European Journal of Pediatrics

, Volume 167, Issue 2, pp 155–160

Scimitar syndrome: incidence, treatment, and prognosis

Authors

  • Ching-Chia Wang
    • Department of PediatricsNational Taiwan University Hospital
  • En-Ting Wu
    • Department of PediatricsNational Taiwan University Hospital
  • Shyh-Jye Chen
    • Department of RadiologyNational Taiwan University Hospital
  • Frank Lu
    • Department of PediatricsNational Taiwan University Hospital
  • Shu-Chien Huang
    • Department of TraumatologyNational Taiwan University Hospital
  • Jou-Kou Wang
    • Department of PediatricsNational Taiwan University Hospital
  • Chung-I Chang
    • Department of TraumatologyNational Taiwan University Hospital
    • Department of PediatricsNational Taiwan University Hospital
Original Paper

DOI: 10.1007/s00431-007-0441-z

Cite this article as:
Wang, C., Wu, E., Chen, S. et al. Eur J Pediatr (2008) 167: 155. doi:10.1007/s00431-007-0441-z

Abstract

This study is based on a database of 16 years; we sought to define the incidence and outcome of scimitar syndrome. Of 8,771 patients, 5 (0.057%) with scimitar syndrome were identified and constituted the study population. Follow-up ranged from 1 to 16 years (median: 10 years). Diagnosis was assured by computed tomography in four patients and by cardiac catheterization in one. Two patients presented with respiratory distress soon after birth and required early pneumonectomy in one case and coil embolization of the abnormal feeding arteries to the right lower lung followed by surgical rerouting of the abnormal pulmonary vein and repair of the atrial septal defect in the other case. The former was supported by ventilator therapy for 3 years after pneumonectomy, but was finally weaned from the ventilator. Among the other three, two had repeated pneumonia that resolved after rerouting of the abnormal right pulmonary vein and cardiac repair. The asymptomatic child did not receive any intervention. In spite of the abnormal orientation of the airways, none of the four patients with detailed computed tomography imaging showed any significant compression of the airways. All five patients were doing well as of the last follow-up. In conclusion, scimitar syndrome is a very rare disease in this Asian country and the varied symptoms, such as tachypnea and repeated infection, could be improved after interventions.

Keywords

Scimitar syndromeCongenital heart diseaseAirway anomalyPulmonary veins

Copyright information

© Springer-Verlag 2007