Intraoperative Ultrasound: A Tool to Support Tissue-Sparing Curative Pancreatic Resection in Focal Congenital Hyperinsulinism

Abstract

Background: Focal congenital hyperinsulinism (CHI) may be cured by resection of the focal, but often non-palpable, pancreatic lesion. The surgical challenge is to minimize removal of normal pancreatic tissue.Aim: To evaluate the results of intraoperative ultrasound-guided, tissue-sparing pancreatic resection in CHI patients at an international expert center.Methods: Retrospective study of CHI patients treated at Odense University Hospital, Denmark, between January 2010 and March 2017.Results: Of 62 consecutive patients with persistent CHI, 24 (39%) had focal CHI by histology after surgery. All patients had a paternal ABCC8 or KCNJ11 mutation and a focal lesion by 18F-DOPA-PET/CT. Intraoperative ultrasound localized the focal lesion in 16/20 patients (sensitivity 0.80), including one ectopic lesion in the duodenal wall. Intraoperative ultrasound showed no focal lesion in 11/11 patients with diffuse CH (specificity 1.0). The positive predictive value for focal histology was 1.0, negative predictive value 0.73.Tissue-sparing pancreatic resection (focal lesion enucleation, local resection of tail or uncinate process) was performed in 67% (n = 16). In 11/12 having tissue-sparing resection and intraoperative ultrasound, the location of the focal lesion was exactly identified. Eight patients had resection of the pancreatic head or head/body, four with Roux-en-Y, three with pancreatico-gastrostomy and one without reconstruction. None had severe complications to surgery. Cure of hypoglycaemia was seen in all patients after one (n = 21) or two (n = 3) pancreatic resections.Conclusion: In focal CHI, tissue-sparing pancreatic resection was possible in 67%. Intraoperative ultrasound was a helpful supplement to the mandatory use of genetics, preoperative 18F-DOPA-PET/CT and intraoperative frozen sections.

The difficult management of persistent, non‐focal congenital hyperinsulinism: A retrospective review from a single, tertiary center

“…In general, the rate and severity of complications depend on both the reporting criteria used, patient co‐morbidity, type of CHI and the complexity of the surgical procedure. We believe that the Clavien‐Dindo classification is an appropriate reporting tool for surgical complications as used once before in CHI …”

“…Hence, a non‐dominant, heterozygous paternal K ATP ‐channel mutation in blood analysis is indicative of focal CHI, whereas homozygous, compound heterozygous, or heterozygous maternal ABBC8/KCNJ11 mutations indicate diffuse CHI . In focal CHI, the focal lesion can be localized by 18 FDOPA PET/CT scan aiding surgeons to perform curative limited pancreatic resection, in addition to per‐operative frozen sections and ultrasound …”

Update of variants identified in the pancreatic β‐cell K ATP channel genes KCNJ11 and ABCC8 in individuals with congenital hyperinsulinism and diabetes

“…18 F‐DOPA PET/CT scanning can identify and localize a focal lesion before surgery (Otonkoski et al, 2006). Intraoperative ultrasound may further aid the surgeon to perform tissue‐sparing pancreatic resection in focal CHI, which is potentially curative (Bendix et al, 2018).…”

Suspected congenital hyperinsulinism in a Shiba Inu dog

“…Congenital hyperinsulinism is the most common cause of persistent hyperinsulinemic hypoglycemia in infants 12 and can occur in both focal and diffuse forms. The focal form lends itself to partial pancreatectomy, but the focal nature is challenging to confirm, requiring genotyping, fluorine‐18 L‐3,4‐dihydroxyphenylalanine positron emission tomography, 13 intraoperative ultrasonography, 14 intraoperative frozen section histopathology, 15 or some combination of these for confirmation. The diffuse form may require subtotal pancreatectomy as part of management, but it is infrequently curative 15,16 …”