Role of Computed Tomography Angiography in the Short-Term Follow-up of Aortic Coarctation Repair.

INTRODUCTION: Coarctation of the aorta (CoA) is a narrowing of the thoracic aorta that often manifests as discrete stenosis but may be tortuous or in long segment. The study aimed to evaluate pre and post-surgical aspects of pediatric patients submitted to CoA surgical correction and to identify possible predisposing factors for aortic recoarctation. METHODS: Twenty-five patients were divided into groups according to presence (N=8) or absence (N=17) of recoarctation after surgical correction of CoA and evaluated according to clinical-demographic profile, vascular characteristics via computed angiotomography (CAT), and other pathological conditions. RESULTS: Majority of males (64%), ≥ 15 days old (76%), ≥ 2.5 kg (80%). There was similarity between groups with and without recoarctation regarding sex (male: 87% vs. 53%; P=0.277), age (≥ 15 days: 62.5 vs. 82%; P=0.505), and weight (≥ 2.5 kg: 87.5 vs. 76.5; P=0,492). Altered values of aortic root/Valsalva diameter, proximal transverse arch, and distal isthmus, and normal values for aorta prevailed in preoperative CAT. Normal values for the aortic root/Valsalva sinus diameter were observed with and without recoarctation, the same for both groups regarding ascending and descending aorta in postoperative CAT. No significant difference for altered values of proximal transverse arch and alteration in distal isthmus was observed. CONCLUSION: No predictive risk for recoarctation was observed. CTA proved to be important in CoA diagnosis and management, since CoA is mainly related with altered diameter of aortic root/sinus of Valsalva and proximal and distal aortic arch/isthmus, however, it failed to show predictive risk for recoarctation.

Role of Computed Tomography Angiography in the Short-Term Follow-up of Aortic Coarctation Repair

ABSTRACT Introduction: Coarctation of the aorta (CoA) is a narrowing of the thoracic aorta that often manifests as discrete stenosis but may be tortuous or in long segment. The study aimed to evaluate pre and post-surgical aspects of pediatric patients submitted to CoA surgical correction and to identify possible predisposing factors for aortic recoarctation. Methods: Twenty-five patients were divided into groups according to presence (N=8) or absence (N=17) of recoarctation after surgical correction of CoA and evaluated according to clinical-demographic profile, vascular characteristics via computed angiotomography (CAT), and other pathological conditions. Results: Majority of males (64%), ≥ 15 days old (76%), ≥ 2.5 kg (80%). There was similarity between groups with and without recoarctation regarding sex (male: 87% vs. 53%; P=0.277), age (≥ 15 days: 62.5 vs. 82%; P=0.505), and weight (≥ 2.5 kg: 87.5 vs. 76.5; P=0,492). Altered values of aortic root/Valsalva diameter, proximal transverse arch, and distal isthmus, and normal values for aorta prevailed in preoperative CAT. Normal values for the aortic root/Valsalva sinus diameter were observed with and without recoarctation, the same for both groups regarding ascending and descending aorta in postoperative CAT. No significant difference for altered values of proximal transverse arch and alteration in distal isthmus was observed. Conclusion: No predictive risk for recoarctation was observed. CTA proved to be important in CoA diagnosis and management, since CoA is mainly related with altered diameter of aortic root/sinus of Valsalva and proximal and distal aortic arch/isthmus, however, it failed to show predictive risk for recoarctation.

IS -94INS/DELATTG POLYMORPHISM IN THE NUCLEAR FACTOR KAPPA-B1 GENE (NFKB1) ASSOCIATED WITH NECROTIZING ENTEROCOLITIS?

BACKGROUND: Abnormalities in the different stages of the intestinal maturation process cause metabolic and molecular changes. Among the genetic alterations associated with necrotizing enterocolitis, the -94ins/delATTG polymorphism in NFKB1 gene leads to unregulated activation of the NFKB protein due to an increase in the inherent pro-inflammatory state of the premature intestine. AIMS: To determine the prevalence of the -94ins/delATTG polymorphism in NFKB1 gene in neonates with and without necrotizing enterocolitis. METHODS: This is a case-control study, in which 25 neonates were evaluated as the case group and 50 neonates as the control group, of both genders. DNA was extracted from peripheral blood leukocytes, and the site encompassing the polymorphism was amplified by molecular techniques (polymerase chain reaction/polymorphism in restriction fragment length). RESULTS: Necrotizing enterocolitis was diagnosed in 25 (33%) neonates and, of these, 3 (12%) died. Male gender was more prevalent in both groups (p=0.1613): cases (52%) and controls (62%). Moderate and extreme preterm newborns were predominant in both groups: cases (80%) and controls (88%) (p=0.3036). Low birth weight and extremely low birth weight newborns were the most prevalent in cases (78%), and very low birth weight and extremely low birth weight were the most prevalent in controls (81%) (p=0.1073). Clinical treatment was successful in 72%, and hospital discharge was achieved in 88% of newborns with NEC. The -94ins/delATTG polymorphism in NFKB1 gene was not identified in all the 150 alleles analyzed (100%). CONCLUSIONS: The absence of the -94ins/delATTG polymorphism in NFKB1 gene in newborns with and without necrotizing enterocolitis does not rule out the possibility of alterations in this and/or in other genes in newborns with this condition, which reinforces the need for further research.

Is -94ins/delattg polymorphism in the nuclear factor kappa-b1 gene (nfkb1) associated with necrotizing enterocolitis? / Polimorfismo -94ins/delattg no gene do fator nuclear kappa-b1 (nfkb1) está associado à enterocolite necrosante?

ABSTRACT BACKGROUND: Abnormalities in the different stages of the intestinal maturation process cause metabolic and molecular changes. Among the genetic alterations associated with necrotizing enterocolitis, the -94ins/delATTG polymorphism in NFKB1 gene leads to unregulated activation of the NFKB protein due to an increase in the inherent pro-inflammatory state of the premature intestine. AIMS:To determine the prevalence of the -94ins/delATTG polymorphism in NFKB1 gene in neonates with and without necrotizing enterocolitis. METHODS:This is a case-control study, in which 25 neonates were evaluated as the case group and 50 neonates as the control group, of both genders. DNA was extracted from peripheral blood leukocytes, and the site encompassing the polymorphism was amplified by molecular techniques (polymerase chain reaction/polymorphism in restriction fragment length). RESULTS:Necrotizing enterocolitis was diagnosed in 25 (33%) neonates and, of these, 3 (12%) died. Male gender was more prevalent in both groups (p=0.1613): cases (52%) and controls (62%). Moderate and extreme preterm newborns were predominant in both groups: cases (80%) and controls (88%) (p=0.3036). Low birth weight and extremely low birth weight newborns were the most prevalent in cases (78%), and very low birth weight and extremely low birth weight were the most prevalent in controls (81%) (p=0.1073). Clinical treatment was successful in 72%, and hospital discharge was achieved in 88% of newborns with NEC. The -94ins/delATTG polymorphism in NFKB1 gene was not identified in all the 150 alleles analyzed (100%). CONCLUSIONS:The absence of the -94ins/delATTG polymorphism in NFKB1 gene in newborns with and without necrotizing enterocolitis does not rule out the possibility of alterations in this and/or in other genes in newborns with this condition, which reinforces the need for further research.

RESUMO RACIONAL:Anormalidades nas diferentes fases do processo de maturação intestinal causam alterações metabólicas e moleculares. Dentre as alterações genéticas associadas à enterocolite necrotizante, o polimorfismo -94ins/delATTG no gene NFKB1 leva à ativação desregulada da proteína NFKB devido ao aumento do estado pró-inflamatório inerente ao intestino prematuro. OBJETIVOS:Determinar a prevalência do polimorfismo -94ins/delATTG no gene NFKB1 em neonatos com e sem enterocolite necrotizante. MÉTODOS: Trata-se de um estudo caso-controle, no qual foram avaliados 25 neonatos como grupo caso e 50 neonatos como grupo controle, de ambos os sexos. O DNA foi extraído de leucócitos do sangue periférico e o sítio que engloba o polimorfismo foi amplificado por técnicas moleculares (reação em cadeia da polimerase/polimorfismo no comprimento do fragmento de restrição). RESULTADOS: Enterocolite necrosante foi diagnosticada em 25 (33%) neonatos e, destes, 3 (12%) foram a óbito. O gênero masculino foi mais prevalente em ambos os grupos (p=0,1613): casos (52%) e controles (62%). Os prematuros moderados e extremos foram predominantes em ambos os grupos: casos (80%) e controles (88%) (p=0,3036). Recém-nascidos de baixo peso e extremo baixo peso foram os mais prevalentes nos casos (78%) e de muito baixo peso e extremo baixo peso foram os mais prevalentes nos controles (81%) (p=0,1073). O tratamento clínico foi bem-sucedido em 72% e a alta hospitalar foi obtida em 88% dos recém-nascidos com enterocolite necrotizante. O polimorfismo -94ins/delATTG no gene NFKB1 não foi identificado em todos os 150 alelos analisados (100%). CONCLUSÕES: A ausência do polimorfismo -94ins/delATTG no gene NFKB1 em recém-nascidos com e sem enterocolite necrosante não afasta a possibilidade de alterações neste e/ou em outros genes em recém-nascidos com esta condição, o que reforça a necessidade de novas pesquisas.

Leucomalácia periventricular e correlação com citocinas pro e antiinflamatórias / Periventricular leukomalacia and correlation with pro and anti-inflammatory cytokines

Introdução:Polimorfismos em genes de citocinas inflamatórias (TNF-α e IL-1ß) e antiinflamatórias (IL-10) intensificam a resposta inflamatória, após anóxia, aumentando as afecções decorrentes da síndrome hipóxico-isquêmica como a leucomalácia periventricular (LPV). Objetivos: Investigar a associação entre ambos os polimorfismos inflamatórios (-1031T/C no gene TNF-α e -511C/T no gene IL-1ß) e o antiinflamatório (-1082G/A no gene IL-10) e a etiopatogênese/risco da LPV em neonatos com esta afecção. Material e Métodos: Estudo prospectivo de casos-controle em 50 neonatos prematuros e a termo (Grupo Casos) e em 50 neonatos a termo (Grupo Controle), de ambos os sexos. DNA foi extraído de leucócitos de sangue periférico e a análise molecular realizada pela Reação em Cadeia da Polimerase/Análise de Restrição Enzimática (PCR/RFLP). Resultados: A idade gestacional média entre casos e controles foi, respectivamente, de 31,0 semanas e 39,4 semanas (p<0,0001). O peso médio, em gramas, foi de 1561,1 para os casos e 3509,9 para controles (p<0,0001). Foi encontrada associação entre o genótipo TC (produtor intermediário de citocina inflamatória) (OR: 2.495; IC95%: 1,10-5,63; p=0,043) assim como entre os genótipos TC+CC (produtores inflamatórios intermediário+alto) (OR: 2,471; IC95%: 1,10-5,55; p=0,044) no gene TNF-α e o risco de LPV. Estatisticamente significante associação foi encontrada entre os genótipos (CT+TT) (produtores inflamatórios intermediário+alto) (OR: 23,120; IC95%: 1,31-409,4; p=0,003) no gene IL-1ß e o risco de LPV. No gene IL-10, foi encontrada reduçãosignificativa do risco de LPV para o genótipo GG (alto produtor antiinflamatório) (OR: 0,07407; IC95%: 0,02-0,34; p<0,0001)assim como para o alelo G (OR: 0,5098; IC95%: 0,29-0,91; p=0,030). Conclusão: há associação entre os polimorfismosinflamatórios (-1031T/C no gene TNF-α e -511C/T no gene IL-1ß) e o risco de desenvolvimento de LPV e associação entre opolimorfismo antiinflamatório (-1082G/A no gene IL-10) na proteção ao desenvolvimento da LPV, na população estudada.

The association between pro- and anti-inflammatory cytokine polymorphisms and periventricular leukomalacia in newborns with hypoxic-ischemic encephalopathy.

BACKGROUND: Periventricular leukomalacia (PVL) is a frequent consequence of hypoxic-ischemic injury. Functional cytokine gene variants that result in altered production of inflammatory (tumor necrosis factor-alpha [TNF-α] and interleukin-1beta [IL-1ß]) or anti-inflammatory (interleukin-10 [IL-10]) cytokines may modify disease processes, including PVL. OBJECTIVE: The aim of this study was to evaluate if there is a relationship between the two proinflammatory polymorphisms (TNF-α-1031T/C and IL-1ß-511C/T) and the anti-inflammatory polymorphism IL-10-1082G/A and PVL risk in Brazilian newborns with and without this injury. MATERIALS AND METHODS: A cross-sectional case-control study performed at the Neonatal Intensive Care Unit of the Children's Hospital and Maternity of the São José do Rio Preto Medical School (FAMERP). Fifty preterm and term newborns were examined as index cases and 50 term newborns as controls, of both sexes for both groups. DNA was extracted from peripheral blood leukocytes, and the sites that encompassed the three polymorphisms were amplified by polymerase chain reaction-restriction fragment length polymorphism. RESULTS: Gestational age ranged from 25 to 39 weeks, in the case group, and in the control group it ranged from 38 to 42.5 weeks (P<0.0001). Statistically significant association was found between TNF-α-1031T/C high expression genotype TC (odds ratio [OR], 2.495; 95% confidence interval [CI], 1.10-5.63; P=0.043) as well as between genotypes (TC + CC) (OR, 2.471; 95% CI, 1.10-5.55; P=0.044) and risk of PVL. Statistically significant association was found between IL-1ß-511C/T high expression genotypes (CT + TT) (OR, 23.120; 95% CI, 1.31-409.4; P=0.003) and risk of PVL. Statistically significant association between IL-10-1082G/A high expression genotype GG (OR, 0.07407; 95% CI, 0.02-0.34; P<0.0001) as well as between IL-10-1082G high expression allele (OR, 0.5098; 95% CI, 0.29-0.91; P=0,030) and PVL reduced risk was observed. There was a statistically significant association between TC/CT/GA genotype combination and the risk of PVL (OR, 6.469; 95% CI, 2.00-20.92; P=0.001). CONCLUSION: There is evidence of an association between the polymorphisms TNF-α-1031T/C, IL-1ß-511C/T, and IL-10-1082G/A and PVL risk in this Brazilian newborn population studied.

When Closure Fails: What the Radiologist Needs to Know About the Embryology, Anatomy, and Prenatal Imaging of Ventral Body Wall Defects.

Ventral body wall defects (VBWDs) are one of the main categories of human congenital malformations, representing a wide and heterogeneous group of defects sharing a common feature, that is, herniation of one or more viscera through a defect in the anterior body wall. Gastroschisis and omphalocele are the 2 most common congenital VBWDs. Other uncommon anomalies include ectopia cordis and pentalogy of Cantrell, limb-body wall complex, and bladder and cloacal exstrophy. Although VBWDs are associated with multiple abnormalities with distinct embryological origins and that may affect virtually any system organs, at least in relation to anterior body wall defects, they are thought (except for omphalocele) to share a common embryologic mechanism, that is, a failure involving the lateral body wall folds responsible for closing the thoracic, abdominal, and pelvic portions of the ventral body wall during the fourth week of development. Additionally, many of the principles of diagnosis and management are similar for these conditions. Fetal ultrasound (US) in prenatal care allows the diagnosis of most of such defects with subsequent opportunities for parental counseling and optimal perinatal management. Fetal magnetic resonance imaging may be an adjunct to US, providing global and detailed anatomical information, assessing the extent of defects, and also helping to confirm the diagnosis in equivocal cases. Prenatal imaging features of VBWDs may be complex and challenging, often requiring from the radiologist a high level of suspicion and familiarity with the imaging patterns. Because an appropriate management is dependent on an accurate diagnosis and assessment of defects, radiologists should be able to recognize and distinguish between the different VBWDs and their associated anomalies. In this article, we review the relevant embryology of VBWDs to facilitate understanding of the pathologic anatomy and diagnostic imaging approach. Features will be illustrated with prenatal US and magnetic resonance imaging and correlated with postnatal and clinical imaging.

Is magnetic resonance spectroscopy capable of detecting metabolic abnormalities in neurofibromatosis type 1 that are not revealed in brain parenchyma of normal appearance?

BACKGROUND: Results of magnetic resonance spectroscopy studies in normal-appearing brain and in non-neoplastic brain lesions in individuals with neurofibromatosis type 1 (NF1) have been discrepant. OBJECTIVE: We used magnetic resonance spectroscopy to analyze the metabolic patterns in the basal ganglia of patients with NF1 and examine their correlation with focal hyperintense lesions in T2-weighted images (T2-weighted hyperintensities). METHODS: We used magnetic resonance spectroscopy data of 42 individuals with NF1 (18 with and 24 without T2- weighted hyperintensities) and 25 controls matched for gender and age. A single-voxel technique was employed by manually placing a region of interest with a uniform size over a predetermined anatomical region including the globus pallidum and putamen (capsulolenticular region). We further analyzed the ratios of choline/creatine, N-acetyl aspartate (NAA)/creatine, and myoinositol/creatine metabolites and the occurrence of T2-weighted hyperintensities in these regions in individuals with NF1. RESULTS: There was a significant difference between the NF1 and control groups with regard to the mean values of myoinositol/creatine and choline/creatine, with higher metabolite values observed in the NF1 group (P < 0.001). Only the myoinositol/creatine ratio was able to discriminate between NF1 subgroups with and without T2-weighted hyperintensities. For the NAA/creatine ratio, there was no significant difference between the NF1 and the control groups. CONCLUSION: Magnetic resonance spectroscopy allows the characterization of tissue abnormalities not demonstrable in the structural images of individuals with NF1 through choline and myoinositol metabolite analysis. Yet the preserved NAA values argue against demyelination and axonal degeneration occurring in the region, suggesting instead a functional neuronal stability. Taken in association with the findings of lack of clinical manifestations and the known transient nature of T2-weighted hyperintensities in NF1 as demonstrated by other studies, our results support the current histopathologically driven hypothesis that such T2-weighted hyperintensities may be related to intramyelinic edema.

Pulmonary interstitial emphysema: a case report and review of the literature / Enfisema intersticial pulmonar: relato de caso e revisão da literatura

Pulmonary interstitial emphysema is a rare condition that generally affects low-weight preterm infants submitted to mechanical ventilation. The prognosis is variable, depending on early diagnosis and treatment. The radiologist plays a key role in this scenario. The authors report a case of persistent pulmonary interstitial emphysema, describing the main characteristics of such entity.

O enfisema intersticial pulmonar é uma condição rara que acomete, em geral, recém-nascidos pré-termos de baixo peso submetidos a ventilação mecânica. O prognóstico é variável e dependente do diagnóstico e tratamento precoces, ressaltando-se a importância do radiologista neste cenário. Os autores relatam um caso de enfisema intersticial pulmonar persistente, abordando as principais características desta entidade.

Unidentified bright objects in neurofibromatosis type 1: conventional MRI in the follow-up and correlation of microstructural lesions on diffusion tensor images.

PURPOSE: To evaluate the evolution of unidentified bright objects (UBOs) in individuals with neurofibromatosis type 1 (NF1) by serial magnetic resonance imaging (MRI), and to relate this to regional fractional anisotropy (FA). MATERIALS AND METHODS: The signal pattern of the T2-weighted sequences in the basal ganglia, thalamus, brain stem, and cerebellum for 27 NF1 individuals and a control group were analyzed by diffusion tensor imaging (DTI). The presence or absence of UBOs in 2 consecutive MRI examinations was related to FA. RESULTS: We demonstrated significant differences in FA for the basal ganglia, cerebellum, and thalamus between NF1 patients and controls (P ≤ 0.05), even with a reduction or disappearance of UBOs. CONCLUSIONS: MRI allows for adequate monitoring of the temporal and spatial distribution of UBOs in patients with NF1. DTI confirmed changes in FA despite the disappearance or reduction of UBOs, thereby confirming the hypothesis that microstructural damage occurs in specific brain regions of NF1 patients.

Tomografia computadorizada na avaliação tardia do tratamento cirúrgico da conexão anômala total de veias pulmonares / Computed tomography in late evaluation of surgical treatment of pulmonary veins total anomalous connection

OBJETIVO: Verificar se os achados da tomografia computadorizada de múltiplos detectores (TCMD) apresentam associação com os parâmetros clínicos e exames complementares rotineiramente empregados na avaliação tardia das crianças submetidas ao tratamento cirúrgico da conexão anômala total de veias pulmonares (CATVP). MÉTODOS: No período de janeiro 2002 a dezembro de 2007, 12 pacientes operados de CATVP foram avaliados tardiamente com anamnese, exame físico, radiografia de tórax, eletrocardiograma, ecocardiograma e TCMD. Alterações específicas de cada um desses exames foram identificadas e comparadas com os achados qualitativos da TCMD. RESULTADOS: Onze pacientes estavam em classe funcional I (NYHA), três apresentavam sopros inespecíficos, três estavam abaixo do percentil 15 de desenvolvimento pôndero-estatural. À radiografia de tórax, dois pacientes tinham alteração dos campos pulmonares e três, aumento discreto da área cardíaca. Ao eletrocardiograma, um paciente apresentava sobrecarga ventricular direita e um, ritmo juncional. Todos os ecocardiogramas mostraram-se dentro dos limites de normalidade, exceto em um paciente com estenose entre a veia cava superior e o átrio direito. A TCMD foi totalmente normal em quatro pacientes, em três demonstrou compressão de veias pulmonares e em quatro, redução de calibre considerada significativa, as quais se correlacionaram com os demais achados. Assim, a TCMD para demonstrar alterações anatômicas, quando comparadas a alterações do exame físico ou outros exames complementares testados, apresentou sensibilidade de 87,5 por cento, especificidade de 75 por cento, valor preditivo positivo 87,5 por cento, valor preditivo negativo de 75 por cento e acurácia de 83,3 por cento. CONCLUSÃO: No acompanhamento tardio dos pacientes submetidos à correção cirúrgica de CATVP, a TCMD pode fornecer subsídios valiosos e complementar o diagnóstico de eventuais alterações anatômicas e funcionais.

OBJECTIVE: To evaluate if the findings of multislice computed tomography (MSCT) are associated with clinical and laboratory tests routinely used in the late follow-up of children undergoing surgical treatment of total anomalous pulmonary venous connection (TAPVC). METHODS: From January 2002 to December 2007, 12 patients operated due to CATVP were evaluated with history, physical examination, chest X-ray, electrocardiogram, echocardiography and MSCT. Specific changes observed in each one of these tests were identified and compared with MSCT qualitative findings. RESULTS: Eleven patients were in functional class I (NYHA), three had nonspecific murmurs, and three were below the 15th percentile of weight and height. Two had pulmonary field abnormalities and three had a slight increase of the cardiac area in the X-ray examination. In the electrocardiogram, one patient had right ventricular overload and one had junctional rhythm. All echocardiograms were within the normal range, except for one patient with stenosis between the superior vena cava and right atrium. MSCT was completely normal in four patients, three had compression of the pulmonary veins and four had significant caliber reduction, which correlated with the other findings. Thus, MSCT showed a sensitivity of 87.5 percent, specificity of 0.75 percent, positive predictive value of 87.5 percent, negative predictive value of 75 percent and accuracy of 83.3 percent to demonstrate anatomic changes compared to changes in the physical examination or other additional tests. CONCLUSION: MSTC may provide valuable information and complement the diagnosis of possible anatomical and functional changes in the late follow-up of patients undergoing surgical repair of TAPVC.

Left pulmonary vein atresia: the contribution of multislice computed tomography.

Unilateral pulmonary vein atresia is a rare congenital heart disease. Its symptoms begin to manifest in childhood and may be similar to those of other left-side heart obstructions. The diagnosis of this disorder is difficult and usually requires several imaging methods. This report presents the case of a 7-year-old girl whose diagnosis was aided through the use of multislice computed tomography.

Computed tomography in late evaluation of surgical treatment of pulmonary veins total anomalous connection.

OBJECTIVE: To evaluate if the findings of multislice computed tomography (MSCT) are associated with clinical and laboratory tests routinely used in the late follow-up of children undergoing surgical treatment of total anomalous pulmonary venous connection (TAPVC). METHODS: From January 2002 to December 2007, 12 patients operated due to CATVP were evaluated with history, physical examination, chest X-ray, electrocardiogram, echocardiography and MSCT. Specific changes observed in each one of these tests were identified and compared with MSCT qualitative findings. RESULTS: Eleven patients were in functional class I (NYHA), three had nonspecific murmurs, and three were below the 15th percentile of weight and height. Two had pulmonary field abnormalities and three had a slight increase of the cardiac area in the X-ray examination. In the electrocardiogram, one patient had right ventricular overload and one had junctional rhythm. All echocardiograms were within the normal range, except for one patient with stenosis between the superior vena cava and right atrium. MSCT was completely normal in four patients, three had compression of the pulmonary veins and four had significant caliber reduction, which correlated with the other findings. Thus, MSCT showed a sensitivity of 87.5%, specificity of 0.75%, positive predictive value of 87.5%, negative predictive value of 75% and accuracy of 83.3% to demonstrate anatomic changes compared to changes in the physical examination or other additional tests. CONCLUSION: MSTC may provide valuable information and complement the diagnosis of possible anatomical and functional changes in the late follow-up of patients undergoing surgical repair of TAPVC.

Aplicação clínica da ultrassonografia craniana com Doppler em neonatos prematuros de muito baixo peso / Clinical application of transcranial Doppler ultrasonography in premature, very-low-birth-weight neonates

OBJETIVO: Analisar o valor do diagnóstico precoce de alterações hemodinâmicas em hemorragias e eventos hipóxico-isquêmicos pela avaliação de imagens e mensuração do índice de resistência por meio da ultrassonografia craniana com Doppler em neonatos prematuros de muito baixo peso. MATERIAIS E MÉTODOS: Cinquenta neonatos prematuros de muito baixo peso foram submetidos a ultrassonografia craniana com Doppler com a técnica transfontanela anterior e transtemporal sequenciais. RESULTADOS: Foram detectadas alterações cerebrais em 32 por cento dos prematuros, sendo 22 por cento com hemorragia intracraniana, 8 por cento com leucomalácia periventricular e 2 por cento com toxoplasmose. Dentre os 34 casos (68 por cento), do total de neonatos, nos quais não foram detectadas lesões cerebrais pela ultrassonografia craniana, 18 (53 por cento) apresentaram alterações no índice de resistência. O índice de resistência variou conforme a época do exame. CONCLUSÃO: Existe correlação entre a presença de alterações na hemodinâmica cerebral e subsequente desenvolvimento de hemorragias e lesões hipóxico-isquêmicas, pela mensuração do índice de resistência. Alterações do índice de resistência, embora não preditoras de morte, estão relacionadas com a gravidade do quadro clínico em neonatos prematuros de muito baixo peso.

OBJECTIVE: The present study was aimed at analyzing the value of the early diagnosis of hemodynamic changes in hemorrhages and hypoxic-ischemic events in premature, very-low-birth-weight neonates through the evaluation of images and resistance index measurement by means of transcranial Doppler ultrasonography. MATERIALS AND METHODS: Fifty premature, very-low-birth-weight neonates were submitted to transcranial Doppler ultrasonography with sequential transfontanellar and transtemporal techniques. RESULTS: Cerebral abnormalities were detected in 32 percent of the neonates (22 percent with intracranial hemorrhage, 8 percent with periventricular leukomalacia, and 2 percent with toxoplasmosis). Among the 34 cases (68 percent) of neonates in whom no brain lesion was detected at transcranial Doppler ultrasonography, 18 (53 percent) presented changes in the resistance index. Such resistance index varied according to the time of the examination. CONCLUSION: There is a correlation between the presence of cerebral hemodynamic changes demonstrated by resistance index measurements and the subsequent development of hemorrhages and hypoxic-ischemic lesions. Although not being a death predictor, changes in the resistance index are associated with the severity of the clinical conditions in preterm, very-low-birth-weight neonates.

Neurofibromatose tipo 1: aspectos radiológicos do tórax / Type 1 neurofibromatosis: radiological findings of the chest

OBJETIVO: Identificar alterações e frequências nas radiografias simples do tórax sugestivas de neurofibromatose tipo 1 e avaliar a possibilidade de inclusão de massa no mediastino posterior como critério de diagnóstico de neurofibromatose tipo 1. MATERIAIS E MÉTODOS: Foram realizadas radiografias com técnica padrão de tórax em póstero-anterior e em perfil de 141 pacientes com neurofibromatose tipo 1, atendidos no Serviço de Radiologia do Hospital de Base e Faculdade de Medicina de São José do Rio Preto, SP. Os resultados obtidos foram avaliados por métodos não paramétricos ao nível de 0,05 de significância (p = 0,05). RESULTADOS: No presente estudo, 141 pacientes com neurofibromatose tipo 1 realizaram radiografia de tórax, sendo as alterações mais frequentes: erosão óssea das costelas (19,8 por cento), peito escavado (12,0 por cento), cifoescoliose (3,5 por cento) e massas no mediastino posterior (7,1 por cento). Esses resultados sugerem que as massas (neurofibroma e meningocele) devem ser incluídas como critério diagnóstico para neurofibromatose tipo 1, juntamente com displasia do osso esfenoide, pseudoartrose e afinamento do córtex de ossos longos, conforme definido pelo National Institutes of Health. CONCLUSÃO: A presença das massas no mediastino posterior associada às alterações ósseas características definidas pelo National Institutes of Health indicam ser um achado consistente para se considerar como critério diagnóstico da doença.

OBJECTIVE: To identify chest radiography findings suggestive of type 1 neurofibromatosis, establishing their frequency and evaluating the possibility of including the presence of posterior mediastinal masses as a criterion for the diagnosis of type 1 neurofibromatosis. MATERIALS AND METHODS: The present study included 141 patients with type 1 neurofibromatosis assisted at the Service of Radiology of Hospital de Base and Faculdade de Medicina de São José do Rio Preto, SP, Brazil, and submitted to standard chest radiography in postero-anterior and lateral views. The results were analyzed by non-parametric methods and the level of statistical significance was set at 0.05 (p = 0.05). RESULTS: The most frequent findings were the following: ribs erosion (19.8 percent), pectus excavatum (12.0 percent), kyphoscoliosis (3.5 percent) and posterior mediastinal masses (7.1 percent). Such results suggest that posterior mediastinal masses (neurofibroma and meningocele) should be included as a diagnostic criterion of type 1 neurofibromatosis, in conjunction with dysplasia of the sphenoid wing, pseudoarthrosis and thinning of long bone cortex, as defined by the National Institutes of Health. CONCLUSION: The presence of posterior mediastinal masses in association with the typical bone changes defined by the National Institutes of Health is a consistent finding to be considered as a diagnostic criterion of the disease.