Polysplenia Syndrome

POLYSPLENIA SYNDROME
44 year old female with history of polysplenia syndrome,s/p ASD secundum repair, s/p left mastectomy for breast carcinoma, and with new neuroendocrine lung tumor.
Frontal X-ray shows a bilobed soft tissue abnormality above the right main stem bronchus The region of the main and left pulmonary artery is also prominent.
POLYSPLENIA SYNDROME
44 year old female with history of polysplenia syndrome,s/p ASD secundum repair, s/p left mastectomy for breast carcinoma, and with new neuroendocrine lung tumor.
CXR shows a soft tissue density above the right mainstem bronchus (dark blue arrowhead , b) a high lying RPA (light blue arrowhead b) and a prominent region of the MPA and left main pulmonary artery (light blue arrowhead left side ,b). Although the left and right main bronchi are suboptimally visualised they appear as symmetric structures (black overlay, b)
The dark blue arrowhead point to the enlarged azygos vein, and the symmetric light blue arrowheads point t the bilateral hyparterial bronchi, indicating bilateral left sided lungs.
Ashley Davidoff MD

NORMAL FRONTAL CXR and NORMAL POSITION OF THE RPA AND LPA
The normal CXR shows the superior position and relationship of the LPA to the left mainstem bronchus. The bronchus is hyparterial ie below the artery.
The RPA (light blue and right in b) runs below the right mainstem bronchus ie the bronchus is eparterial . The azygos vein lies superior to the right mainstem bronchus at the take off of the right mainstem from the trachea.
Ashley Davidoff MD
POLYSPLENIA SYNDROME
44 year old female with history of polysplenia syndrome,s/p ASD secundum repair, s/p left mastectomy for breast carcinoma, and with new neuroendocrine lung tumor.
LEFT SIDED RIGHT LUNG – SINGLE ODD FISSURE
POLYSPLENIA SYNDROME
44 year old female with history of polysplenia syndrome,s/p ASD secundum repair, s/p left mastectomy for breast carcinoma, and with new neuroendocrine lung tumor.
POLYSPLENIA SYNDROME
44 year old female with history of polysplenia syndrome,s/p ASD secundum repair, s/p left mastectomy for breast carcinoma, and with new neuroendocrine lung tumor.

AZYGOUS EXTENSION – POLYSPLENIA SYNDROME
44-year-old female with history of polysplenia syndrome, s/p ASD secundum repair, s/p left mastectomy for breast carcinoma, and with new neuroendocrine lung tumor.
Axial images through the heart and great vessels, show an enlarged azygous vein (dark blue arrowheads a,b) a normal appearing aorta (red arrowheads, a,b) and the MPA (light blue arrowhead a), and RVOT (light blue arrowhead,)
Ashley Davidoff MD
POLYSPLENIA SYNDROME
44 year old female with history of polysplenia syndrome,s/p ASD secundum repair, s/p left mastectomy for breast carcinoma, and with new neuroendocrine lung tumor.
POLYSPLENIA SYNDROME
44 year old female with history of polysplenia syndrome,s/p ASD secundum repair, s/p left mastectomy for breast carcinoma, and with new neuroendocrine lung tumor.
Fig. 6C —Images from ECG-gated CT scan of 20-year-old man with dextrocardia and polysplenia syndrome. Reformatted coronal image shows bilateral hyparterial bronchi. Pulmonary arteries (P) are enlarged from chronic pulmonary artery hypertension secondary to long-standing intracardiac left-to-right shunt. A = aorta, arrow = arch of hemiazygos vein connecting to left-sided superior vena cava (not shown).
Read More: https://www.ajronline.org/doi/10.2214/AJR.06.1179
Fig. 6A —Images from ECG-gated CT scan of 20-year-old man with dextrocardia and polysplenia syndrome. Axial image through upper abdomen shows multiple splenules in right upper quadrant posterior to right-sided stomach (St). Liver is predominantly left-sided. Vessel (arrow) adjacent to descending thoracic aorta is enlarged hemiazygos vein from hemiazygos continuation of inferior vena cava. Note absence of intrahepatic portion of inferior vena cava.
Read More: https://www.ajronline.org/doi/10.2214/AJR.06.1179
Fig. 6B —Images from ECG-gated CT scan of 20-year-old man with dextrocardia and polysplenia syndrome. Axial image through cardiac chambers shows complete endocardial cushion defect comprised of absence of atrial septum (common atrium) and upper portion of ventricular septum. Arrows indicate leaflets of common atrioventricular valve.
Read More: https://www.ajronline.org/doi/10.2214/AJR.06.1179