Total pancreatectomy with islet autotransplantation in a pancreatic-sufficient cystic fibrosis patient

Abstract

For children with Cystic Fibrosis (CF) suffering from acute recurrent pancreatitis (ARP), abdominal pain can be severe, difficult to treat, impair their quality of life, affect participation at school, and can lead to chronic opioid dependence. Total pancreatectomy with islet autotransplantation (TPIAT) is an uncommon treatment that is reserved for refractory cases of ARP. We present a case of a 4 year old female with pancreatic-sufficient CF, refractory ARP, frequent hospital admissions for abdominal pain, and continued growth failure despite gastrostomy tube and parenteral nutrition. One year after successful TPIAT, the patient is insulin-independent, growing well, and has not been re-hospitalized for abdominal pain. To our knowledge, this is the youngest patient with CF to undergo TPIAT for debilitating ARP. With CFTR modulators restoring some pancreatic function, CF clinicians should have increased vigilance for the development of ARP.

Total Pancreatectomy with Autologous Islet Cell Transplantation—The Current Indications

“…Clinical contraindications for pancreatectomy with autologous islet transplantation in patients with pancreatic neoplasms are controversial. There are reports showing the successful use of this procedure in patients with pancreatic neoplasms (including IPMN, insulinoma, pancreatic and ampullary cancer, renal cell carcinoma (RCC) metastasis to pancreas) in the literature [ 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 ]. Ris et al [ 34 ] described 25 patients undergoing extended pancreatectomy and islet autotransplantation for benign pancreatic tumors.…”

“…Desai et al [ 48 ] described the first report of successful TPAIT for pancreatic cystosis on a 21-year-old woman with pancreatic cystic fibrosis, with insulin independence 11 months following surgery. St Onge et al [ 49 ] presented TPAIT on a 4-year-old female with pancreatic cystic fibrosis, with insulin independence one year following surgery.…”