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Background and Objectives: 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. Methods: We retrospectively examined all children diagnosed with perinatal ischemic stroke, who were ≥18 months old at the time of last follow-up at a single institution from 2008 through 2021. We classified patients as having autism spectrum disorder if they were diagnosed by a neurologist, neuropsychologist, clinical psychologist, or developmental pediatrician. Multivariable logistic regression was performed to examine the association between ischemic stroke subtype and autism spectrum disorder. Results: Among 260 children with perinatal stroke, 19 children (7.3%) also had autism spectrum disorder. Children with perinatal venous stroke had 3-fold higher odds of autism spectrum disorder compared to those with perinatal arterial ischemic stroke (adjusted odds ratio: 3.01, 95% confidence interval: 1.07-8.47). Conclusion: In our perinatal ischemic stroke population, children with venous stroke had higher odds of autism spectrum disorder compared to those with arterial ischemic stroke alone. Prospective studies are needed to further investigate the role of perinatal stroke in autism spectrum disorder development.
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Transtorno do Espectro Autista , AVC Isquêmico , Acidente Vascular Cerebral , Feminino , Gravidez , Humanos , Criança , Lactente , Transtorno do Espectro Autista/complicações , Transtorno do Espectro Autista/diagnóstico , Transtorno do Espectro Autista/epidemiologia , Estudos Retrospectivos , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/epidemiologia , Estudos ProspectivosRESUMO
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. Imaging commonly revealed involvement of some combination of mediastinal, pulmonary, pleural, and pericardial compartments and most often included spleen and skeleton. Histopathology was characterized by dilated, redundant, and abnormally configured lymphatic channels typically accompanied by dispersed clusters of variably canalized, and often hemosiderotic, spindled lymphatic endothelial cells that were immunopositive for D2-40, PROX1, and CD31. An activating lesional NRAS variant was documented in 9 of 10 patients. The clinical course was typically aggressive, marked by hemorrhage, thrombocytopenia, diminished fibrinogen levels, and a mortality rate of 21%.
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Células Endoteliais , Pulmão , Boston , Criança , HumanosRESUMO
[This corrects the article DOI: 10.3389/fped.2020.573023.].
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Imunossupressores/efeitos adversos , Mucosa/efeitos dos fármacos , Sirolimo/efeitos adversos , Dermatopatias/induzido quimicamente , Administração Oral , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Imunossupressores/administração & dosagem , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Mucosa/patologia , Estudos Retrospectivos , Sirolimo/administração & dosagem , Dermatopatias/patologia , Malformações Vasculares/tratamento farmacológico , Adulto JovemRESUMO
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. This review focuses on the accurate diagnosis and the relative roles of the various therapeutic options. Observations: Vascular anomalies are classified by the International Society for the Study of Vascular Anomalies into 2 major groups: vascular tumors and vascular malformations. Vascular tumors encompass proliferative lesions ranging from infantile and congenital hemangiomas to kaposiform hemangioendothelioma. Alternatively, vascular malformations are embryologic errors in vasculogenesis. This article focuses on the management of vascular malformations. The 3 primary vascular malformation subclassifications are lymphatic, venous, and arteriovenous. The burden of disease, diagnosis, and current management options are discussed in detail for each subtype. Conclusions and Relevance: Most vascular malformations of the head and neck require a multidisciplinary approach. Available medical, interventional radiologic, and surgical interventions are constantly evolving. Optimization of function and cosmesis must be balanced with minimization of treatment-associated morbidity. Otolaryngologists-head and neck surgeons must remain up to date regarding options for diagnosis and management of these lesions.
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Cabeça/irrigação sanguínea , Pescoço/irrigação sanguínea , Malformações Vasculares/diagnóstico , Malformações Vasculares/terapia , Humanos , Malformações Vasculares/classificaçãoRESUMO
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. Malignant vascular tumors are also rarely seen in the pediatric population, and may be associated with various syndromes.
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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. VTE during hospitalization for surgery was assessed through abstraction and review of ultrasound (U/S) and computed tomography results by 2 independent reviewers. VTEs related to pre-existing central venous catheters were excluded. RESULTS: There were 4,583 children with NCCC who underwent orthopaedic surgery during the study period at 6 centers. Most were male (56.3%), non-Hispanic whites (72.7%), and had private insurance (52.2%). The most common NCCC diagnoses were cerebral palsy (46.7%), brain and spinal cord malformations (31.1%), and central nervous system degenerative conditions (14.5%). Forty children (0.9%) underwent U/S to assess VTE. Eighteen children (0.4%) underwent computed tomography to assess VTE. Four children (with cerebral palsy) had a positive U/S for a lower-extremity VTE (10-18 y of age), yet 2 had their VTE before surgery. Therefore, the adjusted VTE rate for children with NCCC undergoing orthopaedic lower-extremity or spine surgery was 4 per 10,000 (2 cases per 4583 surgeries). Each of the 2 cases had a known coagulation disorder preoperatively. Only 10% of the cohort used compression devices, 3% enoxaparin, and 1.6% aspirin for prophylaxis. CONCLUSION: The rate of non-central-venous-catheter-related VTE associated with orthopaedic surgery in children with NCCC is very low and lower than rates reported in healthy children. SIGNIFICANCE: To our knowledge, this is the first multi-institutional study reporting the incidence of VTE in children with NCCCs undergoing elective hip and spine surgery. These data support no additional prophylaxis is required in children with NCCC undergoing elective hip and spine surgery unless other known risk factors are also present.
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Encéfalo/cirurgia , Paralisia Cerebral/cirurgia , Procedimentos Ortopédicos/estatística & dados numéricos , Medula Espinal/cirurgia , Tromboembolia Venosa/epidemiologia , Tromboembolia Venosa/prevenção & controle , Adolescente , Encéfalo/anormalidades , Criança , Pré-Escolar , Procedimentos Cirúrgicos Eletivos/estatística & dados numéricos , Feminino , Humanos , Incidência , Lactente , Extremidade Inferior/cirurgia , Masculino , Estudos Retrospectivos , Fatores de Risco , Medula Espinal/anormalidades , Coluna Vertebral/cirurgia , Tromboembolia Venosa/diagnóstico por imagem , Adulto JovemRESUMO
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.
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Prova Pericial , Anormalidades Linfáticas/diagnóstico , Guias de Prática Clínica como Assunto/normas , Malformações Vasculares/diagnóstico , Adulto , Criança , Consenso , Diagnóstico por Imagem , Humanos , Anormalidades Linfáticas/diagnóstico por imagem , Prognóstico , Malformações Vasculares/diagnóstico por imagem , Adulto JovemRESUMO
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. We report on outcomes following surgical excision for patients with this condition. METHODS: Between 2007 and 2016, all 35 patients that underwent excision of lower-extremity FAVA were retrospectively reviewed using a combination of medical records, radiologic findings, and telemedicine reviews. RESULTS: Mean age at initial presentation was 12.3±6.8 years. Mean follow-up from time of definitive diagnosis at our institution was 66 months (range: 12 to 161 mo). Mean follow-up after surgery was 35 months (range: 6 to 138 mo). Females were affected more than males (71% vs. 29%). The most common location of FAVA was in the calf (49%), followed by the thigh (40%). The most commonly involved muscle was gastrocnemius (29%), followed by the quadriceps (26%). At latest follow-up after surgery, there was an improvement in the proportion of patients with pain at rest (63% vs. 29%), pain with activity (100% vs. 60%), as well as analgesia use (94% vs. 37%). Fourteen patients (40%) had symptomatic residual disease or recurrence of FAVA requiring further treatment. Six patients (17%) required further surgery and 6 (17%) required further interventional radiologic procedures. Three patients (9%) required eventual amputation for intractable pain and loss of function. Lesions with direct nerve involvement were associated with persistent neuropathic symptoms at latest follow-up (P=0.002) as well as symptomatic residual disease and/or recurrence requiring further treatment (P=0.01). Seventeen patients (49%) had 19 preoperative joint contractures. Eighteen of the 19 contractures (95%) had sustained improvement at latest follow-up. CONCLUSIONS: In carefully selected patients, surgical excision of FAVA results in improvement of symptoms. However, symptomatic residual disease and/or recurrence are not uncommon. Direct nerve involvement is associated with a worse outcome. LEVEL OF EVIDENCE: Level IV-case series.
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Extremidade Inferior , Músculo Esquelético , Doenças Musculares , Dor , Malformações Vasculares , Criança , Dissecação/métodos , Feminino , Humanos , Extremidade Inferior/irrigação sanguínea , Extremidade Inferior/diagnóstico por imagem , Extremidade Inferior/patologia , Extremidade Inferior/cirurgia , Masculino , Músculo Esquelético/patologia , Músculo Esquelético/cirurgia , Doenças Musculares/congênito , Doenças Musculares/patologia , Doenças Musculares/cirurgia , Dor/diagnóstico , Dor/etiologia , Manejo da Dor/métodos , Recidiva , Reoperação/métodos , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Resultado do Tratamento , Malformações Vasculares/diagnóstico , Malformações Vasculares/fisiopatologia , Malformações Vasculares/cirurgiaRESUMO
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. RESULTS: We reviewed 19 patients with FAVA of the upper limb. Pain, stiffness, swelling, and flexion contractures were the most common presentations. Except for one lesion confined to the hand, all lesions either presented with or developed a contracture within 10 years. Ten patients underwent surgical debulking. Six required tendon transfer reconstruction and 3 necessitated a free functional muscle transfer. CONCLUSIONS: Fibroadipose vascular anomaly in the upper extremity requires an accurate diagnosis and may benefit from early referral to a multidisciplinary vascular anomaly center with experienced hand surgeons. Compression garments, propranolol, and sclerotherapy seem to be ineffective. Surgical resection focused on symptomatic regions with appropriate reconstruction may have benefit in salvage of limbs with compromised function. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.
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Extremidade Superior , Malformações Vasculares , Humanos , Estudos Retrospectivos , Escleroterapia , Resultado do Tratamento , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/terapiaRESUMO
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 17â¯year period with biopsy proven EHE. RESULTS: We evaluated 29 patients (17â¯F) with median age of 16â¯years (range 2-76 y). The most common presenting symptoms were pain (nâ¯=â¯13) and palpable mass (nâ¯=â¯7). 22 (70%) had multifocal disease. Most common sites of involvement were lung (nâ¯=â¯25), liver (nâ¯=â¯16), bone (nâ¯=â¯12), soft tissue (nâ¯=â¯3) and lymph nodes (nâ¯=â¯1). Of patients with single site disease, 3 had lung, 3 liver, and 1 had bone lesions. In 18/25 with lung disease, there were multiple nodules of varying sizes and characteristics. In 14/16 with hepatic disease there were multiple nodules with predominantly peripheral distribution. Subcapsular retraction was seen in 10/16 and a "lollipop" sign (hepatic or portal vein tapering at the edge of a well-defined hypoenhancing lesion) identified in 5/16. Of 12 osseous lesions, 11 were lytic, 8 involved vertebrae and 9 involved the axial skeleton. CONCLUSION: EHE has varied imaging findings. The most common sites are lungs, liver, and bone, with multi-organ involvement seen in most. Lung disease is most commonly characterized by multiple nodules. Hepatic lesions demonstrate the most distinctive findings, with peripheral distribution, lack of early enhancement, subcapsular retraction and "lollipop" sign. Osseous lesions are commonly lytic and more prevalent in the axial skeleton.
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Neoplasias Ósseas/patologia , Hemangioendotelioma Epitelioide/patologia , Neoplasias Hepáticas/patologia , Neoplasias de Tecidos Moles/patologia , Adolescente , Adulto , Idoso , Biópsia , Criança , Pré-Escolar , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto JovemRESUMO
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. METHODS: The PubMed, MEDLINE, Embase, Web of Science, Cochrane, and CINAHL databases were screened with inclusion and exclusion criteria applied to all citations. Manuscripts investigating the genetic determinants, histopathogenesis, and heritability of juvenile nasopharyngeal angiofibroma were included. Non-English studies, case reports, and articles focusing on clinical management without original data were excluded. Full text articles were obtained. A qualitative synthesis of data was performed. RESULTS: A total of 59 articles met criteria for inclusion. These were divided into 6 categories based on the primary topic or target discussed, (1) steroid hormone receptors, (2) chromosomal abnormalities, (3) growth factors, (4) genetic targets, (5) molecular targets, (6) Wnt cell signaling, and (7) studies that overlapped multiple of the aforementioned categories. Although relatively low n values prevent definitive conclusions to be drawn, a predominance of certain molecular targets such as vascular endothelial growth factor (VEGF) and Wnt/ß-catenin pathway intermediaries is apparent. CONCLUSIONS: Although the etiology of JNA remains elusive, contemporary molecular genetic investigation holds promise for risk stratification and could form the basis of a modernized staging system. A multicenter clinical registry and linked tissue bank would further promote the search for JNA specific biomarkers.
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Angiofibroma , Testes Genéticos/métodos , Neoplasias Nasofaríngeas , Fator A de Crescimento do Endotélio Vascular/genética , Proteínas Wnt/genética , Adolescente , Angiofibroma/diagnóstico , Angiofibroma/genética , Angiofibroma/metabolismo , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Humanos , Neoplasias Nasofaríngeas/diagnóstico , Neoplasias Nasofaríngeas/genética , Neoplasias Nasofaríngeas/metabolismo , Fator A de Crescimento do Endotélio Vascular/metabolismo , Proteínas Wnt/metabolismoRESUMO
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. Chylous leaks occurred in multiple different anatomic sites. All patients had CCLA and failure of thoracic duct emptying demonstrated by preoperative intranodal lymphangiography. RESULTS: Five patients had complete resolution of symptoms, and two patients had partial improvement. There were no major complications. Of 5 patients with PLE, only one improved after lymphaticovenous bypass. Repeat traditional lymphangiography was performed in 4 patients who did not improve, demonstrating patency of the bypass in all cases with persistent sluggish drainage. One patient had repeat MR lymphangiography that did not show the thoracic duct well. CONCLUSIONS: Bypass of the terminal thoracic duct is a novel procedure that offers improvement and a chance of cure for some patients with devastating manifestations of CCLA who lack other effective therapeutic options. LEVEL OF EVIDENCE: IV.
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Anastomose Cirúrgica/métodos , Quilotórax/cirurgia , Anormalidades Linfáticas/cirurgia , Enteropatias Perdedoras de Proteínas/cirurgia , Ducto Torácico/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Quilotórax/etiologia , Feminino , Humanos , Lactente , Anormalidades Linfáticas/complicações , Vasos Linfáticos , Linfografia/métodos , Masculino , Pessoa de Meia-Idade , Enteropatias Perdedoras de Proteínas/etiologia , Ducto Torácico/anormalidades , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Procedimentos Cirúrgicos Vasculares/métodos , Adulto JovemRESUMO
OBJECTIVE: To define the types of hepatic hemangiomas using the updated International Society for the Study of Vascular Anomalies classification and to create a set of guidelines for their diagnostic evaluation and monitoring. STUDY DESIGN: We used a rigorous, transparent consensus protocol defined by an approved methodology, with input from multiple pediatric experts in vascular anomalies from hematology-oncology, surgery, pathology, radiology, and gastroenterology. RESULTS: In the first section, we define the subtypes of hepatic hemangiomas based on the clinical course, histology, and radiologic characteristics. We recommend against using the term "hemangioma" for any vascular malformations affecting the liver or any hypervascular tumors that are not characterized by the approved definitions. We recommend against using the term "hemangioendothelioma" for infantile or congenital hemangioma. The following 2 sections dedicated to infantile hepatic hemangioma and to congenital hepatic hemangioma individually describe these subtypes in further detail, including complications to be considered during monitoring and respectively recommended screening evaluations. CONCLUSIONS: Although institutional variations may exist for specific clinical details, a clear understanding of the diagnosis of hepatic hemangiomas affecting children and the possible complications that require screening during the monitoring period should be standard. As children with hepatic hemangiomas are managed by different medical and surgical specialties, we offer an expert opinion multidisciplinary consensus based on current literature and on data extracted from the liver hemangioma registry.
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Hemangioma/classificação , Hemangioma/diagnóstico , Neoplasias Hepáticas/classificação , Neoplasias Hepáticas/diagnóstico , Pré-Escolar , Feminino , Hemangioendotelioma , Humanos , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética , Masculino , Programas de Rastreamento , Oncologia , Pediatria/normas , Guias de Prática Clínica como Assunto , Sistema de Registros , Ultrassonografia Doppler , Estados Unidos , Malformações Vasculares/classificação , Malformações Vasculares/diagnósticoRESUMO
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. The structural compromise threatened spinal cord compression. Preoperative sirolimus therapy was initiated to stabilize the disease prior to corrective instrumentation. A biopsy specimen that was obtained at the time of instrumentation showed lymphatic vascular spaces consistent with GSD. Sirolimus therapy with the addition of bisphosphonate therapy was continued postoperatively. CONCLUSION: To our knowledge, this case report is the first to describe sirolimus therapy combined with surgery for GSD of the spine. The patient did well with consecutive medical optimization and surgical intervention, including postoperative sirolimus and bisphosphonate therapy.
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Difosfonatos/administração & dosagem , Osteólise Essencial/tratamento farmacológico , Escoliose/complicações , Sirolimo/administração & dosagem , Vértebras Torácicas/diagnóstico por imagem , Adolescente , Quimioterapia Adjuvante , Humanos , Masculino , Osteólise Essencial/diagnóstico por imagem , Osteólise Essencial/patologia , Osteólise Essencial/cirurgia , Escoliose/cirurgia , Fusão Vertebral , Vértebras Torácicas/patologiaRESUMO
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. Of these, the patients who had PE were screened for thromboembolic risk factors in addition to phlebectasia and the presence of persistent embryonic veins. Treatment outcomes following subsequent endovascular and medical therapies were reported. RESULTS: A total of 12 KTS patients of 96 (12.5%) and 10 CLOVES syndrome patients of 110 (9%) suffered PE. Fourteen patients (64%) developed PE after surgery or sclerotherapy. All of the patients had abnormally dilated central or persistent embryonic veins; 20 patients were treated with anticoagulation (1 died at the time of presentation, and no information was available for 1) after PE, and 14 (66%) patients underwent subsequent endovascular treatment. Five patients developed recurrent PE despite anticoagulation. Two of the patients died of PE. No patients treated with endovascular closure of dilated veins had subsequent evidence of PE. CONCLUSIONS: Patients with KTS and CLOVES syndrome are at high risk for PE, particularly in the postoperative period.
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Síndrome de Klippel-Trenaunay-Weber/epidemiologia , Lipoma/epidemiologia , Anormalidades Musculoesqueléticas/epidemiologia , Nevo/epidemiologia , Embolia Pulmonar/epidemiologia , Varizes/epidemiologia , Malformações Vasculares/epidemiologia , Veias/anormalidades , Adolescente , Adulto , Anticoagulantes/uso terapêutico , Boston/epidemiologia , Criança , Pré-Escolar , Protocolos Clínicos , Angiografia por Tomografia Computadorizada , Dilatação Patológica , Procedimentos Endovasculares/efeitos adversos , Feminino , Humanos , Incidência , Lactente , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Síndrome de Klippel-Trenaunay-Weber/terapia , Lipoma/diagnóstico , Lipoma/terapia , Masculino , Anormalidades Musculoesqueléticas/diagnóstico , Anormalidades Musculoesqueléticas/terapia , Nevo/diagnóstico , Nevo/terapia , Flebografia/métodos , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/terapia , Estudos Retrospectivos , Fatores de Risco , Escleroterapia/efeitos adversos , Fatores de Tempo , Varizes/diagnóstico por imagem , Varizes/terapia , Malformações Vasculares/diagnóstico , Malformações Vasculares/terapia , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Veias/diagnóstico por imagem , Adulto JovemRESUMO
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. METHODS: We prospectively studied pediatric patients treated with UFH after cardiac surgery over an 11-month period. Bleeding events were prospectively assessed and adjudicated. The highest aPTT and corresponding anti-Xa for the 24 hours before bleeding events were collected to assess for association with bleeding. Statistical analysis was performed using generalized additive logistic regression. RESULTS: A total of 202 patients received UFH over 1488 patient-days. The median age at surgery was 0.4 years (interquartile range, 0.1-2.2). A total of 45 major or clinically relevant bleeding events were observed. The correlation between aPTT and anti-Xa was of moderate strength (R = 0.58; P < .001). The odds of bleeding increased significantly when aPTT exceeded 150 (odds ratio, 1.71 per 10-second increase in aPTT, 95% confidence interval, 1.21-2.42; P = .003). Anti-Xa was not associated with bleeding (odds ratio, 1.11 per 0.1 IU/mL increase, 95% confidence interval, 0.89-1.29; P = .34). CONCLUSIONS: In heparinized pediatric patients after cardiac surgery, increased risk of bleeding is more closely associated with elevated aPTT levels than elevated anti-Xa levels. In addition to anti-Xa, monitoring of aPTT levels should be considered during titration of UFH in pediatric patients after cardiac surgery.
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Anticoagulantes/efeitos adversos , Coagulação Sanguínea/efeitos dos fármacos , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Monitoramento de Medicamentos/métodos , Fator Xa/metabolismo , Cardiopatias Congênitas/cirurgia , Heparina/efeitos adversos , Tempo de Tromboplastina Parcial , Hemorragia Pós-Operatória/diagnóstico , Trombose/prevenção & controle , Fatores Etários , Anticoagulantes/administração & dosagem , Biomarcadores/sangue , Pré-Escolar , Heparina/administração & dosagem , Humanos , Lactente , Hemorragia Pós-Operatória/sangue , Hemorragia Pós-Operatória/induzido quimicamente , Valor Preditivo dos Testes , Estudos Prospectivos , Medição de Risco , Fatores de Risco , Trombose/sangue , Trombose/diagnóstico , Trombose/etiologia , Fatores de Tempo , Resultado do TratamentoRESUMO
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. In patients treated with unfractionated heparin, anti-factor Xa activity level as well as activated partial thromboplastin time were used for dose titration. Clinical outcomes, including bleeding and thrombosis events, were prospectively collected for 5 months before and after protocol implementation and adjudicated as either minor, clinically relevant nonmajor, or major. RESULTS: Among 792 surgical patients followed during the study period, a total of 203 patients (87 preimplementation, 116 postimplementation) were treated with therapeutic unfractionated heparin over a total of 1481 patient days. Of these, 28% were neonates and 35% were infants (29 days to 1 year), with a trend toward fewer neonates and lower Risk Adjustment for Congenital Heart Surgery (RACHS) scores after protocol implementation. Among 1321 time-matched pairs, activated partial thromboplastin time and antifactor Xa activity levels were poorly correlated (r2 = 0.33). Clinically relevant bleeding events, which required increased medical care, including blood transfusion, decreased after protocol implementation (4.14 vs 1.62 bleeding events per 100 patient-days; risk ratio, 0.39 [0.20-0.75]; P = .005), even after correcting for differences in age and RACHS scores (P = .006). This finding was primarily found after RACHS category 1 to 3 procedures (risk ratio, 0.27 [0.10-0.73]; P = .0099) and in noninfants (risk ratio, 0.25 [0.09-0.65]; P = .005). There were no significant differences in the incidences of major bleeding (P = .88) or any thrombosis (P = .55). CONCLUSIONS: The use of a standardized anticoagulation protocol is feasible and might reduce the incidence of bleeding and thrombosis events in postcardiotomy patients.
Assuntos
Anticoagulantes/administração & dosagem , Coagulação Sanguínea/efeitos dos fármacos , Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas/cirurgia , Heparina/administração & dosagem , Trombose/prevenção & controle , Fatores Etários , Anticoagulantes/efeitos adversos , Biomarcadores/sangue , Boston , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Protocolos Clínicos , Esquema de Medicação , Monitoramento de Medicamentos/métodos , Fator Xa/metabolismo , Feminino , Cardiopatias Congênitas/sangue , Cardiopatias Congênitas/diagnóstico , Hemorragia/induzido quimicamente , Heparina/efeitos adversos , Humanos , Lactente , Recém-Nascido , Masculino , Tempo de Tromboplastina Parcial , Estudos Prospectivos , Fatores de Risco , Trombose/sangue , Trombose/diagnóstico , Trombose/etiologia , Fatores de Tempo , Resultado do TratamentoRESUMO
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 χ2 and Fisher exact t test. Correlations used Spearman correlation coefficient. RESULTS: Case placentas were more likely than controls to meet criteria for ≥1 of 5 major categories of pathologic abnormality (89% vs 62%; OR, 5.1; 95% CI, 1.9-14.0; P = .0007) and for ≥2 categories (38% vs 8%; OR, 7.3; 95% CI, 2.9-19.0; P < .0001). Fetal vascular malperfusion occurred in 50% of cases and 17% of controls (OR, 4.8; 95% CI, 2.2-10.5; P = .0001). Amniotic fluid inflammation occurred in 46% of cases with arterial ischemic stroke vs 25% of controls (OR, 2.6; 95% CI, 1.1-6.1; P = .037). There was evidence of a "stress response" (meconium plus elevated nucleated red blood cells) in 24% of cases compared with 1% of controls (OR, 31; 95% CI, 3.8-247.0; P < .0001). CONCLUSIONS: Placental abnormality was more common in children with neonatal stroke compared with controls. All placental findings represent subacute-to-chronic intrauterine stressors. Placental thrombotic processes were associated with both arterial and venous stroke. Our findings provide evidence for specific mechanisms that may predispose to acute perinatal stroke. Amniotic fluid inflammation associated with neonatal arterial ischemic stroke deserves further investigation.