Situs Ambiguus, Situs Ambiguous or Heterotaxy, with Polysplenia Syndrome

Situs Ambiguus, Situs Ambiguous or Heterotaxy
(refers to the abnormal arranging of the body organs)


Heterotaxy with Polysplenia Syndrome
(The following may be assocaite w/Heterotax, Polysplenia)

Polysplenia
(2 or more spleens)
Bilateral Left-sideness
Funtional Asplenia

Cardiovascular Malformations with Polysplenia
About 40% Partial anomalous pulmonary Venous Return
About 70% Intrahepatic Interruption of the Inferior Vena Cava with connection to the azygous or hemiazygous vein and left ventricular outflow tract obstruction

Other Problems maybe Associated with Heterotaxy

Pulmonary Isomerism
Vascular Rings
Mid Line Liver
Annular Pancreas
Right side stomach
Biliary Atresia
Tracheo – Esophageal Fistula
Omphalocoele
Intestinal Malrotation with obstruction or Volvulus (Vascular Obstruction)

Other Midline Malformations Maybe Associated with Heterotaxy

Central Nervous Systems
Encephalocele
Neural Tube Defects
Porencephalic Cyst
Cerebellar Agenesis

Axial Skeletal Defects
Hemi vertebra
Sacral Agenesis

Genito – Urinary Tract Anomalies
Hypolastic Kidney
Hypospadias
Duplicated Uterus
Vaginal Atresia
Fusions of Adrenals in Midline

Bindgut Abnormalities
Anal Atresia
Stenosis

Craniofacial Anomalies
Cleft lip/palate Agnathia
Micrognathia
Choanal Atresia
Laryngeal Cleft