Fetal abdominopelvic cystic lesions have various etiologies but are most commonly ovarian or gastrointestinal in origin. Congenital pancreatic cysts are rare entities that typically arise from the pancreatic body and tail, more often in females. In contrast, pancreatic heterotopia or an ectopic pancreas is defined as pancreatic tissue found outside the normal region of the pancreas without a vascular or anatomic connection to the pancreas. It is most commonly associated with the gastrointestinal tract, with >90% of cases located in the stomach, duodenum, or jejunum, and has been known to form cystic structures. We describe an unusual case of a term female neonate with a prenatal diagnosis of an abdominopelvic cyst. The patient had episodes of hypoglycemia secondary to hyperinsulinism requiring a continuous glucose infusion. On surgical resection, the cyst was found to extend from the retroperitoneum towards the left colon without connection to the pancreas or gastrointestinal tract. Histopathological evaluation showed components of pancreatic heterotopia including islets of Langerhans surrounded by acinar cells and dilated pancreatic ducts. The immunostain for Chromogranin showed numerous alpha and beta cells confirming pancreatic tissue. Hypoglycemic episodes improved following cyst resection. This is the first reported case of retroperitoneal cystic pancreatic heterotopia in a neonate in the English literature. We will present the ultrasound findings as well as surgical and histopathologic images.
SPR 2018 Annual Meeting & Postgraduate Course