title: 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.
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Queen Elizabeth Hospital, Kowloon, Hong Kong, Hong Kong.
authors.forename: Michael
authors.initials: M
authors.lastname: Leung
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Queen Elizabeth Hospital, Kowloon, Hong Kong, Hong Kong.
authors.forename: Nicholas Sih Yin
authors.initials: NS
authors.lastname: Chao
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Queen Elizabeth Hospital, Kowloon, Hong Kong, Hong Kong.
authors.forename: Paula Man Yee
authors.initials: PM
authors.lastname: Tang
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Queen Elizabeth Hospital, Kowloon, Hong Kong, Hong Kong.
authors.forename: Kelvin
authors.initials: K
authors.lastname: Liu
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Queen Elizabeth Hospital, Kowloon, Hong Kong, Hong Kong.
authors.forename: Kenneth Lap Yan
authors.initials: KL
authors.lastname: Chung
keywords: Kasabach–Merritt phenomenon; Whipple operation; kaposiform hemangioendothelioma; newborn; pancreas