Intrapancreatic accessory spleen in newborn with multiple congenital malformations

Abstract

A new case of intrapancreatic accessory spleen located in the tail of the pancreas in a premature male newborn is reported. The anomaly was unexpectedly found at the autopsy together with many other malformations including heart defects, polycystic kidneys, oxycephaly, facial dysmorphy, low set ears, webbing of neck and accessory fingers of right hand and foot. Microscopic examination of the pancreas revealed accessory spleen composed of red and white pulp. The spleen was partly well-demarcated but partly intermingled with pancreatic lobules. Many goblet cell-containing intralobular pancreatic ducts, some of them entrapped within spleen were noted. Splenopancreatic field abnormalities are known to be highly specific for trisomy 13 syndrome. Although karyotyping was not performed, on the basis of reveled malformations the syndrome can be suspected in the presented case.