Congenital portosystemic shunts: imaging findings and clinical presentations in 11 patients.
OBJECTIVE
To evaluate the clinical anatomy and presentations of congenital portosystemic shunts, and determine features that promote recognition on imaging.


METHODS
Institutional review board approval was obtained for this HIPAA-compliant study. The requirement for written informed consent was waived. Radiology reports were retrospectively reviewed from non-cirrhotic patients who underwent imaging studies from January 1999 through February 2009. Clinical sources reviewed included electronic medical records, archived images and histopathological material.


RESULTS
Eleven patients with congenital portosystemic shunts were identified (six male and five female; age range 20 days to 84 years). Seven patients had extrahepatic and four patients had intrahepatic shunts. All 11 patients had absent or hypoplastic intrahepatic portal veins, a feature detected by CT and MRI, but not by US. Seven patients presented with shunt complications and four with presentations unrelated to shunt pathophysiology. Three adult patients had four splenic artery aneurysms. Prospective radiological evaluation of five adult patients with cross-sectional imaging had failed prospectively to recognize the presence of congenital portosystemic shunts on one or more imaging examinations.


CONCLUSIONS
Congenital portosystemic shunts are associated with splenic artery aneurysms, a previously unrecognized association. Portosystemic shunts were undetected during prospective radiologic evaluation in the majority of adult patients, highlighting the need to alert radiologists to this congenital anomaly.
Konstas
Angelos A
AA
Department of Radiology, Massachusetts General Hospital and Harvard Medical School, 55 Fruit St, Boston, MA 02114, United States. akonstas@partners.org
Digumarthy
Subba R
SR
Avery
Laura L
LL
Wallace
Karen L
KL
Lisovsky
Mikhail
M
Misdraji
Joseph
J
Hahn
Peter F
PF
D000293:Adolescent; D000328:Adult; D000369:Aged, 80 and over; D000783:Aneurysm / Q000150:complications / Q000175:diagnosis*; D001164:Arteriovenous Fistula / Q000150:complications / Q000175:diagnosis*; D001706:Biopsy; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011168:Portal System / Q000002:abnormalities*; D012189:Retrospective Studies; D013157:Splenic Artery / Q000002:abnormalities*; D014057:Tomography, X-Ray Computed