Korean J Anesthesiol.  1997 Jun;32(6):1031-1035. 10.4097/kjae.1997.32.6.1031.

Anesthetic Management of an Infant with Nesidioblastosis: A case report

• Kim DK
• Bahk JH
• Kim JS
• Min SW

• KMID: 2237773
• DOI: http://doi.org/10.4097/kjae.1997.32.6.1031

Abstract

Nesidioblastosis, persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is a disorder characterized by diffuse pancreatic islet cell hyperplasia arising from the ductal epithelium. Patients usually present during the neonatal or infantile period with apnea, hypotonia, poor feeding, lethargy, or seizure. Despite of greater awareness, one in three has some degree of mental retardation by the time the diagnosis is made. The diagnosis is established by demonstrating high plasma insulin concentration during an episode of hypoglycemia. This hypoglycemia is initially managed medically, but these medical treatment modalities are failed in more than half of nesidioblastosis. Patient who failed to respond to optimal medical treatment should be referred for surgery early, if permanent neurologic damage is to be avoided. The surgical procedure of choice is near total pancreatectomy (95~98% resection). We herein discuss the anesthetic management of a patient with nesidioblastosis who presented for near total pancreatectomy.

Keyword

Metabolism; hyperinsulinism; nesidioblastosis; Surgery; near total pancreatectomy

MeSH Terms

Apnea
Congenital Hyperinsulinism
Diagnosis
Epithelium
Humans
Hyperinsulinism
Hyperplasia
Hypoglycemia
Infant*
Insulin
Intellectual Disability
Islets of Langerhans
Lethargy
Metabolism
Muscle Hypotonia
Nesidioblastosis*
Pancreatectomy
Plasma
Seizures
Insulin