Hadeel Al Rukban1, Mohammed Al Ghaihab1, Omar Tamimi2, Suhail Al-Saleh1
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
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.
Hadeel Al Rukban
Department of Pediatrics, King Abdul-Aziz Medical City, Riyadh
Source of Support: None, Conflict of Interest: None
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