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1.
Artigo em Inglês | MEDLINE | ID: mdl-38199293

RESUMO

OBJECTIVE: To describe clinical, functional, surgical, and outcomes data in pediatric patients with a myocardial bridge (MB) evaluated and managed following a standardized approach. METHODS: Prospective observational study included patients evaluated in the Coronary Artery Anomalies Program. Anatomy was determined by computed tomography angiography, myocardial perfusion by stress perfusion imaging, and coronary hemodynamic assessment by cardiac catheterization. RESULTS: In total, 39 of 42 patients with a complete evaluation for MB were included (December 2012 to June 2022) at a median age of 14.1 years (interquartile range, 12.2-16.4). Sudden cardiac arrest occurred in 3 of 39 (8%), exertional symptoms in 14 (36%), and no/nonspecific symptoms in 7 (18%) patients. Exercise stress test was abnormal in 3 of 34 (9%), stress perfusion imaging in 8 of 34 (24%), and resting instantaneous wave-free ratio ≤0.89 or diastolic dobutamine fractional flow reserve ≤0.80 in 11 of 21 (52%) patients. As a result, 15 of 39 (38%) patients were determined to have hemodynamically significant MB, 1 of 15 patients started beta-blocker, and 14 of 15 were referred for surgery. Myotomy (n = 11) and coronary bypass (n = 1) were performed successfully, resulting in improved symptoms and stress testing results. One patient required pericardiocentesis postoperatively, and all were discharged without other complications. At median follow-up time of 2.9 (1.8-5.8) years, all (except 2 pending surgery) were doing well without exercise restriction. CONCLUSIONS: Pediatric patients with MB can present with myocardial ischemia and sudden cardiac arrest. Provocative stress test and intracoronary hemodynamic tests helped risk-stratify symptomatic patients with MB and concern for ischemia. Surgical repair was safe and effective in mitigating exertional symptoms and stress test results, allowing patients to return to exercise without restriction.

2.
Cancers (Basel) ; 15(19)2023 Sep 29.
Artigo em Inglês | MEDLINE | ID: mdl-37835484

RESUMO

Current understanding and classification of pediatric hepatocellular adenomas (HCA) are largely based on adult data. HCAs are rare in children and, unlike in adults, are often seen in the context of syndromes or abnormal background liver. Attempts to apply the adult classification to pediatric tumors have led to several "unclassifiable" lesions. Although typically considered benign, few can show atypical features and those with beta-catenin mutations have a risk for malignant transformation. Small lesions can be monitored while larger (>5.0 cm) lesions are excised due to symptoms or risk of bleeding/rupture, etc. Management depends on gender, age, underlying liver disease, multifocality, size of lesion, histologic subtype and presence of mutation, if any. In this review, we summarize the data on pediatric HCAs and highlight our experience with their diagnosis and management.

3.
Pediatr Cardiol ; 44(7): 1566-1572, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37326858

RESUMO

Right ventricle-pulmonary artery (RV-PA) conduits are used in the treatment of certain congenital heart disease (CHD). RV-PA conduit complications might develop over time and require intervention. To evaluate how well cardiac computed tomographic angiography (CCTA) performs compared to transthoracic echocardiography (TTE) in evaluating RV-PA conduit complications by using surgical findings as the reference standard. A retrospective chart review of all patients over a 5-year period who underwent CCTA for RV-PA conduit evaluation was performed. Patient demographics and clinical data were recorded. Preoperative CCTA and TTE findings were compared to the operative findings for concordance or discordance. Forty-one patients were included, 51% females. The complications were conduit stenosis (28.68%), infection (7.17%) and aneurysm/pseudoaneurysm (6.15%). TTE and CCTA were consistently able to visualize focal conduit stenosis (96%). The greatest discrepancy between TTE and CCTA was in evaluating for aneurysm/pseudoaneurysm, where TTE detected only 2/6 (33%) compared to CCTA which detected 6/6 (100%) of the cases. However, TTE was slightly better at detecting conduit infection (3/7, 43%) compared to CCTA (2/7, 29%). Note that 5 out of 7 patients with endocarditis had bovine jugular graft. CCTA and TTE provide similar diagnostic accuracy evaluating certain types of RV-PA conduit complications. However, certain complications were only visualized on CCTA or TTE making both modalities complementary to each other during diagnostic evaluation.


Assuntos
Falso Aneurisma , Ventrículos do Coração , Feminino , Humanos , Animais , Bovinos , Masculino , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Constrição Patológica , Estudos Retrospectivos , Angiografia , Prótese Vascular , Resultado do Tratamento
4.
Front Psychiatry ; 14: 1034246, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36998625

RESUMO

Background: Although psychological interventions for stress relief, such as cognitive behavioral therapy (CBT) and mindfulness-based stress reduction (MBSR), have been developed, they have not been widely used in treating depression. The use of mobile devices can increase the possibility of actual use by integrating interventions and reducing the difficulty and cost burden of treatment application. This study aims to determine whether "inMind," an integrated mobile application for stress reduction, developed for the general population, decreases stress for patients with mild to moderate major depressive disorder during the pharmacological treatment period. Methods: This study is a single-blind, multicenter, randomized, controlled crossover trial. The App, developed in Republic of Korea, provides integrated interventions for stress reduction for the general population through three modules based on mindfulness-based stress reduction, cognitive behavior therapy, and relaxation sounds that are known to be effective in stress reduction ("meditation," "cognitive approach," and "relaxation sounds," respectively). Participants (n = 215) recruited via medical practitioner referral will be randomized to an App first group (fAPP) or a wait list crossover group (dAPP). The study will be conducted over 8 weeks; the fAPP group will use the App for the first 4 weeks and the dAPP group for the next 4 weeks. During all study periods, participants will receive their usual pharmacological treatment. The Depression Anxiety Stress Scale-21 is the primary outcome measure. The analysis will employ repeated measurements using a mixed-model approach. Discussion: The App can potentially be an important addition to depression treatment because of its applicability and the comprehensive nature of the interventions that covers diverse stress-relieving models. Clinical trial registration: https://clinicaltrials.gov/ct2/show/NCT05312203, identifier 2021GR0585.

5.
Clin Imaging ; 95: 74-79, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36657378

RESUMO

BACKGROUND AND OBJECTIVE: There is limited literature comparing TTE and CCTA in children with suspected AAOCA. To determine the distribution of various coronary anomalies comparing TTE and CCTA data, and define the added value advanced imaging brings in clinical decision-making. MATERIALS AND METHODS: Retrospective review of data was obtained in patients aged 0-18 years who underwent TTE and CCTA for suspected AAOCA. Patient demographics, CCTA and TTE findings, and interventions performed were recorded. RESULTS: 100 consecutive patients were included (60% male), mean age 11 years (7 days-18 years old). In 93 patients, CCTA detected 94 anomalous coronaries. Definitive coronary abnormality was reported on TTE in 77 patients; 76 of which were confirmed by CCTA, 1 patient was found to have a normal variant. Suspected anomalous origin was reported in 16 patients on TTE, 13 of which were abnormal on CCTA. The coronary origin was not seen on TTE in 6 patients; of these, 3 had AAOCA on CCTA and 3 had hypoplastic RCA with left dominant system. Only 1 patient who had a normal TTE was found to have AAOCA on CCTA. CCTA was better than TTE in defining ostial characteristics and the course of the anomalous coronary artery, and detecting myocardial bridge. CONCLUSIONS: CCTA adds value in diagnosing AAOCA when the coronary origins are not well assessed or suspected anomalous origin is suggested on TTE. In addition, when a confident definitive diagnosis of AAOCA is reported on TTE, CCTA demonstrates better performance in determining additional features of AAOCA.


Assuntos
Anomalias dos Vasos Coronários , Vasos Coronários , Criança , Humanos , Masculino , Feminino , Vasos Coronários/diagnóstico por imagem , Anomalias dos Vasos Coronários/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Angiografia por Tomografia Computadorizada , Estudos Retrospectivos , Angiografia , Angiografia Coronária/métodos
6.
Eur Heart J Cardiovasc Imaging ; 24(5): 598-606, 2023 04 24.
Artigo em Inglês | MEDLINE | ID: mdl-36441164

RESUMO

AIMS: We set out to design a reliable, semi-automated, and quantitative imaging tool using cardiac magnetic resonance (CMR) imaging that captures LV trabeculations in relation to the morphologic endocardial and epicardial surface, or perimeter-derived ratios, and assess its diagnostic and prognostic utility. METHODS AND RESULTS: We queried our institutional database between January 2008 and December 2018. Non-compacted (NC)-to-compacted (C) (NC/C) myocardium ratios were calculated and our tool was used to calculate fractal dimension (FD), total mass ratio (TMR), and composite surface ratios (SRcomp). NC/C, FD, TMR, and SRcomp were assessed in relation to LVNC diagnosis and outcomes. Univariate hazard ratios with cut-offs were performed using clinically significant variables to find 'at-risk' patients and imaging parameters were compared in 'at-risk' patients missed by Petersen Index (PI). Ninety-six patients were included. The average time to complete the semi-automated measurements was 3.90 min (SEM: 0.06). TMR, SRcomp, and NC/C were negatively correlated with LV ejection fraction (LVEF) and positively correlated with indexed LV end-systolic volumes (iLVESVs), with TMR showing the strongest correlation with LVEF (-0.287; P = 0.005) and SRcomp with iLVESV (0.260; P = 0.011). We found 29 'at-risk' patients who were classified as non-LVNC by PI and hence, were missed. When compared with non-LVNC and 'low-risk' patients, only SRcomp differentiated between both groups (1.91 SEM 0.03 vs. 1.80 SEM 0.03; P = 0.019). CONCLUSION: This method of semi-automatic calculation of SRcomp captured changes in at-risk patients missed by standard methods, was strongly correlated with LVEF and LV systolic volumes and may better capture outcome events.


Assuntos
Miocárdio Ventricular não Compactado Isolado , Imagem Cinética por Ressonância Magnética , Humanos , Criança , Imagem Cinética por Ressonância Magnética/métodos , Função Ventricular Esquerda , Valor Preditivo dos Testes , Imageamento por Ressonância Magnética , Volume Sistólico
7.
Pediatr Radiol ; 53(3): 509-522, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36221034

RESUMO

This article reviews the physiology of the ductus arteriosus, the pathophysiology of the patent ductus arteriosus (PDA), and the role advanced imaging such as computed tomography (CT) and magnetic resonance imaging (MRI) can play in guiding diagnosis and percutaneous or surgical intervention. A PDA can have variable clinical and radiologic presentations and can be important to characterize in patients with vascular rings, aortic maldevelopment and congenital heart disease. An understanding of the PDA and the application of CT and MRI can allow the radiologist to provide key information to physicians who plan to close a PDA or maintain PDA patency in the setting of ductal-dependent congenital heart disease.


Assuntos
Permeabilidade do Canal Arterial , Canal Arterial , Cardiopatias Congênitas , Humanos , Canal Arterial/patologia , Aorta , Tomografia Computadorizada por Raios X
8.
Pediatr Blood Cancer ; 70 Suppl 4: e29955, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36083866

RESUMO

Cardiac tumors in children are rare and the majority are benign. The most common cardiac tumor in children is rhabdomyoma, usually associated with tuberous sclerosis complex. Other benign cardiac masses include fibromas, myxomas, hemangiomas, and teratomas. Primary malignant cardiac tumors are exceedingly rare, with the most common pathology being soft tissue sarcomas. This paper provides consensus-based imaging recommendations for the evaluation of patients with cardiac tumors at diagnosis and follow-up, including during and after therapy.


Assuntos
Neoplasias Cardíacas , Rabdomioma , Esclerose Tuberosa , Criança , Humanos , Ressonância de Plasmônio de Superfície , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/complicações , Rabdomioma/diagnóstico por imagem , Rabdomioma/complicações , Diagnóstico por Imagem
9.
Pediatr Blood Cancer ; 69 Suppl 3: e29802, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35709330

RESUMO

Vascular anomalies represent a diverse group of complex disorders that can cause significant complications, including coagulopathies, pain, and decreased function. The diagnosis of vascular anomalies is often challenging due to heterogeneity of presenting phenotypes and overlapping clinical features with other pediatric conditions. Pediatric hematologists/oncologists (PHO) are uniquely positioned for an essential role in diagnosing, managing, and coordinating the multidisciplinary care required to maximize the quality of life of these patients. Here, we review the diagnostic approach involved in patients with vascular anomalies and utilize cases to highlight the challenges involved, and how PHOs can play a vital part in the care of these patients.


Assuntos
Hemangioendotelioma , Malformações Vasculares , Humanos , Qualidade de Vida , Malformações Vasculares/diagnóstico , Malformações Vasculares/terapia
11.
J Pediatr Surg ; 57(8): 1544-1553, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34366130

RESUMO

INTRODUCTION: Up to a third of children undergoing partial hepatectomy for primary hepatic malignancies experience at least one perioperative complication, with a presumed deleterious effect on both short- and long-term outcomes. We implemented a multidisciplinary treatment protocol in the management of these patients in order to improve complication rates following partial hepatectomy. METHODS: A retrospective chart review was completed for all patients < 18 years of age who underwent liver resection at our institution between 2002 and 2019 for primary hepatic cancer. Demographic, intraoperative, postoperative, pathologic, and outcome data were analyzed for perioperative complications using the CLASSIC and Clavien-Dindo (CD) scales, event-free survival (EFS) and overall survival (OS). RESULTS: A total of 73 patients were included in the analysis with 33 prior-to and 40 after dedicated provider protocol implementation. Perioperative complication rates decreased from 52% to 20% (p = 0.005) with major complications going from 18% to 10% (p = 0.31). On multivariable logistic regression, protocol implementation was associated with a reduction in any (OR 0.29 [95% CI 0.09 - 0.89]) but not major complications. On multivariate cox models, post protocol implementation was associated with improved event free survival (EFS) (HR 0.19 (0.036 - 0.195). Among patients with a diagnosis of hepatoblastoma (n = 62), the occurrence of a major perioperative complication was associated with a worse EFS (HR=5.45, p = 0.03) on multivariate analysis, however this did not translate into an impact on overall survival. CONCLUSIONS: Our results demonstrate that, for children with primary liver malignancies, a dedication of patients to high-volume surgeons can improve rates of complications of liver resections and may improve the oncological outcome of hepatoblastoma.


Assuntos
Hepatoblastoma , Neoplasias Hepáticas , Criança , Hepatectomia/métodos , Hepatoblastoma/patologia , Hepatoblastoma/cirurgia , Humanos , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/cirurgia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
12.
Pediatr Radiol ; 51(8): 1299-1310, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33755749

RESUMO

BACKGROUND: Morphological features including interarterial course, intramural course, high ostial location and slit-like ostium are presumed risk factors for sudden cardiac death in children with anomalous aortic origin of the coronary artery (AAOCA). To facilitate clinical risk stratification, the diagnostic accuracy of CT angiography for individual risk factors in the setting of AAOCA must be established. OBJECTIVE: We assessed diagnostic accuracy of standardized CT angiography interpretation for morphological characteristics that might determine risk in children with AAOCA by comparing them to surgical findings. MATERIALS AND METHODS: We created a standardized protocol for CT angiography of AAOCA and retrospectively evaluated diagnostic performance in 25 consecutive surgical patients. Relevant morphological variables in AAOCA were assessed by three independent blinded readers, with surgery as the reference standard. We used Cohen kappa coefficients and accuracies to assess agreement between readers and surgical findings, and we calculated intraclass correlation coefficients to compare length of the intramural course. RESULTS: CT angiography correctly identified AAOCA in all patients. For the three readers, accuracies for detecting ostial stenosis were 84%, 94% and 96%; for high ostial origin, accuracies were 76%, 78% 82%; for intramurality using the peri-coronary fat sign, accuracies were 98%, 96% and 92%; and for intramurality using oval shape of coronary artery, accuracies were 98%, 94% and 92%. The intraclass correlation coefficients (ICCs) for predicting intramural length among the three readers were 0.67, 0.75 and 0.81 using peri-coronary fat, and 0.69, 0.50 and 0.81 using oval shape, respectively. CONCLUSION: CT angiography reliably identified AAOCA in all children and detected the presence of intramurality with high accuracy.


Assuntos
Angiografia por Tomografia Computadorizada , Anomalias dos Vasos Coronários , Aorta , Criança , Angiografia Coronária , Anomalias dos Vasos Coronários/diagnóstico por imagem , Humanos , Estudos Retrospectivos
13.
Pediatr Radiol ; 51(7): 1185-1191, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33538849

RESUMO

BACKGROUND: The Norwood procedure is the first part of a three-stage surgical palliation for patients with functionally single ventricle anatomy. Complications after the stage I operation are not uncommon. Transthoracic echocardiography (TTE) is traditionally the mainstay for evaluation. OBJECTIVE: The purpose of our study is to compare gated cardiac computed tomographic angiography (CCTA) with TTE when evaluating for postoperative complications after stage I Norwood procedure and to describe management implications. MATERIALS AND METHODS: A retrospective chart review of all patients over a 4-year period who underwent nonelective urgent CCTA for suspected complications related to stage I Norwood procedure was performed. Elective CCTA studies before stage II palliation were excluded. Patient demographics, CCTA and TTE findings, as well as interventions performed, were recorded. RESULTS: Thirty-four patients were included. The mean age at CCTA was 63 days (range: 4-210 days). All patients had a recent TTE with a mean time interval between TTE and CCTA of 2 days. CCTA detected 56 abnormalities in 30 patients, with 23 directly related to postsurgical complications, including shunt-related complications (10/23, 43%), Damus-Kaye-Stansel anastomotic narrowing (2/23, 9%) and neo-aortic arch/branch vessel abnormalities (11/23, 48%). These complications were managed as follows: surgery (9, 39%), catheter-based intervention (7, 30%), medical (4, 17%) and no change in management (3, 13%). TTE did not detect 8/23 (35%) findings found on CCTA, of which 75% were either managed with surgery (4/8, 50%) or catheter-based intervention (2/8, 25%). CONCLUSION: CCTA plays an important role in detecting surgical complications after stage I Norwood procedure and demonstrates additional findings that have direct management implications.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico , Procedimentos de Norwood , Angiografia , Ecocardiografia , Coração , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Cuidados Paliativos , Estudos Retrospectivos , Resultado do Tratamento
14.
J Invasive Cardiol ; 33(1): E45-E51, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33385986

RESUMO

BACKGROUND: Invasive fractional flow reserve (FFR) is considered the gold standard to evaluate coronary artery flow. Stress cardiovascular magnetic resonance (sCMR) is an emerging non-invasive tool to evaluate myocardial perfusion in children. We sought to compare sCMR with FFR to determine impaired intracoronary flow in children with anomalous aortic origin of a coronary artery (AAOCA) and/or myocardial bridge (MB) who presented concern for myocardial ischemia. METHODS: From December 2012 to May 2019, AAOCA and/or MB patients (<20 years old) were prospectively enrolled and underwent sCMR and FFR. Abnormal sCMR included perfusion/regional wall-motion abnormality in the involved coronary distribution. FFR was performed at baseline and with dobutamine/regadenoson and considered abnormal if <0.8 in the affected coronary segment. RESULTS: Of 376 patients evaluated, a total of 19 (age range, 0.2-17 years) underwent 24 sets of sCMR and FFR studies, with 5 repeat studies following intervention. Types of anomalies included 6 isolated MB/normal CA origins, 5 single CAs, 5 left AAOCAs, and 3 right AAOCAs. Seventeen patients (89.5%) had MB/intramyocardial course - 14 involving the left anterior descending coronary artery and 3 with multivessel involvement. sCMR correlated with FFR in 19/24 sets (7 sCMR and FFR positive, 12 sCMR and FFR negative) and it did not correlate in 5/24 sets. The positive percent agreement was 77.8%, negative percent agreement was 80.0%, and overall percent agreement was 79.2%. CONCLUSIONS: Assessment of myocardial perfusion using non-invasive sCMR concurred with FFR, particularly if performed with close proximity in time, and may contribute to risk stratification and decision making in children with AAOCA and/or MB.


Assuntos
Doença da Artéria Coronariana , Reserva Fracionada de Fluxo Miocárdico , Imagem de Perfusão do Miocárdio , Adolescente , Criança , Pré-Escolar , Angiografia Coronária , Estenose Coronária , Humanos , Lactente , Perfusão , Valor Preditivo dos Testes
15.
Pediatr Radiol ; 51(3): 419-426, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33151345

RESUMO

BACKGROUND: Children with suspected renal artery stenosis (RAS) are screened with renal Doppler ultrasonography or computed tomography (CT) angiography/magnetic resonance (MR) angiography depending on institutional preference. CT angiography produces images with superior resolution, allowing higher quality multiplanar two-dimensional reformats and three-dimensional reconstructions. However, there is a paucity of data in the literature regarding the utility and diagnostic performance of renal CT angiography in pediatric RAS. OBJECTIVE: The objective of this study is to retrospectively review our experience with renal CT angiography in the diagnosis of pediatric RAS relative to digital subtraction angiography (DSA) as the reference standard. MATERIALS AND METHODS: All patients 0-18 years of age who underwent CT angiography for evaluation of RAS as a cause of hypertension between January 2012 and May 2019 were identified for the study. A total of 131 patients were identified, 23 of whom had DSA correlation. RESULTS: Twenty-three patients (17 boys, 6 girls) with a mean age of 6 years 3 months (range: 3 months to 14 years 7 months) were included in this study. Of the 59 renal arteries studied by DSA, 22 were abnormal on CT angiography and 20 were abnormal on DSA. Of the 59 renal arteries, CT angiography was true positive in 18 and true negative in 35. The sensitivity and specificity of CT angiography for RAS diagnosis were 90.0% and 89.7%, respectively. CT angiography identified all cases of main RAS. CONCLUSION: Renal CT angiography has a high sensitivity and specificity for pediatric RAS diagnosis in patients referred for DSA.


Assuntos
Obstrução da Artéria Renal , Angiografia Digital , Criança , Angiografia por Tomografia Computadorizada , Meios de Contraste , Feminino , Humanos , Lactente , Angiografia por Ressonância Magnética , Masculino , Obstrução da Artéria Renal/diagnóstico por imagem , Estudos Retrospectivos , Sensibilidade e Especificidade , Tomografia Computadorizada por Raios X
16.
J Pediatr Surg ; 55(12): 2828-2835, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32792165

RESUMO

BACKGROUND: The separation of conjoined twins is a challenging and rare operation. Recent technological advances in imaging and three-dimensional printing (3DP) have allowed for enhancements in preoperative surgical planning and intraoperative anatomical orientation for complex operations. This report aims to consolidate the current clinical evidence utilizing 3DP models as an effective tool for surgical planning of conjoined twin separation and to detail our surgical approach for complex hepatic separation and management. METHODS: A literature review was conducted for conjoined twin separations with preoperative use of 3D models including age at attempted separation, operative outcome, 3D modality, and postoperative course between 1998 and 2020. We also conducted a chart review of our electronic medical record for conjoined twin separations between January 2015 and December 2019. RESULTS: We report two cases of conjoined twin separation with preoperative use of 3DP models from our institution: one set in the thoracoomphaloischiopagus orientation and the other set in the thoracoomphalopagus orientation with the presence of intrahepatic vascular anomalies. The literature review produced 10 case reports of conjoined twin separation with use preoperative 3D models accounting for 17 individual separation procedures. We summarize our preoperative radiological planning, the evidence of 3DP models as an educational and preoperative tool, ideal timing for separation, and our surgical approach for complex hepatic separation. CONCLUSIONS: Conjoined twin separation requires a multidisciplinary effort to address the multisystem surgical and medical needs of these patients. These complex patients require extensive preoperative imaging for planning separation, and we strongly recommend utilizing 3D printed models when possible for better surgeon understanding of complex variable anatomy. We have found numerous reports of successful conjoined twin separation using 3DP technology in preoperative planning. The use of three-dimensional printed models for preoperative assessments is an invaluable tool and is rapidly improving in fidelity. TYPE OF STUDY: Operative technique and case series. LEVEL OF EVIDENCE: Level IV.


Assuntos
Modelos Anatômicos , Impressão Tridimensional , Gêmeos Unidos , Humanos , Fígado/cirurgia , Gêmeos Unidos/cirurgia
18.
Radiology ; 296(3): 493-497, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32602829

RESUMO

Appropriate imaging is imperative in evaluating children with a primary hepatic malignancy such as hepatoblastoma or hepatocellular carcinoma. For use in the adult patient population, the American College of Radiology created the Liver Imaging Reporting and Data System (LI-RADS) to provide consistent terminology and to improve imaging interpretation. At present, no similar consensus exists to guide imaging and interpretation of pediatric patients at risk for developing a liver neoplasm or how best to evaluate a pediatric patient with a known liver neoplasm. Therefore, a new Pediatric Working Group within American College of Radiology LI-RADS was created to provide consensus for imaging recommendations and interpretation of pediatric liver neoplasms. The article was drafted based on the most up-to-date existing information as interpreted by imaging experts comprising the Pediatric LI-RADS Working Group. Guidance is provided regarding appropriate imaging modalities and protocols, as well as imaging interpretation and reporting, with the goals to improve imaging quality, to decrease image interpretation errors, to enhance communication with referrers, and to advance patient care. An expanded version of this document that includes broader background information on pediatric hepatocellular carcinoma and rationale for recommendations can be found in Appendix E1 (online).


Assuntos
Carcinoma Hepatocelular/diagnóstico por imagem , Hepatoblastoma/diagnóstico por imagem , Neoplasias Hepáticas/diagnóstico por imagem , Biópsia , Criança , Pré-Escolar , Consenso , Humanos , Lactente , Imageamento por Ressonância Magnética , Guias de Prática Clínica como Assunto , Sistemas de Informação em Radiologia/organização & administração , Tomografia Computadorizada por Raios X
19.
Leuk Lymphoma ; 61(10): 2419-2427, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32519904

RESUMO

Survivors of childhood Hodgkin lymphoma (HL) are at risk for pulmonary late effects, but whether survivors also experience pulmonary dysfunction early off therapy is not well understood. We determined the incidence of pulmonary dysfunction in children/adolescents with HL at entry into survivorship, as well as risk factors related to this outcome. Survivors in clinical remission and with a pulmonary function test (PFT) obtained 2-6 years off therapy were included. Seventy-five of 118 subjects met eligibility criteria (mean age at diagnosis: 13 years, mean time off therapy: 40 months). Survivors of HL had a higher than expected incidence of pulmonary dysfunction at entry into survivorship (40/75 [53%] had an abnormal DLCO and/or a restrictive or obstructive impairment). Evidence for diffusion impairment was associated with female sex (odds ratio [OR] = 3.19, p = .04). Longitudinal follow-up studies are needed to determine if early evidence of pulmonary dysfunction predicts risk for later onset pulmonary outcomes.


Assuntos
Doença de Hodgkin , Adolescente , Criança , Feminino , Seguimentos , Doença de Hodgkin/complicações , Doença de Hodgkin/epidemiologia , Doença de Hodgkin/terapia , Humanos , Incidência , Fatores de Risco , Sobreviventes
20.
Pediatr Radiol ; 50(8): 1095-1101, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32242280

RESUMO

BACKGROUND: Demand for pediatric cardiac computed tomography (CT) angiography is increasing due to recent advances that reduce the need for sedation and radiation exposure while enhancing diagnostic accuracy. This has resulted in the increasing use of cardiac CT angiography emergently during weekends and after hours. The unexpected demand for these services can be challenging, as most hospitals are not staffed to provide 24/7 pediatric cardiovascular imaging. OBJECTIVE: To describe a large single-center experience of providing emergent cardiac CT angiography services in children. MATERIALS AND METHODS: We identified all patients who underwent after--hours weekday and weekend emergent cardiac CT angiography between January 2017 and August 2018. Cardiac CT angiography in the settings of congenital heart disease and coronary imaging were included. Data collected included day and time of cardiac CT angiography, patient age, referral unit, indication, surgical history, need for sedation, need for surgery, intervention and/or change in medical management based on the cardiac CT angiography. RESULTS: Forty-seven studies were identified, 26 (55%) of which were performed on a weekend or holiday and 21 (45%) after 5 p.m. on a weekday. Based on cardiac CT angiography findings, 20 (43%) patients underwent either surgery or an interventional procedure, and 9 (19%) had a change in medical management. The time between cardiac CT angiography and the related surgery/intervention ranged from 0 to 29 days with a median of 3.5 days. CONCLUSION: Emergent pediatric cardiac CT angiography is a valuable service. Larger multi-institutional studies with standardized referral and utilization patterns are needed to determine if outcomes are affected by this service, which in turn will influence hospital staffing patterns for emergent imaging.


Assuntos
Angiografia por Tomografia Computadorizada/métodos , Angiografia Coronária/métodos , Cardiopatias Congênitas/diagnóstico por imagem , Técnicas de Imagem de Sincronização Cardíaca , Criança , Pré-Escolar , Meios de Contraste , Eletrocardiografia , Emergências , Feminino , Cardiopatias Congênitas/terapia , Hospitais Pediátricos , Humanos , Imageamento Tridimensional , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos
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