Nesidioblastosis associated with congenital malformations of the heart. Morphological and immunohistochemical study of 5 necropsy cases.

Pancreatic tissue from 5 children with congenital heart disease (CHD) with morphological alterations of the endocrine components at autopsy were compared in a double-blind semiquantitative study, which included light microscopy and immunocytochemistry, to pancreatic tissue of 5 children of similar age but without CHD. Hyperplasia and hypertrophy of islets of Langerhans, scattered endocrine cells within the exocrinic acini, cluster of endocrine cells budding from ductular epithelium or within interlobular connective tissue, and hyperplasia of ductular epithelium were found to be present in the patients with congenital cardiac defects. The observed findings resembled those present in a surgically resected pancreas from a child with clinically overt nesidioblastosis. Although this study suggests a higher incidence of endocrine pancreas disturbances in certain types of CHD, the clinical significance of these morphological alterations will require further studies.