Rare Indications of Splenectomy: A Case Series and Literature Review

Splenectomy is a procedure, most commonly performed for trauma, hematological disorders, and certain malignancies. Rare indications such as congenital bleeding disorders, pancreatitis complications, and splenic cysts may also warrant splenic removal, especially in life-threatening conditions or diagnostically unclear scenarios. In this retrospective descriptive case series, we report cases of six patients who underwent splenectomy for rare indications. The first case involves a 13-year-old boy with congenital afibrinogenemia who presented with signs of acute abdomen and class 3 hypovolemic shock. Computed tomography (CT) imaging of the abdomen and pelvis revealed a large perisplenic hematoma and hemoperitoneum suggestive of spontaneous hemorrhage. Despite fibrinogen replacement therapy, the patient remained hemodynamically unstable, and emergency open splenectomy was performed, which proved to be life-saving. The second and third cases involved middle-aged men with chronic alcoholic pancreatitis who developed large intrasplenic pseudocysts with spontaneous rupture, evidenced by contrast extravasation on CT. Both underwent emergency splenectomy with distal pancreatectomy. Histopathology revealed pseudocysts with no evidence of malignancy. These cases confirm how the anatomical proximity between the pancreatic tail and spleen can facilitate enzymatic injury leading to splenic rupture, an infrequent but dangerous complication of chronic pancreatitis. The remaining two cases were two young female patients who presented with large splenic cysts, and one of them had undergone prior percutaneous catheter drainage and developed infection. Both underwent elective splenectomy due to persistent symptoms and diagnostic uncertainty. Histopathology revealed a benign epithelial cyst in one and a splenic abscess in the other, illustrating the importance of definitive surgical management in selected cystic lesions of the spleen. The final and most aggressive case involved a 44-year-old woman who presented in hemorrhagic shock. Imaging revealed a ruptured splenic mass with multiple lesions in the liver. Emergency splenectomy was performed, and histopathology confirmed primary angiosarcoma of the spleen. Despite surgical intervention, the patient succumbed postoperatively, underscoring the dismal prognosis associated with this rare vascular malignancy. Each case highlights the diagnostic and therapeutic role of splenectomy in atypical scenarios. Awareness of such rare indications can aid timely surgical decision-making when imaging or conservative strategies are inconclusive or ineffective. Though uncommon, conditions such as congenital bleeding disorders, pancreatitis-related complications, and primary benign splenic cyst must be considered in the differential diagnosis of splenic pathology. Splenectomy remains a definitive solution in selected cases.