min in the single-incision group and 1.3 min in the conventional group (p = 0.085). CONCLUSION: The single-incision technique for central venous access has comparable procedure time and fluoroscopy time with no difference in complication rates between the two techniques in a pediatric population.

Multimodality Image-Guided Sclerotherapy of Low-Flow Orbital Vascular Malformations: Report of Single-Center Experience.

PURPOSE: To evaluate the role of multimodality imaging tools for intraprocedural guidance and outcome evaluation during sclerotherapy of low-flow orbital vascular malformations. MATERIALS AND METHODS: A retrospective review was performed of 17 consecutive patients with low-flow orbital malformations (14 lymphatic, two venous, and one venolymphatic) who underwent multimodality image-guided sclerotherapy between November 2012 and May 2015. Sclerotherapy technique, image guidance tools, and complications were recorded. Sclerotherapy outcome was evaluated using clinical response, magnetic resonance (MR) image-based lesion volumetry, and proptosis quantification. RESULTS: There were 22 sclerotherapy sessions performed. Intraprocedural ultrasound (US), fluoroscopy, cone-beam computed tomography (CT) and MR image fusion were used for image guidance with 100% technical success. Resolution of presenting symptoms was observed in all patients at 1-month follow-up. Four major sclerotherapy complications were successfully managed. Statistically significant reduction in lesion volume (P = .001) and proptosis (P = .0117) by MR image analysis was achieved in all patients in whom 3-month follow-up MR imaging was available (n = 13/17). There was no lesion recurrence at a median follow-up of 18 months (range, 8-38 mo). CONCLUSIONS: Multimodality imaging tools, including US, fluoroscopy, cone-beam CT, and MR fusion, during sclerotherapy of low-flow orbital malformations provide intraprocedural guidance and quantitative image-based evaluation of treatment outcome.

Prenatally diagnosed neck masses: long-term outcomes and quality of life.

PURPOSE: To determine long-term outcomes of fetuses with neck masses (NM), including functional and cosmetic results. METHODS: A retrospective review was performed of all fetuses evaluated for NM from November 2001 to March 2014. Quality of life (QOL) was evaluated using the validated PedsQL™ questionnaire. RESULTS: Of 35 fetuses evaluated, 9 died perinatally and 1 died late from tracheostomy complications. NM ranged from 4 to 20cm (mean, 9.1cm); 18 were delivered by EXIT. Of 25 surviving patients, 22 had mass resection, 7 requiring more than one procedure. Surviving patients with lymphatic malformations (LM) had the highest incidence of moderate and severe disfigurement and a higher rate of persistent/recurrent disease (100% vs. 31%, p=0.002) and cranial nerve dysfunction (50% vs. 0%, p=0.005) compared to those with non-LM diagnoses. Of 9 children attending school, 78% achieve grades of A/B's. QOL for 13 patients revealed a mean score of 83/100 for physical and 78/100 for psychosocial functioning. Median follow-up was 6 years (7 months-17 years). CONCLUSION: Unlike those with teratoma or other lesions, children with congenital cervicofacial LM are at high-risk for persistent disease, nerve dysfunction and moderate-severe disfigurement. There is substantial perinatal morbidity for fetuses with neck masses, but for those surviving, the long-term functional and cognitive outcomes are good.

Sildenafil for microcystic lymphatic malformations of the head and neck: A prospective study.

BACKGROUND: Microcystic lymphatic malformations (LM) are congenital birth defects that can cause severe functional or esthetic deformity. At this time, several treatment interventions are possible, but there is no ideal therapy. A recently published article noted a coincidental improvement in microcystic LMs with the use of sildenafil for pulmonary hypertension, but conclusive and reproducible data is lacking regarding its efficacy. METHODS AND RESULTS: A prospective study was conducted to examine the subjective and objective results associated with sildenafil use in the treatment of microcystic LMs. Patients under the age of 18 were enrolled, and after evaluation with pre-intervention magnetic resonance imaging (MRI) studies, each was given a 6-week course of sildenafil. Subjective outcomes were obtained, and postoperative MRIs were used to objectively quantify changes. Five patients between the ages of 4 and 11 were enrolled in the study. All patients had microcystic LMs of the head and neck. All patients had undergone previous treatment interventions utilizing various modalities, and each was now seeking treatment for functional and cosmetic purposes. All patients had minimal to no response from a subjective standpoint. Comparison of pre- and post-treatment MRIs also demonstrated minimal to no response. CONCLUSIONS: The use of sildenafil for the treatment of microcystic lymphatic malformations did not have any appreciable effect on our selected population of pediatric patients who had been resistant to previous therapies. Further studies would be necessary to determine if other cohorts may benefit.

Imaging the central conducting lymphatics: initial experience with dynamic MR lymphangiography.

PURPOSE: To describe a dynamic magnetic resonance (MR) lymphangiography technique after intranodal injection of gadolinium-based contrast agent and to assess its feasibility for evaluation of the central conducting lymphatics (CCL) in patients with pathologic disorders that involve the CCL. MATERIALS AND METHODS: A retrospective evaluation of experience with the dynamic MR lymphangiographic technique in six consecutive patients was performed after institutional review board approval. Written informed consent for the percutaneous procedure was obtained from the patient, parent, or the legally responsible guardian. The dynamic MR lymphangiographic technique involves ultrasonographically guided intranodal injection of gadolinium-based contrast material into the inguinal lymph nodes, combined with sequential imaging of the chest and abdomen with a three-dimensional sequence optimized for soft tissue with high spatial resolution that provides time-resolved imaging of lymphatic transit through the CCL. Qualitative assessment of the images was performed for reliability of CCL visualization and for associated findings that could explain the clinical symptoms, including lymphangiectasia, chylolymphatic reflux, and chylous leak. RESULTS: The procedure was technically successful in all six patients. The dynamic MR lymphangiographic findings confirmed the presence of normal CCL morphologic structure in two patients and provided a possible explanation for clinical manifestations in the remaining four patients. The dynamic MR lymphangiographic procedure led to a change in management in two patients, continuation of conservative treatment in three patients, and confirmation of an alternative nonlymphatic diagnosis in one patient. Image quality for visualization of the CCL was considered good in all cases by the two readers. There were no known adverse effects related to the procedure. CONCLUSION: The dynamic MR lymphangiographic technique with intranodal injection of gadolinium-based contrast material is feasible and can provide useful information in a variety of lymphatic flow abnormalities involving the CCL.

Medical therapy for pediatric vascular anomalies.

Vascular anomalies (VAs) comprise a large variety of individual diagnoses that in different phases of treatment require a diverse number of medical specialists to provide optimal care. Medical therapies include agents usually associated with cancer chemotherapy, such as vincristine, as well more immunomodulatory types of drugs, such as glucocorticoids and sirolimus. These immunomodulating drugs are being successfully applied in cases that are typically categorized as vascular tumors, including kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), as well as some of the more invasive types of vascular malformations (i.e., microcystic lymphatic malformations and blue rubber bleb nevus syndrome (BRBNS). These therapies need to be combined with good supportive care, which often involves anticoagulation, antimicrobial prophylaxis, and comprehensive pain and symptom-relief strategies, as well as appropriate drug monitoring and management of side effects of medical treatment. The optimal care of these patients frequently involves close collaboration between surgeons, interventional and conventional radiologists, medical subspecialists, and nurses.

Vascular malformations: approach by an interventional radiologist.

Children with vascular malformations are best managed with a multidisciplinary team of specialists. Interventional radiology may deliver primary treatment such as staged sclerotherapy and embolization for malformations that are poor candidates for primary surgical resection or play a supportive role such as preoperative or intraoperative embolization. A thorough understanding of vascular morphology and flow dynamics is imperative to choosing the best treatment tool and technique. In this review, the author discusses the selection of techniques and tools used to treat vascular malformations based on their angiographic morphology.

Traumatic hepatic artery laceration managed by transarterial embolization in a pediatric patient.

While blunt abdominal trauma with associated liver injury is a common finding in pediatric trauma patients, hepatic artery transection with subsequent treatment by transarterial embolization has rarely been reported. We present a case of a child who suffered from a hepatic artery injury which was successfully managed by supraselective transarterial microcoil embolization, discuss management strategies in these patients, and provide a review of currently available literature.