Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature

Abstract.

Background: To present the computed tomographic (CT) features of the abdominal anomalies consistent with polysplenia syndrome in adults. Awareness of these abnormalities may avoid misdiagnosing characteristic findings as separate pathological processes.

Methods: Imaging studies, mainly abdominal CT scans, of eight patients were reviewed. Attention was directed to the location of the multiple spleens, stomach, and liver and to the possible presence of a short pancreas, malrotation of the intestine, and venous anomalies. We also reviewed the CT findings of 15 adult patients described in the literature.

Results: Three men and five women underwent CT for various unrelated conditions. The most common findings were multiple spleens along the greater curvature of the stomach, which were located in the right upper quadrant in six patients. The inferior vena cava was seen on the left side in seven subjects, with azygos/hemiazygos continuation in six. A preduodenal portal vein was present in seven subjects. The liver was in the midline in four patients and on the left side in two. A short pancreas was seen in four patients, intestinal nonrotation in five, and dextrocardia in two. The prevalence of these anomalies was similar to that of the reviewed cases.

Conclusions: CT proved to be an excellent imaging modality in the diagnosis of the abdominal anomalies. Some of these (a short pancreas, multiple spleens, and azygos continuation) can simulate pathological processes. Hence the importance of recognizing these CT findings as part of a syndrome.