Heterotaxy Syndrome

Case contributed by Ayman Mustafa


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

Age: 25 years
Gender: Female

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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