Apandisit mi, ailevi Akdeniz ateşi atağı mı: nasıl ayırt edebiliriz?

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Introduction
Familial Mediterranean fever
(FMF) is one of the most common
causes of acute abdominal pain in
children among the Mediterranean
countries (1-3). Because of similar
clinical and laboratory findings, it
may be confused with appendicitis
(2,4). There is no article about the
role of computed tomography (CT)
on differential diagnosis of acute
abdominal attack of FMF and appendicitis in children. We herein report aCilt 49 · Sayý 1 · Gülhane TD How to distinguish? · 59
diameter tubular structure arising
from medial side of cecum. There is a
calcific opacity more than 7 mm in
diameter, in the proximal luminal
side of the tubular segment (Figure
1).
The patient underwent the operation with the provisional diagnosis of
perforated appendicitis. Exudative
free fluid, multiple abscess focuses
between intestinal segments and perforated appendix were observed at the
exploration. Then appendectomy was
performed. Ceftriaxone and metronidazole were administered postoperatively for 7 days. The patient was discharged at the postoperative 7th day
uneventfully.
Discussion
FMF is an autosomal recessive
inherited disease characterized by
recurrent attacks of fever and pain
secondary to polyserositis, mainly of
the abdomen and joints (3,4). FMF
resulting from mutation in the gene,
which is located in the short arm of
chromosome 16, is one of the most
common causes of acute non-surgical
abdominal pain in children among
the Mediterranean countries (1-3).
Abdominal attacks are characterized
by severe abdominal pain with fever
and abdominal tenderness in 95% of
patients (2,4-6). Abdominal pain with
peritoneal signs is the first clinical
presentation in 50% of the patients.
Laboratory findings include leukocytosis, increased level of acute phase
reactants (2,4,5). Approximately twothird of FMF patients undergo surgical intervention with the provisional
diagnosis of appendicitis in any period of life and the appendix is found
normal in most of the cases (4,7).
Acute appendicitis due to obstruction of the lumen by fecaliths, lymphoid hyperplasia, foreign bodies and
parasites is the most common cause
of emergency abdominal surgery in
childhood (8). It is also characterized
by lack of appetite, abdominal pain,
vomiting, abdominal tenderness and
rigidity. Laboratory findings are similar to laboratory findings, which are
detected in FMF. If it is not diagnosed timely, this condition can manifest with some complications such
as perforation, periappendicular abscess and peritonitis.
It may be difficult to differentiate
FMF and other surgical acute abdominal conditions during abdominal
attack, especially for acute appendicitis due to similar symptoms and laboratory findings (5,7). Because of nonspecific clinical and laboratory findings such as leukocytosis, proteinuria
and leukocyturia, our case was followed-up with the diagnosis of urinary tract infection and FMF at the
Department of Pediatrics. To hinder
complication, urgent exploratory laparotomy is recommended by some
centers in patients with acute abdominal attack of FMF (4,5). Some authors recommend elective laparoscopic appendectomy to prevent misdiagnosis and unnecessary emergency
surgery (4,7). In this respect preoperative radiological evaluation gets
importance utmost to prevent unnecessary surgical approach in FMF and
may be helpful for accurate diagnosis
and treatment.
Ultrasonography is the generally
preferred diagnostic radiological tool
because of availability. Although
there are several clinical trials and
articles about the value of ultrasonography in the diagnosis of acute abdominal conditions in children, it is
not useful in some patients; such as
when the appendix is perforated or
retrocecal. In addition, some technical problems such as obesity, abdominal tenderness, increased bowel gas,
and guarding are other difficulties
(9,10). Also the accuracy of the diagnosis depends on the radiologist (8-
10). In these complex cases appendiceal CEAT scan is more helpful
than ultrasonography for evaluating
patients with acute abdominal attack
(9). It is fast, cost effective, has a high
positive predictive value and identifies abscess and phlegmon better. In
such cases the findings on CEAT
scan are blind-ending tubular segment in connection with cecum with
a diameter of more than 6 mm and
thickened wall containing contrast
material. Heterogenity and hypertrophy in surrounding adipose tissue are
visible that indicate severe inflammation. Periappendicular fluid collection and intraabdominally free fluid
can be seen in perforated cases. In
some cases fecal impaction may be
visible in the lumen of appendix
(6,8). The diagnostic accuracy of CT
has been reported to range between
93% to 98%. This rate was reported as
71% to 97% for US (8).
In our patient the clinical findings
were similar to both of acute abdominal attack of FMF and appendicitis.
Ultrasonographic findings were nonspecific and not helpful. So we decided to perform CEAT to make definitive diagnosis. Findings were almost
similar with the literature findings,
which are aforementioned.
As a conclusion, some FMF cases
with acute abdominal attack may be
confused with acute abdominal conditions, and some FMF cases actually
have an acute abdominal condition,
such as an acute appendicitis simultaneously as in our case. Although ultrasonography is a valuable diagnostic
tool for the differential diagnosis of
Figure 1. Computed tomographic appearance
of the fecalith in the appendix vermiformis60 · Mart 2007 · Gülhane TD Atabek ve ark.
abdominal attack in FMF and acute
appendicitis, it is not effective in some cases who do not have specific
ultrasonographic findings. In these
cases CEAT may be very useful for
the evaluation of acute abdominal
conditions.