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Clinical spectrum of infantile scimitar syndrome: A tertiary center experience


1 Department of Pediatrics, King Abdul-Aziz Medical City; King Abdullah International Medical Research Centre, Riyadh, Saudi Arabia
2 King Saud Bin Abdul-Aziz University for Health Sciences; Cardiac Center, pediatrics division, King Abdul-Aziz Medical City, Riyadh, Saudi Arabia

Correspondence Address:
Hadeel Al Rukban
Department of Pediatrics, King Abdul-Aziz Medical City, Riyadh
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.126547

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Year : 2014  |  Volume : 7  |  Issue : 1  |  Page : 29-33

 

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Aim: Infantile scimitar syndrome is a rare condition, with most of the literature reports being limited to case reports and a few case series. The aim of this study was to review patients with infantile scimitar syndrome who presented to our hospital from July 2000 to January 2011. Materials and Methods: In this retrospective study, we evaluated the medical records of patients aged 0-14 years who were symptomatic before the age of 1 year and were subsequently diagnosed with the syndrome. A total of 16 patients with the infantile form of scimitar syndrome were identified from the database. Results: The median age at presentation was 14 days, with a median age at diagnosis of 55 days. Fifty-six percent of the patients were females. Tachypnea was the major presenting symptom and 13 out of 16 patients had pulmonary hypertension. Of the 13 patients with pulmonary hypertension, 7 had systemic collaterals, which were treated by coil occlusion together with medications, and 3 had corrective surgery. The mortality rate was 3/16 (18.8%) over the 10.5 years study period. Conclusions: Infantile scimitar syndrome is a rare congenital anomaly that needs a high degree of suspicion for early referral and management. The association of the syndrome with pulmonary hypertension leads to recurrent and prolonged hospitalization.






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1 Department of Pediatrics, King Abdul-Aziz Medical City; King Abdullah International Medical Research Centre, Riyadh, Saudi Arabia
2 King Saud Bin Abdul-Aziz University for Health Sciences; Cardiac Center, pediatrics division, King Abdul-Aziz Medical City, Riyadh, Saudi Arabia

Correspondence Address:
Hadeel Al Rukban
Department of Pediatrics, King Abdul-Aziz Medical City, Riyadh
Saudi Arabia
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.126547

Rights and Permissions

Aim: Infantile scimitar syndrome is a rare condition, with most of the literature reports being limited to case reports and a few case series. The aim of this study was to review patients with infantile scimitar syndrome who presented to our hospital from July 2000 to January 2011. Materials and Methods: In this retrospective study, we evaluated the medical records of patients aged 0-14 years who were symptomatic before the age of 1 year and were subsequently diagnosed with the syndrome. A total of 16 patients with the infantile form of scimitar syndrome were identified from the database. Results: The median age at presentation was 14 days, with a median age at diagnosis of 55 days. Fifty-six percent of the patients were females. Tachypnea was the major presenting symptom and 13 out of 16 patients had pulmonary hypertension. Of the 13 patients with pulmonary hypertension, 7 had systemic collaterals, which were treated by coil occlusion together with medications, and 3 had corrective surgery. The mortality rate was 3/16 (18.8%) over the 10.5 years study period. Conclusions: Infantile scimitar syndrome is a rare congenital anomaly that needs a high degree of suspicion for early referral and management. The association of the syndrome with pulmonary hypertension leads to recurrent and prolonged hospitalization.






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