Acute appendicitis, heterotaxy syndrome

Case contributed by Michael P Hartung
Diagnosis certain


Lower abdominal pain.

Patient Data

Age: 40 years
Gender: Female

Heterotaxy syndrome: Right polysplenia, right stomach, right pancreas, midline liver and gallbladder, IVC crosses left of midline to intrahepatic segment.

Small and large bowel have some degree of malrotation, although normal relationship with SMV and SMA is preserved. 

Cecum is in the midline pelvis with dilated, thick-walled and inflamed appendix with appendicolith at the base. 

Case Discussion

Typical appearance of acute appendicitis made more interesting due to heterotaxy syndrome with polysplenia/left isomerism. This is a good reminder that patients with variant anatomy may have different presentations of acute abdominal pain due to differences in position of abdominal organs. 

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