Presentation
Past medical history of orthotopic heart transplantation for complex congenital heart disease complicated by chronic antibody-mediated rejection. Surgical history of distal pancreatectomy and right hemicolectomy.
Patient Data
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Liver: Enlarged and nodular in contour. Transverse liver.
Spleen: Absent
Pancreas: Postsurgical changes related to distal pancreatectomy. Similar size of hypodense lesions within the pancreatic head measuring up to 2.4 cm.
GI Tract: Enteric tube terminates within the stomach. Postsurgical changes elated to right hemicolectomy and left upper quadrant ileostomy.
Adrenal Glands: Unremarkable
Kidneys: Unremarkable
Bladder: Contrast within the lumen of the bladder. Thickened and irregular Contour
Reproductive Organs: Unremarkable
Peritoneum/Retroperitoneum: No free air. Stable position of 4 percutaneous abdominal drains. Trace pelvic and mesenteric free fluid. Similar size of a 5.5 <2.5 cm perigastric fluid collection.
Lymph Nodes: Unremarkable
Vessels: Duplicated infrarenal IVC
Impression: Abdominal heterotaxy with asplenia.
Case Discussion
Heterotaxy syndrome (situs ambiguous) is a disturbance in the usual left-right distribution of the thoracoabdominal organs. Unlike situs inversus, heterotaxy does not entirely translate into a complete mirror image. This syndrome can be disturbed into one of two subsets:
1. heterotaxy with isomerism of the right atrial appendages (asplenia syndrome)
2. heterotaxy with isomerism of the left atrial appendages (polysplenia syndrome)
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