Atraumatic splenic rupture: a case report
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Key findings
• The rare finding of atraumatic splenic rupture (ASR).
What is known and what is new?
• Atraumatic spontaneous splenic rupture is often low on the differential diagnoses due to its rarity. It carries a high risk of morbidity and mortality, and thus timely diagnosis and intervention is imperative.
What is the implication, and what should change now?
• This case highlights the importance of early diagnosis and treatment of ASR, as delayed diagnosis and treatment is associated with significant morbidity and mortality.
Introduction
Atraumatic splenic rupture (ASR) is a rare yet potentially life-threatening condition that often presents with nonspecific and insidious symptoms, posing a challenge to diagnosis (1-10). ASR has been reported to present with abdominal pain with radiation to the back, thoracic spine visceral referral pain, and hemodynamic instability. ASR has a wide array of causes with a nonspecific insidious onset of symptoms which significantly elevates the morbidity and mortality of this diagnosis. The etiology of an ASR can be pathologic involving a diseased spleen, or non-pathologic (true spontaneous rupture), with non-pathologic being less common (1-3). In this article we present a case involving a true spontaneous splenic rupture. The presented patient had no previous abdominal traumas and no known splenic pathologies leading to the rupture. This case highlights the importance of early diagnosis and treatment of ASR, as delayed diagnosis and treatment is associated with significant morbidity and mortality. Given its rare nature, further research and awareness are warranted to enhance understanding and management of this condition. We present this case in accordance with the CARE reporting checklist (available at https://acr.amegroups.com/article/view/10.21037/acr-24-64/rc).
Case presentation
We present the case of a 73-year-old female with past medical history significant for hypothyroidism, hypertension, squamous cell carcinoma of the chest, and history of tobacco use who presented to the emergency department with abruptly worsening left-sided flank pain that radiated to her shoulder. Her blood pressure on admission was 138/78 mmHg. Labs revealed a hemoglobin of 14.1 g/dL, hematocrit 39.9%, platelets 302,000 per mcL, white blood count 7.3×109/L. Computed tomography (CT) of the abdomen and pelvis without contrast was ordered by the emergency department clinicians revealing an acute splenic injury concerning for rupture with contour obscured by blood products with associated mild to moderate hemoperitoneum (Figure 1). The surgical team was then consulted for these findings. Upon further evaluation the patient stated that the pain originally began with less intensity 3 days prior. She endorsed nausea associated with progressively worsening malaise throughout this time. She denied recent trauma. Her systolic blood pressure was noted to rapidly deteriorate to 72/42 mmHg with associated somnolence. Labs revealed decrease of hemoglobin to 10.7 g/dL, hematocrit 31.5%, platelets 137,000 per mcL and white blood count increased to 16.2×109/L. Furthermore, labs revealed monospot negative. She received two liters of crystalloid intravenous fluids in addition to two units of packed red blood cells (RBCs) without significant improvement. The decision was made to proceed with emergent exploratory laparotomy and splenectomy due to her clinical instability. This pathway was chosen rather than nonoperative management with endovascular embolization due to lack of resource availably at the presenting facility. Intraoperatively, there was two liters of hemoperitoneum found on exploration and the spleen was noted to have serosal damage. Surgical pathology demonstrated spleen weighing 59 grams, rupture of spleen, unremarkable splenic parenchymal tissue, negative for atypical proliferation. Patient received her post splenectomy vaccinations. On follow-up appointment, patient was noted to be doing well.
All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent for publication of this case report and accompanying images was not obtained from the patient or the relatives after all possible attempts were made.
Discussion
Spontaneous splenic rupture, a condition initially described in 1874 by English surgeon Atkinson, has remained an infrequent, but significant presentation of acute abdominal pathology throughout medical literature. Despite subsequent case reports, it continues to be a rare occurrence in clinical practice (1). The classification of spontaneous splenic rupture is divided into two categories: pathological, involving a diseased spleen, and spontaneous, involving a healthy spleen (2). Spontaneous splenic rupture is usually associated with hematologic, neoplastic, or infectious disorders, with infectious mononucleosis associated with Epstein-Barr virus being the most common cause worldwide. Spontaneous splenic rupture without underlying cause is thought to be exceedingly rare (3).
In this context, a thorough literature review has established that the presentation of ASR can vary among age groups and in symptomatology. However, the most common clinical features include epigastric or left upper quadrant pain, tachycardia, and a progressive transition to shock. These clinical signs serve as important indicators for early recognition of the condition (4).
Orloff and Peskin’s seminal 1958 study defined four specific criteria for diagnosing spontaneous splenic rupture. Subsequent research introduced a fifth criterion to create precise criteria for the diagnosis of non-pathological spontaneous splenic rupture. These criteria emphasize several essential factors, including the absence of trauma or unusual movements that could disrupt the spleen, no evidence of diseases that could affect the spleen, macroscopic and microscopic examination confirming normal spleen except for hemorrhage and rupture, the absence of adhesions or scarring suggestive of prior trauma (5), and a comprehensive virological study showing no significant rise in antibody titers indicating recent viral infections associated with splenic involvement (6). According to Ahbala et al., it is thought that the underlying cause of spontaneous rupture may be secondary to increase in intrasplenic tension leading to hyperplasia and enlargement, compression secondary to increases in intraabdominal pressure, or occlusion of vasculature secondary to endothelia hyperplasia leading to infarction (7).
In our presented case, the patient fits these criteria, strongly suggesting a true atraumatic spontaneous rupture of the spleen. No identifiable pathologic causes were found in the patient’s history, reinforcing the notion that this is a non-pathological, spontaneous event. It is worth noting that the differential diagnosis of the acute abdomen rarely includes ASR due to its infrequency, but it should not be dismissed entirely. ASR can lead to serious complications if not detected early. Therefore, maintaining awareness of this condition is vital for timely intervention.
In contemporary medical practice, the diagnosis of ASR is primarily achieved through advanced imaging techniques, with CT imaging being the mainstay. This diagnostic tool provides invaluable insights into splenic injuries and ruptures, aiding in assessing the extent of hemorrhage and guiding appropriate management. In our case, a CT scan played a pivotal role, revealing an acute splenic injury/rupture accompanied by a mild to moderate amount of hemoperitoneum. Ultrasound has also been noted to play a role in diagnosis (4). Patients may also present with thoracic spine pain caused by visceral referral pain (8).
Traditionally, splenic injuries, regardless of severity, were predominantly managed surgically through splenectomy. This approach, while effective in controlling hemorrhage, leads to a permanent state of asplenia with associated complications, such as susceptibility to infections and other long-term health issues. Splenic artery embolization (SAE) has emerged as a valuable tool in the management of ASR, although its role is less well-defined compared to traumatic splenic injuries. SAE is a promising and effective treatment modality for ASR, offering a minimally invasive option that preserves splenic function while controlling hemorrhage. As evidence grows, SAE may become an integral part of the management strategy for ASR, similar to its established role in traumatic splenic injuries. Continued research and clinical studies are essential to refine its application and optimize patient outcomes. This includes identifying the underlying conditions that may predispose patients to this type of rupture, determining the appropriate timing for intervention, establishing criteria for patient selection, and selecting the most effective embolization techniques (9).
Operative intervention including splenectomy remains the mainstay of treatment, in addition to appropriate resuscitation. Some authors have reported cases in which hemodynamically stable patients are able to be treated non operatively, however, the long-term consequences are unknown (10).
Conclusions
In conclusion, ASR is an uncommon yet potentially life-threatening condition that often presents with nonspecific and insidious symptoms, posing a challenge to diagnosis. The etiology of ASR can be categorized as pathologic, involving a diseased spleen, or non-pathologic, as seen in true spontaneous rupture. This case highlights the importance of recognizing and diagnosing atraumatic spontaneous splenic rupture, as early identification and intervention can be lifesaving. Given its rarity, further research and awareness are warranted to enhance understanding and management of this condition.
Acknowledgments
Funding: None.
Footnote
Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://acr.amegroups.com/article/view/10.21037/acr-24-64/rc
Peer Review File: Available at https://acr.amegroups.com/article/view/10.21037/acr-24-64/prf
Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-24-64/coif). A.I.G. serves as an unpaid editorial board member of AME Case Reports from February 2023 to January 2025. The other authors have no conflicts of interest to declare.
Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent for publication of this case report and accompanying images was not obtained from the patient or the relatives after all possible attempts were made.
Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.
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Cite this article as: Santarelli J, Alattar R, Elmore LR, Gritsiuta AI, Henwood JR. Atraumatic splenic rupture: a case report. AME Case Rep 2024;8:94.