Hereditárny
Digestive
endoscopy:
angioedém
case
akoreport
príčina bolestí brucha
doi: 10.14735/amgh2016422
Watermelon stomach as the first symp­tom
of liver cir­rhosis
„Watermelon stomach” jako první příznak cirhózy jater
M. Kukulska, I. Smoła, I. Gromny, E. Poniewierka, L. Paradowski
Department of Gastroenterology and Hepatology, Wroclaw Medical University, Poland
Sum­mary: Background: Gastric antral vascular ectasia (GAVE), so-cal­led watermelon stomach, is an uncom­mon cause of refractory
gastrointestinal bleed­ing or iron deficiency anaemia with unclear etiology. GAVE is as­sociated with a number of conditions such as por­
tal hypertension, chronic kidney disease and col­lagen vascular diseases. Most com­monly GAVE is dia­gnosed dur­ing gastroscopy. Red
antral stripes radiat­ing in linear ar­rays in the antrum are the most characteristic endoscopic feature. Furthermore, the clinical course
and histological changes are equal­ly important in the dia­gnostic proces­s. The treatment of GAVE is based on medical, endoscopical and
surgical methods. Case: We present an unusual case of GAVE which was the first symp­tom of liver cir­rhosis detected intraoperatively
dur­ing a gastric resection due to recur­rent blood los­s. The liver bio­psy taken confirmed the dia­gnosis. Before the surgery, numerous
laboratory tests, computed tomography scan, and abdomen ultrasound were performed which did not indicate liver disease.
Key words: argon plasma coagulation – bleeding – gastric antral vascular ectasia
Souhrn: Východiska: Cévní ektazie žaludečního antra (GAVE) patří mezi vzácné příčiny gastrointestinálního krvácení nereagujícího
na léčbu nebo anémie v důsledku nedostatku železa nejasné etiologie. GAVE je spojena s celou řadou stavů, jako je portální hy­
pertenze, chronické onemocnění ledvin a vaskulární kolagenová onemocnění. GAVE je nejčastěji dia­gnostikována při gastroskopii.
Typickým endoskopickým znakem jsou červené lineárně uspořádané pruhy na žaludečním antru. Pro dia­gnostiku je rovněž významný
klinický průběh a histologické změny. Léčba GAVE využívá farmaceutické, endoskopické a chirurgické metody. Případ: Představu­
jeme neobvyklý případ GAVE, která se objevila jako první příznak cirhózy jater, zjištěný intraoperativně při resekci žaludku z důvodu
opakované ztráty krve. Dia­gnóza byla potvrzena bio­psií jater. Před chirurgickým zákrokem byla provedena řada laboratorních vyšet­
ření, CT a ultrazvukové vyšetření břicha, které však onemocnění jater neodhalily.
Klíčová slova: argonová plazmová koagulace – krvácení – cévní ektazie žaludečního antra
Introduction
Gastric antral vascular ectasia (GAVE),
so-cal­led watermelon stomach, is an
uncom­mon but important cause of se­
vere acute or chronic gastrointestinal
blood los­s. It occurs more frequently in
women than in men, typical­­ly in mid­
dle- to old-aged females [1]. We do
not yet have enough knowledge on
this topic [2]. GAVE’s etiology remains
unclear. The im­m unological back­
ground might play a role in the cause
of watermelon stomach. GAVE is more
frequently present in such conditions
as systemic lupus, scleroderma, syste­
mic sclerosis, pernicious anaemia and
primary biliary cir­rhosis [3].
422
Gastroent Hepatol 2016; 70(5): 422– 425
The dia­gnosis is based on an endo­
scopic pattern and for doubtful cases
on histology. Tel­l tale watermelon
stripes are a pathognomonic endosco­
pic pattern [4] as GAVE is characterised
by longitudinal rows of ecstatic twisted
mucosal ves­sels in the antrum [5]. En­
larged submucosal ves­sels may erode
through the gastric mucosa, caus­ing
chronic blood loss and consequently
anaemia [6].
Case report
We describe an unusual case of GAVE.
The 66-year-old man with hyperten­
sion was admitted to the Department
of Gastroenterology and Hepatology
after be­ing observed for the previous
six months with anaemia, with a low­
est haemoglobin level of 6 g/ dL which
required many blood transfusions.
From gastroscopies performed dur­ing
hospitalisation, haemor­rhagic inflam­
mation of antrum and cardia was dia­g­­nosed. The colonoscopy was normal.
The blood test performed on admis­
sion revealed iron deficiency anaemia
with a haemoglobin level of 10.0 g/ dL,
a haematocrit of 32.9%, thrombocy­
topenia, normal hepatic function and
slightly prolonged prothrombin time.
The gastroscopy performed dur­ing
hospitalisation indicated a suspicion
of GAVE as a dia­gnosis, as the endo­
Watermelon
Hereditárny
stomachangioedém
as the first symp­
ako príčina
tom ofbolestí
liver cir­
brucha
rhosis
active hepatitis and advanced fibrosis
on the border of necrosis (incomplete
cir­rhosis).
Discus­sion
Fig. 1. Endoscopic appearance
of watermelon stomach – erosions
in antrum.
Obr. 1. Endoskopický obraz „watermelon stomach”– eroze v antru.
scopy revealed erosive gastritis, with
numerous erosions in cardia and an­
trum, with thicken­ing of the gastric
folds. Changes were also located with­
­in a diaphragmatic hernia.
Dur­ing the procedure, tis­sue sam­
ples were col­lected for histopatholo­
gical analysis. GAVE syndrome was
considered as a cause of recur­rent
anae­mia from gastrointestinal blood
los­s. Due to a GAVE dia­gnosis, the mu­
cosa was cauterised and ablated us­ing
argon plasma coagulation (APC). The
patient was discharged, with a recom­
mendation of tak­ing proton pump in­
hibitors and Sucralfate. After three
weeks the patient was admitted to the
clinic for a check-up. In blood morphol­
ogy the haemoglobin level was stable
(11.5 g/ dL), but there was no visible
improvement dur­ing the gastroscopy
as the images were similar to those
from three weeks before. A contrast
computed tomography scan of the abdo­
men revealed a thicken­ing of the pyloric
antrum wall and hepatosplenomegaly.
Due to recur­r­ing anaemia with a haemo­
globin level of 5.7 g/ dL and exhaust­
­ed conservative treatment options,
a few months later the patient under­
went a total gastrectomy and cho­
lecystectomy due to gal­lstones. Intra­
operatively, liver cir­rhosis was noticed,
and a bio­psy was taken. A histopathol­
ogy exam confirmed GAVE, chronic
Rider et al. were the first who described
GAVE in 1953. They analysed the case
of a patient with chronic iron-defi­
ciency anaemia. The gastroscopy re­
vealed changes in the antrum. It was
“fiery red with specific hyperthropic
mucosal changes”. Chronic inflam­
mation and an oedematous submu­
cosa with dilated veins were noticed
under the microscope [7]. The term
watermelon stomach was first re­port­
­ed by Jabbari et al. [8].
GAVE is typical­l y located in the
gastric antrum, although sometimes
and uncom­monly it may be noticed in
the cardia. Involvement of the proxi­
mal part of the stomach is also rare and
unusual [9,10], but in our case it actual­
­ly occur­red and was located with­­in
a diaphragmatic hernia.
It is estimated that GAVE causes
up to 4% of non-variceal upper gas­
trointestinal bleeding, as shown in
a prospective study of 744 patients;
31% of these also had portal hyper­
tension (PHT). Patients with coexist­
ing GAVE and PHT have more dif­
fuse gastric angiomas in endoscopic
view [11].
Watermelon stomach is more com­
mon in certain health conditions such
as hepatic cir­rhosis, chronic renal fail­
ure, hypertension, chronic pulmonary
disease and diabetes. Furthermore, it
is also related to some autoim­mune
disorders such as Raynaud’s pheno­
menon, rheumatoid arthritis, polymy­
algia rheumatica, primary biliary cir­
rhosis, and systemic sclerosis – both
dif­fuse and limited [9,12]. Among the
patients with GAVE antinuclear anti­
bodies, anti-centromere and anti-RNA
helicase II were detected [13]. There
is a theory that states that those an­
tibodies could cros­s-react with pro­
teins in the mucosal and submuco­
sal layer of the stomach [2]. Various
studies show that there are some risk
factors of GAVE. For instance, hyper­
gastrinaemia, proliferation of neuro­
endocrine cel­ls, changes in hormone
levels of prostaglandin E2 (PGE2),
5-hydroxytryptamine and vasoactive
intestinal polypeptide may be a cause
of the development of GAVE [14]. The
loose con­nection between the distal
gastric mucosa and muscularis externa
may be one of the pos­sible causes of
GAVE [15]. Some authors suggest that
strong peristaltic movements may re­
sult in prolaps­ing of the antral mucosa.
It can lead to blood ves­sel obstruction
result­ing in secondary fibromuscular
hyperplasia and vascular ectasia [16].
About 30% of patients with GAVE
have liver disease. The prevalence of
GAVE in liver cir­rhosis is nearly 2– 3%
of patients [17– 19]. In our patient the
cause of GAVE was at first unknown
as he did not suf­fer from any disease
which might predispose to water­
melon stomach. GAVE was the first
sign of identified intraoperatively liver
cir­rhosis, which was confirmed by his­
topathological exam of liver bio­psy.
Chronic anaemia occurs frequently
in cir­rhotic patients [18], so it is impor­
tant to perform a dif­ferential dia­gnosis
and identify the cause to administer
appropriate treatment. Patients can
develop both GAVE and portal hyper­
tensive gastropathy (PHG) but these
are two dif­ferent conditions which can
cause gastrointestinal bleed­ing among
patients with liver failure [20]. GAVE is
located in the antrum, and PHG in the
fundus and body of the stomach [19].
PHG is more com­mon among liver
cir­rhosis patients with a prevalence
of 20– 80% and develops in the back­
ground of PHT [21]. The hepatic ve­
nous pres­sure gradient is related to the
presence and severity of PHG [22].
The pathophysiology of GAVE is still
unclear, nevertheless it is indisputable
PHT is not involved in GAVE develop­
ment, as it has been reported to occur
in non-cir­rhotic patients. It can be ab­
sent in up to 70% of patients. The re­
Gastroent Hepatol 2016; 70(5): 422– 425
423
Hereditárny angioedém
Watermelon
stomach asako
the príčina
first symp­
bolestí
tom brucha
of liver cir­rhosis
Fig. 2. Endoscopic appearance
of watermelon stomach – erosions
in cardia.
Obr. 2. Endoskopický obraz „watermelon stomach” – eroze v cardiu.
duction of PHT also changes the course
of the disease [23]. Furthermore, it has
been reported that liver transplanta­
tion, despite constant PHT, genera­
tes disappearance of the antral vascu­
lar lesion. The relationship of GAVE
with liver insuf
­fi ciency remains unclear
but it seems to have a more signifi­
cant role. It is pos­sible that liver failure
changes the metabolism of as yet un­
identified substances [19].
Tel­ltale watermelon stripes are the
pathognomonic endoscopic pattern for
GAVE [4], while a PHG endoscopy re­
veals snakeskin mucosa with dif­fuse red
or brown spots, usual­ly in the funds or
gastric body, but sometimes a bio­psy
is required to dif­ferentiate GAVE from
PHG [24]. In PHG, histology reveals di­
lated submucosal and mucosal veins,
along with ectatic capil­laries without
inflam­mation or thrombi [21]. In GAVE,
the presence of intravascular fibrin
thrombi, mucosal inflam­mation, fibro­
muscular hyperplasia of lamina propria,
epithelial regeneration, and mucosal
architectural distortion is shown [25].
In the treatment of GAVE we use
pharmacotherapy, endoscopic pro­
cedures and, in some cases, also sur­
gery. However, refractory GAVE still
presents a therapeutic chal­lenge. The
most com­mon surgical type of GAVE
treatment is antrectomy. There are
no cases of recur­rence of bleed­ing in
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Gastroent Hepatol 2016; 70(5): 422– 425
the postoperative period observed.
The disadvantage of this surgical ap­
proach is a high risk of mortality and
morbidity. Portacaval shunts are not
recom­mended in the treatment of
GAVE [5]. The gold standard is endo­
scopic treatment. One of the most po­
pular endoscopic approaches is Neo­
dymium:yttrium-aluminium-garnet
(Nd:YAG) laser or APC. The others are
endoscopic band ligation, sclerother­
apy with polidocanol [6] and radio­
frequency ablation [26]. Another en­
doscopic method used to treat GAVE
might be cryotherapy. It seems to
provide encourag­ing results but lar­
ger, prospective studies are still
needed [27].
The treatment of PHG is based on
pharmacotherapy to reduce hepa­
tic venous pres­sure gradients. The
first line of treatment is non-selective
β-blockers [19]. The ef
­fi ciency of hormo­
nal therapy – estrogen-progesterone,
a long-act­ing somatostatin analogue – octreotide, methylprednisolone cyclo­
phosphamide was also as­ses­sed. None
of them appear capable of ef­fectively
control­l­ing GAVE-related bleeding [2].
Conclusion
GAVE can be a rare cause of chronic
gastrointestinal bleed­ing which we
should consider when we exclude other
causes. The dia­gnostic process and
therapeutic management of GAVE,
however, still remains a chal­lenge, as
endoscopic images are not always dis­
tinctive. GAVE can be as­sociated with
PHT, chronic kidney dis­ease and col­
lagen vascular diseases.
One third of patients with liver dam­
age have detected GAVE, therefore
it is important to consider that a dif­
ferential dia­gnosis of PHG may also
coexist. Both of these conditions
can cause anaemia. The treatment
of GAVE is often inef­fective, despite
us­i ng pharmacotherapy and endo­
scopic procedures; recur­rent bleed­ing
has been observed and in some cases
a gastrectomy is needed.
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liver cir­
brucha
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The authors declare they have no poten­
tial conflicts of interest concerning drugs,
products, or services used in the study.
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do bio­­­­medicínských časopisů.
Submitted/Doručeno: 28. 7. 2016
Accepted/Přijato: 18. 9. 2016
Monika Kukulska
Department of Gastroenterology
and Hepatology
Wroclaw Medical University
Borowska 213
50-556 Wrocław
Poland
[email protected]
18. ENDOSKOPICKÝ DEN IKEM
7. PRAŽSKÝ ENDOSKOPICKÝ DEN
Prof. Dr. Alexander Meining
Department of Internal Medicine I
Ulm University, Ulm, Germany
14. 2. 2017 IKEM – Praha
Výkony: polypektomie, ligace varixů, submukózní disekce, ERCP, EUS, G-POEM,
submukózní tunelová resekce, konfokální endomikroskopie, radiofrekvenční ablace,
cholangioskopie, bariatrická endoskopie
Hands-on training 13. 2. 2017
Registrace: [email protected]
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