AIM: A small but significant percentage of vascular tumors may develop at extracutaneous location. They are difficult to detect on the physical exam and usually they require immediate intervention. Pediatric surgeons must have acknowledge of its prognostic and therapeutic implications. PATIENTS AND RESULTS: We report 4 of these patients. Patient 1 was a healthy newborn who presented in the second week of life, recurrent severe gastrointestinal bleeding, thrombocytopenia and anemia. Diagnosis of multifocal linfangioendoteliomatosis with thrombocytopenia was established. Patient 2 had prenatal diagnosis of ascites and presented at birth sepsis, anemia, thrombocytopenia and hypoproteinemia. Upon laparotomy hemorrhagic ascites and thickening of rectum-sigmoid wall and mesentery were found. Pathologic diagnosis was Kaposiform hemangioendothelioma and the clinical course was consistent with Kassabach-Merrit phenomenon. Patient 3 had at birth, multifocal hepatic GLUT1- hemangiomatosis with severe cardiac insufficiency and coagulopathy. She died while waiting for a liver transplantation. Patient 4 is a girl who presented in the newborn period with vomiting and hematochezia. She required several transfusions and endoscopic biopsies showed a vascular tumor that infiltrated duodenum, jejunum and mesentery. Imaging studies and histologic findings on biopsy led to the diagnostic of juvenile hemangioma GLUT-1+. CONCLUSIONS: Vascular tumors of the digestive tract may be difficult to diagnosis and their classification is still incomplete. Pediatric surgeons must be acquainted with these varieties of tumors because they are always involved in diagnosis and therapeutic decision making.
AIM: A small but significant percentage of vascular tumors may develop at extracutaneous location. They are difficult to detect on the physical exam and usually they require immediate intervention. Pediatric surgeons must have acknowledge of its prognostic and therapeutic implications. PATIENTS AND RESULTS: We report 4 of these patients. Patient 1 was a healthy newborn who presented in the second week of life, recurrent severe gastrointestinal bleeding, thrombocytopenia and anemia. Diagnosis of multifocal linfangioendoteliomatosis with thrombocytopenia was established. Patient 2 had prenatal diagnosis of ascites and presented at birth sepsis, anemia, thrombocytopenia and hypoproteinemia. Upon laparotomy hemorrhagic ascites and thickening of rectum-sigmoid wall and mesentery were found. Pathologic diagnosis was Kaposiform hemangioendothelioma and the clinical course was consistent with Kassabach-Merrit phenomenon. Patient 3 had at birth, multifocal hepatic GLUT1- hemangiomatosis with severe cardiac insufficiency and coagulopathy. She died while waiting for a liver transplantation. Patient 4 is a girl who presented in the newborn period with vomiting and hematochezia. She required several transfusions and endoscopic biopsies showed a vascular tumor that infiltrated duodenum, jejunum and mesentery. Imaging studies and histologic findings on biopsy led to the diagnostic of juvenile hemangiomaGLUT-1+. CONCLUSIONS:Vascular tumors of the digestive tract may be difficult to diagnosis and their classification is still incomplete. Pediatric surgeons must be acquainted with these varieties of tumors because they are always involved in diagnosis and therapeutic decision making.
Authors: Isabela Picolotto Moraes; Oona Tomiê Daronch; Camila Girardi Fachin; Luiz Paulo Junqueira Rigolon; André Ivan Bradley Dos Santos Dias Journal: Int J Surg Case Rep Date: 2020-10-02