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Scimitar syndrome of atypical, rare drainage of venous vessel to the superior vena cava. A case report

Scimitar syndrome is a rare and complex congenital anomaly characterized by partial or complete anomalous pulmonary venous return from the right or left lung into the inferior vena cava, through drainage into the hepatic vein, right atrium or left atrium. The syndrome is commonly associated with hyp...

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Detalles Bibliográficos
Autores principales: Sybilski, Adam J., Michalczuk, Małgorzata, Chudoba, Anna, Tolak-Omernik, Katarzyna, Bulski, Tomasz, Walecki, Jerzy
Formato: Online Artículo Texto
Lenguaje:English
Publicado: International Scientific Literature, Inc. 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3908509/
https://www.ncbi.nlm.nih.gov/pubmed/24505225
http://dx.doi.org/10.12659/PJR.889913
Descripción
Sumario:Scimitar syndrome is a rare and complex congenital anomaly characterized by partial or complete anomalous pulmonary venous return from the right or left lung into the inferior vena cava, through drainage into the hepatic vein, right atrium or left atrium. The syndrome is commonly associated with hypoplasia of the right lung and right pulmonary artery. We present an 11-year-old female with atypical and rare type of scimitar syndrome. The girl has had cough for 2 months before admission, without fever or abnormalities on medical examination. X-ray films revealed inflammatory and atelectatic changes with mediastinal shift to the right. CT and CT angiography – hypoplasia of the right lung with no visible interlobar fissures. No areas of consolidation in the pulmonary parenchyma. Mediastinum shifted to the right. Single wide venous vessels draining the upper part of the right lung entering the superior vena cava. In our patient, clinical symptoms are mild, but a thorough physical examination could have helped diagnose the syndrome earlier.