Abstract
BackgroundRupture of the spleen in the absence of trauma or previously diagnosed disease is largely ignored in the emergency literature and is often not documented as such in journals from other fields. We have conducted a systematic review of the literature to highlight the surprisingly frequent occurrence of this phenomenon and to document the diversity of diseases that can present in this fashion.MethodsSystematic review of English and French language publications catalogued in Pubmed, Embase and CINAHL between 1950 and 2011.ResultsWe found 613 cases of splenic rupture meeting the criteria above, 327 of which occurred as the presenting complaint of an underlying disease and 112 of which occurred following a medical procedure. Rupture appeared to occur spontaneously in histologically normal (but not necessarily normal size) spleens in 35 cases and after minor trauma in 23 cases. Medications were implicated in 47 cases, a splenic or adjacent anatomical abnormality in 31 cases and pregnancy or its complications in 38 cases.The most common associated diseases were infectious (n = 143), haematologic (n = 84) and non-haematologic neoplasms (n = 48). Amyloidosis (n = 24), internal trauma such as cough or vomiting (n = 17) and rheumatologic diseases (n = 10) are less frequently reported. Colonoscopy (n = 87) was the procedure reported most frequently as a cause of rupture. The anatomic abnormalities associated with rupture include splenic cysts (n = 6), infarction (n = 6) and hamartomata (n = 5). Medications associated with rupture include anticoagulants (n = 21), thrombolytics (n = 13) and recombinant G-CSF (n = 10). Other causes or associations reported very infrequently include other endoscopy, pulmonary, cardiac or abdominal surgery, hysterectomy, peliosis, empyema, remote pancreato-renal transplant, thrombosed splenic vein, hemangiomata, pancreatic pseudocysts, splenic artery aneurysm, cholesterol embolism, splenic granuloma, congenital diaphragmatic hernia, rib exostosis, pancreatitis, Gaucher's disease, Wilson's disease, pheochromocytoma, afibrinogenemia and ruptured ectopic pregnancy.ConclusionsEmergency physicians should be attuned to the fact that rupture of the spleen can occur in the absence of major trauma or previously diagnosed splenic disease. The occurrence of such a rupture is likely to be the manifesting complaint of an underlying disease. Furthermore, colonoscopy should be more widely documented as a cause of splenic rupture.
Highlights
Rupture of the spleen in the absence of trauma or previously diagnosed disease is largely ignored in the emergency literature and is often not documented as such in journals from other fields
We conducted a systematic review of English and French language papers indexed in CINAHL, PubMed and Embase using the medical subject heading (MeSH) search terms “rupture, spontaneous,” and “splenic rupture,” combined with the textword search “undiagnosed” or “first manifestation” or “presenting” or “spontaneous.” This search strategy was combined with an additional strategy including the MeSH terms “rupture, spontaneous” and “spleen” and the free text “normal spleen;” both strategies were used together to extract relevant papers
The relative frequencies of rupture for rare or novel causes are likely over-estimated. Both traumatic and pathological rupture of the spleen are frequently reported in journals and documented in textbooks of emergency medicine
Summary
Rupture of the spleen in the absence of trauma or previously diagnosed disease is largely ignored in the emergency literature and is often not documented as such in journals from other fields. Cases of splenic rupture not fitting the description above are related by their lack of historical cues to suggest the diagnosis at presentation. This distinguishes them from other causes of splenic rupture and highlights the importance to emergency physicians who rely a great deal on the patient history to appropriately triage patients for definitive investigation and referral. We have reviewed the literature on cases of splenic rupture for which there was not an immediately obvious cause apparent on presentation such as significant trauma (either recent or remote) or previously diagnosed disease known to affect the spleen
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