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      Síndrome da cimitarra: relato de caso com falsos diagnósticos e conduta adequada Translated title: Scimitar syndrome: case report with false diagnoses and adequate procedure

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          Abstract

          A síndrome da cimitarra, embora rara, é importante de ser conhecida por pediatras e pneumologistas, pois a imagem radiológica, apesar de sugerir o diagnóstico, pode ser mal-interpretada, levando a falsos diagnósticos. A partir da suspeita diagnóstica, a investigação com ecocardiograma e angiografia é fundamental, mesmo no paciente assintomático, pois pode-se evidenciar hipertensão pulmonar importante, de tal forma que o tratamento cirúrgico é indicado para evitar complicações futuras irreversíveis. Criança, apesar das dificuldades diagnósticas iniciais, foi operada precocemente, havendo excelente evolução durante o seguimento ambulatorial.

          Translated abstract

          Scimitar syndrome is rare, but knowledge about it is important to pediatricians and pneumologists. X-ray can suggest diagnosis, but the interpretation can be wrong. If a diagnosis is made, further anomalies must be investigated with echocardiography and angiography, even if the patient is asymptomatic. This child showed evidence of pulmonary hypertension and required surgery. Postoperative evolution was excellent.

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          Most cited references19

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          The "adult" form of the scimitar syndrome.

          One hundred twenty-two cases of the adult form of the scimitar syndrome were collected from different cardiologic centers. The clinical, radiographic and hemodynamic findings are described. The scimitar syndrome is defined as an anomalous right pulmonary venous drainage, partial or complete, to the inferior vena cava. Additional characteristics of this syndrome such as hypoplasia and abnormalities of the vascular supply to the right lung, dextrocardia and abnormalities of the bronchial segmentation are common; bronchiectases are rare. The left to right shunt was less than 50% in 100 of the 122 patients. The pulmonary arterial pressures were normal in 94 patients and slightly elevated in 28. A follow-up study of these patients showed that, without surgical correction, they lead a normal life. An awareness of this syndrome may avoid unnecessary invasive diagnostic procedures and surgical treatment for most patients.
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            Pulmonary sequestration and related congenital bronchopulmonary-vascular malformations: nomenclature and classification based on anatomical and embryological considerations.

            The pulmonary sequestration spectrum and related congenital lung anomalies present an extremely complex and varied group of bronchopulmonary-vascular malformations. Previous attempts at nomenclature and classification have proved inadequate. In this article we present a classification of the newly named pulmonary malinosculation spectrum, which includes all congenital lung anomalies where there is abnormal connection (that is, malinosculation) of one or more of the four major components of lung tissue--namely, tracheobronchial airway, lung parenchyma, arterial supply, and venous drainage, which in various combinations make up these lesions. We feel that this simple descriptive approach will improve our understanding and management of these complicated lesions and this is supported by the clinical experience we report in the next article.
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              Scimitar syndrome: twenty years' experience and results of repair.

              Thirty-two patients with scimitar syndrome were seen in the period between 1975 and 1995. There were 11 male and 21 female patients. Median age at diagnosis was 7 months (mean 7.7 years, range 1 day to 70 years). Patients in whom the diagnosis was made during the first year of life (infantile group, n = 19) had more severe symptoms and had a higher incidence of heart failure (11/19 vs 0/13) and of pulmonary hypertension (11/19 vs 1/13) than did the patients in whom the diagnosis was made after age 1 year (adult group, n = 13). In 17 patients the anomalous pulmonary venous drainage was repaired by baffling the vein to the left atrium. The median age at this operation was 5.8 years (mean 14.8 years, range 6 months to 70 years). No deaths occurred in this surgical group during a mean follow-up period of 8.9 years (range 1.6 to 17 years). Eight patients (47%), however, had evidence of pulmonary venous stenosis after repair, and two required reoperation for pulmonary venous obstruction. All six children in the infantile group had postoperative pulmonary venous stenosis, compared with two of 11 older patients. Postoperative quantitative pulmonary perfusion scans performed in 15 patients demonstrated reduced flow to the right lung (24%, range 0% to 59%). We conclude that age at detection of scimitar syndrome is important in predicting outcome. Surgical repair seldom results in normal blood flow to the right lung but abolishes left-to-right shunt. Postoperative pulmonary venous obstruction is prevalent, especially in the infants.
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                Author and article information

                Journal
                jpneu
                Jornal de Pneumologia
                J. Pneumologia
                Sociedade Brasileira de Pneumologia e Tisiologia (São Paulo, SP, Brazil )
                0102-3586
                1678-4642
                December 2000
                : 26
                : 6
                : 337-340
                Affiliations
                [01] orgnameUnesp orgdiv1Faculdade de Medicina de Botucatu orgdiv2Departamento de Pediatria
                [02] orgnameUnesp orgdiv1Faculdade de Medicina de Botucatu orgdiv2Departamento de Cirurgia Geral
                Article
                S0102-35862000000600010 S0102-3586(00)02600610
                bc63879b-42ca-4584-bb54-3712c0ec66ee

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 28 March 2000
                : 03 August 2000
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 19, Pages: 4
                Product

                SciELO Brazil

                Categories
                Relatos de Caso

                Pulmonary disease (specialty),Scimitar syndrome,Surgery,Diagnosis,Síndrome da cimitarra,Cirurgia,Doença pulmonar (especialidade),Diagnóstico

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