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

Abstract

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.

Keywords: Deranged coagulation profile; Kasabach–Merritt syndrome; Splenectomy; Splenic hemangioma.

Fig. 1

Upper GI endoscopy showing extrinsic gastric fundal compression. Notice the normal overlying mucosa

Fig. 2

Contrast enhanced CT scan revealed grossly enlarged spleen with mass effect on adjacent organs (as shown by red and orange arrows). Internal architecture normal with prominent sinusoids showing slow filling of contrast as indicated by blue arrow

Fig. 3

Intraoperative image showing the grossly enlarged spleen and dilated splenic vein as indicated by the blue and pink arrows, respectively

Fig. 4

Large cavernous blood filled spaces (black arrow) with intervening splenic red pulp (green arrow). Hematoxylin & Eosin, 100×