Total Pancreatectomy With Islet Autotransplantation Resolves Pain in Young Children With Severe Chronic Pancreatitis

Abstract

Fear of diabetes and major surgery may prohibit referral of young children severely impacted by pancreatitis for total pancreatectomy and islet autotransplant (TPIAT). We evaluated outcomes in our youngest TPIAT recipients, age 3–8 years at surgery. Medical records were reviewed for 17 children (9 female) age ≤8 years undergoing TPIAT from 2000–2014. Most (14/17) had genetic risk factors for pancreatitis. Since 2006, TPIAT recipients were followed prospectively with health questionnaires including assessments of pain and narcotic use, and scheduled HbA1c and mixed meal tolerance tests (6 mL/kg Boost HP) before surgery, and at regular intervals after. Patients are 1–11 years post TPIAT (median 2.2 years). Data are reported as median (25th, 75th percentile). All had relief of pain, with all 17 patients off narcotics at most recent follow up. Hospitalization rates decreased from 5.0 hospitalization episodes per person-year of follow up before TPIAT, to 0.35 episodes per person-year of follow up after TPIAT. Fourteen (82%) discontinued insulin, higher than the observed insulin independence rate of 41% in 399 patients >8 years of age undergoing TPIAT over the same interval (p=0.004). Median post-TPIAT HbA1c was 5.9% (5.6, 6.3%), and within patient post-TPIAT mean HbA1c was ≤6.5% for all but 2 patients. Very young children with severe refractory chronic pancreatitis may be good candidates for TPIAT, with high rates of pain relief and insulin independence, and excellent glycemic control in the majority.

Utility of arginine stimulation testing in preoperative assessment of children undergoing total pancreatectomy with islet autotransplantation

“…[1][2][3][4] The likelihood of achieving insulin independence is closely related to the islet mass transplanted per kilogram of body weight. [1][2][3][4] The likelihood of achieving insulin independence is closely related to the islet mass transplanted per kilogram of body weight.…”

“…Total pancreatectomy and islet autotransplantation (TPIAT) can be a definitive treatment of pancreatitis pain in adults and children with chronic or recurrent acute pancreatitis while mitigating iatrogenic diabetes mellitus. [1][2][3][4] The likelihood of achieving insulin independence is closely related to the islet mass transplanted per kilogram of body weight. 5,6 However, islet mass isolated for transplant is highly variable between patients, due to varying degrees of fibrosis and islet loss.…”

EPC/HPSG evidence-based guidelines for the management of pediatric pancreatitis

“…Organ preservation should be the goal, but total pancreatectomy with islet autotransplant (TPIAT) should be considered as well 131,132 . EUS drainage via endoscopic cystgastrostomy has become the standard of care for drainage of large and symptomatic pancreatic pseudocysts in children 115,133,134 .…”

In-hospital and 90-day outcomes after total pancreatectomy with islet autotransplantation for pediatric chronic and acute recurrent pancreatitis

“…This figure compares the percentage of patients on and off opioids preoperatively and at 90 days postoperatively, with a significant decrease in the proportion of patients requiring opioids at 90 days transmembrane conductance regulator (CFTR), and chymotrypsin C (CTRC) mutations, with 73% of children possessing at least 1 mutation in a pancreatitis-related gene in the INSPPIRE cohort 21,27,28. In these patients specifically, with the high likelihood of progressive CP, the absence of anatomic characteristics that would allow surgical drainage procedures or local resections, and the life-time risk of pancreatic adenocarcinoma,29 early intervention in pediatric patients may have a significant long-term impact.The benefits of TPIAT in pediatric patients include the ability to wean off of opioid medications as well as PN 30,31. PN has significant risk and cost associated with it, including the risk of central line−associated bloodstream infections 32.…”