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Asplenia Syndrome with Congenital Microgastria and Malrotation

Journal of Perinatology volume 24, pages 50–52 (2004)Cite this article

The abdominal sonogram and plain radiographs demonstrated a transverse liver. Additionally the sonogram showed a left upper quadrant gall bladder, normal intrahepatic portion of the inferior vena cava and no discernible spleen. Given the heart disease and sonographic findings, a diagnosis of asplenia syndrome was made. The upper gastrointestinal series demonstrated congenital microgastria with a tubular stomach with a short antrum, located in the midline, and intestinal malrotation. The child was taken to the operating room after having stabilized from the cardiac surgery. A Ladd's procedure with lysis of duodenal adhesions was performed. A gastrostomy tube was placed. The absence of the spleen and microgastria were confirmed. Because of the child's tenuous cardiac status, no further surgery was done.

Asplenia syndrome is a syndrome of right isomerism or attempted bilateral right sidedness.1 Bilateral right-sidedness is manifest as bilateral trilobed lungs, bilateral right atrial appendages, transverse liver and absence of the spleen.1,2 Asplenia syndrome is usually associated with severe congenital heart disease including complete atrioventricular canal, transposition of the great arteries, anomalous pulmonary venous drainage and pulmonic or less commonly aortic stenosis or atresia. Because of the severity of the heart disease and the immune defect from absence of the spleen, the mortality among patients with asplenia is high.3

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  1. Mallinckrodt Institute of Radiology, St. Louis Children's Hospital, St. Louis, MO, USA

    Thomas E Herman MD & Marilyn J Siegel MD

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  1. Thomas E Herman MD
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  2. Marilyn J Siegel MD
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Herman, T., Siegel, M. Asplenia Syndrome with Congenital Microgastria and Malrotation. J Perinatol 24, 50–52 (2004). https://doi.org/10.1038/sj.jp.7211022

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