Neonatal Biliary Obstruction Caused by Pancreatic Kaposi Hemangioendothelioma
Kaposiform hemangioendothelioma (KHE), is a rare benign locally invasive proliferation of blood vessels that forms a mass. Besides the mass effect of KHE, it infiltrates adjacent tissues, it can be associated with severe thrombocytopenia, consumptive coagulopathy with high mortality rate. The manife...
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| Format: | Article |
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Cairo University, Faculty of Medicine, Department of Pediatrics
2022-07-01
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| Series: | Pediatric Sciences Journal |
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| Online Access: | https://cupsj.journals.ekb.eg/article_247042_9dbdc4859f3bb418740e4029a69a0a81.pdf |
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| author | Magd Ahmed Kotb Sherif Kaddah Sally TM El Sorogy Reham Osama Naglaa El Kinaaiee Sayed Khedr Mostafa Gad |
| author_facet | Magd Ahmed Kotb Sherif Kaddah Sally TM El Sorogy Reham Osama Naglaa El Kinaaiee Sayed Khedr Mostafa Gad |
| author_sort | Magd Ahmed Kotb |
| collection | DOAJ |
| description | Kaposiform hemangioendothelioma (KHE), is a rare benign locally invasive proliferation of blood vessels that forms a mass. Besides the mass effect of KHE, it infiltrates adjacent tissues, it can be associated with severe thrombocytopenia, consumptive coagulopathy with high mortality rate. The manifestations of KHE are variable and range from cutaneous lesions with wide varieties of appearances to deep masses without cutaneous signs. We report a case of 3 month old male who presented as extrahepatic biliary atresia in the form of jaundice, hepatomegaly, clay colored stool and elevated liver enzymes. Dynamic Magnetic Resonance Imaging revealed pancreatic head solid mass. Operative findings and biopsy of the highly vascular lesion confirmed that it was KHE of head of pancreas. The mass was excised and hepato-portoenterostomy was successfully performed. One year later the boy is disease free with no organomegaly or any other complication. KHE of head of pancreas is a very rare cause of extrahepatic biliary obstruction early in life. It is amenable to successful surgical intervention. |
| first_indexed | 2024-03-13T00:01:19Z |
| format | Article |
| id | doaj.art-c2c5465515e34d2181bfa157bc0a8fa5 |
| institution | Directory Open Access Journal |
| issn | 2805-279X 2682-3985 |
| language | English |
| last_indexed | 2024-03-13T00:01:19Z |
| publishDate | 2022-07-01 |
| publisher | Cairo University, Faculty of Medicine, Department of Pediatrics |
| record_format | Article |
| series | Pediatric Sciences Journal |
| spelling | doaj.art-c2c5465515e34d2181bfa157bc0a8fa52023-07-13T10:37:28ZengCairo University, Faculty of Medicine, Department of PediatricsPediatric Sciences Journal2805-279X2682-39852022-07-012221021510.21608/cupsj.2022.141453.1056Neonatal Biliary Obstruction Caused by Pancreatic Kaposi HemangioendotheliomaMagd Ahmed Kotb0https://orcid.org/0000-0003-2118-3793Sherif Kaddah1Sally TM El Sorogy2https://orcid.org/0000-0003-0666-0408Reham Osama3https://orcid.org/0000-0001-9192-4089Naglaa El Kinaaiee4https://orcid.org/0000-0002-3752-3708Sayed Khedr5Mostafa Gad6Department of Pediatrics, Faculty of Medicine, Cairo University, EgyptDepartment of Pediatric Surgery, Faculty of Medicine, Cairo University, EgyptDepartment of Pediatrics, Public Mounira Hospital, Cairo, EgyptDepartment of Radiodiagnosis, Faculty of Medicine, Cairo University, EgyptDepartment of Pathology, Cancer Institute, Cairo University, EgyptDepartment of Pediatric Surgery, Faculty of Medicine, Cairo University, EgyptDepartment of Pediatric Surgery, Faculty of Medicine, Cairo University, EgyptKaposiform hemangioendothelioma (KHE), is a rare benign locally invasive proliferation of blood vessels that forms a mass. Besides the mass effect of KHE, it infiltrates adjacent tissues, it can be associated with severe thrombocytopenia, consumptive coagulopathy with high mortality rate. The manifestations of KHE are variable and range from cutaneous lesions with wide varieties of appearances to deep masses without cutaneous signs. We report a case of 3 month old male who presented as extrahepatic biliary atresia in the form of jaundice, hepatomegaly, clay colored stool and elevated liver enzymes. Dynamic Magnetic Resonance Imaging revealed pancreatic head solid mass. Operative findings and biopsy of the highly vascular lesion confirmed that it was KHE of head of pancreas. The mass was excised and hepato-portoenterostomy was successfully performed. One year later the boy is disease free with no organomegaly or any other complication. KHE of head of pancreas is a very rare cause of extrahepatic biliary obstruction early in life. It is amenable to successful surgical intervention.https://cupsj.journals.ekb.eg/article_247042_9dbdc4859f3bb418740e4029a69a0a81.pdfpancreatic kaposiform hemangioendotheliomaextrahepatic biliary obstructionhepato-portoenterostomyneonatal |
| spellingShingle | Magd Ahmed Kotb Sherif Kaddah Sally TM El Sorogy Reham Osama Naglaa El Kinaaiee Sayed Khedr Mostafa Gad Neonatal Biliary Obstruction Caused by Pancreatic Kaposi Hemangioendothelioma Pediatric Sciences Journal pancreatic kaposiform hemangioendothelioma extrahepatic biliary obstruction hepato-portoenterostomy neonatal |
| title | Neonatal Biliary Obstruction Caused by Pancreatic Kaposi Hemangioendothelioma |
| title_full | Neonatal Biliary Obstruction Caused by Pancreatic Kaposi Hemangioendothelioma |
| title_fullStr | Neonatal Biliary Obstruction Caused by Pancreatic Kaposi Hemangioendothelioma |
| title_full_unstemmed | Neonatal Biliary Obstruction Caused by Pancreatic Kaposi Hemangioendothelioma |
| title_short | Neonatal Biliary Obstruction Caused by Pancreatic Kaposi Hemangioendothelioma |
| title_sort | neonatal biliary obstruction caused by pancreatic kaposi hemangioendothelioma |
| topic | pancreatic kaposiform hemangioendothelioma extrahepatic biliary obstruction hepato-portoenterostomy neonatal |
| url | https://cupsj.journals.ekb.eg/article_247042_9dbdc4859f3bb418740e4029a69a0a81.pdf |
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