Endocrine Abstracts (2008) 17 OC16

Surgical outcomes in congenital hyperinsulinism of infancy (CHI) pre and post the introduction of diagnostic 18Fluoro (F)-DOPA positron emission tomography (PET)-CT scanning

M Skae1, J Bruce1, O Blankenstein2, M Jones3, A Morabito1, A Bianchi1, L Rigby1, L Patel1, R Amin1, P Clayton4, I Banerjee1 & C Hall1

1Royal Manchester Children’s Hospital, Manchester, UK; 2Charite University of Medicine, Berlin, Germany; 3Royal Liverpool Children’s Hospital, Liverpool, UK; 4University of Manchester, Manchester, UK.

In severe CHI which is unresponsive to medical therapy, pancreatic resection is undertaken to prevent further hypoglycaemia from dysregulated beta-cell insulin secretion. Traditionally, 95% subtotal pancreatectomy has been the surgery of choice (Lovvorn et al. 1999), with associated risks of post-operative complications such as lifelong diabetes and malabsorption. Since the introduction of 18F-DOPA PET-CT imaging, clinicians have been able to effectively differentiate between focal and diffuse CHI processes, hence altering surgical practice and allowing limited pancreatic resection in focal disease. At the Northern Congenital Hyperinsulinism Service (NORCHI), patients with a genetic analysis suggestive of possible focal disease (paternal or no mutation in the ATP sensitive potassium channel) have undergone PET-CT imaging since 2007.

Aim: To assess the influence of genetic analysis and 18F-DOPA PET-CT on surgical outcomes in CHI patients before and after the introduction of 18F-DOPA PET-CT scanning as part of patient management.

Method: A retrospective case notes analysis of CHI patients referred to the Royal Manchester Children’s Hospital between 1998–2008 was performed.

Results: Eleven patients who had pancreatic surgery were identified. Prior to the introduction of 18F-DOPA PET-CT imaging (1998–2006), 5 CHI patients underwent subtotal pancreatectomies, with 2 requiring further surgery to control hypoglycaemia. Four patients developed malabsorption and 3 developed insulin dependent diabetes post-operatively. Since 2007, 6 patients have undergone pancreatic surgery including 3 patients with a PET-CT diagnosis of focal disease who underwent focal lesionectomies (all curative). Three patients with diffuse disease had 95% subtotal pancreatectomies, with 1 requiring repeat surgery. Post-operatively, none require regular anti-hypoglycaemic medication and none have developed malabsorption or diabetes. All cases were consistent with post-operative histological diagnosis.

Conclusion: Rapid genetic analysis with 18F-DOPA PET-CT imaging can effectively identify patients with focal lesions. This has significantly altered surgical management of CHI and reduced the incidence of life long postoperative complications such as malabsorption and diabetes.