Clinical spectrum of infantile scimitar syndrome: A tertiary center experience

Abstract

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.

Scimitar syndrome and anesthetic implications

“…The largest series of cases reported to date describe 44 cases in adult patients 3 and 16 in children 4 . The prevalence in the general population is low, which reflects the perioperative challenge associated with these patients and the importance of understanding the anesthetic implications due to its high perioperative mortality rate 1,2 .…”

“…This syndrome represents 4% of anomalous pulmonary venous returns. It is associated with alterations in pulmonary arterial supply, right pulmonary hypoplasia, and hypoplasia of the right bronchus and right pulmonary artery 4,5,7 . The most frequent heart alterations are the dextroposition of the heart with displacement of the mediastinum, absence of the pericardium and other malformations, such as atrial septal defect (60-70%) or ventricular septal defect, coarctation of the aorta, and tetralogy of Fallot in 25% of cases 3,6,7 .…”

Surgical management of a scimitar syndrome with severe pulmonary hypertension in 2-year-old child: Case report and review of literature

“…The discovery of the disease occurs at age of 55 days [3] with prominent respiratory symptoms (tachypnea, cyanosis, permanent pulmonary infection...) [2].…”

Scimitar Vein with Anomalous Pulmonary Venous Return in an Adult

“…Pulmonary hypertension with an infantile presentation is associated with a poor outcome. Other associated cardiovascular abnormalities are uniformly found in this form …”

“…Other associated cardiovascular abnormalities are uniformly found in this form. 9 The adult form is often asymptomatic, with diagnosis after the first year of age. Most lead a normal life without surgical correction.…”