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Clinical spectrum of infantile scimitar syndrome: A tertiary center experience
Hadeel Al Rukban1, Mohammed Al Ghaihab1, Omar Tamimi2
1Department of Pediatrics, King Abdul-Aziz Medical City, Riyadh, Saudi Arabia ; King Abdullah International Medical Research Centre, Riyadh, Saudi Arabia.
Insights
Infantile scimitar syndrome, a rare congenital anomaly, often presents with tachypnea and pulmonary hypertension. Early diagnosis and management are crucial due to a significant mortality rate and risk of prolonged hospitalization.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Pulmonary Hypertension
Background:
- Infantile scimitar syndrome is a rare congenital anomaly.
- Existing literature primarily consists of case reports and small case series.
- Early identification and management are critical for affected infants.
Purpose of the Study:
- To review clinical characteristics and outcomes of patients with infantile scimitar syndrome.
- To analyze presentation, diagnosis, and treatment modalities in a cohort of infants.
- To assess the mortality rate associated with this rare condition.
Main Methods:
- Retrospective review of medical records for patients aged 0-14 years.
- Inclusion criteria: symptomatic before 1 year of age and diagnosed with scimitar syndrome.
- Identified 16 patients diagnosed with infantile scimitar syndrome between July 2000 and January 2011.
Main Results:
- Median age at presentation was 14 days; median age at diagnosis was 55 days.
- Tachypnea was the most common presenting symptom; 13 out of 16 patients had pulmonary hypertension.
- Mortality rate was 18.8% (3/16) over the study period; 7 patients with pulmonary hypertension had systemic collaterals treated, and 3 underwent corrective surgery.
Conclusions:
- Infantile scimitar syndrome requires a high index of suspicion for timely diagnosis and intervention.
- Pulmonary hypertension is a significant comorbidity, contributing to recurrent hospitalizations.
- Early referral and comprehensive management are essential for improving outcomes in infants with this condition.
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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