The patient was a 6-year-old boy known to suffer growth retardation and severe cyanosis. The results of cardiac ultrasound suggested that the boy suffers from complex congenital heart disease.
There are no obvious abnormalities in the routine laboratory tests. Chest X-ray indicated the patient had heart hypertrophy and showed a right-sided stomach with laevocardia. Echocardiography suggested the presence of a complex congenital heart disease.
In order to understand this specific case, Computed Tomography Angiography (CTA) has been heavily relied upon because it allows for 3D reconstruction and optimal visualization of those features. CTA was performed on the first generation of dual source CT (Somatom Definition, Siemens Medical Systems, Forchheim, Germany) to further assess the presence of other abnormalities. The result showed that the stomach bubble and pancreas was located in the right abdomen, the spleen was absent. In addention, aortic arch was located on the right side, the thoracic aorta originated from the right ventricle, and the main pulmonary artery was originated from the left ventricle. 3D volume rendering images showed that the bilateral four pulmonary veins flowed into the right atrium,the right superior vena cava flowed into the right atrium and the left superior vena cava flowed into the left atrium (Fig. 1a). The right liver had three abnormal hepatic veins converged into a vascular trunk flowed into the right atrium (Fig. 2a, 2b), and the inferior vena cava flowed into the left atrium (Fig. 1b). CT images showed that the heart contained septal defects (Fig. 3a,3b) with right ventricular hyperplasia. According to CT imaging, in addition to congenital spleen hypoplasia or thoracic and abdominal organs of the abnormal structure and location, the diagnosis was that the great vessel was completely transposed, with complete atrioventricular common channel and multiple venous ectopic drainage.
In order to prevent heart failure, the patient underwent cardiac surgery. The surgery confirmed that the patient had no spleen, and abdominal organs of the anti-place. Furthermore, there is a complete transposition of large vessels,complete atrioventricular common channel and complete pulmonary vein ectopic drainage.
The cause of spleneless syndrome is unknown. Most of teratogenic factors may occur in embryos 4 to 5 weeks, resulting in embryonic development pause or abnormalities and the formation of a variety of deformities, mainly to the deformities of cardiovascular organs. The development of the spleen,lung,pulmonary vein, stomach and duodenum is in the twenty-fourth to 27 weeks of the embryo,once the development of dysplasia or abnormal development will lead to multiple organ congenital malformations.Because of the presence of multiple organ malformations, the patient's mortality is extremely high [7].
Another characteristic disorder of splenic syndrome is the development of bilateral organ symmetry, which may due to abnormal embryonic development. The spleen is developed by the left gastric dorsal mesentery, which can also affect the development of the stomach and omentum, leading to intra-abdominal organ displacement or deformity [8].