Document Detail


Scimitar syndrome: incidence, treatment, and prognosis.
MedLine Citation:
PMID:  17345096     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Ching-Chia Wang; En-Ting Wu; Shyh-Jye Chen; Frank Lu; Shu-Chien Huang; Jou-Kou Wang; Chung-I Chang; Mei-Hwan Wu
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2007-03-08
Journal Detail:
Title:  European journal of pediatrics     Volume:  167     ISSN:  1432-1076     ISO Abbreviation:  Eur. J. Pediatr.     Publication Date:  2008 Feb 
Date Detail:
Created Date:  2007-12-24     Completed Date:  2008-05-22     Revised Date:  2008-06-05    
Medline Journal Info:
Nlm Unique ID:  7603873     Medline TA:  Eur J Pediatr     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  155-60     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, National Taiwan University Hospital, 7 Chung-Shan South Road, Taipei, 100, Taiwan.
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MeSH Terms
Descriptor/Qualifier:
Child
Child, Preschool
Embolization, Therapeutic
Female
Follow-Up Studies
Humans
Imaging, Three-Dimensional
Incidence
Infant
Male
Prognosis
Radiography, Thoracic
Scimitar Syndrome* / epidemiology,  radiography,  therapy
Taiwan / epidemiology
Tomography, X-Ray Computed

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