True congenital cysts of the pancreas in the newborn and infant.

Objectives: True congenital pancreatic cysts (CPC) are extremely rare, only 27 cysts were reported so far in children under 3 years of age. With several new cases reported here, conclusions for diagnostic work up and treatment can be drawn. Material and Methods: 4 cases with CPC are analyzed retrospectively and reviewed together with all previously published cases. Results: Girls were affected by CPC about twice as frequent as boys, cysts were localized more often in the tail than in the head or body of the pancreas. Unilocular cysts are seen more frequent than multilocular cysts. Diagnosis can be made by prenatal or postnatal ultrasound. Usually, total excision of the cyst(s) was performed and prognosis was good in otherwise healthy children. Conclusion: This rare diagnosis should be considered when cystic lesions appear in routine antenatal ultrasound. The benign nature of these cysts together with a certain risk for complications when left in place justifies for a planned surgical approach in the neonate aiming at total excision of the cyst with preservation of the pancreas. Ultrasound is sufficient for preoperative imaging. Keywords: Pancreatic cyst; Congenital; Prenatal diagnosis; Pediatric