L. Sá, T. Pereira, Ana B. Ferreira, A. Reis, A. Madureira, Margarida Azevedo
{"title":"Scimitar syndrome – a case report","authors":"L. Sá, T. Pereira, Ana B. Ferreira, A. Reis, A. Madureira, Margarida Azevedo","doi":"10.1515/crpm-2020-0088","DOIUrl":null,"url":null,"abstract":"Abstract Objectives The aim of this article is to describe a clinical case of Scimitar syndrome, an unusual cause of respiratory distress in the neonatal period. This syndrome is a rare form of partial or total anomalous right lung venous drainage to the inferior caval vein, usually associated with right pulmonary hypoplasia. Case presentation The authors report a case of a 36-week-old newborn admitted to the neonatal intensive care unit with worsening respiratory distress. Chest radiography revealed a characteristic right pulmonary hypoplasia and a tubular structure paralleling the right heart border in the shape of a Turkish sword (“scimitar”). Chest computed tomography angiography confirmed the diagnosis. Conclusions Age of presentation and the severe malformations found, contributed to the patient’s poor outcome in this case.","PeriodicalId":9617,"journal":{"name":"Case Reports in Perinatal Medicine","volume":"673 1","pages":""},"PeriodicalIF":0.1000,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Case Reports in Perinatal Medicine","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1515/crpm-2020-0088","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"OBSTETRICS & GYNECOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Abstract Objectives The aim of this article is to describe a clinical case of Scimitar syndrome, an unusual cause of respiratory distress in the neonatal period. This syndrome is a rare form of partial or total anomalous right lung venous drainage to the inferior caval vein, usually associated with right pulmonary hypoplasia. Case presentation The authors report a case of a 36-week-old newborn admitted to the neonatal intensive care unit with worsening respiratory distress. Chest radiography revealed a characteristic right pulmonary hypoplasia and a tubular structure paralleling the right heart border in the shape of a Turkish sword (“scimitar”). Chest computed tomography angiography confirmed the diagnosis. Conclusions Age of presentation and the severe malformations found, contributed to the patient’s poor outcome in this case.
期刊介绍:
Case Reports in Perinatal Medicine is a double-blind peer-reviewed journal. The objective of the new journal is very similar to that of JPM. In addition to evidence-based studies, practitioners in clinical practice esteem especially exemplary reports of cases that reveal specific manifestations of diseases, its progress or its treatment. We consider case reports and series to be brief reports describing an isolated clinical case or a small number of cases. They may describe new or uncommon diagnoses, unusual outcomes or prognosis, new or infrequently used therapies and side effects of therapy not usually discovered in clinical trials. They represent the basic concept of experiences for studies on representative groups for further evidence-based research. The potential roles of case reports and case series are: Recognition and description of new diseases Detection of drug side effects (adverse or beneficial) Study of mechanisms of disease Medical education and audit Recognition of rare manifestations of disease.