Scimitar Syndrome and Associated Pulmonary Sequestration

Report of a Successfully Corrected Case
  • E. Horcher
  • F. Helmer
Part of the Progress in Pediatric Surgery book series (PEDIATRIC, volume 21)


The scimitar syndrome is characterized by anomalous venous drainage from the right lung to the vena cava inferior. The appearance of this vein on chest X-ray is suggestive of a Turkish sword and is called the “scimitar” sign by Neill et al. (1960). The condition may be associated with anomalies of the right lung (absence of lung fissures, mirror image of the left lung, absence of lobe bronchi), anomalies of the pulmonary artery, and congenital heart defects (Mathey et al. 1968; Mende et al. 1973). The symptoms are similar to those of an atrial septal defect due to a left-to-right shunt, such as repeated respiratory infections. We present an uncommon case of scimitar syndrome combined with pulmonary sequestration; few cases of this anomaly have been reported in the literature (Alivizatos et al. 1985; Ohsaki 1983).


Pulmonary Vein Left Atrium Atrial Septal Defect Lower Lobe Pulmonary Sequestration 
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  1. Alivizatos P, Cheatle T, de Leval M, Stark J (1985) Pulmonary sequestration complicated by anomalies of pulmonary venous return. J Pediatr Surg 20: 76–79Google Scholar
  2. Hallman GL, Cooley DA (1975) Surgical treatment of congenital heart disease, 2nd edn. Lea and Febiger, Philadelphia, pp 194–196Google Scholar
  3. Lichea C. Ursinus W (1983) Die operative Behandlung des Scimitarsyndroms. Zentralbl Chir 108: 159–166Google Scholar
  4. Mathey J, Galey JJ, Logeais Y, et al (1968) Anomalous pulmonary venous return into inferior vena cava and associated bronchovascular anomalies (the scimitar syndrome). Thorax 23: 398–407PubMedCrossRefGoogle Scholar
  5. Mende S, Kallfelz HC, Kreutzberg B (1973) Scimitar-Syndrom. Pathologie, Klinik und Therapie. Klin Padiatr 185: 421–436Google Scholar
  6. Neill CA, Ferenca C, Sabiston DC, Shelton J (1960) The familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage: Scimitar syndrome. John Hopkins Med J 107: 1–20Google Scholar
  7. Ohsaki Y (1983) Case of scimitar syndrome with pulmonary sequestration. Nippon Kyobu Shik-kan Gakkai Zasshi 21: 904–909Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1987

Authors and Affiliations

  • E. Horcher
    • 1
  • F. Helmer
    • 1
  1. 1.Department of Pediatric SurgerySecond Surgical University ClinicViennaAustria

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