Hereditary pancreatitis (HP) is a progressive disease that can manifest in childhood with debilitating, relapsing pain. A total pancreatectomy and islet autotransplant (TPIAT) is a surgical option to relieve chronic pain while preserving the available β-cell mass. The clinical course of HP is fraught with pancreatitis-related sequelae that can both necessitate and complicate a TPIAT. We describe a child with HP who developed a pancreatic pseudocyst–portal vein (PV) fistula. Active hemorrhage of the perforated PV into the pseudocyst and PV thrombosis complicated the planned TPIAT procedure and, preoperatively, required urgent image-guided stenting. During the TPIAT procedure, the endovascular stent was found to be protruding through the PV into the pseudocyst. Using the autologous splenic vein from the TPIAT specimen, we performed a vascular reconstruction of the perforated PV. This case underscores the need for evaluation of children with HP by a multidisciplinary pancreatic TPIAT care team to best prepare for the potential ramifications of pancreatitis-related complications. It also illustrates a useful vascular reconstruction technique for PV complications.
- hereditary pancreatitis
- pediatric chronic pancreatitis
- pseudocyst-portal vein fistula
- total pancreatectomy and islet autotransplant
- vein reconstruction