Pancreatic Kaposiform Hemangioendothelioma Presenting with Duodenal Obstruction and Kasabach–Merritt Phenomenon: A Neonate Cured by Whipple Operation

Abstract

Aim Kaposiform hemangiondothelioma (KHE) is a rare vascular tumor, commonly associated with Kasaback–Merritt phenomenon characterized by thrombocytopenia and consumptive coagulopathy. We report a case of pancreatic KHE presenting with neonatal duodenal obstruction and Kasaback–Merritt phenomenon.

Case Report A full term male baby presented with bile stained vomiting on Day 3 of life. Contrast study and computed tomography scan showed duodenal obstruction by a 5 cm extrinsic hypervascular mass. Platelet count was 23 x 109/L. Laparotomy confirmed a vascular tumor arising from the pancreatic head compressing on the duodenum. Whipple operation was performed.

Results Intestinal obstruction and thrombocytopenia resolved after surgery. There was no post-operative complications. Histology confirmed KHE. The boy was tolerating hydrolyzed milk formula and was thriving at 5 months follow up.

Conclusion We reported a case of pancreatic KHE presented with neonatal intestinal obstruction and Kasaback–Merritt phenomenon. High index of suspicion is necessary for diagnosis. To our knowledge, this is the youngest patient who underwent Whipple operation.

Keywords

pancreas - kaposiform hemangioendothelioma - Kasabach–Merritt phenomenon - Whipple operation - newborn

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