[Scimitar syndrome: a case series]

Bol Med Hosp Infant Mex. 2014 Nov-Dec;71(6):367-372. doi: 10.1016/j.bmhimx.2015.01.002. Epub 2015 May 19.
[Article in Spanish]

Abstract

Background: Scimitar syndrome is a rare and complex congenital anomaly of the lung with multiple variables and is named for its resemblance to the classical radiological crooked sword. Its defining feature is the anomalous pulmonary drainage. It is associated with various cardiothoracic malformations and a wide spectrum of clinical manifestations.

Case series: Nine patients diagnosed with scimitar syndrome found in the database of Hospital Infantil de México between 2009 and 2013 were reviewed. Demographic records, clinical status and hemodynamic parameters reported were collected.

Conclusions: This case series called attention to certain differences between our group of patients and those reported in the international literature. Patients were predominantly female and were diagnosed between 1 and 20 months of life. All were asymptomatic at the time of the study. Half of the patients had a history of respiratory disease and all patients had with pulmonary hypertension. Surgical management was required in on-third of the patient group.

Keywords: Anomalous pulmonary venous return; Anomalías del sistema respiratorio; Malformaciones vasculares; Respiratory system abnormalities; Retorno venoso pulmonar anómalo; Scimitar syndrome; Síndrome de la cimitarra; Vascular malformations.

Publication types

  • English Abstract