Case Report
A Case of Ruptured Inferior Pancreaticoduodenal Artery Aneurysm due to Median Arcuate Ligament Compression
Nicole Ilonzo*, Anik Patel, Nii Koney, Rahul Patel and Grace Kim
Department of Surgery, Mount Sinai West Hospital, USA
*Corresponding author: Nicole Ilonzo, Department of Surgery, Mount Sinai West Hospital, 1000 10th Ave Suite 2B New York, NY 10019, USA
Published: 14 Oct, 2017
Cite this article as: Ilonzo N, Patel A, Koney N, Patel R,
Kim G. A Case of Ruptured Inferior
Pancreaticoduodenal Artery Aneurysm
due to Median Arcuate Ligament
Compression. Ann Clin Case Rep.
2017; 2: 1450.
Abstract
Inferior pancreaticoduodenal artery aneurysms are rare, comprising approximately 2% of splanchnic aneurysms. These aneurysms are more often due to celiac stenosis from atherosclerosis but can also be due to median arcuate ligament syndrome. We report the case of a 68-year-old female with a history of unease with eating, who presented with retroperitoneal hemorrhage of unknown origin. CT angiography revealed a ruptured inferior pancreaticoduodenal artery aneurysm and celiac artery compression with the characteristic “hook” appearance, consistent with median arcuate ligament compression. The ruptured IPDA aneurysm was managed with endovascular coil embolization and the patient was discharged hemodynamically stable with stabilized hematocrits.
Case Presentation
68F with history of hypertension, hypothyroidism, migraines, hemorrhoids, and unease with
eating, presented with 3 days of abdominal pain and bloody stools. The pain was located in the
epigastrium, was 10/10 in severity, and associated with several episodes of bright red blood per
rectum and one syncopal episode. Of note, the patient endorsed “unease with eating” that resulted
in decreased intake for 8-9 months. She attributed this to stress from an ongoing divorce. She also
reported a long history of "problems with gas" for which she takes over the counter medications. The
patient denied any aspirin or anticoagulant use.
In the ER, her vitals were normal. On exam, she appeared fatigued however in no acute distress.
Her abdomen was soft, non-distended. She was noted to have tenderness in the epigastrium and left
lower quadrant however there was no rebound or guarding. On digital rectal exam, there was gross
blood, no internal hemorrhoid, sphincter tone was normal, external hemorrhoids were present,
non-thrombosed and non-bleeding. Her initial hematocrit was 42, however after 1L of crystalloid,
downtrended to 35 and continued to downtrend to as low as 26 within 24 hours. Otherwise, her
lactate level was normal as well as her lipase.
CT of the abdomen and pelvis with IV and PO contrast obtained during workup in the ER
revealed retroperitoneal hemorrhage with extensive retroperitoneal induration which was higher
attenuation than simple fluid measuring on the order 50 Hounsfield units centered about the
pancreas (Figure 1). Additionally, there was colitis from the transverse colon to the descending colon. Surgery was consulted and the decision was made to obtain a CT angiogram of the abdomen
and pelvis. This revealed a ruptured inferior pancreaticoduodenal artery aneurysm (Figure 2
and 3) as well as kinked and hooked appearance of the celiac origin in the absence of significant
atherosclerotic disease raising the possibility of median arcuate syndrome (Figure 4).
Given this diagnosis, the patient underwent a diagnostic
visceral angiogram with subsequent embolization of the inferior
pancreaticoduodenal artery aneurysm. The left radial artery was
accessed with a 21 gauge micro needle and ultrasound guidance.
A wire was placed through the needle and a 5F hydrophilic glides
heath was advanced over the wire. The complex collection measures
39-85 Hounds field units, highly suggestive of blood products. A
.035 wire and 5F diagnostic catheter was used to navigate through
the arm and into the descending aorta. A 5F Sarah Radial catheter
was used to selectively catheterize the SMA and a micro catheter
system was used to catheterize the posterior branch of the inferior
pancreaticoduodenal artery. Coil embolization was used to occlude
the artery distal to the aneurysm and the aneurysm sac. After the
procedure, the patient was monitored in the surgical intensive care
unit (SICU) for two days. She tolerated a regular diet after two days and no longer had bright red blood per rectum. Her hematocrit was
stable, abdominal pain improved, and she was discharged with plans
for follow-up (Figure 5-9).
Figure 1
Figure 2
Figure 2
CTA (arterial phase) Blue: large complex fluid collection displacing
the pancreas (green arrow) anteriorly thus confirming a retroperitoneal space
location. Green: head/neck of the pancreas. Red: lobular clearly delineated
enhancement posterior to the pancreatic head with a density that follows the
aortic blood pool.
Figure 3
Figure 3
CTA (arterial phase). The complex collection measures 39-85
Houndsfield units, highly suggestive of blood products. The lobular region
of enhancement measures 11mm. Given its location with respect to the
pancreatic head, and that it follows the aortic blood pool, it suggests posterior
branch inferior pancreaticoduodenal artery aneurysm.
Figure 4
Figure 4
Sagittal CT angiogram (arterial phase): Kinked and hooked
appearance of the celiac origin in the absence of significant atherosclerotic
disease raises the possibility of median arcuate syndrome.
Figure 5
Figure 5
Arterial phase angiogram with catheter in the SMA. Green:
Superior Mesenteric Artery. Red: Origin of the inferior pancreaticoduodenal
artery. Blue arrow: Aneurysm seen arising from the posterior branch of the
inferior pancreaticoduodenal artery (when correlated with CTA performed
earlier). Orange: Anterior division of the PDA.
Figure 6
Figure 6
Arterial Phase Angiogram with catheter at the origin of the Inferior
PDA. Red arrowhead: Common hepatic Artery. Red arrow: Gastroduodenal
artery. Blue arrow: Posterior superior PDA. Green arrow: Anterior superior
PDA. White arrow: Aneurysm from the posterior inferior PDA. Blue
arrowhead: Posterior inferior PDA. Selective opacification of the anterior and
posterior branches of the Inferior PDA. Inferior PDA anastomosis with the
Superior PDA (arising from GDA) are well seen in this image.
Figure 7
Figure 8
Figure 9
Figure 9
Coils now fill the majority of the aneurysm sac. Contrast is no
longer seen distal to the embolized portion confirming complete embolization.
Discussion
Inferior Pancreaticoduodenal artery aneurysms are very rare, comprising only 2% of visceral aneurysms. Inferior
pancreaticoduodenal artery aneurysms are more frequently identified
given the increase in diagnostic radiographic testing such as computed
tomography (CT) and magnetic resonance imaging (MRI). Inferior
pancreaticoduodenal artery (IPDA) aneurysms when symptomatic
can present with rupture, and in-hospital mortality for patients not
treated with ruptured inferior pancreaticoduodenal artery aneurysm
rupture approaches 80%.
True aneurysms of the pancreaticoduodenal arteries are
distinguished from false aneurysms on the basis of etiology and
location of the bleeding. With respect to location of bleeding,
true aneurysms rupture into the retroperitoneal space whereas
false IPDA aneurysms rupture into the abdomen. True inferior
pancreaticoduodenal artery aneurysms are often secondary to celiac
stenosis, either by atherosclerosis or extrinsic compression from the
median arcuate ligament. In contrast, false IPDA aneurysms occur
in patients with recent surgery, trauma, pancreatitis, or with septic
emboli [1].
Patients with celiac stenosis may develop aneurysms of the
pancreaticoduodenal arcade. The hypertension that results distally to
the stenosis leads to aneurysmal dilation of the pancreaticoduodenal
arcades. Extrinsic compression of the celiac artery can result in
median arcuate syndrome, which is a rare condition which comprises
clinical manifestations e.g. postprandial pain and celiac stenosis. The
median arcuate ligament is a fibrous band that connects both sides of
the diaphragmatic crura. In most people, the median arcuate ligament
passes superior to the celiac axis. However, in a minority of patients,
the median arcuate ligament passes much lower and anterior to the
celiac artery and causes compression. Median arcuate syndrome
consists of post-prandial abdominal pain associated with 5-10 lb.
weight loss. The syndrome, while in part due to stenosis, most likely
has a neuropathic origin as well. Celiac stenosis, alone, is unlikely the
cause, since 47% of patients continue to have recurrence of symptoms
with celiac revascularization alone. In contrast, only 21% of patients
have recurrence with revascularization and surgical release of the
ligament [2].
Median arcuate ligament syndrome is best visualized with CT
angiography where the sagittal view is preferred. With conventional
angiography, the region of narrowing is better identified during
expiration whereas it improves with inspiration. 13-50% of healthy
patients will have extrinsic compression of the celiac artery due to
the median arcuate ligament. However, only 10-24% of patients with
extrinsic compression of the celiac by the median arcuate ligament
will have the syndrome [3].
The goal for our patient during this admission was to control
the bleeding. She was hemodynamically stable and therefore a prime
candidate for endovascular coil embolization of the IPDA aneurysm.
She will need further workup of her previous abdominal pain
symptoms and possibly release and revascularization of the celiac
axis [4].
Conclusion
References
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