Nesidioblastosis treated successfully by 85% pancreatectomy.
Nesidioblastosis is a rare disorder in pediatric surgery. It is caused by hypertrophy and hyperplasia of the islands of Langerhans, and can lead to persistent hyperinsulinemic hypoglycemia. If appropriate treatment is delayed there is a high risk of the development of cerebral palsy, impaired mental development, epilepsy or other forms of irreversible brain damage, so early detection and early treatment to stabilize serum glucose levels is essential. Initial treatment consists of nutritional management with administration of an adequate amount of calories along with drug therapy using diazoxide. In cases that are resistant to drug therapy a subtotal pancreatectomy is performed. Conventional recommended practice has been to perform a 95% pancreatectomy, however this can lead to the onset of diabetes due to abnormal pancreatic endocrine function. We report here a case of nesidioblastosis in which we performed an 85% pancreatectomy. Postoperative clinical course has been excellent, with no recurrence or impaired glucose tolerance during the four years since the operation.
Tanaka
Yoshiaki
Y
Department of Pediatric Surgery, Kurume University School of Medicine, 67 Asahi-machi, Kurume 830-0011, Japan. yoshi456@med.kurume-u.ac.jp
Asakawa
Takahiro
T
Asagiri
Kimio
K
Akiyoshi
Kenjiro
K
Hikida
Shigeki
S
Mizote
Hiroyoshi
H
D001786:Blood Glucose / Q000032:analysis; D006801:Humans; D007223:Infant; D010180:Pancreatectomy / Q000379:methods*; D010182:Pancreatic Diseases / Q000601:surgery*; D010289:Parenteral Nutrition, Total
D001786:Blood Glucose