Total pancreatectomy with islet autotransplantation in children

Chronic pancreatitis (CP) and recurrent acute pancreatitis (RAP) in children most often occur in the setting of genetic mutations that increase the risk for pancreatitis. CP and RAP are associated with high disease burden, including multiple hospitalizations, missed school, and risk of opioid dependence. Medical and endoscopic therapeutic options are limited, and so when disease is severe, total pancreatectomy with islet autotransplant (TPIAT) may be considered. In children undergoing TPIAT, quality of life substantially improves and > 80% of children remain free of opioids long term. Most children are C-peptide positive following islet autotransplantation, and insulin independence is achieved in over 40%. Very young children may have the greatest chance at insulin independence and possibly greater likelihood for complete pain relief. Before and after TPIAT, these children require frequent care from a multidisciplinary team, for glycemic control, malabsorption issues, micronutrient deficiencies, and management of surgical asplenia.