Total Pancreatectomy and Islet Auto-Transplantation for Chronic Pancreatitis Children: Pre-Surgical Evaluation, Patient Selection, the Surgical Procedures, Islet Isolation Procedure, and the Early Inpatient Management

Chronic pancreatitis in children is commonly due to genetic mutations (PRSS1, SPINK 1, and CFTR). Total pancreatectomy with islet auto-transplantation (TP-IAT) is now increasingly being used to treat children with chronic pancreatitis refractory to medical and endoscopic treatment. The total pancreatectomy removes the source of the pain and theoretically eliminates risk of pancreatic cancer. In isolation, however, a total pancreatectomy would lead to a lifetime of brittle diabetes. The islet auto-transplantation prevents or minimizes TP-related diabetes. Isolated islets of Langerhans are infused back into the patient, most typically via the portal vein, and eventually engraft in the sinusoids of the liver. The operative mortality after TP-IAT in pediatric patients is very low (0-1%). Surgical complications occur in 20% of patients and include abdominal hemorrhage (5.3%), bowel obstruction (5.3%), abdominal abscess (4%), enteric leak (2.6%), biliary leak (1.3%), and wound infection (1.3%). Pancreatitis-type pain and the severity of pain significantly improve in >90% of patients following TP-IAT. In the largest series of pediatric patients to date, 41.3% achieved insulin independence following TP-IAT, and 90.3% of these patients did so within 1 year. Younger children (<12 years) are more likely to achieve insulin independence than older children (12-18), 56.0% versus 40.5% (p = 0.05). Quality of life improves dramatically after TP-IAT, with most children reporting full-time return to school.