# Hemoperitoneum in a Cirrhotic Patient Due to Rupture of Retroperitoneal Varix

Authors: Igor Rafael Sincos; Grace Mulatti; Sheila Mulatti; Ilana Cristina Sincos; Sergio Q. Belczak; Valdir Zamboni
Journal: HPB Surgery
 (2009)

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## Abstract

The rupture of retroperitoneal varices is a rare and catastrophic complication of portal hypertension. We describe a case of this nature, the first in Brazilian medical literature, and also reviewing all previous 34 cases. We systematically analyzed all therapeutic approach and propose a management algorithm for diagnosis and treatment of this lethal condition. The majority of the patients presented with abdominal pain, distention and hypotension, and developed hemorrhagic shock. Rupture of retroperitoneal varices can be properly managed if an early diagnosis is made and surgery is performed promptly, which is the only effective treatment. Arteriography should be used when the suspicion is of rupture of hepatocellular carcinoma.


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## Body

## 1. Introduction

The first case of rupture of retroperitoneal varices, a rare and catastrophic
complication of portal hypertension, has been reported in 1958 [1]. According to our research over Medline and PubMed, to date, only 34 similar
cases have been described with high mortality rates even nowadays [1–38]. We used hemoperitoneum and varices as keywords for search. The aim of this
article is to describe a case of this nature, the first in Brazilian medical
literature, and also reviewing all previous cases. We systematically analyzed
all therapeutic approaches and propose a management algorithm for diagnosis and
treatment of this lethal condition. Rupture of retroperitoneal varices can be
properly managed if an early diagnosis is made and surgery is performed
promptly.


## 2. Case Report

A 51-year-old woman entered the Emergency Department of Universitary Hospital of the
University of Sao Paulo (USP) in March 2006 presenting with abdominal pain for
two days, associated with nausea and vomits. She also reported abdominal
distention for the last fourteen days.Her past
medical history showed a chronic abuse of alcohol leading to liver cirrhosis
associated to Hepatitis B. She had been under medical follow-up with a
clinician from 2001 to 2004, when she abandoned medical care.Her
physical examination was remarkable for an ill-appearing, pale, jaundiced, and
dyspneic patient. She had a heart rate of 100 beats/min and a systolic blood
pressure of 95 and diastolic of 65 mm Hg. Abdominal examination revealed
diminished bowel sounds, a slight distention, and diffuse tenderness during
palpation, with no guarding. Admission laboratory values showed hemoglobin
level of 6.4 g/dL. MELD score of 24, Child-Pugh grade C.An
endoscopy was carried out, which showed a healed distal esophageal ulcer, a
hiatal hernia, and erosive gastritis of the body and antrum. There were no signs
of esophageal varices.A few
hours after entering the Emergency Department she developed severe hypotension
of 70/40 mm Hg, Glasgow coma scale 14, tachycardia of 125 beats/min. She
underwent volume resuscitation with no sustained response. Treatment for
Spontaneous Bacterial Peritonitis was initiated with Ceftriaxone. Additional
treatment with norepinephrine was started as she remained hypotensive even
after the continuous infusion of volume. She was then transferred to the
intensive care unit. She had progressive hemodynamic instability, abdominal
distention, and altered mental status, requiring endotracheal intubation.At this
time a surgeon was requested to examine the patient. A paracentesis was carried
out; the peritoneal fluid was hemorrhagic with a hematocrit of 12%. Laboratory
values at this moment were hemoglobin level of 3.8 and INR of 7.29. She was
then transfused with packed RBCs and plasma. Reaching hemodynamic stability she
underwent an exploratory laparotomy.About 5 L of blood were evacuated from the peritoneal cavity. A ruptured
retroperitoneal varix was found to be the cause of bleeding, next to the
mesenteric root. Direct ligation of the vessel led the bleeding to stop (Figure1). The liver was cirrhotic; an exuberant collateral circulation was seen on
the retroperitoneum and on the abdominal wall, with canalization of the
Umbilical vein, with an 8 mm diameter. The Retzius vein was identified in both
paracolic gutters, with a lot of collaterals. A liver biopsy was made. Later
histopathological evaluation revealed steatohepatitis grade IV (alcoholic and
nonalcoholic).Figure 1
Direct ligation of the vessel in retroperitoneum.Returning
to the intensive care unit the patient was massively transfused with packed
RBCs and plasma for anemia and coagulopathy. She continued to be
hemodynamically unstable associated with renal and hepatic failure. On the sixth
posoperatory she died of multiple organ dysfunction syndrome.


## 3. Discussion

Trauma
and nonmalignant gynecological conditions account for more than 90% of
intraperitoneal hemorrhages [2]. The main cause of hemoperitoneum in
women is the rupture of an ectopic pregnancy; in men, the major cause is the
posttraumatic rupture of the liver or spleen [2]. The vascular causes
are also relevant, as the Aorta Aneurysm, rupture of viscera, and hemorrhagic
pancreatitis. Inflammatory and hematological disorders rarely manifest as
hemoperitoneum.In
cirrhotic patients with ascites the intraperitoneal bleeding occurs most of the
times due to structural lesions such as hepatocellular carcinoma or ovary
cancer and rupture of intraperitoneal varices [3].The
intraperitoneal varices rupture is a rare event, whose incidence unknown, and
it is related to severe portal hypertension. We believe that the real incidence
of this pathology is much superior than the 34 cases described in literature due
to misdiagnosis. It also appears in patients with terminal liver disease,
mostly in a fulminate way.Portal
hypertension leads to the development of portosystemic shunts in well-defined
anatomic sites. The most acknowledged sites include the gastroesophageal veins
connecting the azigohemiazigos system, the hemorrhoidary veins from the
inferior mesenteric vein, communicating with the tributaries of the Internal
Iliac Vein and the Umbilical and periumbilical veins draining to the left
Portal Vein and to the epigastric veins of the anterior abdominal wall. The
recanalization of the Umbilical vein is known as Cruveillier-Baumgarten
Syndrome [1, 4]. There are also shunts of the Retzius veins connecting
the Colic veins with the lumbar and the lower intercostals veins; pancreatic
veins connecting the splenic vein and the left renal vein; numerous venous
canals communicating the liver with the diaphragm (Sappey veins) [1].There
are 34 cases of intraperitoneal bleeding due to rupture of varices described in
literature, as shown in Table1. Including our patient, 27 were men and 8 were
women. The age onset was 21 to 76 years old (average of 48.8). Only one case
reported in literature was of a noncirrhotic patient. Table 1 summarizes
the presentation, diagnosis, treatment, and results of the cases described in
literature.Table 1
Summary of Presentation, Management and Results of all 35 cases.
Patientsn: 35
(%)Signs and symptomsHypotension or  shock24
(68.6%)Abdominal pain23
(65.7%)Abdominal  distention15  (42.8%)DiagnosisParacentesis22  (62.8%)Arteriography6  (17.1%)Source  of bleeding*Umbilical veins7  (20%)Retzius veins6  (17.1%)Retroperitoneal  varices5  (14.3%)Other  intraperitoneal sources19  (54.2%)Treatment#Variceal or vein
ligation27 (77.1%)Clinical
management6 (17.1%)Portocaval
shunt3 (8.6%)Arteriography
(embolization)1 (2.8%)OutcomeDeath23 (65,7%)Survival12 (34,3%)Some* patients had more than one source of bleeding.#Some patients were submitted to more than one
treatment.The
majority of the patients presented with abdominal pain, distention and
hypotension, and developed hemorrhagic shock. The diagnosis was established by
paracentesis, angiography, ultrasound Doppler, and tomography. Even so, the
diagnosis was confirmed only by laparotomy.The
hemoperitoneum diagnosis is confirmed by paracentesis when the Ht> 5%. The
paracentesis was important in the diagnosis and surgical indication is most of
the cases. Only two cases out of ten who survived did not undergo the
paracentesis [5, 6]. In one of them, the diagnosis was suggested via
tomography and confirmed at laparotomy [5].The
angiography was used as an attempt to achieve the diagnosis in 6 cases of
hemoperitoneum by varices inside the abdomen [7–12]. The source of the
bleeding was identified only in one patient. However, this patient past away
probably because of recurrent bleeding [8]. In one case the angiogram
may have anticipated the recurrent bleeding and finally his death [9].The only
effective treatment was surgery. None of the 7 patients treated in a
nonchirurgical basis survived. Twenty eight were operated, twelve survived.
The global mortality rate was 65.7%. And for the patients submitted to surgery
it was of 57.1%. The causes leading of death were uncontrollable or recurrent
bleeding, liver failure, kidney failure, heart failure, and aspiration of blood
from ruptured esophageal varices.Out of
28 cases that underwent surgery, in twenty six the ligation of the bleeding
vessels was successful, and eleven survived the after surgery period. In two
cases who underwent surgery, the ligation was not possible [12, 13], and
the patient died of bleeding, even after the use of a portocaval shunt as an
attemptive to relieve the portal hypertension [12]. Only one patient
survived after a porto-systemic shunt [14].The
management of the bleeding from intra-abdominal varices is difficult since
there are no randomized trials due to the rareness of this situation [15]. However, this condition seems to be underestimated, probably because most of
the patients present tense ascitis and hepatic disease in a terminal state
leading rapidly to death [2].The
patients' survival rate seems to be related to three important facts: the
patient's functional hepatic reserve, the importance of the hemorrhagic shock
in its presentation, and the early operative intervention and control of the
bleeding source [3, 16].The
first challenge in the management of these cases is in the differential
diagnosis of acute hemorrhagic abdomen in a cirrhotic patient. We suggest a flowchart
based on the analysis of all the published cases related to intraperitoneal
varices and a review of the articles related to the other causes of
hemoperitoneum in cirrhotic patients (Figure2).Figure 2
Fluxogram for diagnosis
and treatment of hemoperitoneum in cirrhotic patients. SBP: Secondary bacterial
peritonitis; Ht: Hematocrit; US: Ultrassonography; CT: Computerized abdominal
tomography; HCC: Hepatocellular carcinoma; TIPSS: Transjugular intrahepatic
portosystemic shunt.The
paracentesis with Ht over 5% is a precise indicator of intra-abdominal bleeding
that can dimish the risk of unnecessary laparotomies [2]. It may be
repeated in another site to exclude a punction accident and has rarely been to
relate to hemorrhagic complications [16].Once the
bleeding in a cirrhotic patient was identified, the diagnostic orientation is
made on differing HCC rupture, bleeding intra-abdominal varices, vascular
causes such as aorta’s aneurism, and gynecological causes.We
suggest checking the dosage level of HCG in women, followed by Abdominal Duplex
Scan in both sexes as the first diagnostic step. Duplex scan can provide
information on the Aorta and its branches, the abdominal collateral
circulation, the patency of the Portal Vein, hepatic nodules and tumors, and the
ovaries.Computerized
tomographic scanning was suggested by Bataille et al. [15] and Goldstein
et al. [5] as the first diagnostic approach for excluding rupture of
hepatocellular carcinoma and all other causes. It is, though, more expensive
than the Duplex Scan, and it is not always available in all emergency services.
On the other hand, it provides more detailed information on other acute
hemorrhagic abdomen.Arteriography
has proved to be an inefficient investigation for diagnosis and treatment of
retroperitoneal bleeding varices. It postpones the surgical treatment [14], the only one that has been effective in these cases. Thus, it is very important
to notice that arteriography has a fundamental role in the treatment of the
rupture of hepatocellular carcinoma, which is the main differential diagnosis
as mentioned before.The
fundamental treatment of variceal bleeding is the ligation of the vessel.
Nevertheless, the Surgical Portosystemic Shunt or the Transjugular Intrahepatic
Portosystemic Shunt (TIPSS) must be considered for selected patients.In the
operation room, the decision of performing a Portosystemic shunt must take into
consideration the patient's clinical condition and the time needed to perform
the shunt. In unstable patients and with little hepatic functional reserve we
strongly suggest not increasing the surgical time. However, multiple bleeding
varices or the possibility of a new bleed should be analyzed in order to decide
if the shunt must be performed.TIPSS
was not performed in any of the reported cases. Nevertheless, the way we
understand its use for treatment of gastroesophageal varices can help us in
treating variceal bleeding from the retroperitoneum. Therefore TIPSS can be used
mainly for the patient's postoperatory when there is suspicion of a new
bleeding, or for diminishing the portal tension in selected patients serving as
a bridge for liver transplantation. Before deciding if the TIPSS will be
performed, one must be aware of its contraindications and complications as bleeding,
perforation of the liver capsule, and encephalopathy, among others.


## 4. Conclusion

Bleeding
intraperitoneal varix is a rare complication of portal hypertension, but
carries a high mortality rate. Nonetheless, the physician must know this
condition, as the clinical suspicion is the only way of establishing an early
diagnosis and indicating surgery at once, which is the only effective
treatment.We
suggest a flowchart to optimize the treatment of the acute hemorrhagic abdomen
in the cirrhotic patient. Paracentesis followed by ultrassonography with Duplex
Scan or Computerized Tomography seems to be the most important procedure for
establishing the correct diagnosis of abdominal pain, distention, and shock. Thereafter,
surgery must be performed as soon as possible in case of ruptured varices.
Arteriography should be used when the suspicion is of rupture of hepatocellular
carcinoma.



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*Source: 240780-2009-04-23.xml*