Ultrasound Board Review on Asplenia скачать в хорошем качестве

Ultrasound Board Review on Asplenia

Asplenia syndrome, also known as right isomerism or Ivemark syndrome, is a type of heterotax, is a syndrome of right isomerism or attempted bilateral right sidedness. It includes congenital asplenia in association with complex congenital cyanotic heart disease and situs anomalies of the other thoracoabdominal organs. Herein we report a case of asplenia syndrome so as to highlight and refocus attention on the possibility of this rare syndrome that must be considered in a neonate presenting with congenital cyanotic heart disease. General Main characteristic radiographic features include: absence of the spleen bilateral epartial bronchi bilateral trilobed lungs bilateral right atria transverse liver Associations severe/complex congenital heart disease (50%), especially cyanotic congenital cardiac anomalies total anomalous pulmonary venous return (almost 100%) endocardial cushion defect (85%) transposition of the great arteries (72%) single ventricle (51%) double outlet right ventricle (DORV) gastrointestinal gallbladder agenesis intestinal malrotation (up to 100% in small series) 3 microgastria imperforate anus genitourinary horseshoe kidney fused / horseshoe adrenal gland or absent left adrenal gland bicornuate uterus bilobed urinary bladder vascular duplication of the superior vena cava absent coronary sinus juxtaposition of the IVC in front (usually) of the abdominal aorta (piggyback configuration)