Extraperitoneal hemorrhagic rupture of a simple hepatic cyst. A case

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Extraperitoneal hemorrhagic rupture
of a simple hepatic cyst.
Ann. Ital. Chir.
Published online (EP) 30 September 2016
pii: S2239253X16025883
www.annitalchir.com
A case report and literature review
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Andrea Vannucchi*, Andrea Masi**, Gabriele Vestrini*, Francesco Tonelli***
*Department of General Surgery of Centro Oncologico Fiorentino, Sesto Fiorentino, Italy
**Department of Diagnostic Imaging of Centro Oncologico Fiorentino, Sesto Fiorentino, Italy
***Department of Surgery and Translational Medicine, University of Florence, Florence, Italy
Extraperitoneal hemorrhagic rupture of a simple hepatic cyst. A case report and literature review
Hemorrhagic rupture is a very rare complication of a simple hepatic cyst. We report the first case of a totally extraperitoneal rupture of a recurrent cyst, occurred in a 73-year-old man who presented with acute right hypochondralgia.
Computed tomography revealed the rupture of a large hemorrhagic cyst in the right liver lobe and the formation of a
voluminous hematoma in the retroperitoneal space. Despite the absence of hemoperitoneum, the entity of the bleeding
led us to perform an urgent and successful surgical intervention. A review of the literature was conducted. To date, 9
cases of hemorrhagic rupture of simple hepatic cyst have been described and our case is the first one characterized by
extraperitoneal bleeding. Our clinical management was in accordance with the majority of the other Authors. Different
surgical procedures have been proposed and the best approach has not been established yet. However, the laparotomic
approach should be preferred for a better control of the bleeding. In conclusion, the hemorrhagic rupture of a liver cyst
is a life-threatening complication and a prompt surgery is necessary to prevent the hypovolemic shock.
KEY
WORDS:
Extraperitoneal rupture, Hemorrhagic rupture, Simple hepatic cyst
Background
Simple hepatic cysts are congenital or acquired benign
cystic formations, that occur in the general population
with a prevalence of 3-5% in ultrasound (US) studies
and 18% in computed tomography (CT) studies 1,2. The
Pervenuto in Redazione Maggio 2016. Accetttato per la pubblicazione
Giugno 2016.
Correspondence to: Andrea Vannucchi, MD, Centro Oncologico
Fiorentino, Via Ragionieri 101, 50019 Sesto Fiorentino, Italy (e-mail:
[email protected])
pathogenesis of simple cysts is unknown, but the origin
from aberrant bile ducts is the widespread hypothesis 3.
Results of pathological analysis usually confirm the presence of a layer of cuboidal or columnar epithelium
resembling biliary epithelium 4.
Simple cysts remain asymptomatic in most cases.
Symptoms occur in only 10-16% of affected patients
and they are usually related to complications, which are
uncommon unless the cysts reach large sizes 5. The
majority of hepatic cysts are small in size, and less than
20% are more than 1 cm in diameter 6. Abdominal discomfort, early satiety, nausea and vomiting, arise as a result
of a mass effect. Complications such as compression of the
biliary tree, vascular compression, hemorrhage or rupture
are rare and may occur in giant cysts 7. The association
of cystic hemorrhage and rupture is the rarest complication and to date only a few articles are available on
this item.
Published online (EP) 30 September 2016 - Ann. Ital. Chir.
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A. Vannucchi, et al.
Case Report
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A 73-year-old man presented with acute onset of abdominal pain in the right hypochondrium. His medical history included arterial hypertension, life-long treatment
with acenocoumarol for chronic atrial fibrillation, and
laparoscopic cholecystectomy with unroofing of a simple
cyst of the right hepatic lobe performed in 1993. The
hepatic cyst recurred and a percutaneous drainage with
alcohol instillation was executed in 2005.
Physical examination revealed tenderness in the right
upper quadrant and an enlarged palpable right lobe of
the liver. Laboratory analyses showed hemoglobin level
of 13.4 g/dL, white cell count of 12.4×10^6/L, C-reactive protein level of 20.7 mg/dL, International
Fig. 3: The coronal computed tomography scan showed the rupture of
the cyst in the retroperitoneal space.
Fig. 1: The ultrasound image showed an hepatic cystic lesion of 10 cm
diameter in the right hepatic lobe with an hyperechogenic pattern in the
central zone.
Fig. 2: The axial computed tomography scan revealed a thickening of
the cystic wall and a heterogeneous content of the lesion. Free peritoneal fluid was not present.
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Ann. Ital. Chir. - Published online (EP) 30 September 2016
Normalized Ratio (INR) of 2.3 and creatinine level of
2.4 mg/dL. Ultrasonography of the liver demonstrated a
cystic lesion of 10 cm diameter in the right lobe with
an hyperechogenic pattern in the central zone (Fig. 1).
An intracystic hemorrhage was confirmed by the abdominal CT, which was executed without intravenous contrast due to the high creatinine level (Fig. 2). The CT
scan also showed the rupture of the cystic wall in the
subhepatic region with the formation of a voluminous
hematoma in the retroperitoneal space (Fig. 3).
Hemoperitoneum was not identified. The presence of a
neoplasm could not be excluded by the CT images,
which revealed a thickening of the cystic wall and a heterogeneous content of the cyst.
The acenocoumarol was substituted with low-weight
heparin and a conservative management was instituted.
A clinical evaluation after 48 hours showed hemoglobin
level of 9.6 g/dL, INR value of 1.4 and creatinine level of 1.3 mg/d/L. The persistence of symptoms, the
development of tachycardia and the decreased hemoglobin level induced us to perform an urgent laparotomy
with right subcostal access.
Surgical exploration revealed a voluminous hepatic mass
originated from the inferior part of the fifth segment.
The rupture of the cystic wall was totally in the retroperitoneal space, generating an hematoma containing bulky
clots and involving the duodenopancreatic bloc and the
perirenal right space. The cyst was detached from the
right colonic flexure and the resection of the fifth hepat-
Extraperitoneal hemorrhagic rupture of a simple hepatic cyst. A case report and literature review
hemorrhagic liver cyst rupture have been described in
the English literature, including our case (Table I).
Patients affected by Polycystic Liver Disease (PLD) were
excluded from our research. PLD is a rare and debilitating genetic pathology characterized by the formation
of >20 fluid-filled cysts in the liver. A different pathogenesis of the cystic lesions is probably the reason of the
increased risk of complications demonstrated in PLD
compared to simple hepatic cyst 10.
Data presented in Table I are insufficient to identify clear
risk factors for hemorrhagic rupture of a liver cyst. We
can only suppose a role of predisposing factor for age
>60 years and anticoagulant therapy, confirming observations already reported 11.
Two patients were previously treated with percutaneous
ethanol injection (PEI) of the cyst. However, the role of
PEI in the hemorrhagic rupture is difficult to establish
because the time between PEI and the occurrence of the
complication was two years in the first case and ten years
in the second one. Arterial hypertension was described
in two patients and probably it had a role in determining the entity of the bleeding, rather than in the
pathogenesis of the hemorrhagic cyst.
The totally extraperitoneal rupture of the liver cyst is
the peculiarity of our case, which is the first one reported in the English literature. Cases of spontaneous
retroperitoneal hematoma have been described for different pathologies 12-15 but not for hepatic benign diseases. The unusual site of the rupture was probably
favored by the peritoneal adhesions due to the previous
surgical and percutaneous procedures.
Despite the absence of hemoperitoneum, the initial
hemodynamic instability led us to perform an urgent
surgical intervention. As shown in Table I, the surgical
treatment was preferred in most cases and our approach
was in accordance with the majority of the other
Authors. Moreover, one study described the failure of
the conservative management 16 and three patients did
not undergo surgical intervention due to their compromised general conditions 17-20. However, outcome was
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ic segment en-bloc with the cystic wall was progressively carried out. One of the origins of the bleeding was
identified in a branch of the middle suprahepatic vein,
but we cannot exclude a supplementary arterial cause.
After the hemostasis of the hepatic resection surface, all
retroperitoneal blood clots were evacuated. A 24 Fr
drainage was placed in the posterior pararenal space.
During surgery, the patient received 4 packs of red blood
cells. The postoperative (PO) period, including two days
in intensive care, was uneventful. The drainage was
removed in PO day 5 and the patient was discharged
in PO day 6 with hemoglobin level of 11.1 g/dL.
Anatomopathological analyses described a hemorrhagic
benign cystic lesion lined by a bile duct epithelium.
Discussion
Clinical manifestations of simple liver cysts are usually
related to complications, such as compression on adjacent organs (bile ducts, portal vein, digestive tract, lungs),
intracystic hemorrhage, infection and intra- or extraperitoneal cyst rupture.
Intracystic hemorrhage is generally benign and can generate acute and regressive pain or can go unnoticed.
Diagnosis is based on abdominal US or CT scans, which
show an heterogenic content of the cyst. The distinction between a bleeding simple cyst and a hepatobiliary
cystic neoplasm may be difficult on the basis of clinical
and radiological features, due to the presence of intracystic heterogeneity in both situations. Percutaneous aspiration of cystic fluid for cytological examination and
serum dosage of CA19-9 have a low diagnostic accuracy and are not helpful for differential diagnosis 8. The
incidence of spontaneous rupture of an hepatic cyst is
not clearly reported in the literature. This event is far
rarely and usually occurs in lesions >10 cm 6.
The association of cystic hemorrhage and rupture is the
rarest complication and its clinical management has not
been established yet 9. A total of 9 patients affected by
Table I - Cases of hemorrhagic rupture of simple hepatic cyst reported in the English literature.
Study
Lotz11
Yamaguchi8
Ishikawa12
Kanazawa13
Cheung6
Marion9
Wang14
Simon15
Tonelli
Year
Sex
Age
Coexisting Factors
Site of bleeding
Treatment
1989
1999
2002
2003
2005
2011
2015
2015
2016
F
M
F
M
F
F
M
M
M
49
61
42
78
73
37
71
63
73
AHT
No
No
No
Previous PEI
Pregnancy
ACT, uremia, liver cirrhosis
ACT
ACT, AHT, previous PEI
Intraperitoneal
Intraperitoneal
Intraperitoneal
Intraperitoneal
Intraperitoneal
Intraperitoneal
Intraperitoneal
Intraperitoneal
Extraperitoneal
Surgical
Surgical
Surgical*
Conservative
Surgical
Surgical
Conservative
Conservative
Surgical
AHT: Arterial Hypertension; PEI: Percutaneous Ethanol Injection; ACT: Anticoagulant Therapy.
* Surgical intervention was performed after the failure of conservative treatment.
Published online (EP) 30 September 2016 - Ann. Ital. Chir.
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A. Vannucchi, et al.
favorable in both surgical and conservative treatments.
Different surgical procedures have been proposed and the
best approach has not been established yet. In most cases a laparotomic incision was preferred and the laparoscopic approach was performed only in one patient, characterized by hemodynamic stability 6. The laparoscopic
approach to non-hydatid hepatic cystic disease is safe and
efficacious 21, but surgeons should avoid this technique
if the hemorrhagic rupture of a cyst is suspected. In fact,
the possible involvement of a large blood vessel in the
pathogenesis of this complication exposes to a high risk
of major intraoperative bleeding.
We can conclude that in case of hemorrhagic rupture of
a liver cyst, a prompt surgery is necessary to prevent
hypovolemic shock and a laparotomic approach should
be preferred.
zioni, e in accordo con i dati estratti dalla letteratura,
l’intervento chirurgico urgente si rende necessario al fine
di prevenire lo shock ipovolemico.
Riassunto
5. Gigot JF, et al.: Laparoscopic management of benign liver diseases:
where are we? HPB (Oxford), 2004; 6(4):197-212.
Le cisti epatiche semplici sono patologie benigne di frequente riscontro nella popolazione generale, la cui incidenza è in progressivo aumento a seguito della larga diffusione di tecniche di imaging quali ecografia e TC addome. Tra le complicanze cui può andare incontro una cisti
epatica, la rottura emorragica è sicuramente la più rara
e temibile. Nel nostro lavoro riportiamo il caso di un
paziente di 73 anni, giunto alla nostra osservazione per
dolore ingravescente in ipocondrio destro. La TC
dell’addome ha evidenziato la rottura emorragica di una
voluminosa cisti del lobo epatico di destra, con formazione di un grossolano ematoma in sede retroperitoneale. L’instaurarsi di un iniziale quadro di instabilità emodinamica ha reso necessario l’esecuzione di un intervento chirurgico urgente, consistito nella resezione delle
pareti cistiche e di parte del quinto segmento epatico da
cui la cisti originava. Il decorso postoperatorio si è svolto in modo regolare ed il paziente è stato dimesso in
sesta giornata postoperatoria.
A dimostrazione della rarità dell’evento, abbiamo condotto una revisione della letteratura inglese. Ad oggi sono
stati riportati 9 casi, incluso il nostro, di rottura emorragica di cisti epatica semplice. La peculiarità del caso
da noi descritto, che lo rende unico in letteratura, è la
rottura in sede extraperitoneale. Analizzando i pochi dati
a disposizione, abbiamo cercato di individuare alcuni fattori di rischio per la rottura emorragica di una cisti epatica, riscontrando nell’età avanzata e nella terapia anticoagulante orale un ruolo predisponente.
Riguardo al management clinico, la revisione della letteratura conferma il ruolo primario della chirurgia, riservando la gestione conservativa ai soli pazienti compromessi e non suscettibili di intervento. La rottura emorragica di una cisti epatica è infatti un evento “life-threatening”, che può indurre rapidamente uno scompenso
emodinamico nel paziente. Secondo le nostre osserva-
6. Cheung FK, Lee KF, John W, Lai PB: Emergency laparoscopic
unroofing of a ruptured hepatic cyst. JSLS, 2005; 9(4):497-99.
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