Although rupture of the spleen in the absence of previously diagnosed disease or trauma is widely described as rare, given the extensive reports in the literature documented here, we believe that this descriptor should no longer be used. Although its existence is debated
[1, 369, 400–402], sufficient reports from multiple authors are available to strongly suggest that rupture can occur spontaneously in otherwise normal spleens, but that this phenomenon is very rare. Given these two facts, the emergency clinician must be attuned to the possibility of splenic rupture in patients presenting with compatible symptoms without a compatible history. ED physicians must also be aware that such a presentation is very likely to be the manifesting episode of an underlying disease or anatomical abnormality. In the only other reference to these surprising findings, Renzulli found that the underlying cause for 51.2 % of the cases of atraumatic splenic rupture was not elicited until after hospital presentation
In 1958, Orloff and Peskin proposed four criteria to define a true spontaneous rupture of a spleen
, which emphasize that the spleen must appear grossly and histologically normal. In the same paper, they cite 71 reports documenting ruptures of the spleen labelled as spontaneous, only 20 of which fulfilled all of their criteria. Thus, usage of the term spontaneous was inconsistent and continues to be so in the more recent literature, with many authors labeling the rupture of diseased spleens as spontaneous. We highlight this because many of the pathological ruptures that we have documented here (as well as pathological ruptures in patients with previously known disease documented elsewhere
) include the word spontaneous in the title and no information on the associated pathology
[8, 61, 91, 98, 124, 151, 154, 355–357, 365, 400, 403]. Thus, readers skimming titles may be mistaken in thinking that true spontaneous rupture is more common than thought.
As we have shown here, documentation of rupture of the spleen following colonoscopy is relatively common with at least 87 cases reported (Table
2). However, we found only 1 such case reported in the emergency medicine literature
, and no reference to this association in emergency medicine textbooks
[2, 3] or electronic resources
. Although many occurrences of these cases should be evident to the endoscopist at the time of or shortly after the procedure, at least 8 documented cases have presented to the ED greater than 48 hours afterwards
[10, 11, 21–23]. We have therefore elected to include these and other post-procedure cases in this review. Rupture of the spleen after other procedures appears to be very rare.
For the cases presented here with pathology in addition to the splenic rupture, there is a plausible causative relationship between the other pathology and the rupture for the vast majority. However, we have also included cases with a less clear patho-physiological relationship, such as the case reported 3 years after a pancreato-renal transplant
, and that associated with viral hepatitis but no cirrhosis
. We acknowledge that the association in these cases may be coincidental and thus that these cases may better be classified as spontaneous. Although Wilson’s disease does not typically affect the spleen directly, the likely pathologic mechanism of the rupture in the case reported here is splenomegaly caused by portal hypertension
We found only one case of delayed rupture of a normal spleen following trivial trauma reported in the literature in the last 60 years
. One other report of such a rupture in an enlarged but otherwise normal spleen
, and reports of three others do not include information on the presence of splenic disease
[393–395]. One additional case has been reported in a man 14 days after a mild fall, but the patient had also just been given heparin for a presumed myocardial infarction
. Given the dearth of publication in this area, the possibility remains that the associations observed in these reports are coincidental rather than causational. Regardless of the causative mechanism, these cases still meet the inclusion criteria for this review.
The primary goal of this paper is to highlight the occurrence of splenic rupture in patients without risk factors apparent on history. A secondary purpose is to document the diverse nature of illnesses that can present in this manner. However, we have not attempted to obtain papers that were not available to us either electronically, on paper at our library or through inter-library loan. We also have not attempted to have non-English or non-French language abstracts or papers translated. The possibility remains therefore that we have missed some rare causes of splenic rupture. In addition, while a general estimate of the relative frequency of different causes of splenic rupture can be made from the numbers reported here, the numbers for those that are frequently reported such as colonoscopy, malaria and lymphoma are likely underestimated because of publication bias. Conversely, the relative frequencies of rupture for rare or novel causes are likely over-estimated.