Atraumatic Spleen rupture
Chief Complaint:
Abdominal PainHPI
41-year-old male presents to the emergency department with reports of the same exact abdominal pain as prior episodes of pancreatitis described as beginning last night, progressively worsening, 10 out of 10, epigastric, sharp, and stabbing. It is the same as pancreatitis pain he has had in the past due to alcohol use. The new pain, however, is now to the left shoulder from the epigastrium. Reports associated shortness of breath. He states that he was discharged from a prior hospital for pancreatitis 5 days ago. He has not had any alcohol since discharge.Pertinent Exam Findings
General Appearance: Patient is alert, awake and in significant acute distress secondary to abdominal pain Eyes: Pupils equal, eye movements normal, + subconjunctival pallor Respiratory: Lungs are clear to auscultation, patient is in no respiratory distress, not using any accessory muscles and speaking full sentences Cardiovascular: Tachycardic rate and regular rhythm Gastrointestinal: Abdomen is soft, epigastrium tender to palpation, negative Murphy’s, negative McBurney’s and no rebound is presentData Interpretation
Lactate elevated CBC: Leukocytosis and thrombocytosis acute inflammatory response BMP: Acute kidney injury and hyperkalemia LFTs unremarkable Lipase elevated but not consistent with acute pancreatitis Ethanol negative Chart review from outside records: Patient had a CT and MRCP at outside hospital with splenic vein thrombus with extension to proximal branch vessels of superior mesenteric vein. On re-imaging at outside hospital number 2, vessels all patent. Outside hospital number 1 imaging, reviewed by outside hospital number 2 radiology team reviewed and felt that there was an area of clot in the splenic vein and likely SMV that had potentially gotten smaller. At outside hospital number 1 patient treated with Heparin drip. Outside hospital number 2 did not continue this during admission since unclear of clot. Discharged with Rivaroxaban starter pack and 3 month supply to treat clot. Follow up with PCP for continuation as indicated.ED Course
Sepsis alert called after labs resulted. Dilaudid 1mg given and patient still with 8/10 pain, written for an additional dose 1mg. Patient found to have a significant acute kidney injury causing hyperkalemia. He was treated with insulin, D50, albuterol, sodium bicarb and lokelma for hyperkalemia. He was consented prior to transfusing 1 unit packed red blood cells. PCC was ordered to reverse xarelto but did not come down in time to be given in the ED. CT showed blood around the spleen and case discussed with interventional radiologist. Patient went to IR for angiogram and had embolization of main splenic artery due to concern for spontaneous splenic rupture in setting of anticoagulation then was admitted to ICU.
Hospital Course
Status post splenic artery embolization hospital day 1. Patient developed severe systemic inflammatory response syndrome and sepsis likely due to retained massive splenic hematoma following embolization with necrosis along with distal pancreatic necrosis. Patient had continued drop in hemoglobin. Patient underwent exploratory laparotomy, splenectomy and distal pancreatic debridement on hospital day 6. Patient received a total of 7 units PRBC since hospital day 1 secondary to splenic rupture. The patient went on to develop acute respiratory failure following abdominal surgery. A thoracic epidural placed with the hope of achieving better pain control to facilitate decrease in IV medications and maintain adequate ventilation when extubated. He was finally extubated on day 8.DISCUSSION
Background
Atraumatic splenic rupture (ASR) is an exceedingly rare diagnosis, especially when compared to the more common traumatic etiologies for splenic rupture. ASR is a difficult diagnosis to make due to its insidious and uncommon presentation. Even though this diagnosis is rare, it has a high mortality given the high volume of blood that passes through the spleen and approximately 500 mL of blood that is held in the organ at any one time. One systematic review found that approximately 30% of ASR cases are related to a neoplasm (e.g. leukemia or lymphoma), 27% of ASR cases were due to infection (e.g. EBV, HIV, malaria, or CMV), and 20% from inflammation of the spleen or surrounding structures (e.g. acute or chronic pancreatitis). Other less common causes include anticoagulation, pregnancy-related splenomegaly, or congestive splenomegaly. Up to seven percent of cases are idiopathic with no apparent etiology on diagnostic work up. Maintaining a broad differential when working up symptoms and quick recognition of ASR in the emergency department is imperative to prevent the significant morbidity and mortality associated with this condition.Diagnosis
Workup of ASR requires a thorough history and physical examination. The presentation of patients with ASR is variable and not well characterized, but one review of case studies found that most patients presented with nausea, vomiting, and abdominal pain. A smaller case series found that half of patients presented in extremis with shock and peritonitis. Physical exam will occasionally present with Kehr’s Sign, which is a sharp referred pain at the tip of the left shoulder while the patient is lying down and the legs are elevated. This is often associated with splenic rupture, as blood irritates the peritoneal cavity and causes referred pain from the diaphragm. Laboratory studies have limited use in diagnosing ASR. They may reveal a low hematocrit depending on severity and duration of the rupture and a lipase may be elevated in the setting of pancreatitis. Sensitivity in diagnosing ASR with ultrasound and computed tomography (CT) with contrast have been shown to be 57.1% and 85.7%, respectively. This is a much lower sensitivity on CT than when diagnosing traumatic splenic rupture which is nearly 100% sensitive. Imaging will frequently show splenomegaly with the presence of free fluid in the abdominal cavity. Often, the presence of a hematoma can be appreciated on CT. CT with IV contrast should be performed in all suspected ASR patients who are hemodynamically stable enough to undergo imaging.Management in ED
Once there is a high suspicion of ASR or positive findings on CT with contrast or ultrasound, emergent surgical consultation should be placed. As always, the patient’s ABCs should be prioritized in the emergency department. If at any point the patient becomes hemodynamically unstable, promptly initiate resuscitation, preferably with packed red blood cells. According to one large review, most patients with ASR undergo exploratory laparotomy with splenectomy. Therefore the patient should be kept NPO while waiting in the ED. Other less common treatment options include splenorrhaphy, supportive measures, or arterial splenic embolization. Consider reversal of anticoagulation, in conjunction with the surgeons, if the patient is currently taking anticoagulation.Prognosis
One systematic review of 845 patients found the overall ASR-mortality rate to be 12.2%. ASR-associated mortality was higher in patients over 40 and those who presented with splenomegaly. In the same systematic review, the percent of patients undergoing operative management versus conservative management was 85.3% and 14.7%, respectively. Of the conservative group, 17% of them had to have a secondary operation for a total splenectomy due to rebleeding. There was an increased risk of mortality in these patients who were initially treated conservatively, but subsequently had a splenectomy. Prompt recognition of ASR and emergency surgical consultation are important for emergency physicians to best manage this rare condition.Differential Diagnoses
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- Traumatic spleen rupture
- Perforated viscus
- Splenic vein thrombosis
- Infectious mononucleosis
- Malaria
- HIV
- Lymphoma
- Leukemia
- Arteriovenous malformation
- Pancreatitis
- Peptic ulcer disease
Clinical Pearls
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- ASR is a rare, life-threatening condition that is often associated with underlying malignancy, infection, or inflammation.
- Semiology is variable, although it may include abdominal pain, nausea, vomiting, and/or Kehr’s sign.
- ABCs should be a priority in any patient presenting with signs of frank peritonitis or shock.
- CT of the abdomen/pelvis with IV contrast is the preferred diagnostic modality.
- Emergent surgical consultation should be placed if there is high clinical suspicion for ASR or confirmation via imaging.
- Exploratory laparotomy and splenectomy is a common management course in patients with ASR.
- Overall mortality was 12.2% from one systematic review, with higher mortality rates in patients over 40 and those with splenomegaly on presentation.
References
- Ahbala T, Rabbani K, Louzi A, Finech B. Spontaneous splenic rupture: case report and review of literature. Pan Afr Med J. 2020;37:36. Published 2020 Sep 8. doi:10.11604/pamj.2020.37.36.25635
- Carlin F, Walker AB, Pappachan JM. Spontaneous splenic rupture in an intravenous drug abuser. Am J Med. 2014;127(3):e7-e8. doi:10.1016/j.amjmed.2013.09.035
- Lieberman ME, Levitt MA. Spontaneous rupture of the spleen: a case report and literature review. Am J Emerg Med. 1989;7(1):28-31. doi:10.1016/0735-6757(89)90079-x
- Liu J, Feng Y, Li A, Liu C, Li F. Diagnosis and Treatment of Atraumatic Splenic Rupture: Experience of 8 Cases. Pilone V, ed. Gastroenterology Research and Practice. 2019;2019:5827694. doi:10.1155/2019/5827694
- Renzulli P, Hostettler A, Schoepfer AM, Gloor B, Candinas D. Systematic review of atraumatic splenic rupture. Br J Surg. 2009;96(10):1114-1121. doi:10.1002/bjs.6737
- Tonolini M, Ierardi AM, Carrafiello G. Atraumatic splenic rupture, an underrated cause of acute abdomen [published correction appears in Insights Imaging. 2016 Aug;7(4):647]. Insights Imaging. 2016;7(4):641-646. doi:10.1007/s13244-016-0500-y
- Yau HV, Pradhan S, Mou L. Atraumatic splenic rupture in a patient treated with apixaban: A case report. Int J Surg Case Rep. 2020;71:270-273. doi:10.1016/j.ijscr.2020.04.050
Authors
Aaron Wolfe, DO, FACEP, Director of Education for Emergency Medical Minute and Clinical Associate Faculty for Rocky Vista University College of Osteopathic Medicine
Jack Spartz MSIV
Peter Haskins MSIV
Josh Wahba MSIV