RESUMO
Splenomegaly is one of the complications of sickle cell disease (SCD) occurring in early childhood. This risk is reduced by the age of five years as the spleen undergoes auto splenectomy as a consequence of recurrent vasooclusion and splenic infarction. However, in some variants of SCD, the persistence of the spleen occurs. This can be complicated rarely by the formation of a splenic pseudocyst. We report a 17-year-old teenager with sickle cell anemia who presented with an 8-year history of gradually increasing left-sided abdominal swelling and a 2-month history of recurrent left upper abdominal pain. A computerized tomography scan revealed splenomegaly and multiple splenic cysts, not responsive to opioid analgesics which necessitated a total splenectomy. The histology report found an absence of epithelial lining confirming splenic pseudocysts. SCD patients with splenomegaly have underlying splenic infarction, which is a predisposition to splenic pseudocyst formation, though a rare occurrence. Many patients with splenic pseudocyst are usually asymptomatic and do not need intervention. However, the index patient developed serious symptoms indicating a need for a total splenectomy. A splenic pseudocyst is rare and found in <1% of splenectomies.
