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Introduction: Spontaneous rupture of the spleen (SSR) is a rare but potentially fatal emergency, typically linked to trauma, infections, or hematological conditions. Its occurrence as an initial manifestation of the vascular form of Ehlers-Danlos syndrome (vEDS) is particularly uncommon and presents significant diagnostic and therapeutic challenges. Given the life-threatening nature of SSR and the complexities introduced by vEDS, early recognition is crucial.

Methods: We report the case of a 34-year-old previously healthy female who presented with acute severe pain in the left upper quadrant and left shoulder tip, accompanied by profound hypotension and clinical features of hypovolaemic shock. Diagnostic imaging via computed tomography revealed a large subcapsular splenic hematoma with signs of active extraperitoneal bleeding. An emergency splenectomy was performed.

Results: Postoperatively, the patient exhibited delayed wound healing, hyperextensible skin, and generalized joint laxity—clinical signs that raised suspicion of a connective tissue disorder. Subsequent genetic testing confirmed the presence of a pathogenic mutation in the COL3A1 gene, establishing the diagnosis of vascular Ehlers-Danlos syndrome (vEDS).

Conclusion: This case underscores the importance of considering rare connective tissue disorders like vEDS in the differential diagnosis of atraumatic SSR. Early diagnosis can influence emergency surgical decisionmaking and ensures timely genetic counseling, ongoing multidisciplinary management, and proactive family risk assessment.