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Splenic Rupture

Splenic rupture carries a high mortality rate in the absence of an early diagnosis. It can occur due to both traumatic and non-traumatic causes. Pain in the upper left abdominal quadrant, hypotension, pallor, tachycardia and abdominal distention are main symptoms. A complete blood count revealing a decreased hematocrit and imaging studies, mainly ultrasonography and computed tomography, are used for confirmation, whereas history taking is essential for determining the underlying cause.

Presentation

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].

Immune System

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]
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]
She proved to be infected with cytomegalovirus causing idiopathic thrombocytopenic purpura, splenomegaly and splenic rupture. She was treated for 14 days with ganciclovir and meropenem and discharged on recovery.[ncbi.nlm.nih.gov]
At hospital admission, abdominal tenderness with splenomegaly was present with free fluid in abdomen. Haematological investigations established the coinfection of Plasmodium falciparum and Plasmodium vivax.[ncbi.nlm.nih.gov]

Entire Body System

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]
We report a unique case of a renal transplant patient with PB19 associated PRCA who developed a spontaneous splenic rupture after receiving IVIg for persistent anemia. He subsequently required splenectomy.[ncbi.nlm.nih.gov]
Spontaneous splenic rupture complicated by malignant lymphoma may cause exacerbation of anemia and hypovolemic shock.[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.[ncbi.nlm.nih.gov]
There is strong evidence that intravenous immunoglobulin causes sequestration of erythrocytes in the spleen and extravascular hemolytic anemia.[ncbi.nlm.nih.gov]

Infectious Mononucleosis

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]
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]
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]
This case demonstrates the risk of splenic rupture following EBV infection, even in the absence of trauma, and highlights the importance of prompt diagnosis and appropriate counselling in patients with infectious mononucleosis.[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]
Clinical examination revealed signs of tachycardia, pallor and splenomegaly. He had no evidence of peripheral stigmata of chronic liver disease.[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]

Raynaud Phenomenon

On review of symptoms with the patient, a history of oligoarthralgia, photosensitivity, xerostomia and Raynaud phenomenon was elicited.[ncbi.nlm.nih.gov]

Gastrointestinal

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]
Splenic rupture should be considered in cases of primary varicella in young adults presenting with abdominal pain.[ncbi.nlm.nih.gov]
A 22-year-old man presented with a 1-day history of severe abdominal pain. He developed a generalised vesicular rash 12 h prior to admission. On examination, he was maximally tender with peritonism in the lower abdomen.[ncbi.nlm.nih.gov]
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]
Here we present another case of this uncommon complication, occurring in a 54-year-old woman in clinical follow-up for VEDS who presented with sudden onset of abdominal pain and hypotension.[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]
A woman aged 36 years presented to a London teaching hospital's Accident and Emergency department with severe abdominal pain following a 6-day history of diarrhoea and vomiting, which had been under review by her GP.[ncbi.nlm.nih.gov]
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]
A 28-year-old Sinhalese, Sri Lankan man presented with a history of fever, myalgia and vomiting of 4 days duration. Investigations revealed a diagnosis of dengue fever with no signs of plasma leakage.[ncbi.nlm.nih.gov]
A 62-year-old woman presented with a 10-day history of right iliac fossa pain radiating to the right leg and associated vomiting.[ncbi.nlm.nih.gov]

Kehr's Sign

sign) are highly suggestive of a splenic pathology.[symptoma.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]
Physical examination may reveal tenderness in left hypochondrium, enlarged spleen and signs of diaphragmatic irritation (Kehr’s sign). Signs of hypotension and hypovolemic shock may also be present.[tropicalgastro.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]
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]
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]
Other symptoms include: lightheadedness confusion blurred vision fainting signs of shock, including restlessness, anxiety, nausea, and paleness These symptoms result from loss of blood and a drop in blood pressure.[medicalnewstoday.com]

Severe Abdominal Pain

A 22-year-old man presented with a 1-day history of severe abdominal pain. He developed a generalised vesicular rash 12 h prior to admission. On examination, he was maximally tender with peritonism in the lower abdomen.[ncbi.nlm.nih.gov]
A 26 years old Moroccan woman para II gravida II presented at the third stage of pregnancy with acute onset of severe abdominal pain. She developed immediately a hypovolemic shock.[ncbi.nlm.nih.gov]
A woman aged 36 years presented to a London teaching hospital's Accident and Emergency department with severe abdominal pain following a 6-day history of diarrhoea and vomiting, which had been under review by her GP.[ncbi.nlm.nih.gov]
We describe the clinical case of an 83-year-old white man who complained of sudden severe abdominal pain 5 days into a hospital stay for acute-on-chronic congestive heart failure and exacerbation of chronic obstructive pulmonary disease.[ncbi.nlm.nih.gov]

Jaw & Teeth

Xerostomia

On review of symptoms with the patient, a history of oligoarthralgia, photosensitivity, xerostomia and Raynaud phenomenon was elicited.[ncbi.nlm.nih.gov]

Cardiovascular

Hypotension

While undergoing evaluation for his facial trauma, the patient became severely hypotensive. The patient was diagnosed with a delayed splenic rupture and underwent splenectomy.[ncbi.nlm.nih.gov]
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]
Here we present another case of this uncommon complication, occurring in a 54-year-old woman in clinical follow-up for VEDS who presented with sudden onset of abdominal pain and hypotension.[ncbi.nlm.nih.gov]
Pain in the upper left abdominal quadrant, hypotension, pallor, tachycardia and abdominal distention are main symptoms.[symptoma.com]
This is a case of a 65-year-old woman who underwent endoscopic submucosal dissection (ESD) for lateral spreading tumor in the descending colon, and 36 h afterwards presented symptoms and signs of severe hypotension due to SR.[ncbi.nlm.nih.gov]

Musculoskeletal

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

Workup

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].

Serum

Thrombocytosis

(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]

Microbiology

Babesia Microti

Babesiosis, an infection caused by the protozoan Babesia microti and transmitted by the Ixodes scapularis tick, is commonly described in the literature with an approximate incidence of 1000 cases per year (Herwaldt et al., 2012).[ncbi.nlm.nih.gov]
Babesia microti is the main pathogen causing human disease and is endemic in the northeastern and upper midwestern parts of the USA.[ncbi.nlm.nih.gov]

Treatment

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]
Splenic rupture is a rare complication of primary cytomegalovirus infection, but has not been reported after administration of intravenous immunoglobulin or in the setting of the Guillain-Barré syndrome and its many variants, which often lead to treatment[ncbi.nlm.nih.gov]
A conservative treatment was chosen using a peritoneal cleansing during laparoscopic surgery. Clinicians should be aware of this rare toxicity as it could be severe, but easily reversible using appropriate surgical treatment.[ncbi.nlm.nih.gov]
Physicians should have greater awareness of the possibility of splenic rupture following colonoscopy in order to avoid delay of diagnosis and treatment for this life-threatening complication.[ncbi.nlm.nih.gov]
This is the first reported case of a neonate who had spontaneous splenic rupture and severe hemophilia A, and underwent successful medical treatment without any surgical intervention.[ncbi.nlm.nih.gov]

Prognosis

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]
Different from other Ehler-Danlos Syndrome subtypes, VEDS has poor prognosis due to severe fragility of connective tissues and association with life-threatening vascular and gastrointestinal complications.[ncbi.nlm.nih.gov]
Even if prognosis remains poor for patients with lung cancer, invasive procedures could be considered in this rapidly evolving setting, especially in case of reversible adverse event.[ncbi.nlm.nih.gov]
[…] lobulation of the spleen after development in contrast to a laceration, a cleft is usually smooth with a rounded edge and are not associated with an adjacent subcapsular hematoma or perisplenic fluid some larger clefts may contain fat Treatment and prognosis[radiopaedia.org]
[…] hospitalization -Prescribed a 6 month recovery before returning to hockey -At 2 months-light to medium physical activity and a CT scan -At 4 months- follow up assessment, intense physical noncontact training began -At 6 months- full contact was permitted Prognosis[physio-pedia.com]

Etiology

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]
The case raised also other problems regarding the etiology of splenic rupture, in establishing a causal relationship between a intrapartum splenic injury, and the three episodes of inferior genital tract hemorrhaging, in establishing the cause of the[ncbi.nlm.nih.gov]
Deciphering the etiology can be challenging with many cases remaining unclear despite full investigation.[ncbi.nlm.nih.gov]
It was considered that the etiology of bleeding was directly from the spleen. However, due to the temporary malposition of the peritoneal catheter, catheter-induced splenic trauma could not be ruled out.[ncbi.nlm.nih.gov]
Etiology was considered to be idiopathic in 1 patient. Two patients died in the postoperative period.[ncbi.nlm.nih.gov]

Epidemiology

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]

Sex distribution

Age distribution

Pathophysiology

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]

Prevention

In the prevention and treatment of thromboembolic disease, novel oral anticoagulants have emerged as alternatives to warfarin.[ncbi.nlm.nih.gov]
Emergency total splenic embolization was performed, and total splenectomy was conducted later to prevent re-bleeding or abscess formation. His postoperative course was uneventful, and he was discharged on postoperative day 22.[ncbi.nlm.nih.gov]
Early diagnosis and intervention can prevent mortality.[ncbi.nlm.nih.gov]
Prior to discharge he was commenced on recommended splenectomy secondary prevention treatment. There are several reported complications of varicella infection, more commonly in the immunocompromised population.[ncbi.nlm.nih.gov]
The routine use of ultrasound for this purpose would cost more than 1 million to prevent 1 traumatic rupture. 6 Evidence-based answers from the Family Physicians Inquiries Network[mdedge.com]

References

Article

Gedik E, Girgin S, Aldemir M, Keles C, Tuncer MC, Aktas A. Non-traumatic splenic rupture: Report of seven cases and review of the literature. World J Gastroenterol. 2008;14(43):6711-6716.
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.
Freiwald S. Late-Presenting Complications After Splenic Trauma. Perm J. 2010;14(2):41-44.
Habib E. Ruptured spleen: an unusual complication of laparoscopy. HPB (Oxford). 2004;6(1):55-56.
Bartlett A, Williams R, Hilton M. Splenic rupture in infectious mononucleosis: A systematic review of published case reports. Injury. 2016;47(3):531-538.
Roche M, Maloku F, Abdel-Aziz TE. An unusual diagnosis of splenic rupture. BMJ Case Rep. 2014;2014:bcr2014204891.
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.
Hui CM, Tsui KY. Splenic rupture in a newborn. J Pediatr Surg. 2002;37(4):E3.

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Last updated: 2018-06-22 10:01