Pylephlebitis, also called portal venous septic
thrombophlebitis, is a rare pathological entity, characterized by
suppurative thrombophlebitis of the porto-mesenteric venous system [1].
The incidence is not adequately clarified; however, a study from 2009 reports
it at 2.7 per 100,000 people, with a preference to the male sex [2, 3].
The clinical manifestations are not very specific and can manifest
itself asymptomatically or as an acute abdomen [4]. Any organ whose venous
drainage ends in the portal system is susceptible to generating
pylephlebitis. Thus, we have diverticulitis, appendicitis, inflammatory
bowel disease, pancreatitis, infectious enteritis,
intestinal perforation and neoplasms as potential sources [5]. The
treatment, currently not standardized, is based on antibiotic therapy,
surgical control of the abdominal infectious focus and early
anticoagulation. Over the years, each of these has been questioned,
resulting in the judicious use of these therapies [6-9]. The objective of
this report is to present the clinical case of an asymptomatic
pylephlebitis as an incidental diagnosis by tomography.
Case report
A 50-year-old
patient was admitted to the emergency room, referred from the radiology service
due to the finding of suggestive signs of an abdominal
inflammatory process compatible with acute appendicitis, and septic
thrombophlebitis in the upper mesenteric and right ileocolic veins, seen
on the contrast abdominal-pelvic multislice spiral tomography (fig. 1).
The patient was undergoing an imaging study as a result of an
anatomopathological report of a gastric biopsy, which led to a suggestive
lesion of infiltrating tubular adenocarcinoma. There were no other
comorbidities or previous surgeries.
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Fig. 1. Contrast abdominal MSCT on admission. Coronal and
axial images show upper mesenteric vein with non-contrast capturing hypodense image,
suggestive of thromboembolism with alteration of mesenteric fat density
The review of the
emergency clinical history found that 15 days before, he presented with
colicky abdominal pain of sudden onset and moderate intensity, associated
with nausea, vomiting and mucous diarrhea; being treated as an acute
gastroenteroco-litis with antibiotic therapy (ciprofloxacin) and an-tispasmodics
(scopolamine) orally for 5 days. The abdominal pain progressively decreased
within 7 days, when he arrived at the oncologist's outpatient
clinic, without any pain; however, he had recurrent fevers up to
38.5°C daily, which subsided with Paracetamol 1 gr orally.
The physical
examination disclosed a soft, de-pressible abdomen, with mild pain on deep
palpation in the right lower quadrant, no palpable masses, and negative Mc
Burney and Blumberg signs. The laboratory tests found on admission are
detailed in table. The treatment implemented was begun based on
the diagnosis of pylephlebitis secondary to complicated acute appendicitis.
Antibiotic therapy was started with Piperacillin-Tazobactam 4.5gr IV every
6 hours, analgesia with Metamizole 2gr IV C/8h. Enoxaparin 60UI
subcutaneously every 12 hours was instituted and he was taken to the
operating theater for an exploratory laparotomy via a midline
infraumbilical incision. The intraoperative findings revealed a retroileal
appendicular mass with a 8 x 1cm cecal appendix, perforated in its distal
third and necrotic in its entire extension with an appendicular base
in good condition. A 5cc abscess was contained within the
mesoappendix. Histopathology of the appendix reported acute suppurative appendicitis.
Laboratory
evolution
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On the first
postoperative day a diet was started, the surgical wound was without signs of
phlogosis or bleeding, the abdomen was soft, without peritoneal signs
and active ambulation was begun, with only mild pain in the surgical site.
Piperacylin-Tazobactam 4.5gr IV was continued every 6 hours for 7 days
and at discharge, laboratory results obtained were within normal
limits (table) with tomographic findings reported as pericecal inflammatory
sequelae and other organs evaluated without significant
alterations (fig. 2). Rivaroxaban 20mg Q/24h x 30 days, Ciprofloxacin
500mg Q/12h x 3 more weeks, Paracetamol 1gr Q8h x 4 days are indicated; he
returned to his outpatient visit 10 days later, without discomfort,
and was referred back to his oncologist to continue with his gastric
cancer treatment.
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Fig. 2. Contrast abdominal MSCT control. Coronal and axial
images show contrast capturing upper mesenteric vein, compatible with permeable
vessel
Discussion
Pylephlebitis was
described for the first time by Waller, in 1846, with the findings of liver
abscesses in autopsies, considering it as an unusual complication of
intra-abdominal infections [10]. In spite of the small number of cases, he
reported a high mortality; from 30 to 50% of the cases [6, 11, 12].
Currently, sigmoid diverticulitis is the most frequent
etiology (30%), followed by appendicitis (19%), inflammatory bowel
disease (6%), pancreatitis (5%), infectious enteritis (4%), intestinal
perforation and neoplasms (6%) [5].
A cohort of 95
patients identified the right portal vein as the most frequent site of
thrombophlebitis, in 33% of the cases followed by the inferior mesenteric
vein as the least common site with 8%. Septic thromboembolism in the
superior mesenteric vein was documented in the literature with 30.5% [13].
The clinical
manifestations are characterized by being ambiguous and not very specific; from
the asymptomatic character, to the presence of abdominal pain (80.5%),
fever (31.7%), muscular defense, nausea, diarrhea, anorexia or jaundice, with
an average duration of 12.34±11.23 days [4, 13, 14]. Our
patient described here presented with a 15-day clinical evolution, of
regressive course and low intensity from the beginning, leading to a
diagnostic error. The laboratory findings are also atypical, finding
leukocytosis (80%), positive blood cultures (44-88%), alteration of
hepatic enzymes (40-69%), elevation of total bilirubin (55%) and elevation
of CRP [4, 13]. The organism isolated most frequently in blood cultures
is Bacteroides fragilis over Escherichia coli and Streptococcus sp,
resulting in bacteremia in 23 to 88% of patients [12]. Imaging studies
have an important role and it is necessary to be attentive to their
findings. The portal venous doppler ultrasound is able to identify portal
venous thrombosis and, in association with the CT scan, are able to
demonstrate the possible intra-abdominal septic focus and its
affectation to the porto-mesenteric venous system. Both techniques
facilitate and prioritize early diagnosis [15].
Currently there
are no established protocols for the management of pylephlebitis, however,
antibiotic therapy, anticoagulation and surgical control of
the septic focus are recommended [8]. Antibiotic therapy should cover
gram-negative and anaerobic flora utilizing metronidazole, gentamicin,
piperacillin-tazobactam, imipenem-cylastatin, ceftizoxime and ampicillin
for a period of 4 weeks in patients without liver abscess, and for 6 weeks
if necessary [7]. The most controversial point of the therapeutic algorithm is
undoubtedly anticoagulation. L. Naymagon et al., state that it should be
used early whenever possible since it produces resolution rates of 58% vs
21% [16]. Likewise, T.C. Hall et al. describe the synergy with antibiotic
therapy in the recanalization process [17].
On the other hand,
N. Baril et al. suggest its use in selected patients diagnosed with
pylephlebitis who meet criteria of hypercoagulability such as
neoplasia or coagulation factor deficit. Our patient met the first described
criterion and the diagnosis of pylephlebitis, thus justifying the use of this
strategy [6, 18]. Upon discharge, Rivaroxaban VO was prescribed
orally for 1 month, although some recommend Apixaban because of its
greater range of safety in patients with atrial fibrillation and in the
prevention of stroke over vitamin K antagonists K [19-21]. The surgical
strategy for draining the septic focus is also not well clarified. Cases have
been reported successfully treated by laparotomy or laparoscopy, both emergently
and deferred; but all agree that intravascular septic emboli should not be
delayed [22, 23].
Conclusion
This report
illustrates a rare pathological finding, with potentially high morbidity and
mortality complications, pylephlebitis. The poor specificity of the
clinical picture points out that the imaging studies must be scrutinized. Acute
appendicitis is the leading cause of surgical acute abdomen
worldwide, so its presence should be suspected when there
is thrombosis of the porto-mesenteric venous system. An incidental
radiological diagnosis was made in our patient.
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