Dependence of pancreas resection volume on PET-tomography and express biopsy findings

Abstract

Introduction. Recently, achievements in molecular genetics, imaging techniques (PET/CT), medicamentous therapy as well as in surgical treatment have promoted a better control of hypoglycemia and, consequently, better outcomes in children with congenital hyperinsulinism.Purpose. To specify indications, volume and outcomes of surgical treatment in patients with congenital hyperinsulinism depending on pathology form, differential diagnostics with PET / CT and intraoperative express biopsy.Material and methods. 41 children with congenital hyperinsulinism were operated in the department of pediatric surgery in Almazov National Medical Research Centre (Saint-Petersburg) during 2011 – September 2020. In Group 1, there were 6 children who had standard treatment with subtotal resection of the pancreas (95%). In Group 2, there were 35 patients who had PET tomography with 18-F-DOPA before surgery and intraoperative express biopsy of pancreas tissue. The analyzed patients were operated on in 2017-September 2020. PET tomography with 18-F-DOPA findings revealed that these children had 10 diffuse forms and 22 focal forms; the other 3 children had a disputable picture.Results. After surgery, diffuse form was confirmed in 10 (29%) children; focal form - in 21 (60%) children; 4 (11%) patients had the atypical form which was diagnosed intraoperatively. Of 35 children from Group 2, 33 (94%) had complete hyperinsulinism reversal, significant improvement in their psycho-motor function; however, 10 (29%) of them require insulin replacement therapy with minimal dosages - 8 children with the diffuse form of hyperinsulinism and 2 children with the atypical one. There were no intra- and postoperative complications. 20 (95%) out of 21 children with focal forms recovered completely. The authors also describe problems and peculiarities of urgent histological examination.Conclusion. Thus, partial pancreatectomy in focal forms, subtotal in atypical ones and almost total in diffuse forms allows to cope with hypoglycemia caused by congenital hyperinsulinism.