title: Intraoperative sonography: a technique for localizing focal forms of congenital hyperinsulinism in the pancreas.
abstract: Congenital hyperinsulinism (CHI), syn. nesidioblastosis, is the most frequent cause of persistent, recurrent hypoglycemia in infancy. One third of patients show a single circumscribed focus. Enucleation of the focus and the removal of all affected β-cells with preservation of healthy tissue is the treatment of choice. The intrapancreatic choledochus as well as the ductus pancreaticus major must remain intact. The diagnostic gold standard is 18F-DOPA-PET/CT. Intraoperative sonography is carried out to correctly visualize the focus preoperatively localized by PET/CT in situ during the operation. The enucleation of the focus was carried out 3 - 20 days after PET/CT in 5 patients at an age of 3.5 - 14 months. Intraoperative ultrasound was carried out with high-capacity devices of different manufacturers under use of broadband probes (9 - 14 MHz). The localization by intraoperative ultrasound was accurate in all 5 patients with focal CHI, with regard to the intraoperative localization as previously described by PET/CT and histology. D. choledochus and D. pancreaticus major were separated intraoperatively by ultrasound. 3 of 5 patients were cured by complete enucleation of the focus. Nevertheless, the entire intraoperative identification of the segmented focus is still problematic. Characteristic sonographic features of a CHI focus are: hypoechogenicity, variable homogeneous and inhomogenous texture, blurred, irregular limitation without capsule, filiform, lobular processes, and insular dispersal into the surrounding tissue. Intraoperative high-resolution sonography helps the pediatric surgeon to determine size, configuration and topography of a CHI focus.
authors.affiliation: Kinderradiologie, Klinik für Radiologie, Germany. susann.empting@med.ovgu.de
authors.forename: L
authors.initials: L
authors.lastname: von Rohden
authors.forename: K
authors.initials: K
authors.lastname: Mohnike
authors.forename: H
authors.initials: H
authors.lastname: Mau
authors.forename: T
authors.initials: T
authors.lastname: Eberhard
authors.forename: W
authors.initials: W
authors.lastname: Mohnike
authors.forename: O
authors.initials: O
authors.lastname: Blankenstein
authors.forename: S
authors.initials: S
authors.lastname: Empting
authors.forename: M
authors.initials: M
authors.lastname: Koch
authors.forename: F
authors.initials: F
authors.lastname: Füchtner
authors.forename: W
authors.initials: W
authors.lastname: Barthlen
mesh_terms: D003135:Common Bile Duct / Q000000981:diagnostic imaging / Q000601:surgery; D004867:Equipment Design; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted / Q000295:instrumentation / Q000379:methods*; D021621:Imaging, Three-Dimensional; D007223:Infant; D050417:Insulin-Secreting Cells / Q000000981:diagnostic imaging; D007432:Intraoperative Period; D008297:Male; D046768:Nesidioblastosis / Q000000981:diagnostic imaging* / Q000601:surgery*; D010179:Pancreas / Q000000981:diagnostic imaging* / Q000601:surgery*; D010183:Pancreatic Ducts / Q000000981:diagnostic imaging / Q000601:surgery; D049268:Positron-Emission Tomography; D012680:Sensitivity and Specificity; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography / Q000295:instrumentation / Q000379:methods*