Pneumoperitoneum in a patient with heterotaxy syndrome (left isomerism)
Diagnosis certain
Presentation
Sudden onset of central chest pain. Background of sick sinus syndrome and permanent pacemaker.
Patient Data
Age: 40
Gender: Female
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Continuous diaphragm sign and free gas inferior to the right hemidiaphragm indicating pneumoperitoneum.
Dual lead permanent pacemaker projected over the right chest. No evidence of complication.
CT abdomen and pelvis
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- Free gas is present, predominately within the right upper abdomen. A site of perforation is not evident. Mild bowel wall thickening involves jejunal loops without significant surrounding inflammatory change.
- Malrotation. Jejunal loops are located within the right upper quadrant. The cecum is left-sided. The duodenum can be traced with D3 extending anteriorly and to the right from the inferior duodenal flexure.
- The portal vein passes anterior to the neck of the pancreas.
- Polysplenism.
- Left-sided IVC.
Case Discussion
The patient underwent laparoscopy but the site of perforation was not identified. There were no plans to surgically correct the malrotation.
Left isomerism (also called polysplenism) is a type of heterotaxy syndrome characterized by multiple spleens, malposition of the abdominal viscera, abnormalities of the IVC, and cardiac abnormalities.
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