Intraoperative Sonography: A Technique for Localizing Focal Forms of Congenital Hyperinsulinism in the Pancreas

Zusammenfassung

Der kongenitale Hyperinsulinismus (CHI), Synonym Nesidioblastose, ist die häufigste Ursache persistierender, rezidivierender Hypoglykämien im Säuglingsalter. Ein Drittel der Patienten weist einen umschriebenen Fokus auf. Die Enukleation und vollständige Entfernung aller betroffenen β-Zellen ist die Therapie der Wahl. Gesundes Gewebe muss soweit als möglich geschont, intrapankreatischer Choledochus sowie Ductus pancreaticus major intakt bleiben. Diagnostischer Goldstandard ist das [¹⁸F] F DOPA PET/CT. Die Sonografie wird durchgeführt, um den im präoperativen PET/CT lokalisierten Fokus während der Operation korrekt in situ darzustellen. Bei 5 Patienten im Alter von 3œ – 14 Monaten erfolgte 3 – 20 Tage nach PET/CT die Enukleation des Herdes. Die intraoperative Ultraschalluntersuchung wurde mit Hochleistungsgeräten verschiedener Hersteller unter Verwendung von Breitbandschallköpfen (9 – 14 MHz) vorgenommen. Bei allen 5 Patienten mit fokaler CHI ist, gemessen an PET/CT, intraoperativem Lokalbefund und Histologie mithilfe des intraoperativen Ultraschalls, der Herd korrekt lokalisiert und von den Ductus choledochus et pancreaticus major sicher abgegrenzt worden. 3 von 5 Patienten wurden durch komplette Fokusenukleation geheilt. Problematisch ist die vollständige intraoperative Darstellung stark segmentierter Herde. Sonografische Charakteristika des CHI-Fokus sind: konstante Hypoechogenität, variable homogene/inhomogene Gewebetextur, unscharfe, unregelmäßige Begrenzung ohne Kapsel, filiforme, lobuläre Fortsätze und insuläre Absiedlungen in das umgebende Gewebe. Die intraoperative hochauflösende Sonografie hilft dem Kinderchirurgen, Größe, Konfiguration und Topografie eines CHI-Fokus zu bestimmen.

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.

Key words

congenital hyperinsulinism - pancreas - intraoperative - ultrasound - PET-CT

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