AJR 2005; 184:S123-S125
© American Roentgen Ray Society
Percutaneous Balloon Angioplasty of Superior Mesenteric Artery Stenosis in an Adolescent
Dagmar Honnef1,
Gundula Staatz1,
Andreas H. Mahnken1,
Urte Hannig2 and
Rolf W. Günther1
1 Department of Diagnostic Radiology, University of Technology of the RWTH
Aachen, Pauwelsstraße 30, Aachen 52057, Germany.
2 Department of Pediatrics, University of Technology of the RWTH, Aachen,
Germany.
Received April 21, 2004;
accepted after revision July 1, 2004.
Address correspondence to D. Honnef.
Introduction
In the pediatric age group, ischemic bowel disease is rare and
mainly seen in children with intestinal volvulus or necrotizing enterocolitis.
Chronic mesenteric ischemia is a result of a lack of blood supply in the
splanchnic region caused by stenosis or occlusion of one or more visceral
arteries. Because the symptoms are sometimes unspecific, a diagnosis can be
delayed or even missed. We present the case of an adolescent with
Williams-Beuren syndrome (WBS) with symptomatic mesenteric ischemia secondary
to superior mesenteric artery (SMA) stenosis.
Case Report
A 17-year-old male adolescent had recurrent abdominal pain for several
weeks that occurred shortly after meals. The patient showed the typical elfin
facies of WBS, was mentally retarded, and had a known mild supravalvular
aortic stenosis. Physical examination of the slender teenager yielded a 2/6
systolic bruit and a supraumbilical 3/6 systolic murmur. Gastroscopy and blood
samples were normal apart from a slightly elevated hematocrit (Hct 60%).
Abdominal sonographic examination revealed a malrotation of the right kidney,
a renal aplasia on the left side, and a vesical diverticulum. Color Doppler
sonography showed a significantly increased peak systolic velocity (PSV) of
600 cm/sec at the ostium of the SMA (Fig.
1A), a PSV of 250 cm/sec in the abdominal aorta, and a low-grade
stenosis of the celiac trunk (300 cm/sec). The SMA measurements were made
during fasting.
Subsequently, an abdominal four multislice helical CT angiography (MSCTA)
was performed with a Somatom Volume Zoom CT scanner (Siemens Medical
Solutions). The abdomen was imaged with 165 mAs, 120 kV, 4 x 2.5 mm
collimation, 12.5 mm/sec table speed, and a rotation time of 0.5 sec. The
reconstructed slice thickness was 3 mm with a 1 mm reconstruction interval.
The scan length was 39.2 cm and the overall examination time was 16 sec; 80 mL
of nonionic water-soluble contrast medium (iopromide, Ultravist 370, Schering)
was infused IV with an automatic injector at a rate of 3 mL/sec. Data
acquisition was performed using a CARE bolus technique, which enables as an
automatic bolus tracking program, the start of the spiral scan at the optimal
contrast enhancement of the visceral vessels. Three-dimensional volume
rendering was subsequently performed on a 3D Virtuoso work-station (Siemens
Medical Solutions). The axial and volume-rendered CT images revealed that the
caliber of SMA ostium was significantly reduced and that a mild stenosis of
the celiac trunk was also present (Figs.
1B and
1C). A hypoplastic aorta and a
malrotated single kidney with an abnormal low origin and course of the right
renal artery were evident (Fig.
1B). Contrast enhancement of the small bowel was normal with no
signs of bowel ischemia.
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Fig. 1B. —17-year-old male adolescent with Williams-Beuren syndrome.
Multislice helical CT angiogram shows narrowing of aorta, high-grade stenosis
of superior mesenteric artery, aplasia of the left kidney, and low origin of
right renal artery (white arrow).
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Fig. 1C. —17-year-old male adolescent with Williams-Beuren syndrome. 3D
sagittal oblique volume-rendered CT image shows significant stenosis at the
orfice of superior mesenteric artery (arrow) and minimal narrowing of
celiac trunk (arrowhead).
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Intraarterial abdominal aortography and selective angiography of the
visceral arteries were performed under general anesthesia using a 5-French
multipurpose catheter (Aachen-2-catheter, William Cook Europe). Manual
injection of a total of 60 mL of Ultravist 370 showed a nonsignificant
stenosis of the celiac trunk and a 70% stenosis of the SMA orifice
(Fig. 1D). Consequently,
percutaneous transluminal angioplasty (PTA) was performed with a 5-mm balloon
catheter (Schneider Boston Scientific) inflated up to 14 atm two times
(Fig. 1E). The following
angiography of the SMA showed good results from the angioplasty with a
significant improvement of the blood flow
(Fig. 1F). During the
intervention, 2,000 IU of low-molecular-weight heparin was administered IV;
500 IU/hr heparin was applied for the following 24 hr. Follow-up color-coded
Doppler sonography showed the success of the angioplasty, as the maximum PSV
within the ostium of the SMA was reduced to 220 cm/sec
(Fig. 1G). After the
intervention, the patient remained asymptomatic for at least 24 months.
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Fig. 1D. —17-year-old male adolescent with Williams-Beuren syndrome.
Lateral projection of selective arteriogram shows 70% reduction in the
diameter of superior mesenteric artery at its origin (arrow).
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Fig. 1G. —17-year-old male adolescent with Williams-Beuren syndrome.
Color Doppler sonogram follow-up 3 months after the procedure shows no
significant stenosis of the superior mesenteric artery.
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Discussion
Chronic mesenteric ischemia is a rare childhood entity and only a few cases
of stenosis of the mesenteric artery are reported
[1-3].
Abdominal angina is an early clinical symptom for mesenteric arterial
insufficiency and includes postprandial abdominal pain, weight loss, bowel
habit disturbances, abdominal bruit, or signs of occlusive disease elsewhere.
Progressive disease may result in acute mesenteric ischemia. SMA stenosis can
be present as a result of fibromuscular dysplasia
[1] or Takayasu's arteriitis
[2]. It is also described in
patients with midaortic syndrome and WBS
[3].
The most common locations of vascular stenosis in WBS are the ascending
aorta, the aortic arch, and the peripheral pulmonary arteries
[4]. In our patient, the
abdominal angina was probably caused by stenosis of the SMA and an elevated
hematocrit level. In WBS, the incidence of the structural anomalies of the
renal system is increased 12- to 36-fold compared with the normal population
[5]. Our patient had a single
kidney and a bladder diverticulum. Consequently, sonography of the urinary
tract and the kidneys should always be part of the first evaluation of
patients with WBS.
The key to the diagnosis of SMA stenosis was an abdominal bruit and an
abnormal duplex sonographic examination of the visceral arteries. Duplex
sonography is the preferred noninvasive screening test for abdominal vessels.
A PSV of more than 275 mm/sec predicts a 70% or more angiographic stenosis of
the SMA with a sensitivity of 92% and a specificity of 96%
[6]. However, PSV may increase
postprandially and is dependent upon changes in central and peripheral vessel
resistance. Although Doppler sonography depicted a high-grade stenosis of the
SMA, we performed an MSCTA to exclude further narrowing of the peripheral
segments of the vessels and to evaluate the contrast enhancement of the bowel
wall. In our case, in contrast to MRI, MSCTA had several advantages: general
anesthesia was avoided, contrast enhancement of the bowel walls was easily and
quickly assessed, and evaluation of the parenchymal organs was possible within
a 16-sec examination. MSCTA allows high-resolution images without compromising
z-axis coverage in a single breath-hold, even in patients with mental
retardation. We chose a 4 x 2.5 mm collimation to cover a large volume
in a short time. Bolus tracking and overlapping reconstruction enable 3D
visualization of splanchnic vessels and detection of stenosis even with a 4
x 2.5 collimation.
Balloon angioplasty of the renal artery or the aorta is commonly performed
in children. To our knowledge, a balloon angioplasty of the SMA in a child or
adolescent has not been described in the literature. Multiple series have
shown that PTA is a safe and effective alternative treatment to manage SMAs.
PTA alone has initial technical and clinical success rates up to 88% in adults
[7,
8]. Visceral angioplasty in
adolescence seems to be justified in relation to the low incidence of
complications arising from PTA and the high surgical mortality
[8].
In summary, even in childhood, intermittent abdominal pain may be a result
of chronic mesenteric ischemia. In patients with appropriate symptoms of
chronic visceral ischemia, Doppler sonography should be used as an initial
screening method. MSCTA allows optimal depiction of the anatomy of the
splanchnic vessels and the evaluation of the vascularization of the bowel
wall. Percutaneous angiography should be reserved for children in whom
invasive treatment is considered and can be performed before surgery in cases
of short SMA stenosis near the ostium.
References
- Sandmann W, Schulte KM. Multivisceral fibromuscular dysplasia in
childhood: case report and review of the literature. Ann Vasc
Surg 2000;14:496
-502[Medline]
- Van Elburg RM, Henar EL, Bijleveld CM, Prins TR, Heymans HS.
Vascular compromise prior to intestinal manifestations of Crohn's disease in a
14-year-old girl. J Pediatr Gastroenterol Nutr1992; 14:97
-100[Medline]
- Lillehei CW, Shamberger RC. Staged reconstruction for middle aortic
syndrome. J Pediatr Surg2001; 36:1252
-1254[Medline]
- Rose C, Wessel A, Scholz KH, Pankau R. Strömungsgeräusche
im Oberbauch. Monatsschr Kinderheilkd2000; 148:496
-502
- Pankau R, Partsch CJ, Winter M, Gosch A, Wessel A. Incidence and
spectrum of renal abnormalities in Williams-Beuren syndrome. Am J
Med Genet 1996;63:301
-304[Medline]
- Moneta GL. Screening for mesenteric vascular insufficiency and
follow-up of mesenteric artery bypass procedures. Semin Vasc
Surg 2001;14:186
-192[Medline]
- Matsumoto AH, Angle JF, Spinosa DJ, et al. Percutaneous
transluminal angioplasty and stenting in the treatment of chronic mesenteric
ischemia: results and long term follow-up. J Am Coll
Surg 2002;194[suppl 1]:S22
-S31[Medline]
- Maspes F, Mazzetti di Pietralata G, Gandini R, et al. Percutaneous
transluminal angioplasty in the treatment of chronic mesenteric ischemia:
results and 3 years of follow-up in 23 patients. Abdom
Imaging 1998;23:358
-363[Medline]
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Introduction
Case Report
