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Submitted on February 28, 2006
Accepted on May 2, 2006
London Centre for Paediatric Endocrinology and Metabolism, Great Ormond Street, Hospital for Children NHS Trust, WC1N 3JH, and The Institute of Child Health, London WC1N 1EH UK; Turku PET Centre, Turku University Hospital, Turku, Finland; Department of Pediatrics, Turku University Hospital, Turku, Finland; Division of Surgeryand EndocrinologyChildren's Hospital of Philadelphia USA; Divison of Endocrinology and Diabetes, Cook Childrens Medical Center, Fort Worth, Texas USA; Department of Oncology and Radiotherapy, Turku University Hospital, Turku, Finland
* To whom correspondence should be addressed. E-mail: K.Hussain{at}ich.ucl.ac.uk.
Background: Congenital hyperinsulinism 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 as the surgical management is radically different. The focal form of the disease can be cured if the focal lesion can be accurately localized and surgically completely resected.
Aim: We report the case of a child who underwent three pancreatectomies with a choledochoduodenostomy and a cholecystectomy but continued to have severe hyperinsulinaemic hypoglycemia.
Methods/Results: Radiological investigations including imaging with 18Fluoro-L-Dopa PET (Positron Emission Tomography) scan showed a clear focus of increased 18F-Fluoro-L-DOPA uptake in the vicinity of the former head of the pancreas. On the MRI scan this focal uptake appeared to localize adjacent/next to duodenum (in the wall/cavity of the duodenum).
Conclusions: This unique case highlights the importance of correctly localizing and completely resecting the focal lesion in patients with congenital hyperinsulinism. 18Fluoro-L-Dopa PET scan can identify ectopic focal lesions in patients with congenital hyperinsulinism.
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