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.
The first case of rupture of retroperitoneal varices, a rare and catastrophic
complication of portal hypertension, has been reported in 1958 [
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 (Figure
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.
Trauma
and nonmalignant gynecological conditions account for more than 90% of
intraperitoneal hemorrhages [
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 [
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 [
There
are 34 cases of intraperitoneal bleeding due to rupture of varices described in
literature, as shown in Table
Summary of Presentation, Management and Results of all 35 cases.
Patients | |
---|---|
Hypotension or shock | 24 (68.6%) |
Abdominal pain | 23 (65.7%) |
Abdominal distention | 15 (42.8%) |
Paracentesis | 22 (62.8%) |
Arteriography | 6 (17.1%) |
Umbilical veins | 7 (20%) |
Retzius veins | 6 (17.1%) |
Retroperitoneal varices | 5 (14.3%) |
Other intraperitoneal sources | 19 (54.2%) |
Variceal or vein ligation | 27 (77.1%) |
Clinical management | 6 (17.1%) |
Portocaval shunt | 3 (8.6%) |
Arteriography (embolization) | 1 (2.8%) |
Death | 23 (65,7%) |
Survival | 12 (34,3%) |
#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
The
angiography was used as an attempt to achieve the diagnosis in 6 cases of
hemoperitoneum by varices inside the abdomen [
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 [
The
management of the bleeding from intra-abdominal varices is difficult since
there are no randomized trials due to the rareness of this situation [
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 [
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 (Figure
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 [
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. [
Arteriography
has proved to be an inefficient investigation for diagnosis and treatment of
retroperitoneal bleeding varices. It postpones the surgical treatment [
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.
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.