Pancreatic kaposiform hemangioendothelioma presenting with duodenal obstruction and kasabach-merritt phenomenon: a neonate cured by whipple operation

European Journal of Pediatric Surgery Reports
Michael LeungKenneth Lap Yan Chung

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.

Citations

Aug 28, 2020·World Journal of Pediatrics : WJP·Wei YaoHe-Ying Yang
Feb 6, 2020·Orphanet Journal of Rare Diseases·Yi JiLi Li
Nov 23, 2019·SA Journal of Radiology·Denny Mathew, Nasreen Mahomed
Oct 2, 2021·Radiographics : a Review Publication of the Radiological Society of North America, Inc·Lisa QiuJonathan R Dillman

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