Autism Spectrum Disorder in Children With Perinatal Ischemic Stroke Varies by Stroke Type.

Perinatal stroke leads to significant morbidity over a child's lifetime, including diagnosis of various neurodevelopmental disorders. Specific studies examining the prevalence of autism spectrum disorder in children with perinatal stroke are scarce. Following the clinical observation of autism spectrum disorder in a pediatric referral stroke center, we evaluated the rate of autism spectrum disorder diagnosis after perinatal ischemic stroke, including analysis by subtypes of perinatal ischemic stroke.

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.

Corrigendum: Childhood Vascular Tumors.

[This corrects the article DOI: 10.3389/fped.2020.

Mucocutaneous Adverse Events Associated With Oral Sirolimus for the Treatment of Vascular Anomalies.

This case series examines the clinical spectrum and frequency of mucocutaneous adverse events related to oral sirolimus in populations with vascular anomalies.

Contemporary Management of Vascular Anomalies of the Head and Neck-Part 1: Vascular Malformations: A Review.

Importance: Vascular anomalies of the head and neck are relatively rare lesions. Management is challenging because of the high likelihood of involvement of functionally critical structures. Multiple modalities of treatment exist for vascular anomalies of the head and neck, including medical therapies, sclerotherapy and embolization procedures, and surgery.

Childhood Vascular Tumors.

Vascular tumors in pediatric patients are an important entity for the clinician to recognize and correctly diagnose. They may present at birth or develop at any point during infancy, childhood, or adolescence. Most are benign, but even benign lesions may have significant morbidity without proper intervention.

Incidence of Pediatric Venous Thromboembolism After Elective Spine and Lower-Extremity Surgery in Children With Neuromuscular Complex Chronic Conditions: Do we Need Prophylaxis?

Background: The incidence of venous thromboembolism (VTE) after elective surgery in children with mobility impairments, including those with a neuromuscular complex chronic condition (NCCC), is unknown. Therefore, our objectives were to assess the incidence of VTE after elective spine and lower-extremity surgery in children with NCCC.

Methods: A retrospective analysis of children with NCCC undergoing elective lower-extremity and/or spinal surgeries from 2005 to 2009 included in the Pediatric Health Information Systems Plus (PHIS+) database.

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.

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.

The Genetic and Molecular Determinants of Juvenile Nasopharyngeal Angiofibroma: A Systematic Review.

Objective: Juvenile nasopharyngeal angiofibroma (JNA) is a rare vascular tumor of unknown etiology. Studies investigating the molecular and genetic determinants of JNA are limited by small sample size and inconsistent approaches. The purpose of this study is to examine all eligible JNA studies in aggregate, applying qualitative analysis to highlight areas of particular relevance, including potential targets for therapeutic intervention.

Lymphaticovenous bypass of the thoracic duct for the treatment of chylous leak in central conducting lymphatic anomalies.

Background: Central conducting lymphatic anomalies (CCLA) may cause chylous leaks and protein-losing enteropathy (PLE) owing to dysfunction of the central lymphatic channels. Most of the treatment strategies for these conditions are palliative and provide transient improvement.

Methods: We treated 14 patients with intractable chylous leak and/or PLE using a novel technique of lymphaticovenous bypass of the terminal portion of the thoracic duct.

Sirolimus Therapy as Perioperative Treatment of Gorham-Stout Disease in the Thoracic Spine: A Case Report.

Case: Gorham-Stout disease (GSD) is a rare entity that is marked by progressive osteolysis and bone resorption. A 14-year-old boy who was being followed for scoliosis presented with a marked curve progression and kyphoscoliosis. Imaging revealed osteolysis of the posterior elements and the ribs, suggestive of GSD.

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.

Partial thromboplastin time is more predictive of bleeding than anti-Xa levels in heparinized pediatric patients after cardiac surgery.

Objectives: Anticoagulation with unfractionated heparin (UFH) after pediatric cardiac surgery can be monitored using either activated partial thromboplastin time (aPTT) or anti-factor Xa activity (anti-Xa). However, correlation of bleeding with either of these laboratory values has not been established. We sought to determine the correlation between bleeding events and aPTT and anti-Xa in patients who undergo anticoagulation after congenital heart surgery.

An anticoagulation protocol for use after congenital cardiac surgery.

Background: Patients undergoing surgery for congenital heart disease are at high risk for bleeding as well as thrombosis in the postoperative period. The objective of the study was to describe the design and effects of implementing a standardized unfractionated heparin anticoagulation protocol for children after congenital heart surgery.

Methods: We created a tiered guideline for the postoperative management of bleeding and thrombosis.

Placental Pathology in Neonatal Stroke: A Retrospective Case-Control Study.

Objective: To assess the association of placental abnormalities with neonatal stroke.

Study Design: This retrospective case-control study at 3 academic medical centers examined placental specimens for 46 children with neonatal arterial or venous ischemic stroke and 99 control children without stroke, using a standard protocol. Between-group comparisons used χ and Fisher exact t test.

Imaging features and prognostic factors in fetal and postnatal torcular dural sinus malformations, part I: review of experience at Boston Children's Hospital.

Background: Even for the most common dural sinus malformation (DSM), the torcular DSM (tDSM), generalizable statements about etiology and prognosis are difficult because neurosurgeons/neuroradiologists and obstetrical imagers have focused on different patient age groups, have reported different outcomes, and have offered differing pathophysiologic explanations.

Objective: To examine the imaging features and outcomes of a local cohort of tDSMs across fetal-neonatal life for commonalities.

Methods: Review of imaging and clinical outcome for a local cohort of 12 tDSM patients (9 fetal, 3 postnatal).

Imaging features and prognostic factors in fetal and postnatal torcular dural sinus malformations, part II: synthesis of the literature and patient management.

Background: Torcular dural sinus malformations (tDSMs) are described as slow flow dural arteriovenous fistulae with frequently poor outcomes in the neuroangiographic literature, but other etiologies have been proposed in the obstetric literature, where outcomes have been more favorable.

Objective: To review tDSMs reported in the literature of multiple specialties for features that support a common etiology, and to identify key prognostic factors, with an emphasis on tDSM trajectory highlighted in part I.

Methods: Analysis of imaging features and clinical outcome for 77 prenatal and 22 postnatal tDSMs reported in 37 papers from the literature.

Sirolimus as treatment for 19 patients with refractory cervicofacial lymphatic malformation.

Objective: Lymphatic malformations (LMs) are challenging to manage, particularly those involving the cervicofacial region and airway. Traditional therapy is sclerotherapy and/or resection. We aim to establish the emerging therapeutic role of sirolimus.

Vascular anomaly cases for the pediatric hematologist oncologists-An interdisciplinary review.

Vascular anomalies (VAs) are classified as tumors or malformations depending on their clinical characteristics, pathological diagnosis, and genomic information. Diagnosis can be challenging because of the heterogeneity of clinical presentation; thus, the best diagnosis and care are provided by an interdisciplinary team of specialists. Over the past 10 years, an increasing number of pediatric hematologist/oncologists are caring for patients with VAs secondary to new medical therapy options and clinical trials.