Multimedia
 | Media file 1: Polysplenia. Ultrasonogram of the left upper quadrant reveals multiple, widely spaced splenules above the left kidney (same patient in Images 1 and 3 in Multimedia). Reproduced with permission. |
 | Media file 2: Polysplenia. Ultrasonogram of the left upper quadrant reveals few, closely spaced splenules above the left kidney. The adrenal gland lies medial and immediately adjacent to the splenules. Reproduced with permission. |
 | Media file 3: T1-weighted magnetic resonance image obtained in a patient with polysplenia (same patient in Images 1 and 3 in Multimedia). Multiple widely spaced splenules are obvious along the greater curvature of the left-sided stomach. Reproduced with permission from Hernanz-Schulman M et al. AJR Am J Roentgenol. 1990;154(4):797-802. |
 | Media file 4: Computed tomography scan obtained in the excretory phase during the intravenous administration of contrast material in a patient with polysplenia. This image demonstrates multiple splenules in the right upper quadrant, along the greater curvature of the right-sided stomach. Note that the liver extends over the splenic mass. |
 | Media file 5: Abnormalities of rotation. Upper gastrointestinal image demonstrates inverted nonrotation and a right-sided stomach in a patient with polysplenia (same patient in Images 5-6 in Multimedia). |
 | Media file 6: Abnormalities of rotation. Image obtained during a barium enema examination in a patient with polysplenia (same patient in Images 5-6 in Multimedia). This radiograph demonstrates inverted nonrotation. |
 | Media file 7: Interruption of the inferior vena cava. Transverse ultrasonogram of the abdomen in a patient with polysplenia (same patient in Images 7-8 in Multimedia). This image demonstrates a large retrocrural vessel, which is similar in size and adjacent to the right side of the aorta, a finding that indicates azygous continuation and caval interruption. |
 | Media file 8: Interruption of the inferior vena cava. Axial T1-weighted magnetic resonance image of the abdomen (same patient in Images 7-8 in Multimedia). This image demonstrates a large retrocrural vessel, which is similar in size and adjacent to the right side of the aorta, a finding that indicates azygous continuation and caval interruption. |
 | Media file 9: Interruption of the inferior vena cava. Coronal T1-weighted magnetic resonance image of the abdomen in a patient with polysplenia. This image demonstrates the enlarged azygous vein, which is parallel to the aorta and to its right. A partial volume-averaging artifact is present in the azygous vein and arch. |
 | Media file 10: Intact inferior vena cava. Transverse ultrasonogram of the abdomen in patient with polysplenia (same patient in Images 10-11 in Multimedia). This image demonstrates an antecrural vessel, a finding that indicates an intact inferior vena cava, which is in the process of crossing over anterior to the aorta to enter the atrium. |
 | Media file 11: Intact inferior cava. Sagittal ultrasonogram of the abdomen in a patient with polysplenia (same patient in Images 10-11 in Multimedia). This image demonstrates an antecrural vessel, a finding that indicates an intact inferior cava, which is in the process of crossing over anterior to the aorta to enter the atrium. Compare this ultrasonogram with the transverse ultrasonogram in Image 10. |
 | Media file 12: Intact inferior cava. Sagittal ultrasonogram of the abdomen in a patient with polysplenia. This image demonstrates an intact intrahepatic cava with a normal appearance. Because the abdominal caval laterality was congruent with that of the atria in this patient, crossing over did not occur. Reproduced with permission. |
 | Media file 13: Intact inferior vena cava. Transverse ultrasonogram in a patient with asplenia. This image demonstrates the aorta and vena cava on either side of the midline, an appearance that simulates solitus anatomy. Reproduced with permission. |
 | Media file 14: Total anomalous pulmonary venous connection (TAPVC). Sagittal abdominal ultrasonogram in a patient with asplenia and TAPVC below the diaphragm (same patient in Images 14-16 and 22-23 in Multimedia). This image shows that the anomalous vessel bypasses the portal vein and connects to a hepatic vein. Note the abrupt caliber change in the draining pulmonary vein in this patient with severe pulmonary venous outflow obstruction. Reproduced with permission. |
 | Media file 15: Total anomalous pulmonary venous connection (TAPVC). Axial T1-weighted magnetic resonance image in a patient with asplenia and TAPVC below the diaphragm (same patient in Images 14-16 and 22-23 in Multimedia). This image shows the anomalous vessel anterior to the gastroesophageal junction (high-intensity focus). The hepatic draining vein is the smaller-caliber vessel immediately anterior to the anomalous vessel. The caliber difference indicates the presence of an obstruction. The intrahepatic vena cava is seen to the right, and the aorta is anterior to the spine to the left of the midline. |
 | Media file 16: Total anomalous pulmonary venous connection (TAPVC). Coronal T1-weighted magnetic resonance image obtained in a patient with asplenia and TAPVC below the diaphragm (same patient in Images 14-15, 22-23 in Multimedia). The anomalous vessel receives the pulmonary veins in the chest and courses below the diaphragm. Reproduced with permission. |
 | Media file 17: Bilateral superior cavae. Coronal T1-weighted magnetic resonance image in a patient with polysplenia. This image demonstrates bilateral superior cavae, a large atrial septal defect, and a single-ventricle anatomy. Portions of a transverse aortic arch and pulmonary outflow tract are also visible. |
 | Media file 18: Preduodenal portal vein. Transverse ultrasonogram obtained just caudal to the pancreas in an infant with polysplenia (same patient in Images 18-20 in Multimedia). This image demonstrates the superior mesenteric vein just ventral to and to the left of the superior mesenteric artery (denoted by the characteristic hyperechoic halo) (see Image 19 in Multimedia). The relationship of the artery and vein is abnormal; the vein is to the left and anterior to the artery, rather than to the right and in the same plane as the artery in the normal relationship. Reproduced with permission. |
 | Media file 19: Preduodenal portal vein. Transverse ultrasonogram obtained at the level of the pancreas in an infant with polysplenia (same patient in Images 18-20 in Multimedia). This image demonstrates that the superior mesenteric vein lies ventral to the pancreas and is separated from the superior mesenteric artery (denoted by the characteristic hyperechoic halo) by the pancreas. Reproduced with permission. |
 | Media file 20: Preduodenal portal vein. Sagittal ultrasonogram obtained in an infant with polysplenia (same patient in Images 18-20 in Multimedia). This image demonstrates the superior mesenteric vein, which courses ventral to the pancreas, in a staircase configuration. Reproduced with permission. |
 | Media file 21: Preduodenal portal vein. Sagittal ultrasonogram in an infant with asplenia. This image demonstrates the characteristic staircase configuration of the ventral course of the superior mesenteric–portal junction anterior to the pancreas and duodenum. The hyperechoic triangular structure indicates gas within the duodenal cap. |
 | Media file 22: Conotruncal anomalies and total anomalous pulmonary venous connection (TAPVC). Axial T1-weighted magnetic resonance image in a patient with asplenia (same patient in Images 14-16 and 22-23 in Multimedia). This image demonstrates truncus arteriosus type II, with pulmonary arteries arising from the arterial trunk. A single right-sided superior vena cava is noted to the right of the truncus (see also Image 23 in Multimedia). |
 | Media file 23: Conotruncal anomalies and total anomalous pulmonary venous connection (TAPVC). Axial T1-weighted magnetic resonance image in a patient with asplenia (same patient in Images 14-16 and 22-23 in Multimedia). This image demonstrates the common pulmonary vein is dorsal to the ambiguus atrium and receives pulmonary venous return. The signal intensity of the lung fields is relatively high; this finding indicates increased water content in the edematous lung, which is due to obstruction of pulmonary venous return. Note the single-atrium, single-ventricle anatomy. The descending aorta lies to the left of the spine. The cardiac apex is to the right. |
 | Media file 24: Chest and abdominal radiograph in a neonate with asplenia. This image demonstrates a midline liver, mesocardia, and a right-sided stomach. Severe pulmonary edema is present, with a normal heart size; this finding is strongly suggestive of total anomalous pulmonary venous connection (TAPVC) with obstruction, which most likely drains subdiaphragmatically. Note the extension of the liver into both upper quadrants. |
 | Media file 25: Chest radiograph in a female adolescent with polysplenia. This image demonstrates characteristic findings such as an enlarged, left-sided azygous arch (black arrows) that indicates inverted caval interruption anatomy. Bilateral hyparterial bronchi also strongly suggest heterotaxy with a left-sided tendency. The aortic arch (A) is on the right, and the cardiac apex and stomach are on the left. The white arrowheads point to symmetrical epibronchial pulmonary arteries. Reproduced with permission. |
 | Media file 26: Radiograph in an infant with polysplenia. This image seems to demonstrate solitus anatomy. The cardiac apex and stomach lie on the left side. A large thymic shadow overlies and obscures the hilar anatomy. A surgical clip is noted on the left. Reproduced with permission. |
 | Media file 27: Axial T1-weighted magnetic resonance image in a patient with asplenia (same patient in Images 27-29 in Multimedia). This image demonstrates hepatic extension over the greater curvature of the stomach. Mesogastria and microgastria are also present. Reproduced with permission. |
 | Media file 28: Left coronal ultrasonogram obtained along the greater curvature of the stomach in a patient with asplenia (same patient in Images 27-29 in Multimedia). Although identification of a solid viscus in the expected location of the spleen may be misleading, note the absence of a splenic hilum and the characteristic portal echoes that parallel the long axis of the organ. These findings reveal that the solid viscus represents extension of the liver rather than splenic tissue. Reproduced with permission. |
 | Media file 29: Anterior scintigraphic examination obtained in a patient with asplenia (same patient in Images 27-29 in Multimedia). Hepatic extension into the left upper quadrant may simulate nonexistent splenic tissue. Reproduced with permission. |
 | Media file 30: Total anomalous pulmonary venous connection (TAPVC). Radiograph from a newborn following insertion of an umbilical catheter. This image shows dextrocardia, a midline liver, an abnormal course of the umbilical catheters, and a venous line that courses to the left of the arterial line. Findings of heterotaxy or situs ambiguus are present. This radiograph also reveals severe pulmonary edema. In this setting, the findings strongly suggest the presence of TAPVC with obstruction to the venous outflow. |
 | Media file 31: Polysplenia. Upper gastrointestinal (UGI) radiograph in a patient who presented with vomiting. Although many patients with heterotaxy present with severe congenital cardiac malformations, (eg, truncus arteriosus, anomalous pulmonary venous connections, atrioventricular septal defects), not all do. Some patients have milder forms of congenital heart disease, such as ventricular septal defect (VSD), or no cardiac disease. Patients with these conditions may have other anomalies, such as vomiting due to malrotation, duodenal stenosis, or jaundice due to biliary atresia. UGI studies in the patient who presented with vomiting revealed a right-sided stomach, malrotation, and a duodenal diaphragm. Findings from further evaluation were diagnostic of polysplenia. Rarely, asymptomatic manifestations, such as interruption of the inferior vena cava and polysplenia, may be discovered during medical imaging performed for unrelated complaints. |
 | Media file 32: Sagittal ultrasonogram in a patient with asplenia and total anomalous pulmonary venous connections (TAPVCs) below the diaphragm. This image demonstrates an anomalous vessel that enters the abdomen and courses into the liver. Although pulmonary angiograms could delineate the anomalous pulmonary venous connection, the anomalous vessel can be easily identified with less-invasive imaging. Ultrasonography, magnetic resonance imaging, or computed tomography scanning can demonstrate the anomalous vessel as it enters the abdomen anterior to the esophagus. |
More on Asplenia/Polysplenia |
| Overview: Asplenia/Polysplenia |
| Imaging: Asplenia/Polysplenia |
 Multimedia: Asplenia/Polysplenia |
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Further Reading
Related eMedicine topics
Heterotaxy, Asplenia
Heterotaxy, Polysplenia
Situs Inversus
Intestinal Malrotation
Biliary Atresia
Keywords
asplenia, polysplenia, heterotaxy, heterotaxia, laterality, isomerism, right-sided isomerism, left-sided isomerism, right isomerism, left isomerism, Ivemark syndrome, situs ambiguus, situs ambiguous, partial situs inversus, polyasplenia, polyasplenia syndrome, situs solitus, visceral symmetry, visceral asymmetry, situs inversus, laterality, situs inversus totalis, Kartagener syndrome, total anomalous pulmonary venous connection, TAPVC, viscerovascular anomalies, Xq26, 18p, interrupted inferior vena cava
