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The Diagnosis of Ectopic Focal Hyperinsulinism of Infancy with [¹⁸F]-Dopa Positron Emission Tomography

London Center for Pediatric Endocrinology and Metabolism (K.H.), Hospital for Children National Health Service Trust, London WC1N 3JH, United Kingdom; The Institute of Child Health (K.H.), London WC1N 1EH, United Kingdom; Turku PET Center (M.S.) and Departments of Pediatrics (K.N.-S.) and Oncology and Radiotherapy (H.M.), Turku University Hospital, FIN 20521 Turku, Finland; Divisions of Surgery (N.S.A.) and Endocrinology (C.S.), Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104; and Division of Endocrinology and Diabetes (P.T.), Cook Children’s Medical Center, Fort Worth, Texas 76104

Address all correspondence and requests for reprints to: Dr. K. Hussain, Unit of Biochemistry, Endocrinology and Metabolism, Institute of Child Health, University College London, 30 Guilford Street, London WC1N 1EH, United Kingdom. E-mail: K.Hussain{at}ich.ucl.ac.uk.

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.

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

Methods/Results: Radiological investigations including imaging with ¹⁸fluoro-L-Dopa 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 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. ¹⁸Fluoro-L-Dopa positron emission tomography scan can identify ectopic focal lesions in patients with CHI.

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