Venous Anomalies in Overgrowth Syndromes.

Overgrowth syndromes, particularly within the PIK3CA-related overgrowth syndrome (PROS) spectrum, are commonly associated with venous anomalies. The anomalies include spongiform venous malformations and persistent embryonic veins, such as the lateral marginal vein (of Servelle). The anomalous veins pose a significant risk of thromboembolic disease and should be occluded, preferably earlier in life.

Image-Guided Transrectal Drainage of Pelvic Abscesses in Children.

Objective:  To describe our technique and share our experience with image-guided transrectal drainage (TRD) of pelvic abscesses in children.

Materials And Methods:  Retrospective review and analysis of indications for image-guided TRD and examination of procedural outcomes in pediatric patients with pelvic abscesses over 8 years.

Results:  A total of 69 patients (33 males and 36 females) with symptomatic pelvic abscesses underwent image-guided TRD.

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

Background: Due to the rarity of pediatric diseases, collaborative research is the key to maximizing the impact of research studies. A research needs assessment survey was created to support initiatives to foster pediatric interventional radiology research.

Objective: To assess the status of pediatric interventional radiology research, identify perceived barriers, obtain community input on areas of research/education/support, and create metrics for evaluating changes/responses to programmatic initiatives.

Kaposiform Lymphangiomatosis: Pathologic Aspects in 43 Patients.

Kaposiform lymphangiomatosis is an uncommon generalized lymphatic anomaly with distinctive clinical, radiologic, histopathologic, and molecular findings. Herein, we document the pathology in 43 patients evaluated by the Boston Children's Hospital Vascular Anomalies Center from 1999 to 2020. The most frequent presentations were respiratory difficulty, hemostatic abnormalities, and a soft tissue mass.

Cerebrospinal fluid leak in epidural venous malformations and blue rubber bleb nevus syndrome.

Objective: Clinical manifestations of blue rubber bleb nevus syndrome (BRBNS) and multifocal venous malformation (MVM) vary depending on the location of the lesions. The aim of this study was to assess the risk of developing CSF leaks in patients with epidural venous malformations (VMs).

Methods: The authors retrospectively investigated the relationship between the development of a CSF leak and the presence of epidural VMs.

The use of the internal mammary vein for central venous access.

Purpose: To describe the use of the internal mammary vein as an alternative access for central venous catheters.

Methods: We performed a retrospective review of patients who underwent placement of central venous catheters via the internal mammary vein. Patient demographics, indication for venous access, technical success, catheter type, dwell time and indication for exchange or removal were recorded.

Surgical management of pediatric renovascular hypertension and midaortic syndrome at a single-center multidisciplinary program.

Objective: To evaluate the outcomes of various surgical approaches in the treatment of renovascular hypertension and midaortic syndrome (MAS) in children.

Methods: We performed a retrospective medical record review of patients who had undergone surgery for renovascular hypertension from 2010 to 2018 at our center under the care of a multidisciplinary team. The operative interventions included mesenteric artery growth improves circulation (MAGIC), tissue expander-stimulated lengthening of arteries (TESLA), aortic bypass using polytetrafluorethylene, renal artery reimplantation, and autotransplantation.

Vascular anomalies of the head and neck.

The head and neck are the most common site of involvement for vascular tumors and malformations, with more than half of all vascular anomalies seen in this region. Lesions in this location can cause significant disfigurement and can be associated with airway obstruction, impairment in vision or hearing, swallowing disorders and hemorrhage. Accurate diagnosis is critical in determining treatment, and interdisciplinary care is essential for optimal management.

Two-hour Observation After Liver Biopsy in Children: Clinical and Economic Outcome of a Quality Improvement Intervention.

Objective: Following percutaneous liver biopsy performed at our institution on an outpatient basis, children traditionally were observed for 4 hours then discharged after verifying a stable hematocrit level. In June 2015, we adopted a quality improvement project with shorter 2-hour observation for patients with no known risks and the hematocrit test was abandoned.The purpose of this study is to evaluate the clinical and economic outcomes of early discharge of children following liver biopsy.

Single Procedure Saline Lavage for Treatment of Inspissated Bile.

Inspissated bile syndrome is a rare cause of cholestatic jaundice in infancy, occurring due to obstruction of the biliary ducts and gallbladder by biliary sludge. Traditional methods of treatment include surgical drainage or cholecystostomy drain placement. Both can be associated with complications and prolonged admission.

Congenital Disseminated Pyogenic Granuloma: Characterization of an Aggressive Multisystemic Disorder.

Objective: To describe the clinical, radiologic, and histopathologic features of "congenital disseminated pyogenic granuloma" involving various organs with high morbidity related to cerebral hemorrhagic involvement.

Study Design: We searched the database of the Vascular Anomalies Center at Boston Children's Hospital from 1999 to 2019 for patients diagnosed as having multiple vascular lesions, visceral vascular tumors, congenital hemangiomatosis, multiple pyogenic granulomas, or multiple vascular lesions without a definite diagnosis. A retrospective review of the medical records, photographs, histopathologic, and imaging studies was performed.

Complex Lymphatic Anomalies and Therapeutic Options.

Complex lymphatic anomalies include a variety of disorders with overlapping clinical, histological and imaging features. The often-confusing nomenclature used for lymphatic anomalies limits timely diagnosis and treatment. The updated 2018 classification of the International Society for the Study of Vascular Anomalies divides lymphatic anomalies into several subsets.

Clinical characterization and long-term outcomes in pediatric epithelioid hemangioendothelioma.

There is a paucity of information about the clinical characteristics and long-term outcomes of pediatric epithelioid hemangioendothelioma (EHE), a rare vascular neoplasm commonly presenting in adulthood. In our case series of 24 patients with EHE aged 2-26 years, the majority presented with multi-organ disease. Progression was seen in 63% of patients with a mean time to progression of 18.

Fibroadipose Vascular Anomaly in the Upper Extremity: A Distinct Entity With Characteristic Clinical, Radiological, and Histopathological Findings.

Purpose: Fibroadipose vascular anomaly (FAVA) is an intramuscular vascular malformation that has been recently described as a distinct clinical entity. The clinical, radiological, and histopathological characteristics of FAVA in the upper extremity are reviewed.

Methods: This was a retrospective case series of upper-extremity FAVA lesions.

Imaging findings in epithelioid hemangioendothelioma.

Purpose-objective: Epithelioid hemangioendothelioma (EHE) is a rare vascular malignancy with varying biologic behavior. The purpose of this study was to identify imaging findings most characteristic of EHE.

Methods: Retrospective review of clinical and imaging records in patients referred to our Vascular Anomalies Center over a 17 year period with biopsy proven EHE.

Surgical Management of Fibroadipose Vascular Anomaly of the Lower Extremities.

Background: Fibroadipose vascular anomaly (FAVA) is a recently-defined vascular malformation often involving the extremities and presenting in childhood. Patients may present to orthopaedic surgeons with pain, swelling, joint contractures, and leg length discrepancy. There is no established therapy or treatment paradigm.

A somatic activating NRAS variant associated with kaposiform lymphangiomatosis.

Purpose: Kaposiform lymphangiomatosis (KLA) is a rare, frequently aggressive, systemic disorder of the lymphatic vasculature, occurring primarily in children. Even with multimodal treatments, KLA has a poor prognosis and high mortality rate secondary to coagulopathy, effusions, and systemic involvement. We hypothesized that, as has recently been found for other vascular anomalies, KLA may be caused by somatic mosaic variants affecting vascular development.

Pulmonary thromboembolic events in patients with congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal abnormalities and Klippel-Trénaunay syndrome.

Objective: Patients with Klippel-Trénaunay syndrome (KTS) and congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal abnormalities (CLOVES) syndrome have central phlebectasia and enlarged persistent embryonic veins that are often incompetent and prone to thromboembolism. The purpose of the study was to determine the presence of phlebectasia and the incidence of symptomatic pulmonary embolism (PE).

Methods: A retrospective review was conducted of patients referred to the Vascular Anomalies Center at our institution during a 21-year period who were diagnosed with KTS and CLOVES syndrome.

Preoperative Embolization of Venous Malformations Using n-Butyl Cyanoacrylate.

Purpose: The purpose of this study was to evaluate the safety and efficacy of preoperative percutaneous n-butyl cyanoacrylate (nBCA) embolization of venous malformations in children.

Material And Methods: Clinical data were retrospectively reviewed in children who underwent embolization using nBCA followed by resection of venous malformations.

Results: A total of 17 embolizations were performed in 14 patients (9 females, mean age: 5.