Asplenia is combined with bilateral right-sided
organs. There are two right lungs, two right lobes of the liver, two right
atria, and bilateral superior vena cava. Various abnormal localizations of
single organs are observed. This syndrome cannot be qualified as situs
inversus. Clinical manifestations include sensibility to infection with
encapsulated germs and presence of Heinz and Howell-Jolly bodies in the
blood because of asplenia. Cardiac malformations (single ventricle,
transposition of great vessels, truncus arteriosus, atrioventricular defect)
are frequent with their own signs; arrhythmia as a result of the presence of
two sinoatrial nodes is specific. Neurologic signs could be observed in
relation with anophthalmia, holoprosencephaly, hydrocephalus, or
meningocele. Hepatogastrointestinal structures can be abnormal and
complicate evolution of the syndrome: biliary atresia or stenosis, hiatus
hernia, megaesophagus or brachyesophagus, volvulus as a result of
malrotation of the gut, and malformation of the pancreas. Up to 70% of
patients die within the first year of life.