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Int J Surg Case Rep
Int J Surg Case Rep
International Journal of Surgery Case Reports
2210-2612
Elsevier

S2210-2612(24)00960-X
10.1016/j.ijscr.2024.110179
110179
Case Report
Spontaneous rupture of a normal spleen: A case report and a review of the literature emphasizing diagnostic and surgical challenges
Ben Ismail Imen imen_bi@yahoo.fr
a⁎
Sghaier Marwen a
Zaafouri Elmontassar Belleh b
Rebii Saber c
Helal Imen d
Jouini Raja d
Zoghlami Ayoub c
a University of Tunis El Manar, Department of General Surgery, Trauma Center Ben Arous, Tunisia
b Department of General Surgery, Trauma and Burns Center, Ben Arous, Tunisia
c Department of General Surgery, Trauma and Burns Center, Ben Arous, University of Tunis El Manar, Tunisia
d University of Tunis El Manar, Department of Pathology, Habib Thameur Hospital, Tunisia
⁎ Corresponding author at: Department of General Surgery, Trauma Center, Ben Arous, Faculty of Medicine of Tunis, University of Tunis El Manar, Tunisia. imen_bi@yahoo.fr
15 8 2024
10 2024
15 8 2024
123 11017930 6 2024
12 8 2024
14 8 2024
© 2024 The Authors
2024
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction

Spontaneous splenic rupture (SSR) is a rare but potentially fatal condition. It is commonly linked to underlying conditions such as infections, neoplasms, or hematologic diseases. SSR can also occur in a healthy spleen without any associated pathology, termed idiopathic splenic rupture. Symptoms range from non-specific abdominal pain to hemodynamic instability, often requiring emergency splenectomy. Early recognition using CT is crucial for improving outcomes.

Case presentation

A 32-year-old male presented with severe abdominal pain for 24 h. Examination showed stable hemodynamics but tenderness in the left upper quadrant. CT revealed a subcapsular hematoma and moderate hemoperitoneum, leading to a diagnosis of SSR. Initially managed conservatively, the patient developed hemorrhagic shock 24 h later, with hemoglobin decreasing to 6.2 g/dL. An exploratory laparotomy confirmed a superior pole splenic fracture with significant hemoperitoneum, necessitating a total splenectomy. Postoperative recovery was uneventful, and the patient was discharged on postoperative day 6 with prophylactic vaccinations and lifelong penicillin.

Discussion

SSR in a normal spleen is extremely rare and poses significant diagnostic and therapeutic challenges. The exact mechanisms are unclear, with theories including vascular anomalies, microtrauma, increased splenic pressure, and idiopathic factors. SSR symptoms are often non-specific, leading to misdiagnosis. Timely diagnosis using imaging, particularly contrast-enhanced CT, is essential. Management varies from conservative approaches to splenectomy, based on hemodynamic stability and splenic damage.

Conclusion

Spontaneous rupture of a normal spleen is a critical condition requiring high clinical suspicion for timely diagnosis and management. Further research is needed to understand its pathophysiology and risk factors.

Highlights

• Spontaneous splenic rupture (SSR) can occur without underlying pathology or trauma.

• Diagnosis of SSR requires high clinical suspicion and prompt imaging with CT or ultrasound.

• SSR symptoms range from abdominal pain to hemodynamic instability, complicating diagnosis.

• Prophylactic measures post-splenectomy include vaccinations and lifelong antibiotics.

• Management of SSR ranges from conservative treatment to splenectomy.

Keywords

Spontaneous
Atraumatic
Splenic rupture, idiopathic, case report
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pmc1 Introduction

Spontaneous splenic rupture, also known as, “atraumatic splenic rupture”, is a rare but potentially life-threatening condition with an approximate mortality rate of 12 % [1]. It is often associated with underlying pathological conditions such as infectious, neoplastic, or hematologic diseases [2]. The use of anticoagulants is a major risk factor. In some cases, such as in patients undergoing hemodialysis, spontaneous splenic rupture may be attributed to uremic coagulopathy and heparin-induced coagulopathy [3]. It may develop less frequently in a normal spleen without being associated with any pathology and refers to “idiopathic splenic rupture” [4]. Symptoms can range from non-specific acute abdominal pain to hemodynamic instability, necessitating emergency surgical intervention for splenectomy. Early recognition through diagnostic tools like computed tomography and ultrasound is crucial for improving patient outcomes. Given the importance of timely diagnosis and appropriate surgical management in such cases, this article will emphasize the diagnostic challenges, imaging techniques, and surgical approaches necessary for managing spontaneous splenic rupture. This case report aims to provide insight into the clinical presentation, diagnostic process, and surgical management of a spontaneous rupture in a normal spleen. We will discuss the surgical techniques employed, including the challenges encountered during the procedure, and review the literature to highlight the best practices for managing this rare but critical condition. Clinicians should maintain a high index of suspicion for spontaneous splenic rupture, especially in cases involving hematologic disorders, to ensure timely management and better prognosis.

This work has been reported in line with the SCARE criteria [5].

2 Case presentation

A 32-year-old male, without past medical history, presented to the emergency department with a sudden onset of severe abdominal pain evolving for 24 h before admission. The patient denied any recent trauma, history of anticoagulant drug therapy, or any significant medical conditions such as liver disease, blood disorders, or infections. He also reported no history of recent heavy lifting, coughing, or other activities that could potentially lead to increased intra-abdominal pressure.

On physical examination, he was afebrile with stable hemodynamics (blood pressure of 110/70 mmHg and pulse rate of 80/min) but exhibited tenderness in the left upper quadrant of his abdomen.

Laboratory investigations revealed a hemoglobin level of 8.1 g/dL. Computed tomography of the abdomen demonstrated a subcapsular hematoma compressing the normal splenic parenchyma and moderate hemoperitoneum (Fig. 1). The diagnosis of spontaneous splenic rupture was made. As the patient was hemodynamically stable, he was managed conservatively with intravenous fluid infusion and analgesic.Fig. 1 Axial reconstruction of the abdominal CT scan with contrast showing a subcapsular hematoma (yellow arrow) in the spleen, moderate hemoperitoneum (white arrow) and splenic fracture (red arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Fig. 1

Twenty-four hours later, the patient showed symptoms of hemorrhagic shock, including low blood pressure and an accelerated heart rate and the repeat hemoglobin level dropped to 6.2 g/dL. A massive transfusion protocol was initiated and the patient was urgently rushed to the operating room to do an exploratory laparotomy.

A midline laparotomy was performed, providing adequate exposure to the abdominal cavity. Upon entering the peritoneum, approximately 1.5 l of blood was evacuated. Exploration revealed a superior pole fracture of the spleen with significant hemoperitoneum (Fig. 2). No other abdominal viscera were injured. Given the extent of the splenic injury, a total splenectomy was performed. Unfortunately, an image of the spleen post-resection was not captured. The surgery was performed by a senior general surgeon who specializes in trauma surgery, and works in a dedicated trauma center. The surgeon has extensive experience in managing complex abdominal injuries, including splenic trauma. Histopathological examination of the spleen revealed a non-pathological aspect, confirming the diagnosis of spontaneous splenic rupture (Fig. 3).Fig. 2 Intra-operative image showing splenic laceration.

Fig. 2

Fig. 3 Histopathological examination showing: (a) Subcapsular ruptured splenic hematoma (HEx20), (b) Normal splenic parenchyma surrounding the hematoma (HEx20).

Fig. 3

Postoperatively, the patient was managed in the intensive care unit (ICU) with close monitoring of vital signs, hemoglobin levels, and overall hemodynamic status. He was administered broad-spectrum antibiotics (ceftriaxone) for seven days to prevent infection, with an initial dose of 2 g daily, followed by prophylactic vaccinations against pneumococcal, meningococcal, and Haemophilus influenzae infections. Lifelong penicillin prophylaxis was initiated at discharge.

The patient had an uneventful recovery and was discharged on postoperative day 6 with instructions for follow-up care. Follow-up included clinical assessments and blood work at 1 week, 1 month, and 6 months post-surgery to monitor for any complications, including infection or thromboembolic events. The patient also received regular counseling on the importance of vaccinations and adherence to antibiotic prophylaxis. Imaging studies, including a follow-up abdominal CT scan performed 6 months post-surgery, showed no evidence of complications or abnormalities (Fig. 4).Fig. 4 Follow-up abdominal CT scan performed 6 months post-surgery, showing no evidence of collection in the splenic area.

Fig. 4

3 Discussion

Spontaneous rupture of a healthy spleen is an exceptionally rare clinical event. Unlike traumatic splenic rupture, which has clear precipitating factors such as physical injury [6], spontaneous rupture occurs without any apparent cause or underlying pathology. This phenomenon poses significant diagnostic and therapeutic challenges, often leading to delayed recognition and management. Its incidence is estimated to be less than 0.5 % of all cases of splenic rupture [7].

The entity of spontaneous splenic rupture has been recognized for a long time. It was first described by Rokitansky in 1861 [8].

The pathophysiological mechanisms behind spontaneous rupture of a healthy spleen remain poorly understood. Several theories have been proposed:• Vascular Anomalies: Congenital or acquired vascular abnormalities might predispose the spleen to spontaneous rupture. Microaneurysms or arteriovenous malformations could potentially weaken the splenic parenchyma.

• Microtrauma and daily Activities: Minor, often unrecognized, microtrauma from activities such as heavy lifting or coughing might contribute to splenic rupture in susceptible individuals.

• Increased splenic pressure: Situations leading to a sudden increase in intra-abdominal pressure, such as vomiting or strenuous physical exertion, could precipitate rupture in an otherwise healthy spleen.

• Idiopathic factors: In many cases, no clear etiology is identified, and these are classified as idiopathic spontaneous splenic ruptures.

Literature reports have highlighted various causes of spontaneous splenic rupture, including neoplastic, hematological, and inflammatory conditions. In his review Srihari et al. [9] demonstrated that a variety of bacterial, viral and parasitic agents have been reported to cause splenic enlargement and predispose to spontaneous rupture. The most involved infections are: Hepatitis, Bacterial endocarditis, Tuberculosis, Brucellosis, Syphilis, cytomegalovirus and especially Infectious mononucleosis caused by the Epstein-Barr virus and Malaria which are associated with a high mortality rate ranging from 9 to 13 % [10]. In a systematic literature review (1980–2008) [11] including 632 publications and reporting 845 patients, six major aetiological groups were defined: neoplastic (30·3 %), infectious (27·3 %), inflammatory, non-infectious (20·0 %), drug- and treatment-related (9·2 %) and mechanical (6·8 %) disorders, and normal spleen (6·4 %). The latter group was classified as “atraumatic–idiopathic splenic rupture”.

Symptoms of spontaneous splenic rupture may include abdominal pain, nausea, bloating, altered consciousness, and signs of peritonitis and hypovolemic shock in severe cases [12]. However, the absence of trauma history frequently leads to misdiagnosis or delayed diagnosis. Differential diagnoses might include gastrointestinal perforation, ectopic pregnancy, or aortic dissection, depending on the patient's demographics and clinical presentation.

Prompt diagnosis is the key to successful treatment. Imaging modalities are essential for diagnosing splenic rupture and related hemoperitoneum, and contrast-enhanced computed tomography (CT) scans is the modality of choice. Ultrasonography may also be useful, especially in hemodynamically unstable patients, due to its rapidity and accessibility [13].

Magnetic resonance imaging (MRI) has also been explored as a diagnostic tool for visceral injuries, including splenic rupture. While CT remains the gold standard for acute settings due to its speed and ability to detect active bleeding, MRI offers superior soft-tissue contrast and can provide additional information in cases where CT findings are inconclusive or when radiation exposure is a concern, such as in pregnant patients or repeated imaging studies [14]. MRI is particularly sensitive in detecting small amounts of intraparenchymal hemorrhage, subtle capsular tears, and other soft tissue injuries. However, its use in the acute setting is limited due to longer acquisition times, patient stability requirements, and less accessibility compared to CT [15]. Therefore, MRI is typically reserved for follow-up evaluations or specific clinical scenarios where its advanced tissue characterization capabilities are needed.

Management strategies for spontaneous splenic rupture range from conservative observation with close monitoring, bed rest, and serial imaging, to splenectomy going through embolization of the splenic artery [1]. The choice of the appropriate treatment option depends on the patient's hemodynamic stability and the extent of splenic damage.

Though there are no established guidelines for treating spontaneous splenic rupture, it is recommended to perform a total splenectomy when SSR is secondary to a neoplastic disease. In the setting of an infectious etiology conservative management is advised [11]. But we can clearly notice that splenectomy is the preferred strategy. In a Turkish series [16] including 12 patients, all patients but one underwent total splenectomy (91 %), these results are in line with those of Renzulli et al., were the reported rate of splenectomy was 84.1 % [11].

The prognosis of spontaneous splenic rupture varies based on the timeliness of diagnosis and the adequacy of treatment. Early recognition and appropriate management are critical to improving outcomes. Potential complications include re-bleeding, infection, and postsplenectomy sepsis. Patients who undergo splenectomy require long-term prophylactic measures, such as vaccinations and antibiotics, to mitigate the risk of overwhelming postsplenectomy infection (OPSI).

4 Conclusion

Spontaneous rupture of a healthy spleen, though rare, is a critical condition that requires high clinical suspicion for timely diagnosis and management. The exact pathophysiological mechanisms remain elusive, necessitating further research to understand the underlying causes and risk factors. Clinicians should remain vigilant and consider this diagnosis in patients presenting with unexplained acute abdominal pain and hemodynamic instability.

Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Ethical approval

All procedures performed in studies involving human participants were by the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Ethical clearance was not necessary as the format of this paper is a case report.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author contribution

Manuscript writing: Dr. Imen Ben Ismail.

Study concepts: Dr. Imen Ben Ismail and Dr. Marwen Sghaier.

Helped in data interpretation and manuscript evaluation: Dr. Saber Rebii.

Data acquisition: Dr. Elmontasser Bellah Zaafouri.

Critical revision: Dr. Ayoub Zoghlami.

Guarantor

Dr. Imen Ben Ismail.

Conflict of interest statement

Authors declare no conflict of interest.
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