Kasabach-Merritt Syndrome Associated With a Large Cavernous Splenic Hemangioma Treated With Splenectomy: A Surgeon's Introspection of an Uncommon, Little Read, and Yet Complex Problem-Review Article.

Cavernous hemangiomas of the spleen are small benign lesions found incidentally, majority of times while patients are being investigated for some other disease and patients remain asymptomatic otherwise for this condition. The natural history of cavernous hemangiomas of spleen is slow, symptoms or complications, when present, occur late, they are rarely large and can manifest as a palpable non-tender mass in the left upper quadrant. A very rare syndrome is associated with this condition called Kasabach-Merritt syndrome (KMS), which is defined as diffuse cavernous hemangioma of the spleen alongwith anemia, thrombocytopenia, and coagulopathy. Perioperative diagnosis can be confirmed by imaging study which can be CT, MRI, or ultrasound. Splenectomy is considered the treatment of choice for such patients with symptoms. To our knowledge, a very few cases have been reported so far. The purpose of writing this review article is the reporting of this rare case and to provide some experience related to the management of this condition in a patient with KMS.