Hyperhomocysteinemia and Evan’s syndrome with uncal herniation for emergency splenectomy
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.