Scimitar syndrome associated with aberrant right subclavian artery, diaphragmatic hernia, and urinary anomalies - case report and review of the literature

Rom J Morphol Embryol. 2018;59(2):625-630.

Abstract

Scimitar syndrome is a form of a partially or totally right pulmonary venous return to the inferior vena cava, which may associate variably right lung hypoplasia, right pulmonary artery hypoplasia, pulmonary sequestration together with the presence of aortopulmonary collaterals from the descending aorta towards the right lung. In many cases, there are also other cardiac anomalies associated. We present a unique association of a partially anomalous pulmonary venous return to the inferior vena cava with other vascular and thoracic anomalies: inferior sinus venosus and secundum atrial septal defect, retroesophageal right subclavian artery, obstructed accessory right bronchus, diaphragmatic hernia with ectopic liver, "S"-type thoracic scoliosis and malformations of the urinary tract (duplication of the right ureter and of the left basinet). The patient had a reimplantation of the "scimitar" vein to the left atrium and closure of the inferior sinus venosus and secundum atrial septal defect.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Cardiovascular Abnormalities / etiology*
  • Cardiovascular Abnormalities / pathology
  • Echocardiography / methods*
  • Female
  • Hernias, Diaphragmatic, Congenital / etiology*
  • Hernias, Diaphragmatic, Congenital / pathology
  • Humans
  • Scimitar Syndrome
  • Subclavian Artery / abnormalities*
  • Subclavian Artery / pathology
  • Urogenital Abnormalities / etiology*
  • Urogenital Abnormalities / pathology
  • Young Adult

Supplementary concepts

  • Aberrant subclavian artery
  • Genitourinary Tract Anomalies