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ABDOMINAL SURGERY

Official Journal of the American Society of Abdominal Surgeons, Inc.

This article originally appeared in the Winter 20011 / Spring 2012 issue of the Journal.

Non-traumatic Rupture of the Spleen: An Atypical Presentation of the Acute Abdomen

Michiel Leijnen, M.D., M.Sc.1
Wobbe O. de Steur, M.D.2
Wouter Brekelmans, M.D.1
Andy da Costa, M.D., Ph.D.1

1. Department of Surgery, Rijnland Hospital,
Leiderdorp, The Netherlands.
2. Department of Surgery, Leiden University
Medical Center, Leiden, The Netherlands.

Address for correspondence:
Michiel Leijnen, M.D., M.Sc.
Rijnland Ziekenhuis
Department of Surgery
Simon Smitweg 1
2353 GA Leiderdorp
Tel: +31715828045
E-mail: m.leijnen@rijnland.nl or leijnen@hotmail.com

ABSTRACT

In most cases splenic rupture is due to trauma, however, spontaneous ruptures do occur. The reasons for these ruptures can be of diverse origin. These include diseases such as infection, neoplasia and infiltrative process. This can be one of the causes of an acute abdomen when a patient presents with abdominal pain, especially in the upper left quadrant. We present four patients and a review of the literature on this rare entity. The therapy of choice can vary between patients depending on grade of splenic rupture, hemodynamic instability, availability of endovascular treatment and preference of the treating physician. Treatment should be focused on preserving splenic tissue. Non-traumatic rupture of the spleen must be considered by physicians in patients presenting with acute abdominal pain even without evident history of trauma, since early recognition and treatment can prevent serious morbidity and mortality.

INTRODUCTION

A rupture of the spleen is one of many causes of the acute abdomen and in most cases of traumatic origin. Nontraumatic causes include diseases such as infection, neoplasia and infiltrative process.1, 2 A non-traumatic ruptured spleen is a condition less common in patients presenting with acute abdominal pain and can result in severe morbity and mortality when diagnosed late due to doctor’s delay. Overall mortality rate is 10-15%.3 In this article we would like to present four patients with a spontaneous rupture of the spleen to illustrate that non-traumatic ruptures of the spleen do occur and remind physicians of this rare cause of the acute abdomen. Additionally we will present a review of the current literature on this rare entity.

CASE 1

A 54-year-old male presented with acute onset of pain abdominal pain since one day in the left quadrant. He experienced nausea. No fever was reported. His history reported Lyme’s disease, Hepatitis A, five periods of ablation of the left retina and shortly before presentation he was diagnosed with lung carcinoma with metastases in the adrenal glands. On physical examination a sweaty, pale man was seen with the following vital signs: tension 120/80 mmHg, heart rate 120 beats per minute, respiratory frequency 30 to 35 per minute, temperature 36.8 degrees Celsius and oxygen saturation of 98% with supplementation of oxygen 2 L/min. Abdominal tenderness was experienced in the left upper quadrant without rebound tenderness. A computed tomography (CT) scan of the abdomen was performed (Figure 1). This showed an active subcapsulary bleeding of the spleen and progression of the metastases in the adrenal glands with additonally skeletal metastases in the left iliac bone. Splenectomy was performed during emergency laparotomy. Histopathological examination of the spleen showed a normal spleen with a small subcapsulary bleeding, weight 250 grams. No other pathological features were described. After surgery he developed a paralytic ileus. He suffered from pneumonia and heart failure which were treated with diuretics and antibiotics. The dyspnea did not decrease and the next step would be to use endotracheal intubation and ventilation. However, he clearly stated that considering his relatively short prognosis due to lung carcinoma he refused to be mechanically ventilated. He died the same day.

CASE 2

Figure 1

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Figure 2

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A 64-year-old male presented with abdominal pain in the left upper quadrant since two weeks. That morning the pain was progressive without nausea or vomiting. His history reported correction of the nasal septum, left tympanoplasty and Helicobacter pylori infection. On physical examination a sweaty, pale man was seen with the following vital signs: tension 80/57 mmHg, heart rate 110 beats per minute, temperature 36.2° Celsius and oxygen saturation of 100% with supplementation of oxygen 2 L/min. Abdominal tenderness was experienced in the left upper quadrant and epigastric region with rebound tenderness. After fluid resuscitation a focused abdominal sonographic technique (FAST) was performed which showed free fluid in the abdominal cavity and inhomogeneous aspect of the spleen (size: 12 centimeters); no aneurysm of the abdominal aorta was seen. In addition an abdominal CT scan was performed. This showed an active bleeding of the spleen and a large amount of free fluid in the abdominal cavity especially around the liver. Liver, gall bladder, pancreas, abdominal aorta and kidneys were normal. Angiography showed an active bleeding from arteries lateral and cranial in the spleen. Endovascular embolisation of these arteries was performed with no persistent bleeding after the procedure. Due to hemodynamic instability an angiography was repeated later that day, which revealed residual active bleeding. This time the entire splenic artery was embolised, and again the bleeding was stopped (Figure 2). During that night he remained hemodynamically stable, however the next morning hypotension returned. An emergency laparotomy was performed, and the spleen was extirpated in multiple fragments. Histopathological examination of the spleen showed normal spleen tissue. Postoperatively he suffered from an abscess in the spleen cavity, which was drained percutaneously. Six months after surgery he reported with jaundice, elevated liver enzymes, elevated serum amylase and pain in the epigastric region. These findings were most likely due to pancreatitis. A CT of the abdomen showed signs of a pancreatic carcinoma in the head of the pancreas, since this area was enlarged on the CT-scan. Differential diagnosis was pancreatitis. Through endoscopic retrograde cholangiopancreatography a stent was placed in de choledochal duct after papillotomy and a cytological brush sample was obtained for analysis. There were no malignant cells in this brush sample. After three months this procedure was repeated and again there were no malignant cells in the brush sample, therefore, the diagnosis that remains until publication is stenosis of the choledochal duct after pancreatitis.

CASE 3

A 60-year-old male reported to our emergency department (ED). He had a history of appendectomy, osteotomy of the left femur and excision of an inguinal lymphoma which was being analyzed when he presented with pain in the upper abdomen and back. On presentation he was hemodynamically stable with blood pressure 107/40 mmHg, heart rate 84/minute and 100% saturation with supplemental oxygen. On physical examination there was abdominal tenderness in the upper abdomen with rebound tenderness. A half hour later his vital signs worsened, the blood pressure dropped to 85/44 mmHg with good response to fluid resuscitation. A FAST showed a multilobulary aspect of the spleen with a large amount of free fluid in the peritoneal space. An emergency splenectomy was performed of which he recovered without significant problems. The histopathological analysis of the spleen revealed localisation of a malignant lymphoma. This was concurrent with the findings of the excised inguinal lymphoma where a mantle cell lymphoma (blastoid variant) was diagnosed. He is currently being treated with chemotherapy

CASE 4

A 20-year-old female presented to our ED with abdominal pain on the left side. Her history reported asthma, scoliosis correction, infarction of the cerebellum bilaterally (probably due to immobilisation during the corrective surgery for the scoliosis), migraine and two months before her presentation at our ED she fell of her bicycle with a cerebral concussion after a head trauma. There was no abdominal trauma in her recent history. The abdominal pain increased with inhaling. She had stable vital signs and no signs of shock. At first a pulmonary embolism was suspected; this was excluded by a spiral CT of the thorax. On this CT free fluid surrounding the liver was noticed and she was admitted for further analysis. The free fluid surrounding the liver was thought to be due to a viral infection; therefore a full viral serologic analysis was performed including cytomegalovirus and Epstein-Barr virus (EBV). The next day a CT of the abdomen was performed where a large volume of ascites was seen and splenomegaly with irregularity of the capsule of the spleen. The ascites was aspirated and it was hemorrhagic. There was a drop in serum hemoglobin levels (from 7.2 mmol/L to 6.3 mmol/L). Based on the findings mentioned above the diagnosis splenic rupture was made. At first this was thought to be a delayed traumatic rupture due to the bicycle accident she suffered two months before, however when the results of the viral serologic analysis came back, it showed an acute infection with EBV. Therefore the diagnosis was changed to non-traumatic rupture of the spleen due to an acute infection with EBV. She remained stable during her admission and recovered fully without the need for intervention.

DISCUSSION

A spontaneous rupture of the spleen was first described in 1874 by the English surgeon Atkinson.4 His publication was followed by numerous case reports until a review of all these cases was published in 1958, which was the most comprehensive study at that time.1 They concluded that ‘a small number of cases which, when subjected to careful scrutiny, defy any conclusion other than that they represent instances of spontaneous rupture of the normal spleen’. They defined four criteria to diagnose a spontaneous splenic rupture:

  1. On thorough questioning, either prior to operation or in retrospect after operation, there should be no history of trauma or of unusual effort which conceivably could injure the spleen.
  2. There should be no evidence of disease in organs other than the spleen that is known to affect the spleen adversely and, thereby, could cause the rupture.
  3. There should be no perisplenic adhesions or scarring of the spleen which suggests that it had been traumatised or had ruptured previously.
  4. Other than the findings of hemorrhage and rupture, the spleen should be normal on both gross and histological examination.

Crate and Payne added a fifth criterion: Full virological studies of the acute phase should show no significant rise in viral antibody titers suggesting recent viral infection of the type associated with splenic involvement.5We can state that in our cases only the latter two do not fit within the criteria mentioned above. However, in the acute moment when a patient presents with a non-traumatic rupture of the spleen it is impossible to come to the diagnosis of spontaneous splenic rupture based on these criteria, since most criteria mentioned above are obtained after treatment the hemodynamically unstable patient. When a spleen is ruptured spontaneously and not due to trauma a wide range of conditions including viral infections has been reported to be responsible. These conditions can be divided in five groups: hematological, metabolic, iatrogenic, infective and other (Table 1).6 93% of spontaneous ruptures are associated with histopathologically altered spleens.3 Splenic injury is graded using the standards published by the Organ Injury Scaling Committee of the American Association for the Surgery of Trauma (AAST). Categories range from grade I (minor) to grade V (major) and correlate to kind of intervention (Table 2).7 The classification is developed for traumatic ruptures of the spleen, however, in our opinion this can be used as a clinical decision tool for the treatment of non-traumatic ruptures as well (conservative approach, endovascular embolisation, spleen-preserving surgery or splenectomy).

Table 1(part1)

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Table 1(part1)

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Table 2

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When examining a non-trauma patient with abdominal pain in the left upper quadrant a doctor has to bear the diagnosis of a non-traumatic rupture of the spleen in mind. Typically, patients present with hypovolemic shock and signs of peritonitis. Two other signs that are suggestive of splenic rupture are Kehr’s sign (left diaphragmatic irritation resulting in referred pain to the left shoulder) and Balance’s sign (palpable tender mass in the left upper quadrant). 2 Radiological imaging can play an important role in diagnosing when a ruptured spleen is suspected. Plain abdominal radiographs can show enlargement of the spleen with medial displacement of the gastric bubble and elevation of the left hemidiaphragm with impaired motion.8 Angiographic findings include extravasation of contrast, a “mottled” parenchymal phase and arteriovenous shunts with early venous filling and bowing, stretching and crowding of the vessels.8 CT has replaced angiography as the preferred diagnostic tool and can clearly show splenic hematoma or rupture (Figure 1). CT has a sensitivity and specificity of at least 95% in detecting splenic injury.9 Problems in using CT include the necessity to move the patient out of the ED with associated prolonged time required to complete the CT scan. FAST is another diagnostic tool that can be performed in the ED without moving the patient to the radiology department and it is a method that is performed more rapid than CT. This is a safe method to look for the presence of fluid in the peritoneal cavity. Although it is an operator-depended technique, it is still considered the best imaging method for the initial evaluation of unstable patients. The limitations of FAST are its inability to identify the type and source of the fluid and it may not detect fluid volumes less than 200 to 500 milliliters. FAST can be combined with aspiration of the free fluid when the fluid is easy accessible to determine the type of fluid. Beside these radiologic tools, diagnostic peritoneal lavage (DPL) is another fast, inexpensive and safe method when in experienced hands. FAST is considered superior to DPL by many authorities.2,10

Historically the treatment of choice for all kinds of splenic rupture used to be splenectomy; however, nowadays it remains subject to debate. Splenectomy remains the treatment of choice in patients with a hemoperitoneum and severe hypovolemic shock. During laparotomy repair of the spleen or tamponade by the use of an absorbable mesh (splenorraphy) should be considered to preserve splenic tissue.11,12

An alternative to surgery is emergency transcatheter arterial embolisation, when patients are responsive to initial fluid resuscitation since preservation of viable splenic tissue is considered preferable.13-15 There are some reports that indicate that spleen function remains even after embolisation of the proximal splenic artery due to collateral circulation.16-18 Post-splenectomy sepsis occurs in 3.2% of patients after splenectomy and is a potentially lethal complication of splenectomy with a mortality rate of 40-50%, hence the need for tissue preservation.19 The third possibility when treating splenic rupture is a conservative approach. This is a good option when patients are hemodynamically stable. Conservative management consists of observation for 7 to 14 days in the hospital, strict bed rest and administration of fluid and blood as needed.10 Rupture usually occurs within the first week of illness, although it may happen in any time of the course of the disease (1-30 days).20 Stephenson and DuBois conclude in a recent report that patients are considered suitable for the conservative approach when patients require less than 4 units or 40 ml/kg of blood for resuscitation.21 It remains unclear how to follow these patients and whether the pathologic spleen can heal and retain its function. The treatment of hemodynamic stable patients with spontaneous splenic ruptures should be assessed individually with a low threshold for surgery or arterial embolisation.

CONCLUSIONS

We described four patients with a non-traumatic rupture of the spleen due to multiple causes. Three of the patients underwent splenectomy due to hemodynamic instability, while one of them was initially treated by embolisation of the splenic artery. The fourth was treated conservatively. Spontaneous ruptures are associated with many underlying conditions. The therapy of choice can vary between patients depending on grade of splenic rupture, hemodynamic instability, availability of endovascular treatment and preference of the treating physician. Treatment should be focused on preserving splenic tissue. Non-traumatic rupture of the spleen must be considered by physicians in patients presenting with acute abdominal pain even without evident history of trauma, since early recognition and intervention can save lives.

REFERENCES

1. Orloff MJ, Peskin GW. Spontaneous rupture of the normal spleen: a surgical enigma. Int Abstr Surg. 1958; 106:1-11.

2. Wehbe E, Raffi S, Osborne D. Spontaneous splenic rupture precipitated by cough: A case report and a review of the literature. Scan J Gastroenterol. 2008; 43:634-7.

3. Renzulli P, Hostettler A, Schoepfer AM, Gloor B, Candinas D. Systematic review of atraumatic splenic rupture. Br J Surg. 2009; 98:1114-21.

4. Atkinson E. Death from an idiopathic rupture of the spleen. BMJ. 1874; 2:403-4.

5. Crate MID, Payne CMJ. Is the diagnosis of spontaneous rupture of a normal spleen valid? J R Army Corps. 1991; 137(1):50-1.

6. Debnath D, Valerio D. Atraumatic rupture of the spleen in adults. J R Coll Surg Edinb. 2002; 47:437-45.

7. http://www.trauma.org/index.php/main/article/806/, last accessed July 25th 2010.

8. Love L, Greenfield GB, Braun TW, Moncada R, Freeark RJ, Baker RJ. Angiography of splenic trauma. Radiology. 1968; 91(1):96-102.

9. Jeffrey RB, Laing FC, Federle MP, Goodman PC. Computed tomography of splenic trauma. Radiology. 1981; 141(3):729-32.

10. Körner M, Krötz MM, Degenhart C, Pfeifer KJ, Reiser MF, Linsenmaier U. Current Role of Emergency US in Patients with Major Trauma. Radiographics. 2008; 28(1):225-42.

11. Hamel CT, Blum J, Harder F, Kocher T. Nonoperative treatment of splenic rupture in malaria tropica: a review of literature and case report. Acta Tropica. 2002; 82(1):1-5.

12. Leemans R, van Mourik JB. A spleen-preserving method in splenic rupture using an absorbable net. Ned Tijdschr Geneeskd. 1988; 132(44):2016-7.

13. Lin WC, Chen YF, Lin CH, Tzeng YH, Chiang HJ, Ho YJ, Shen WC, Chen JH. Emergent transcatheter arterial embolization in hemodynamically unstable patients with blunt splenic injury. Acad Radiol. 2008; 15(2):201-8.

14. Wei B, Hemmila MR, Arbabi S, Taheri PA, Wahl WL. Angioembolization reduces operative intervention for blunt splenic injury. J Trauma. 2008; 64(6):1472-7.

15. Lui B, Schlicht S, Vrazas J. Role of embolization in the management of splenic trauma. Australas Radiol. 2004; 48(3):401-3.

16. Bellingham GA, Kribs S, Kornecki A, Scott L, Leaker M, Fraser DD. Proximal splenic artery embolization in the management of splenic rupture. Pediatr Crit Care Med. 2009; 10(1):e1-4.

17. Loffroy R, Guiu B, Cercueil JP, Lepage C, Cheynel N, Steinmetz E, Ricolfi F, Krausé D. Transcatheter arterial embolization of splenic artery aneurysms and pseudoaneurysms: short- and long-term results. Ann Vasc Surg. 2008; 22(5):618-26.

18. Laganà D, Carrafiello G, Mangini M, Fontana F, Dizonno M, Castelli P, Fugazzola C. Endovascular treatment of splenic artery aneurysms. Radiol Med. 2005; 110(1-2):77-87.

19. Bisharat N, Omari H, Lavi I, Raz R. Risk of infection and death among post-splenectomy patients. J Infect. 2001; 43(3):182-6.

20. Julià J, Canet JJ, Lacasa XM, González G, Garau J. Spontaneous spleen rupture during typhoid fever. Int J Infect Dis. 2000; 4(2):108-9.

21. Stephenson JT, DuBois JJ. Nonoperative management of spontaneous splenic rupture in infectious mononucleosis: a case report and review of the literature. Pediatrics. 2007; 120(2):432-5.



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The American Society of Abdominal Surgeons, Inc.
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Louis F. Alfano, Jr., M.D., Editor-in-Chief
Diane M. Pothier, Executive Editor and
Director of Continuing Medical Education


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Figure 1:
Computed tomography scanning of the abdomen showing a large subcapsular hematoma with extravasation of contrast (**). The bleeding is inside the splenic capsule with deformation of the splenic tissue (*).

Figure 2: Angiography of the splenic artery after coiling.

Table 1(part 1): Conditions associated with non-traumatic rupture of the spleen.4

Table 1(part 2): Conditions associated with non-traumatic rupture of the spleen.4

Table 2: Classification of spleen ruptures according to the AAST.7