kaposiform hemangioendothelioma ( khe ) was first described by zukerberg et al in 1993.1 it is a rare vascular tumor with an estimated prevalence of less than 1 in 100,000 children . it is more commonly seen in neonates and early infancy . in approximately 70% of khe , merritt phenomenon ( kmp ) occurs characterized by consumptive coagulopathy and thrombocytopenia.2 kmp is rarely seen in the majority of common vascular tumors such as infantilehemangioma.3 khe usually has a cutaneous origin affecting extremities , cervicofacial and torso body wall.4 being classified as a vascular neoplasm , it usually infiltrates deeper tissue.5 khe with visceral origin is exceeding rare . a chinese male baby with uneventful antenatal history except maternal gestational diabetes was born at 41 weeks by emergency cesarean section because of cephalopelvic disproportion . apgar score was 8 at 1 and 5 minutes . because of maternal gestational diabetes , he had blood investigation for complete blood count , plasma electrolytes , and glucose . he was transferred to the neonatal unit for incidental finding of thrombocytopenia with platelet count 38    10/l . white cell count was 24.8    10/l with c - reactive protein ( crp ) of 13 mg / l . contrast study showed anterior and lateral displacement of the first and second part of duodenum with appearance suggestive of stretching by an extrinsic mass ( fig . abdominal sonography was performed on next day showing a retroduodenal hyperechoic mass measuring 3.8    3.4    5.2 cm with increased doppler signals . computed tomography confirmed a retroperitoneal hypervascular mass close to the uncinate process of pancreas ( fig . because of the persistent thrombocytopenia ( platelet count of 37    10/l ) and duodenal obstruction , laparotomy was performed on day 7 revealing a 4    4    5 cm retroperitoneal hypervascular tumor encasing the pancreatic head and lower end of common bile duct ( cbd ) , displacing the duodenal c - loop , and causing extrinsic compression . the mass had feeding vessels from the gastroduodenal artery and drained to the superior mesenteric vein ( smv ) . frozen section showed a vascular tumor with no evidence of malignancy . attempted resection of tumor preserving the pancreatic head and cbd failed because of its hypervascular and infiltrative nature . whipple operation was then decided with removal of the gall bladder , cbd , pancreatic head , distal one - third of stomach , pylorus , duodenum , and 15 cm of proximal jejunum with the tumor en bloc safeguarding hepatic artery , smv , and portal vein . oral feeding was started on day 10 and full feeding with normal formula was attained on day 14 . the pancreas was almost completely replaced by a tumor with closely packed lobules composing of proliferated oval and spindly endothelial cells forming whorls of kaposi sarcoma - like sweeping fascicles , compact capillaries , and glomeruloid structures . on immunohistochemical examination , the neoplastic endothelial cells expressed erg and cd31 but were negative for glucose transporter 1 and human herpesvirus 8 . the baby was followed up regularly after the operation . at 3 months , he developed steatorrhea and had failure to thrive with body weight fallen at third percentile . clinically , pancreatic insufficiency was suspected and high caloric , hydrolyzed formula feeding was prescribed . khe is classified as a vascular neoplasm according to the international society for the study of vascular anomalies ( issva).6 it is a rare high flow vascular tumor commonly affecting infants . it is locally aggressive and infiltrates surrounding tissues . to date , less than 300 cases have been reported worldwide.2 
 4 it shares the common histological feature of hemangioma and kaposi sarcoma.7 most khe have cutaneous origin from extremities , torso body wall and cervicofacial regions . retroperitoneal khe is very rare with no more than 30 cases reported in the english literature . from the boston series , approximately 12% of khe originated from retroperitoneum.2 in 2009 , we reported a retroperitoneal khe infiltrating the mesentery causing intestinal obstruction and kmp.8 in approximately 70% of khe , thrombocytopenia occurs as a result of platelet aggregation induced by turbulence blood flow within the distinctive endothelial architecture , leading to consumptive coagulopathy.9 it is not yet known whether kmp is associated with the site and size of khe . in general , khe with kmp is an aggressive lesion with poor prognosis.10 the platelet count will return to normal only after excision of the khe . platelet transfusion can cause painful enlargement of lesion and should be avoided without definitive treatment . differential diagnosis of pediatric pancreatic tumor includes pseudopapillary tumor , pancreatoblastoma , rhabdomyosarcoma , endocrine cell carcinoma , and vascular tumor . muller et al described 18 pancreatic tumors in a 22-year period , with 11 pancreaticoduodenectomy performed.11 vogel et al reported three pancreatic vascular tumors ( two infantile hemangiomas and one khe ) in a 10-year database of over 5,000 vascular anomalies.12 yeap et al reported three children between 1.5 and 8 years with pancreaticoduodenectomy performed over a 9-year period.13 our patient had whipple operation performed on day 7 of life with a body weight of less than 4 kg . to our knowledge , this is the youngest reported patient with successful whipple operation in the english literature . dunking anastomosis is performed , requiring mobilization of the distal pancreatic stump and its invagination into the jejunum . there are very few reports in the literatures describing the nutritional and endocrine status of children after pancreatic resections . two of them had increased hemoglobin a1c.14 from the french review of 18 pediatric pancreatic resections over a median follow - up period of 4.2 years , all patients had normal growth . there was no diabetes mellitus , but one patient had fat intolerance requiring pancreatic enzyme substitution.11 the postoperative plasma glucose level in our patient is normal . however , he developed indolent malabsorption while on normal infant formula with subsequent steatorrhea and failure to thrive . after implementation of hydrolyzed milk formula with increased medium chain triglyceride , the steatorrhea resolved and weight gain improved . we believe that long - term follow - up of this patient is essential to monitor his nutritional and endocrine status .
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 .