Splenic rupture is most commonly caused by abdominal trauma and both immediate and delayed ruptures after traumatic events have been described in the literature [1] [2]. The exact pathogenesis of a delayed rupture is not known, with expanding subcapsular hematomas, clotting events, and rupture of pseudocysts/pseudoaneurysms being the main theories [3]. On the other hand, non-traumatic splenic rupture (NSR) is rare, but can be seen in a range of disorders [1] [2] [4] [5] [6]:

• Infections - Infectious mononucleosis (caused by Epstein-Barr virus - EBV) and malaria are two important diseases in which splenic rupture is a well-known complication [1] [2] [5].
• Surgery-induced - Colonoscopy, emergency laparoscopy, and various types of abdominal surgery [2] [4].
• Iatrogenic - Use of thrombolytic drugs, anticoagulants and recombinant granulocyte-colony stimulating factor (G-CSF) [2].

In addition, several tumors (angiosarcomas, hematologic malignancies), pregnancy-related events, and intrinsic splenic disease (infarction, development of pseudocysts, etc.) have been mentioned as possible etiologies [2]. The clinical presentation may start abruptly or approximately 4-8 days after the injury in the case of delayed rupture [3] [7]. Upper left quadrant abdominal pain accompanied by generalized distension and tenderness is the main symptom, followed by tachycardia, hypotension, oliguria, pallor, and hypovolemic shock [1] [3] [5] [6]. Splenic rupture is particularly dangerous in neonates when bleeding and severe hemoperitoneum follows abdominal distention [8]. When trauma is the reason for splenic rupture, other injuries may be observed, such as lower rib fractures overlying the spleen (seen in up to 40% of cases) [3].

• Hemophilia A

  Successful medical management of a neonate with spontaneous splenic rupture and severe hemophilia A. [scholars.northwestern.edu]

  He was subsequently diagnosed with severe hemophilia A, and was managed medically with recombinant factor VIII replacement therapy without any surgical intervention. [ncbi.nlm.nih.gov]

• Splenomegaly

  Splenomegaly is defined as cranio-caudal length greater than 13 cm by ultrasound [ 7 ]. [omicsonline.org]

  CONCLUSION: This is the second case of spontaneous splenic rupture without preceding splenomegaly in a patient with APL during induction chemotherapy described so far. [ncbi.nlm.nih.gov]

  Abdominal examination revealed tender palpable splenomegaly, without guarding. [em-consulte.com]

• Infectious Mononucleosis

  Evidence summary The annual incidence of infectious mononucleosis is somewhere between 345 and 671 cases per 100,000 in the US; it is highest in the adolescent age group. 1 Splenic rupture is the leading cause of death in infectious mononucleosis, occurring [mdedge.com]

  Abbreviation Yale J Biol Med Full Title Spontaneous splenic rupture in infectious mononucleosis: a review. [populationmedicine.org]

  RUPTURE of the spleen in infectious mononucleosis is a rare complication of a rather common disease. King, 1 in 1941, presented the first authenticated case. [nejm.org]

  As splenic ruptures carry a life-threatening risk (up to 9% of infectious mononucleosis patients), the diagnosis must be made as soon as possible. [symptoma.com]

  Abstract Spontaneous rupture of the spleen is a rare complication of infectious mononucleosis with no clear consensus on appropriate management. [pediatrics.aappublications.org]

• Anemia

  However, she was re-admitted on postoperative day 10 for febrile hemolytic anemia. Further exposure history was obtained and was significant for multiple tick bites 8 weeks preceding the index hospitalization. [ncbi.nlm.nih.gov]

  Here, we present the case of a term neonate who presented with worsening anemia in the setting of known hemolytic disease during the newborn period and was later found to have a spontaneous splenic rupture. [scholars.northwestern.edu]

  A blood analysis showed that the patient presented anemia (9.5 g/dL hemoglobin) and an abdominal CT scan ( Fig. 1 ) displayed a subcapsular hematoma of the spleen that was over 10 cm with active bleeding and hemoperitoneum. [scielo.isciii.es]

• Pallor

  Pain in the upper left abdominal quadrant, hypotension, pallor, tachycardia and abdominal distention are main symptoms. [symptoma.com]

  In the presence of severe anemia with pallor and abdominal distension, particularly in the context of a difficult birth, an abdominal ultrasound must be urgently performed and surgical management promptly considered. [ncbi.nlm.nih.gov]

  On examination, she had severe pallor, tachycardia (pulse 120/min), tachypnea and a blood pressure (BP) of 90/60 mm Hg. There was a marked tenderness over all quadrants of the abdomen with ascites and hepatosplenomegaly. [ijccm.org]

  They may also have a mild pallor. Doctors will note that there is decreased capillary refill, abdominal distension, peritoneal signs, decreased pulse pressure and finally overt shock. [autoaccident.com]

• Amyloidosis

  AL amyloidosis patients undergoing stem cell mobilization need careful monitoring of signs and symptoms of spontaneous splenic rupture. [ncbi.nlm.nih.gov]

  No proliferation of myeloid CD34+ cells, infiltration with myeloma cells or amyloidosis could be demonstrated. Myeloperoxidase-positive cells were numerous, but not obviously increased when compared to the normal spleen. [nature.com]

  Langerhans cell histiocytosis primary splenic neoplasms, e.g. angiosarcoma, hemangioma amyloidosis (primary and secondary) post-colonoscopy 4 sneezing 5 sarcoidosis 6 Radiographic features Features are similar to splenic trauma with hemoperitoneum and [radiopaedia.org]

• Acutely Ill Patient

  However, as a complication of an acute episode it is very rare and can easily be missed. This can lead to a significant delay in the appropriate treatment of an acutely ill patient. [ispub.com]

• Pneumonia

  A 65-year-old man who was hospitalised for eosinophilic pneumonia developed haemodynamically unstable atrial flutter that necessitated urgent synchronised direct current cardioversion (DCCV). [ncbi.nlm.nih.gov]

  Living without the organ is more prone to develop infections, especially precarious ones such as Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae. [casereports.in]

  […] indicated with incomplete rupture splenectomy indicated with complete rupture or intractable bleeding Prognosis, Prevention, and Complications Post-splenectomy immunization patients are at increased risk of encapsulated organism infections including S. pneumoniae [medbullets.com]

  Very rare causes of spontaneous splenic rupture include viral infections such as Hepatitis A 6 and Rubella 7 as well as bacterial pneumonia 8. [ispub.com]

• Abdominal Pain

  The diagnosis of spontaneous splenic rupture is important to consider in a patient using apixaban who presents with abdominal pain and associated signs of hypotension and anemia. [ncbi.nlm.nih.gov]

• Vomiting

  Often, a triggering factor or minor physical event can be ascribed as the aetiology for rupture, including coughing, vomiting or minor medical procedures not involving the spleen. [ncbi.nlm.nih.gov]

• Kehr's Sign

  in the upper left portion of the stomach (abdomen) or under rib cage Tenderness when you touch the upper left portion of the stomach (abdomen) Left shoulder pain (Kehr’s Sign) Kehr's sign: Pain in the left shoulder caused by irritation of the undersurface [physio-pedia.com]

  […] of the diaphragm (the Kehr's sign) are highly suggestive of a splenic pathology. [symptoma.com]

  Signs and symptoms [ edit ] In minor injuries with little bleeding, there may be abdominal pain, tenderness in the epigastrium and pain in the left flank. Often there is a sharp pain in the left shoulder, known as Kehr's sign. [en.wikipedia.org]

  […] injury is the most common cause of bleeding but is more often clinically insignificant Risk factors left-sided rib fractures history of blunt abdominal trauma EBV infection leading to splenomegaly Presentation Symptoms referred pain to left shoulder ( Kehr [medbullets.com]

  […] to identify signs of any other major life-threatening injury in a polytrauma patient! [amboss.com]

• Nausea

  He presented to the hospital with abdominal pain, nausea, and blood-stained dialysate. Laboratory data revealed severe anemia, with a hemoglobin of 4.3 g/dL. [ncbi.nlm.nih.gov]

  On hospital day two, the patient began to experience abdominal pain associated with nausea. Routine laboratory tests were normal. Hospital day three was marked by worsening nausea and vomiting. [scielo.br]

  Right quadrant/left abdominal pain Left shoulder pain Severe or mild pain Rebound tenderness Muscle guarding Nausea Profuse sweating or hot and cold sensations Abdominal distension or ecchymosis (bruising) Light headedness or syncope (fainting) Fatigue [ksi.uconn.edu]

  This can cause: Blurred vision Confusion Light-headedness Fainting Signs of shock, including restlessness, anxiety, nausea, and paleness How Is a Ruptured Spleen Diagnosed? A physical exam may be the only test done to diagnose a ruptured spleen. [webmd.com]

  The major symptoms and signs of splenic rupture include abdominal pain, tenderness and guarding, hypotension, nausea, vomiting, dizziness and syncope ( 9 ). [spandidos-publications.com]

• Abdominal Distension

  In the presence of severe anemia with pallor and abdominal distension, particularly in the context of a difficult birth, an abdominal ultrasound must be urgently performed and surgical management promptly considered. [ncbi.nlm.nih.gov]

  Right quadrant/left abdominal pain Left shoulder pain Severe or mild pain Rebound tenderness Muscle guarding Nausea Profuse sweating or hot and cold sensations Abdominal distension or ecchymosis (bruising) Light headedness or syncope (fainting) Fatigue [ksi.uconn.edu]

  Her abdominal examination revealed lower abdominal distension, uterine size of 28 weeks, free fluid and regular fetal heart rate of 158 beats per minute. [casereports.in]

  Doctors will note that there is decreased capillary refill, abdominal distension, peritoneal signs, decreased pulse pressure and finally overt shock. [autoaccident.com]

• Hypotension

  When splenic rupture is indicated by abdominal pain, tachycardia, or hypotension in a patient with splenomegaly, abdominal examination should be performed immediately, and emergency transcatheter arterial embolization and/or splenectomy should be considered [ncbi.nlm.nih.gov]

  Pain in the upper left abdominal quadrant, hypotension, pallor, tachycardia and abdominal distention are main symptoms. [symptoma.com]

• Shoulder Pain

  He now presented with sudden severe abdominal and left shoulder pain for 1 day, with no history of antecedent trauma. He was in shock, and examination revealed diffuse peritonitis. A computed tomography scan showed a grade IV splenic laceration. [ncbi.nlm.nih.gov]

  The physical examination may be even more important if conducted thoroughly, and the presence of a palpable tender mass in the upper left quadrant of the abdomen (known as the Ballance's sign), as well as referred left shoulder pain due to irritation [symptoma.com]

  and left shoulder pain (referred from phrenic nerve irritation) Hematologic findings - due to decreased splenic clearance thrombocytosis Howell-Jolly bodies Please rate topic. [medbullets.com]

  Right quadrant/left abdominal pain Left shoulder pain Severe or mild pain Rebound tenderness Muscle guarding Nausea Profuse sweating or hot and cold sensations Abdominal distension or ecchymosis (bruising) Light headedness or syncope (fainting) Fatigue [ksi.uconn.edu]

  Free blood can irritate the diaphragm and cause a radiating left shoulder pain (known as Kehr’s sign). [teachmesurgery.com]

• Left Shoulder Pain

  He now presented with sudden severe abdominal and left shoulder pain for 1 day, with no history of antecedent trauma. He was in shock, and examination revealed diffuse peritonitis. A computed tomography scan showed a grade IV splenic laceration. [ncbi.nlm.nih.gov]

  The physical examination may be even more important if conducted thoroughly, and the presence of a palpable tender mass in the upper left quadrant of the abdomen (known as the Ballance's sign), as well as referred left shoulder pain due to irritation [symptoma.com]

  and left shoulder pain (referred from phrenic nerve irritation) Hematologic findings - due to decreased splenic clearance thrombocytosis Howell-Jolly bodies Please rate topic. [medbullets.com]

  Right quadrant/left abdominal pain Left shoulder pain Severe or mild pain Rebound tenderness Muscle guarding Nausea Profuse sweating or hot and cold sensations Abdominal distension or ecchymosis (bruising) Light headedness or syncope (fainting) Fatigue [ksi.uconn.edu]

  Free blood can irritate the diaphragm and cause a radiating left shoulder pain (known as Kehr’s sign). [teachmesurgery.com]

As splenic ruptures carry a life-threatening risk (up to 9% of infectious mononucleosis patients) [5], the diagnosis must be made as soon as possible. A combination of clinical, laboratory and imaging studies are necessary [1], starting with a properly obtained patient history that will point to a traumatic or non-traumatic cause. The physical examination may be even more important if conducted thoroughly, and the presence of a palpable tender mass in the upper left quadrant of the abdomen (known as the Ballance's sign), as well as referred left shoulder pain due to irritation of the diaphragm (the Kehr's sign) are highly suggestive of a splenic pathology [6]. Laboratory studies include a basic biochemical panel and a complete blood count (CBC), which will often show a decreased hematocrit [3]. Imaging studies, however, are pivotal for making the diagnosis. Abdominal ultrasonography is a highly useful first-line procedure that can be done bedside if patients are unstable [1], but a computed tomography (CT) scan should be performed whenever possible [1] [4]. Aspiration of fresh blood from the peritoneum is another diagnostic indicator of splenic rupture [1].

• Thrombocytosis

  […] of splenectomy, acute pancreatitis, trauma, and other abdominal surgeries presents with elevated WBC's, fevers, pleuritic pain, and left shoulder pain (referred from phrenic nerve irritation) Hematologic findings - due to decreased splenic clearance thrombocytosis [medbullets.com]

  (see differential diagnoses below) References: [3] Diagnostics Laboratory tests : low Hb, leukocytosis, and thrombocytosis ; crossmatch for blood transfusion if needed In hemodynamically unstable patients First ultrasound : focused assessment with sonography [amboss.com]

  Complications of Treatment The main complications of conservative treatment or embolisation are: Ongoing bleeding Splenic necrosis Splenic abscess or cyst formation Thrombocytosis * *The thrombotic risk, including DVT and portal vein thrombosis, increases [teachmesurgery.com]

  The activating mutation JAK2V617F is frequently found in myeloproliferative neoplasms (MPNs), including polycythemia vera, essential thrombocytosis and PMF ( 20 ). MPNs share an increased risk of thrombotic and hemorrhagic complications. [spandidos-publications.com]

• Hemoglobin Decreased

  […] every 6 hours for the first 24 hours Nothing by mouth for the first 24 hours May then eat when emergent surgery is unlikely Must first demonstrate stable Vital Sign s and serial Hemoglobin s Consider repeat Abdominal CT imaging Hemoglobin decrease (via [fpnotebook.com]

• Leukocytosis

  […] splenomegaly Presentation Symptoms referred pain to left shoulder ( Kehr sign ) result of irritation of the diaphragm Physical exam hypotension shock Differential Splenic abscess seen in immunocompremised patients and IV drug users presents with fever, leukocytosis [medbullets.com]

  (see differential diagnoses below) References: [3] Diagnostics Laboratory tests : low Hb, leukocytosis, and thrombocytosis ; crossmatch for blood transfusion if needed In hemodynamically unstable patients First ultrasound : focused assessment with sonography [amboss.com]

  The laboratory analysis revealed a normal hemoglobin level (14 g/dl) and a mild leukocytosis (17.4). [wjes.biomedcentral.com]

  On investigations her haemoglobin was 7.2 g% with thrombocytopenia (platelet count of 42,000 per cu mm) and marked leukocytosis (total leukocyte count of 25,000 per cu mm). [tropicalgastro.com]

  Emerging risk factors may include leukocytosis and presence of the JAK2V617F mutation, or an increase in its allelic burden. [spandidos-publications.com]

Further treatment with rituximab led to catastrophic treatment complications that proved to be fatal for the patient. Severe cytokine release syndrome associated with biologics carries a very high morbidity and case fatality rate. [ncbi.nlm.nih.gov]

These patients have a very poor prognosis with a median survival of about 10 months. We present a patient, with a history of CLL in complete remission, who presented with splenic rupture requiring splenectomy. [ncbi.nlm.nih.gov]

Significant advances have been made in our understanding of the natural history, pathogenesis, mechanisms of progression and prognosis of monoclonal gammopathy of undetermined significance (MGUS). [scienceopen.com]

[…] quadrant pain Evaluation Imaging CT scan important in showing extent of splenic injury Exploratory laparotomy Treatment Surgical endovascular embolization indicated with incomplete rupture splenectomy indicated with complete rupture or intractable bleeding Prognosis [medbullets.com]

Spontaneous splenic rupture (SSR) is a rare condition in contrast to traumatic splenic hematoma, and vascular abnormality has rarely mentioned as an etiology in SSR with patients who had chronic kidney disease. [ncbi.nlm.nih.gov]

English : BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications CN congenital DI diagnosis DG diagnostic imaging DH diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology EH ethnology ET etiology [decs.bvs.br]

[…] inj) Allowable Qualifiers English : BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications CN congenital DI diagnosis DG diagnostic imaging DH diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology [decs.bvs.br]

2005, vol. 118 (pg. 759 - 766 ) [7] Time-related distribution, risk factors and prognostic influence of embolism in patients with left-sided infective endocarditis, Int J Cardiol, 2006, vol. 110 (pg. 334 - 339 ) [8] Infective endocarditis: changing epidemiology [ejcts.oxfordjournals.org]

Epidemiology Frequency Determining the actual frequency of splenic injuries with precision in the United States or worldwide is not possible. [emedicine.medscape.com]

Detailed attention is paid to pathophysiology, clinical symptoms and findings, all relevant imaging modalities and other tests employed to evaluate abdominal injuries at the time of admission of the trauma victim. [books.google.com]

[…] splenic salvage maneuver Grade IV Fragmented spleen with major devascularization Surgical intervention: splenectomy, a partial splenic resection if possible Grade V Hilar laceration/shattered spleen Surgical intervention: splenectomy References: [2] Pathophysiology [amboss.com]

Pathophysiology Though normally protected by its anatomic position, preexisting illness or disease can markedly increase the risks and severity of splenic injury. [emedicine.medscape.com]

The pathophysiologic mechanism seems to be due to the luminal occlusion by a fecalith during the endoscopic maneuvers [ 3 ]. The first case of splenic rupture after colonoscopy was published in 1975 by Wherry and Zehner [ 4 ]. [wjes.biomedcentral.com]

In the prevention and treatment of thromboembolic disease, novel oral anticoagulants have emerged as alternatives to warfarin. [ncbi.nlm.nih.gov]

[…] diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology EH ethnology ET etiology GE genetics HI history IM immunology ME metabolism MI microbiology MO mortality NU nursing PS parasitology PA pathology PP physiopathology PC prevention [decs.bvs.br]

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2. Aubrey-Bassler FK, Sowers N. 613 cases of splenic rupture without risk factors or previously diagnosed disease: a systematic review. BMC Emerg Med. 2012;12:11.
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4. Habib E. Ruptured spleen: an unusual complication of laparoscopy. HPB (Oxford). 2004;6(1):55-56.
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6. Roche M, Maloku F, Abdel-Aziz TE. An unusual diagnosis of splenic rupture. BMJ Case Rep. 2014;2014:bcr2014204891.
7. Cocanour CS, Moore FA, Ware DN, Marvin RG, Clark JM, Duke JH. Delayed complications of nonoperative management of blunt adult splenic trauma. Arch Surg. 1998;133(6):619-624.
8. Hui CM, Tsui KY. Splenic rupture in a newborn. J Pediatr Surg. 2002;37(4):E3.