CONGENITAL EXTRAHEPATIC PORTOSYSTEMIC SHUNT IN PANCREATIC CANCER

Objectives: Introduction
Portosystemic shunts are generally related with portal hypertension but in a few cases, they are associated with genetic abnormalities called congenital portosystemic shunts (CPSS). The CPSS is characterized by a large communication between the portal venous system and the systemic venous circulation in the absence of parenchymal or biliary disease. The incidence of CPSS is not well known and estimated to be 1/30,000 live births. Different types of CPSS have been described by abdominal imaging: extrahepatic and intrahepatic. The extrahepatic are classified as type Ia (splenic and the SMV drain separately into the IVC), Ib (portal vein drains into IVC), both without portal perfusion to the liver and II has normal portal vein (may be hypoplastic) draining to the liver and communicating with IVC. Herein, we present an unusual case of CPSS in an asymptomatic patient diagnosed with pancreatic cancer
Case Report
An 85-years-old female with a past medical history of a carotid endarterectomy, hypertension and no other comorbidities presented with an episode of decreased appetitive and obstructive jaundice. Imaging studies were performed showing the presence of a 3 cm mass in the head of the pancreas. Incidentally, there was a large portosystemic shunt extending from the left renal vein to the portal vein as well as a replaced right hepatic artery arising from the SMA noted. Both were asymptomatic to the patient. An upper endoscopic ultrasound was carried out and showed the mass previously described a biopsy from the mass was obtained confirming the presence of an adenocarcinoma. The case was presented in a pancreatic board and was decided to go on neoadjuvant chemotherapy for 5 cycles. After that, a Whipple procedure was performed with no injury to the large CPSS (see image below). The patient did well and was discharged in the fifth day.
Conclusion
Few cases of CPSS are described in the literature, especially with a pancreatic cancer. Some reports show that these patients may have a higher rate of other genetic diseases but no associate with malignancy has been noted. Patients with CPSS may develop complications just like encephalitis, portal hypertension, hepato-pulmonary syndrome and shunt closure during surgery must be considered. However, this patient was 85 and asymptomatic and therefore did not require taking down the shunt. Care should be taken to identify these vascular abnormalities prior to surgery to avoid intraoperative injury of an anomalous vascular structure.

Methods:

Results:

Conclusion: