Atraumatic splenic rupture: An atypical presentation in a previously healthy patient

Abstract

Unlabelled:
Atraumatic splenic rupture represents a life-threatening abdominal event that requires immediate diagnosis and prompt surgical treatment to ensure the survival of the patient. Atraumatic rupture is relatively uncommon and can occur either in pathological spleens or, more rarely, in normal ones. It has a high morbidity, frequently with few and non specific signs suggesting its presence, can be associated to other pathologies incidentally discovered by imaging. We present a case, successfully treated, of a 51-year-old man, previously healthy, that referred to our hospital for arterial hypertension and abdominal pain; the patient, for an idiopathic splenic rupture with haemoperitoneum, underwent an open splenectomy whose histology examination showed a normal spleen.

Key words:
Atraumatic splenic rupture, Normal spleen, Spontaneos haemoperitoneum - Full text is available at: www.annaliitalianidichirurgia.it.

Full text

Atraumatic splenic rupture:
an atypical presentation
in a previously healthy patient
Published online (EP) 23 December 2015 - Ann. Ital. Chir. 1
Ann. Ital. Chir.
Published online (EP) 23 December 2015
pii: S2239253X1502438X
www.annitalchir.com
Pervenuto in Redazione Giugno 2015. Accettato per la pubblicazione
Settembre 2015.
Correspondence to: Fausto Famà, Ph.D., M.D., Complesso MITO -
Residenza Ginestre F/2, 98151, Messina, Italy (e-mail: famafausto@yahoo.it)
Fausto Famà, Giovanni Scibilia, Antonio Ieni, Dario Lo Presti, Alessandra Villari,
Maria Gioffrè Florio
University Hospital of Messina, Department of Human Pathology, Messina, Italy
Atraumatic splenic rupture: an atypical presentation in a previously healthy patient
Atraumatic splenic rupture represents a life-threatening abdominal event that requires immediate diagnosis and prompt
surgical treatment to ensure the survival of the patient. Atraumatic rupture is relatively uncommon and can occur either
in pathological spleens or, more rarely, in normal ones. It has a high morbidity, frequently with few and non specific
signs suggesting its presence, can be associated to other pathologies incidentally discovered by imaging. We present a case,
successfully treated, of a 51-year-old man, previously healthy, that referred to our hospital for arterial hypertension and
abdominal pain; the patient, for an idiopathic splenic rupture with haemoperitoneum, underwent an open splenectomy
whose histology examination showed a normal spleen.
KEY WORDS: Atraumatic splenic rupture, Normal spleen, Spontaneos haemoperitoneum
Introduction
Blunt thoraco-abdominal trauma is the most frequent
cause of splenic rupture (about 90% of cases) and rep-
resents a life-threatening condition if late diagnosed and
treated 1,2. Nevertheless many other systemic disorders
are likely to determine an atraumatic splenic rupture
(ASR) in pathological spleens 3. More rarely than the
latter are the spontaneous ruptures that occur in healthy
spleens; they represent a distinct clinico-pathological enti-
ty and are classified as idiopathic 2,3. Patients may show
a plethora of clinical presentations mimicking other med-
ical emergencies (i.e. myocardial infarction, angina pec-
toris, pulmonary embolism, acute appendicitis, ectopic
pregnancy, acute sigmoid diverticulitis and visceral per-
foration) yet the abdominal pain, localised in the left-
side upper quadrant, along with signs of acute bleeding
or haemorrhagic shock are the most frequent clinical
findings 2,4. Furthermore, ASR is usually diagnosed late,
due to the absence of any history of trauma, and this
delay may affect significantly the patient outcome 2,3.We
report the case of a man, previously in healthy condi-
tion, who presented for abnormal blood pressure values
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and a drug-resistant epigastric pain; after he developed
a haemoperitoneum due to a spontaneous splenic rup-
ture and underwent a successful open splenectomy.
Case Report
A 51-year-old man was admitted for an isolated epigas-
tric pain associated to palpitations and slightly increased
values of blood pressure (155/95 mmHg). He denied
history of abdominal trauma, previous major surgery,
other pathologies and drug treatments. At physical exam-
ination the abdomen was mildly painful only in epigas-
tric region, distended for meteorism, without spleen size
enlargement and signs of peritoneal irritation. Laboratory
routine tests were all within the normal range (platelet
count 187,000/μl and white blood cells count 5,900/μl),
and his initial haemoglobin (Hb) value was 14.7 g/dL.
Furthermore, electrocardiogram and thorax x-ray were
unremarkable. After pharmacological treatment with
clonidine and ranitidine, his clinical status improved and
blood pressure normalised. Notwithstanding, for the sus-
picion of a latent coronary syndrome, patient was fur-
ther observed and monitored with blood and instru-
mental tests. Five hours later, his clinical condition sud-
denly deteriorated, with flare of pain, tachycardia and
hypotension. His Hb-level shrank to 8.1 g/dL, without
any evidence of external bleeding. A contrast enhanced
computed tomography (CT) showed a wide subcapsular
and intraparechimal splenic haematoma with an abun-
dant haemoperitoneum (Fig. 1). Radiologist hypothesize
a spontaneous rupture of an unknown benign vascular
tumour (i.e. haemangioma). Patient underwent an open
splenectomy with aspiration of, approximately, 2000 ml
of blood from the peritoneal cavity. Both, serological,
virological and haematological tests were also negative.
Gross examination of the specimen (measuring 12x11x6
cm and weighing 320 g) showed a fractured spleen with
a deep (3.5 cm) and large (6 cm) wound. Histology
revealed a normal splenic tissue without underlying any
atypia (Figs. 2, 3) and any evidence of benign vascular
tumour, thus allowed the diagnosis of idiopathic rup-
ture. His clinical course was uneventful and the patient
was discharged in 8th post-operative day, with appropri-
ate post-splenectomy antibiotic prophylaxis. Six months
later, he was well and free from significant complaints.
F. Famà, et al.
2Ann. Ital. Chir. - Published online (EP) 23 December 2015
Fig. 1: Contrast-enhanced computed tomography (CT), axial plane:
the white arrow points at splenic rupture with haemoperitoneum.
Fig. 2: Subcapsular haematoma of the spleen (haematoxylin-eosin
stain, original magnification x 180).
Fig. 3: Normal splenic tissue with blood congestion (original mag-
nification x 200).

Discussion
The atraumatic splenic rupture is usually due to a large
group of diseases, accounting approximately 10% of all
splenic ruptures 1,5,6. ASR, had a male/female ratio of
2:1 and may be divided into two categories: pathologi-
cal concerning abnormal spleens and idiopathic occurring
in healthy spleens 3,6. Pathological subtype recognizes the
following multiple aetiological causes: neoplastic (i.e.
lymphoma, leukaemia, metastases, benign or malignant
spleen tumours), infectious (i.e. malaria, infectious
mononucleosis), metabolic (amyloidosis, Gaucher’s dis-
ease), inflammatory non-infectious (i.e. rupture of splenic
cyst, infarction of the spleen, splenomegaly and hyper-
splenism), and drug/treatment-related (i.e. abuse of anti-
coagulants and thrombolytics, endoscopic procedures)
1,3,5. The idiopathic subtype, widely described as rare,
had a reported prevalence of about 7% of spontaneous
splenic rupture 1-3. The four criteria to assess the diag-
nosis of spontaneous splenic rupture were formulated
more than fifty years ago and currently are still consid-
ered as the gold standard: 1) on thorough questioning
either before or after the operation there should be no
history of trauma or unusual effort which conceivably
could injure the spleen; 2) there should be no evidence
of disease in organs other than the spleen, which is
known to affect the spleen adversely; 3) there should be
no evidence of perisplenic adhesions or scarring of the
spleen, which suggest that it has been traumatized or
had ruptured previously; 4) other than the findings of
haemorrhage and rupture, the spleen should be normal
on macroscopic as well as microscopic examination 7.A
fifth criterion was added later: 5) full virological studies
of acute phase and convalescent sera should show no
significant rise in antibody titres suggesting recent viral
infection of types known to be associated with splenic
involvement 8.
The timely ASR recognition has an utmost relevance in
order to well-manage patients with predisposing condi-
tions and reduce mortality rate, that may reach 13% in
worldwide literature 9,10. The major risk factors increas-
ing the mortality rate are: enlarged spleen size, neoplas-
tic diseases and age over forty years 3,11. The spleen rup-
ture can occur in the absence of major trauma or any
previously diagnosed splenic disease. Even minor events,
such as coughing, vomiting, or sneezing may be cause
of pathological spleen ruptures 12. The typical clinical
presentation includes a sudden-onset upper abdominal
pain, worse in the left upper quadrant and also referred
on the left shoulder (Kehr’s sign), abdominal rigidity and
hemodynamic instability 1,3,5. Less frequently is possible
to recognize a tender mass in the splenic region (Balance’s
sign) and non-specific symptoms as nausea, dizziness or
syncope may also be associated 1,12. An atypical presen-
tation or the absence of these signs are likely to lead to
the risk of misdiagnosis. Laboratory investigations may
show a normal or low Hb-level. Ultrasounds are per-
formed routinely and may be useful, notwithstanding CT
scan represents the main diagnosis tool to detect splenic
lesions and possible causative pathological processes 4.
Histological features of idiopathic spleen rupture are sim-
ilar to those found in traumatic ones: subcapsular or
parenchymal haematoma, pedicle laceration, and
perisplenic haematomas 1,3,12. Patients with splenic rup-
ture are more frequently treated with total splenectomy,
realised by open approach or laparoscopy, in order to
remove the bleeding source and make a diagnosis of any
underlying disease, despite the correct management is
still debated 1,3,10,13,14. A conservative procedure or
spleen-preserving may be carried out only in patients
with known underlying splenic cause, whereas the non-
operative management (that requires bed staying and flu-
id resuscitation) can be performed in patients with
haemodynamic stability 1,3,5,6,15.
Conclusions
ASR represents a rare and potentially life-threatening
condition, that should be suspected and considered in
the differential diagnosis of the acute upper abdominal
pain. The diagnosis may be delayed and difficult to
make, especially in patients with atypical features.
Our clinical case highlights that patients with splenic
rupture may present unusual symptoms; in such cases a
possible early recognition associate to a correct surgical
management can be life-saving.
Acknowledgement
For the accurate revision of the manuscript, We wish to
thank Mr Sam Palella, a native speaker, with an exten-
sive experience on scientific papers; furthermore we also
thank Dr. Francesco Armaleo who contributed to the
data collection and to the clinical case management.
Riassunto
La rottura splenica non traumatica rappresenta un even-
to addominale potenzialmente fatale che richiede imme-
diata diagnosi e rapido trattamento chirurgico per garan-
tire la sopravvivenza del paziente. La rottura non trau-
matica è relativamente non comune e può verificarsi sia
in milze patologiche che, più raramente, in milze nor-
mali. Essa presenta un’elevata morbilità, frequentemente
con pochi ed aspecifici segni che possano suggerire la
diagnosi, e può essere associata ad altre patologie inci-
dentalmente scoperte con l’imaging radiologico. Noi pre-
sentiamo un caso, trattato con successo, di un uomo di
51 anni, apparentemente in buona salute, visitato pres-
so il nostro ospedale per rialzo pressorio e dolore addo-
minale; il paziente, a causa di una rottura splenica idio-
Published online (EP) 23 December 2015 - Ann. Ital. Chir. 3
Single port VATS resection of a sessile solitary fibrous tumour of the visceral pleura. A case report

patica con emoperitoneo, è stato sottoposto a splenec-
tomia laparotomica e l’esame istologico ha mostrato un
parenchima splenico normale.
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4Ann. Ital. Chir. - Published online (EP) 23 December 2015