Unraveling a rare splenic pathology: a case report of a benign hemorrhagic spleen with primary congenital splenic epidermoid cysts

Abstract

Introduction and importance: A primary congenital splenic epidermoid cyst is an immensely rare pathology with mostly unknown epidemiological parameters. Misdiagnosis can easily happen and this results in life-threatening ramifications for patients. Considering this pathology as a potential differential diagnosis allows for the required surgical intervention to be timely accomplished. In this case, the authors are documenting this pathology and presenting how it was successfully managed via proper and informed preoperative analysis and meticulous intraoperative technique.

Case presentation: Hereby, we portray the exceptionally rare case of a 7-year-old male who presented to our surgical clinic complaining of a sudden left hypochondriac pain with early satiety for 1 month's duration. The preoperative radiological assessment displayed numerous splenic cystic lesions throughout the splenic parenchyma.

Clinical discussion: Resection of the cysts was accomplished via total splenectomy. The ensuing histopathological analysis via Hematoxylin and Eosin of the resected specimens established the diagnosis of a congested hemorrhagic spleen with multiple primary congenital splenic epidermoid cysts.

Conclusion: Primary congenital splenic epidermoid cysts are an extremely rare type of splenic pathology. There is profound scarcity amidst the published literature regarding it. This merits in-depth study and apt documentation to raise awareness regarding this pathology as a potential differential diagnosis in cases of abdominal pain. Documentation allows us to set up proper and innovative clinical and surgical protocols for these patients. Based on our conclusive review of the published literature, the authors conclude that ours is the first ever documented case from our country of a primary congenital splenic epidermoid cyst.

Keywords: abdominal surgery; case report; epidermoid cyst; primary congenital splenic epidermoid cyst; splenic cyst; total splenectomy.

Figure 1

(A, B): MSCT cross-sectional images of the abdomen and pelvis. Findings include splenomegaly where the spleen measured around (12 cm) with multiple splenic lesions within its parenchyma without calcifications (Identified in both images by the Red Arrows). The largest of said lesions measured approximately (2.5 cm).

Figure 2

A: Microscopic image of the histopathological analysis of the resected specimens. Via H&E staining, we can demonstrate the spleen’s native histological tissue characterized by the white pulp. B: Microscopic image of the histopathological analysis of the resected specimens. Via H&E staining, we can see the splenic histological structure while depicting the cyst’s epithelial lining identified by the Blue Arrow. C: Microscopic image of the histopathological analysis of the resected specimens. Via H&E staining, we can clearly visualize the cystic lumen. D: Microscopic image of the histopathological analysis of the resected specimens. Via H&E staining, we can clearly visualize the cystic lumen along with the thin squamous epithelial lining identified by the Blue Arrow.