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].

• Splenomegaly

  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]

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

• Infectious Mononucleosis

  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]

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

  We report the case of a previously healthy and immunocompetent 52-year-old Caucasian woman with a remote history of clinically diagnosed infectious mononucleosis who experienced sudden atraumatic splenic rupture after an untreated stray cat bite. [ncbi.nlm.nih.gov]

  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]

• 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]

• 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]

  Other common signs are those related to the hemodynamic alterations and vary from pallor, hypotension, tachycardia, dyspnea, to the more dramatic signs of shock. In other cases the clinical presentation is more subtle. [wjes.biomedcentral.com]

• Acutely Ill Patient

  This can lead to a significant delay in the appropriate treatment of an acutely ill patient. This, in turn, may result in increased morbidity and even mortality. [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

  Splenic hemorrhage secondary to retching and vomiting from incarceration of paraesophageal hernia is a rare but life-threatening complication. [ncbi.nlm.nih.gov]

• Kehr's Sign

  sign) are highly suggestive of a splenic pathology. [symptoma.com]

  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, and left upper quadrant pain Evaluation Imaging CT scan [medbullets.com]

  sign : referred pain in the left shoulder Ballance's sign: dullness on percussion in the LUQ Hemorrhagic shock (often delayed) : tachycardia and hypotension In delayed splenic rupture, symptoms may not present until days to weeks after trauma It is important [amboss.com]

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

  Often there is a sharp pain in the left shoulder, known as Kehr's sign. In larger injuries with more extensive bleeding, signs of hypovolemic shock are most prominent. [en.wikipedia.org]

• 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]

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

  That morning the pain was progressive without nausea or vomiting. His history reported correction of the nasal septum, left tympanoplasty and Helicobacter pylori infection. [abdominalsurg.org]

• 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]

  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]

  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]

  With increasing blood loss into the abdominal cavity, abdominal distension, peritoneal signs, and overt shock may be observed. [emedicine.medscape.com]

• Hypotension

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

  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]

• 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]

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

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

  Secondly, left-sided chest pain coupled with left shoulder pain and hemodynamic instability seen in splenic rupture can mimic myocardial infarction, pulmonary embolism or dissecting/ruptured aortic aneurysm. [westjem.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]

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

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

  Secondly, left-sided chest pain coupled with left shoulder pain and hemodynamic instability seen in splenic rupture can mimic myocardial infarction, pulmonary embolism or dissecting/ruptured aortic aneurysm. [westjem.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

  Altered Level of Consciousness ) Consider at 24-48 hours if high grade Splenic Injury (Grade 3 or higher) Indications for surgical intervention Continued Hemoglobin decrease or need for repeated Blood Transfusion Hemodynamic instability (e.g. [fpnotebook.com]

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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.
3. Freiwald S. Late-Presenting Complications After Splenic Trauma. Perm J. 2010;14(2):41-44.
4. Habib E. Ruptured spleen: an unusual complication of laparoscopy. HPB (Oxford). 2004;6(1):55-56.
5. Bartlett A, Williams R, Hilton M. Splenic rupture in infectious mononucleosis: A systematic review of published case reports. Injury. 2016;47(3):531-538.
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.