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 (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.
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 (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.
Authors: Pablo Laje; Lisa J States; Hongming Zhuang; Susan A Becker; Andrew A Palladino; Charles A Stanley; N Scott Adzick Journal: J Pediatr Surg Date: 2013-02 Impact factor: 2.545
Authors: N Scott Adzick; Diva D De Leon; Lisa J States; Katherine Lord; Tricia R Bhatti; Susan A Becker; Charles A Stanley Journal: J Pediatr Surg Date: 2018-10-05 Impact factor: 2.545
Authors: Björn A Blomberg; Mateen C Moghbel; Babak Saboury; Charles A Stanley; Abass Alavi Journal: Mol Imaging Biol Date: 2013-02 Impact factor: 3.488