Open AccessHyperhomocysteinemia and Evan′s syndrome with uncal herniation for emergency splenectomy
Author(s) -
Savitha Keelara Shivalingaiah,
J Arpana,
Mamta Jain,
Vineesh K. Varghese
Publication year - 2015
Publication title -
al-banǧ. maqālāt wa abḥāṯ fī al-taẖdīr wa-al-in’āš
Language(s) - English
Resource type - Journals
ISSN - 0259-1162
DOI - 10.4103/0259-1162.150186
Subject(s) - splenectomy , medicine , pancytopenia , hyperhomocysteinemia , hematoma , pediatrics , surgery , anesthesia , homocysteine , bone marrow , spleen
Hyperhomocysteinemia is a genetic disorder of metabolism and transport of amino acid, commonly present as a pro-coagulant state. Evan's syndrome is an autoimmune disorder with pancytopenia, a diagnosis of exclusion. The present report highlights the anesthetic management of a rare case, where both these clinical entities coexist. A 26-year-old male, a known case of hyperhomocyteinemia on medication for 4 years, came with a history of severe headache, blurring of vision and bleeding gums. Computerized tomography brain report showed subdural hematoma (SDH) of 16 mm with 9 mm right midline shift and on investigation had thrombocytopenia (5000 cells/cumm). Patient was diagnosed to have Evan's syndrome. Because he was refractory to the medical management, taken up for emergency splenectomy, followed by burr hole evacuation of SDH. Successful anesthetic management of the case is presented in this report.