The laparoscopic approach toward hyperinsulinism in children.
Hyperinsulinemic hypoglycemia (HH) in children requiring surgery is rare. Early HH can be the result of focal or diffuse pancreatic pathology. A number of genetic abnormalities in early HH have been identified, but in the majority of patients no abnormality is found. The sporadic focal and diffuse forms as well the autosomal recessive form are particularly therapy-resistant and demand for early surgery. Preoperative discrimination between focal and diffuse disease in early HH is difficult. 18 F DOPA PET in combination with CT is promising as is laparoscopic exploration of the pancreas. Frozen section biopsy analysis has not been uniformly beneficial. If macroscopically no focal lesion is found, limited laparoscopic distal pancreatectomy provides tissue for definitive pathologic examination. Subsequent near total laparoscopic spleen-saving pancreatectomy surgery is not particularly difficult. Later HH may occur in the context of the MEN-1 syndrome and is then multifocal in nature. In MEN-1 patients, a distal spleen-saving pancreatectomy with enucleation of lesions in the head seems justified. Insulin-producing lesions in non-MEN-1 patients should be enucleated. There should always be a suspicion of malignancy. Also, in older children, surgery for hyperinsulinism should be performed laparoscopically.
Bax
Klaas N M A
KN
Sophia Children's Hospital, Erasmus Medical Centre, Rotterdam, The Netherlands. n.bax@erasmusmc.nl
van der Zee
D C
DC
D017668:Age of Onset; D044903:Congenital Hyperinsulinism / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D005629:Frozen Sections; D006801:Humans; D006946:Hyperinsulinism / Q000145:classification / Q000188:drug therapy / Q000453:epidemiology / Q000601:surgery*; D007340:Insulinoma / Q000601:surgery; D010535:Laparoscopy; D018761:Multiple Endocrine Neoplasia Type 1 / Q000601:surgery; D010180:Pancreatectomy; D014057:Tomography, X-Ray Computed