Grand Rounds Vol 9 pages 6–8

Speciality: Critical care, General surgery

Article Type: Case Report

DOI: 10.1102/1470-5206.2009.0002

� 2009 e-MED Ltd

Atraumatic splenic rupture secondary

to infectious mononucleosis: a

case report and literature review

Alex Looseley, Alex Hotouras, Quentin M. Nunes and Antony P. Barlow

Section of Surgery, United Lincolnshire Hospitals NHS Trust, UK

Corresponding address: Dr. Alex Looseley, Derby City General Hospital, Uttoxeter New Road,

Derby DE22 3NE, UK.

E-mail: alex.looseley@nhs.net

Date accepted for publication 20 February 2009

Abstract

Although splenomegaly is found in approximately two thirds of patients with infectious

mononucleosis (IM), splenic rupture is uncommon. However, it constitutes the single largest

cause of mortality in this group. True atraumatic splenic rupture is very rare and is seen in only

0.5% of all cases of IM. We present a case of a 22-year-old man with atraumatic splenic rupture

associated with infectious mononucleosis and highlight key considerations in diagnosing and

managing this potentially fatal complication.

Keywords

Atraumatic splenic rupture; diagnosis; infectious mononucleosis; glandular fever.

Case Report

A 22-year-old man presented to the Accident and Emergency department following collapse.

This was associated with vague lower abdominal pain and vomiting. He emphatically denied any

trauma to his abdomen. He had recently been treated by his General Practitioner with amoxicillin

for a painful right submandibular gland. However, he had developed a macular-papular rash

affecting both upper limbs and accordingly the antibiotic was discontinued. On examination he

was pale with a heart rate of 120/min and a blood pressure of 90/60mm Hg. His abdomen

was not distended, though generally tender with early signs of peritonism. Blood tests revealed an

Hb of 13.0 (g/dl), white cell count of 30.3 (109/L) and lymphocytes of 16.0 (109/L). A positive

heterophil antibody titer (Monospot) confirmed the diagnosis of infectious mononucleosis. The

surgical team on-call reviewed the patient and organised a contrast-enhanced computed

tomography (CECT) scan of his abdomen and pelvis which revealed a large volume of free

fluid/blood within the peritoneal cavity, with the largest collection around an enlarged spleen

(Fig. 1). As the patient was requiring increasing fluid resuscitation to maintain his haemodynamic

stability, a decision was taken for surgical intervention. Intra-operative findings included a

haemoperitoneum with multiple lacerations of the spleen, which was friable. A splenectomy was

performed after which the patient made a good post-operative recovery. The spleen measured

20 x 15 cm and weighed 650g. Microscopically, the normal splenic architecture was preserved

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but there was prominent expansion of the red pulp within which there was diffuse infiltration of

lymphoid cells (Fig. 2).

Discussion

Splenic rupture is most commonly associated with trauma. Atraumatic splenic rupture is an

extremely rare clinical entity which was first documented in the 19th century.[1] Since then

several cases have been reported in the literature as a complication of infectious (e.g. malaria,

glandular fever), gastrointestinal (e.g. pancreatitis), haematological (e.g. lymphoma) and systemic

(e.g. sarcoidosis) disorders. [2,3,4,5] Haematological disorders such as non-Hodgkin lymphoma and

acute and chronic myeloid leukaemia are the most frequently reported causes of atraumatic

splenic rupture.[6] As described in this case, infectious mononucleosis is related to atraumatic

splenic rupture in 0.1–0.5% of patients.[7] It appears to be more common in males, with a male to

female ratio of about 3:1 and occurs almost exclusively in adults.[6]

It has been postulated that there are three pathophysiological factors that may explain

atraumatic rupture. The major underlying factor appears to be the splenic parenchymal

congestion. Coagulation disorders leading to haemorrhage and splenic infarction are also thought

to be involved in the aetiology.[6]

In the absence of trauma, the diagnosis of splenic rupture cannot always rely on the classic

symptomatology: abdominal pain, guarding in the left upper quadrant and haemodynamic

instability. The development of a macular-papular rash following initiation of treatment

with amoxicillin was of great diagnostic value in this case and prompted the consideration of

Epstein-Bar virus. Approximately 70–100% of patients who receive a b-lactam antibiotic while

infected with the Epstein-Bar virus will develop such a rash.[8] Additionally, left shoulder-tip pain

(Kehr’s sign) resulting from intraperitoneal blood causing diaphragmatic irritation is present

in approximately 50% of cases.[7] The clinician should have a high index of suspicion in order to

diagnose atraumatic splenic rupture, not only because of the rarity of the condition but

most importantly due to the gravity of a delayed diagnosis. Clinicians should also recall that

atraumatic splenic rupture could initially present as — and be mistaken for — cardiac ischaemia,

pulmonary embolism, pneumonia, peptic ulceration or ruptured sigmoid diverticulitis.[9,10]

Fig. 1. CECT showing haemoperitoneum with an enlarged and ruptured spleen.

Fig. 2. Histological image showing activated lymphoid cells in the red pulp of the spleen.

Atraumatic splenic rupture secondary to infectious mononucleosis 7

The diagnosis is aided by the use of emergency ultrasonography or CECT which can demonstrate

the presence of haemoperitoneum and an enlarged/ruptured spleen.[2] CECT has around 95%

sensitivity and specificity in detecting splenic injury.[11] Splenectomy is the traditional treatment

although conservative management may be adopted in haemodynamically stable patients to avoid

the potentially severe septic complications post-splenectomy. [12,13]

Teaching point

Classically, though not exclusively, splenic rupture presents with abdominal pain, signs of

peritoneal irritation and haemodynamic instability. If any one of these features occurs in the

absence of trauma, in conjunction with a known or suspected diagnosis of IM, atraumatic splenic

rupture must always be excluded as a priority.

Acknowledgement

No competing interests have been declared by the authors.

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8 A. Looseley et al.