Anesthetic management of pediatric patient with congenital hyperinsulinism undergoing pancreatectomy

Abstract

Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in infants and children. In most affected infants, CHI is caused by a specific genetic defect that results in the altered expression of pancreatic beta cells causing unregulated oversecretion of insulin. Infants with CHI may have either focal or diffuse abnormalities of the pancreatic b-cells. Both forms of CHI manifest as hypoglycemia, usually in the early newborn period. Focal disease can be treated effectively with surgical resection of the affected area, resulting in a total cure or rendering the patient amenable to medical management. Most children with diffuse disease are unresponsive to medical therapy, and require near-total pancreatectomy (Olga et al, 2007). The main aim of anesthetic management is to prevent hypoglycemia until resection of the pancreas and the control of rebound hyperglycemia soon after resection. Various approaches have been described, including use of “artificial pancreas”, which continuously monitors plasma glucose and delivers glucose or insulin to maintain a predetermined glucose level. But, it is not much in use due to its cost and complexity (Manabe et al, 2009). Pancreatectomy is an urgent surgery that is performed to protect the brain. It is a safe procedure that is well tolerated in infants and children, and successful anesthetic management depends on fluid management, to maintain normoglycemia in spite of changing glucose levels, and to maintain hemodynamic stability during the perioperative period. This procedure is always a challenge to many anesthesiologists (Seohiu et al, 2012).