Keywords
stomach - hemorrhage - embolization - intra-arterial therapy
Introduction
Gastric antral vascular ectasia syndrome or GAVE syndrome is a rare condition of the
stomach, which is associated with severe acute or chronic upper gastrointestinal bleed.
The exact pathogenesis of the condition is poorly understood; however, the condition
has been linked to various medical conditions.
This condition was first described by Rider et al in 1953.[1] Wheeler et al[2] reported its characteristic endoscopic features. Based on the distinctive endoscopic
features, the term watermelon stomach was coined by Jabbari et al.[3] Watermelon stomach endoscopically resembles a pattern similar to the watermelon’s
exterior, the prominent erythematous stripe-like pattern radiates from the pylorus
to the antrum resembling the similar design. GAVE syndrome is often misdiagnosed as
either antral gastritis or portal hypertensive gastropathy.[4] Treatment of the condition can be challenging and mainly includes three options
including: (1) medical management, (2) endoscopic management, and (3) surgical management.
Scanty case reports of the use of various pharmacologic therapies in the management
of the condition have been reported. Surgical resection is the definitive option available,
but it comes at the cost of significant morbidity and mortality rates.[4] Endoscopic management is the main conservative treatment option available.[5] Complete eradication of the condition requires two to four sessions based on the
site, extent, and number of lesions. Up to 40% of postprocedure recurrence has been
reported in some studies.[6] We report a unique experience of performing an intra-arterial embolization in a
case of GAVE presenting with uncontrolled bleeding even after multiple rounds of endoscopic
management.
Case Report
A 63-year-old male patient, who is a known case of chronic liver disease (etiology:
cryptogenic) since 2011, presented to the emergency department with complaints of
two episodes of hematemesis and melena. The patient had several such incidents in
the past for which esophageal variceal ligation and argon plasma coagulation (APC)
was performed on several occasions.
On examination, patient’s vitals were stable with evidence of pallor, pedal edema,
and ascites. The patient had a Child–Pugh score B with a MELD (Model for End-Stage
Liver Disease) score of 15. Supportive measures were taken, and an emergency upper
gastrointestinal endoscopy was performed considering bleeding varices as the cause
for the patient’s symptoms. Endoscopy showed eradicated esophageal varices, mild portal
gastropathy in fundus and body, nodular erythematous streaks in the antrum extending
to the proximal body, and lesion in the prepyloric region and the antrum with active
ooze. Duodenum showed features of erythematous duodenopathy with no active ooze. A
diagnosis of GAVE syndrome was made. Forced argon plasma coagulation (APC) was performed
(40 W, 2 L/min) to settle down the active ooze. The patient was symptomatically normal
for 24 hours following which he redeveloped melena with a drop in hemoglobin concentration
(drop from 9 to 8.3 g%). Due to the sudden drop, again an emergency repeat endoscopy
was performed, which showed blood at the former site of APC. A definite bleeding point
could not be found due to blood in the field of view. APC was abandoned, and computed
tomography (CT) was planned. A CT was performed after a multidisciplinary meeting
between the hepatologist, hepatic surgeon, and the interventional radiology team.
Computed tomography abdominal angiography was performed (16 slice, GE Healthcare).
The scan was performed in the supine position in suspended respiration. The contrast
was injected using a pressure injector through an 18 gauge cannula sited in the left
upper limb vein. Omnipaque (Iohexol 350, General Electric Healthcare) was used as
intravenous contrast media. Patient underwent two angiographic phases with the first
phase acquisition taken at 6 seconds and the second phase at 45 seconds following
a bolus trigger. Technical parameters were set as pitch (1.2), collimation (arterial:
16 × 0.6 mm and enous: 24 ×1.2 mm), rotational time (0.6 s), kV (120), and mA (120).
CT angiography showed the patient to have features of chronic liver disease with features
of portal hypertension and ascites. There was a focal mucosal thickening seen at the
antral region of the stomach with multiple prominent vessels supplying this area with
mildly increased vascularity best appreciated on the arterial phase ([Figs. 1]
[2]). No active contrast extravasation was demonstrated on any phase of the CT angiography.
Due to recurrent fall in patients hemoglobin concentration, established the diagnosis
of GAVE syndrome and failed APC, a unique and alternative approach was planned for
the management of the condition. An empirical intra-arterial embolization was proposed
based on the CT angiographic finding of multiple arterial feeders to the thickened
antral region.
Fig. 1 Contrast-enhanced CT abdomen, axial images. (A) Noncontrast scan showing features of chronic liver disease with ascites, (B) section across the pyloric region of stomach, (C) mild focal mucosal thickening at the antral-pyloric region of the stomach with multiple
prominent vessels supplying this area with mildly increased vascularity, and (D) MIP image of the corresponding region showing multiple prominent vessels supplying
the antrum-pyloric region. MIP, maximum intensity projection.
Fig. 2 Contrast-enhanced CT abdomen, coronal, and sagittal reformat images (A and B) showing mild focal mucosal thickening at the antral-pyloric region of the stomach
with multiple prominent vessels supplying this area with mildly increased vascularity.
A super selective empirical intra-arterial embolization was planned and performed
under mild sedation. Right femoral arterial access was achieved under ultrasound guidance,
and a 5F sheath was inserted. Celiac artery and common hepatic artery were cannulated
using a 5F cobra catheter, and angiographic run was acquired. A normal celiac artery
anatomy was noted with the common hepatic artery diving into hepatic artery proper
and gastroduodenal artery. Super selectively catheterization of the gastroduodenal
artery was then performed using a 2.7F microcatheter (Progreat, Terumo). An angiographic
phase was acquired, which demonstrated multiple small vessels supplying the area of
focus in the antral region with these small gastric feeding vessels arising mainly
from the right gastroepiploic artery with early contrast runoff ([Fig. 3]). Empirical embolization was performed using gel foam slurry, which was made by
mixing small gelatin sponge pledgets with contrast using the Tessari technique. Gel
foam slurry was first injected super-selectively into these multiple small feeding
vessels. Check angiography showed stasis of flow across these small feeding vessels.
Following this, coil embolization was performed using three Nester micro-coils (2–70
× 4 mm and 1–140 × 4 mm), which was placed in the right gastroepiploic artery and
the superior pancreaticoduodenal artery, respectively ([Fig. 4]). Cross circulation from the superior mesenteric artery was blocked using gel foam
slurry injection into the inferior pancreaticoduodenal artery, and this was confirmed
on the successive angiographic run. Other sources of cross circulation were checked
by confirming angiographic series from the proper hepatic artery, gastric artery,
splenic artery, and colic arteries. The patient was hemodynamically stable postprocedure
with no further drop in hemoglobin concentration or any complaints of melena. The
patient was shifted to ICU on day 5 due to development of oliguria, increase lactate,
and breathlessness. The patient developed multi-organ failure and bacterial peritonitis
by day 7 and eventually succumbed due to cardiopulmonary arrest. No follow-up imaging
or endoscopy was performed due to deterioration in the patients’ condition.
Fig. 3 Conventional angiography images (A and B), contrast blush with the early draining vein in the antral-pyloric region of the
stomach with feeder vessels arising from the right gastroepiploic artery.
Fig. 4 Postembolization images (A and B).
Discussion
GAVE syndrome or watermelon stomach is a rare condition of the stomach, which can
be misinterpreted as either antral gastritis or portal hypertensive gastropathy.[4] The condition is common in elderly patients with a female preponderance.[7] The mean age at presentation is about 73 years in women and 68 in men, with up to
71% of patients being women.[8] Ninety percent of patients present with nonresponsive iron deficiency anemia and
occult bleed.[9] The exact pathogenesis is not well understood; however, the condition has shown
a link to various conditions like liver cirrhosis, diabetes, end-stage renal disease,
hypothyroidism, connective tissue disorder, and cardiac disease.[1]
[7]
[10] Cirrhosis is one of the most common causes of the condition, with up to 30% of patients
having preexisting cirrhosis.[1]
[10] Patients with GAVE present with chronic iron-deficiency anemia, acute GI bleed,
unexplainable abdominal pain, and rarely as gastric outlet obstruction.[11] Up to 60% of patients with connective tissue disorder have skin telangiectasias.[12] Diagnosis of the condition can be using endoscopy, enteroscopy, and sometimes using
red blood cell scans or capsule endoscopy. GAVE syndrome, classically present as a
red lesion which is often hemorrhagic, which can be either in a linear fashion or
diffusely spread out. These are predominantly located within the gastric antrum.[13] Histologically, the underlying region shows hyperplastic antral mucosa, thrombosed
dilated capillary channels, and hypertrophy of the fibromuscular lamina propria without
inflammatory signs.[14] The histological findings are also appreciated on endoscopic ultrasound. Some studies
have found a 5-year latency period for the diagnosis of the condition.[15] Due to its features, GAVE can be misinterpreted as portal hypertensive gastropathy;
however, there are specific features which help in differentiating the conditions.
Portal hypertensive gastropathy involves the corpus and the fundal region of the stomach,
whereas, GAVE is most limited mainly to the antral region. GAVE has a classical endoscopic
finding. Procedures such as β-blocker therapy or transjugular intrahepatic shunt are
not helpful in case of GAVE syndrome.[1]
[10]
[16]
[17]
Treatment of the condition can be challenging and mainly includes three options including,
(1) medical management, (2) endoscopic management, and (3) surgical management. Medical
management consists of the use of various pharmacological agents, such as steroids,
octreotide, estrogen-progesterone hormonal combination therapy, tranexamic acid, thalidomide,
cyproheptadine, α-interferon, and calcitonin.[1]
[10] The effectiveness of medical management has only been reported in case reports/case
series and need randomized control trials to find efficacy and drug safety.[18] Endoscopic treatment is a preferred treatment option and includes band ligation,
cryotherapy, argon plasma coagulation, neodymium-yttrium-aluminum-garnet laser coagulation,
radiofrequency ablation, and sclerotherapy.[11]
[18] APC is the treatment of choice in patients with GAVE and has shown effective results
in both bleeding and rebleeding.[18] Surgical treatment is reserved as a last resort in these cases; however, it comes
at the cost of significant morbidity and mortality rates.[4] No controlled studies have been performed to suggest if medical-surgical treatment
options are superior. Studies have suggested the use of surgical interventions in
medically refractory cases.[16]
Transarterial embolization has not been well established as a treatment option. Jabbari
et al[3] who first coined the term watermelon stomach failed to demonstrate the lesion on
angiography in three patients. It was suggested that the failure to detect GAVE on
angiography could be subtle and hence missed due to early arterial filling and emptying
which would appear normal in the late venous phase. Second, the surrounding hypervascularity
can also obscure the lesion.[19] Digital angiography allows better visualization of the otherwise subtle lesion.
Terawaki et al reported a productive and successful embolization in the case of GAVE
syndrome.[20] Another case report of successful embolization was published in Japan for refractory
GAVE.[21] Complications of embolization include necrosis and gastric wall ulcerations.[22]
Conclusion
We report a unique medical experience of performing an endovascular embolization in
a case of GAVE which has rarely been published in the past as per our literature review.
Embolization provides an additional treatment option in patients who are not manageable
with endoscopy or have several rebleed episodes or who do not want surgery as a treatment
option.
