The diagnosis of ectopic focal hyperinsulinism of infancy with [18F]-dopa positron emission tomography.
BACKGROUND
Congenital hyperinsulinism (CHI) is a cause of severe hypoglycemia in the neonatal and infancy period. Histologically, there are two subtypes with diffuse and focal disease. The preoperative differentiation of these two forms is very important because the surgical management is radically different. The focal form of the disease can be cured if the focal lesion can be localized accurately and completely resected with surgery.


OBJECTIVE
We report the case of a child who underwent three pancreatectomies with a choledochoduodenostomy and a cholecystectomy but continued to have severe hyperinsulinemic hypoglycemia.


RESULTS
Radiological investigations including imaging with (18)fluoro-L-Dopa positron emission tomography scan showed a clear focus of increased (18)F-fluoro-L-Dopa uptake in the vicinity of the former head of the pancreas. On the magnetic resonance imaging scan, this focal uptake appeared to localize adjacent or next to duodenum (in the wall or cavity of the duodenum).


CONCLUSIONS
This unique case highlights the importance of correctly localizing and completely resecting the focal lesion in patients with CHI. (18)Fluoro-L-Dopa positron emission tomography scan can identify ectopic focal lesions in patients with CHI.
Hussain
Khalid
K
London Center for Pediatric Endocrinology and Metabolism, Hospital for Children National Health Service Trust, London WC1N 3JH, United Kingdom. K.Hussain@ich.ucl.ac.uk
Seppänen
Marko
M
Näntö-Salonen
Kirsti
K
Adzick
N Scott
NS
Stanley
Charles A
CA
Thornton
Paul
P
Minn
Heikki
H