DOI:10.2214/AJR.08.1151
AJR 2009; 192:W311-W316
© American Roentgen Ray Society
Direct CT Venography for Evaluation of the Lower Extremity Venous Anomalies of Klippel-Trénaunay Syndrome
Ertugrul Mavili1,
Mehmet Ozturk1,
Yigit Akcali2,
Halil Donmez1,
Ali Yikilmaz1,
Turgut Tursem Tokmak1 and
Nevzat Ozcan1
1 Department of Radiology, Erciyes University Medical Faculty, 38039 Kayseri,
Turkey.
2 Department of Cardiovascular Surgery, Erciyes University Medical Faculty,
Kayseri, Turkey.
Received May 2, 2008;
accepted after revision November 7, 2008.
Address correspondence to E. Mavili
(ertmavili{at}yahoo.com).
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Abstract
OBJECTIVE. Our aim was to describe the technique of direct CT
venography and to describe various forms of venous anomalies detected with CT
venography in patients with Klippel-Trénaunay syndrome.
CONCLUSION. MDCT is helpful for visualizing the full length of
extremities and for evaluating length and thickness on one image.
Keywords: bone • extremities • hypertrophy • Klippel-Trénaunay syndrome • MDCT • soft tissue • veins
Introduction
Klippel-Trénaunay syndrome (KTS) was first described in 1900. It
consists of enlargement of an extremity as the result of circumferential
soft-tissue hypertrophy, bone elongation, or both; cutaneous vascular lesions,
mostly capillary malformations; and underlying diffuse venous and lymphatic
malformations [1]. Two of these
three symptoms are sufficient for the diagnosis of KTS. All three symptoms are
present in 63% of patients with the diagnosis of KTS
[2]. Cutaneous vascular lesions
usually involve the affected limb. Unilateral involvement occurs in 85% of
patients; 95% of patients have lower extremity involvement
[3]. Bilateral involvement,
upper extremity involvement, and extension into the trunk also occur
[4]. Patients usually come to
medical attention during infancy.
Most patients with KTS are treated conservatively, but surgery (surgical
stripping; phlebectomy; subfascial endoscopic ligation of perforating veins;
and, occasionally, deep venous reconstruction) or imaging-guided intervention
(sclerotherapy with alcohol or foam, selective endovenous thermal ablation) is
performed in selected cases
[5]. The indications for
surgical management of venous abnormalities include functional disability,
cardiac failure, and cosmetic factors
[6–8].
Detailed preoperative evaluation of the venous system is important because the
symptoms can worsen if intervention is performed on dilated superficial
collateral veins when hypoplasia or aplasia of the deep veins also is present
[4,
7,
9].
Various noninvasive and invasive techniques can be used to assess KTS. MR
venography is the principal imaging technique for evaluation of the patency of
the deep veins in patients with KTS. Radiography
[10], Doppler sonography
[11], and conventional
venography also can be used [3,
12,
13]. CT venography is a new
concept for complete assessment of the vascular abnormalities of KTS. We
describe the CT venographic technique for evaluation of pathologic conditions
affecting the veins and the various venous anomalies detected with CT
venography in patients with KTS.
CT Venographic Technique
MDCT examinations were performed with a 16-MDCT scanner (LightSpeed 16, GE
Healthcare). The parameters for CT venography were beam collimation, 20 mm;
pitch 1.75; slice thickness 1.25 mm; and reconstruction interval, 1.25 mm.
Images were obtained with the patient in the supine position. A tourniquet was
not used. Contrast material with a concentration of 350 mg I/mL (iomeprol,
Iomeron 350, Bracco–Altana Pharma) was administered with an automatic
injector into the subcutaneous superficial veins of both feet simultaneously
through a 22-gauge IV line at a rate of 1.5 mL/s for each extremity. The
contrast material was diluted (1:3) to avoid artifacts. The total amount of
contrast material used was 30 mL (105 mg I) for each extremity in adults.
Children received 0.5 mL/kg (1.75 mg I/kg) per extremity. The contrast volume
used varied between 10 and 20 mL (17.5–35 mg I) for each extremity in
children.
The scanning protocol was from distal to proximal, and a bolus-tracking
method was used. Acquisition was triggered automatically when the contrast
material reached the level of the femoral vein or the saphenous vein close to
the main femoral vein. The images were sent to a workstation (Advantage ADW
4.2, GE Healthcare; or View Pro-X version 3, Rogan-Delft). Conventional
transverse images, reformatted multiplanar reconstructions, maximum intensity
projections, and volume-rendered 3D images were used.
Discussion
MDCT venography is helpful for evaluating the features and the anatomic
origin, course, and relations of varicose veins and venous malformations. MDCT
is fast and easy to perform, and the spatial resolution is high, making the
technique valuable for visualization of the whole venous system and for
preoperative mapping. The length of the extremities can be measured on scout
or reconstructed images, and extremity thickness can be evaluated on axial
images [14] (Fig.
1A,
1B,
1C). Multiplanar
reconstructions, maximum intensity projections, and volume-rendered images
depict the entire length of the venous system.
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Fig. 1A —32-year-old woman with Klippel-Trénaunay syndrome,
type 1. Volume-rendered image shows collateral veins (arrowheads) and
suprapubic vein (short arrow) draining into contralateral saphenous
vein (long arrow).
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Fig. 1B —32-year-old woman with Klippel-Trénaunay syndrome,
type 1. Volume-rendered image shows saphenous vein and deep veins are not
visible on left side; inferior vena cava (thin long arrow) is patent;
and on right side iliac veins (thick long arrow) and femoral vein
(thin short arrow) are evident and suprapubic vein (thick short
arrow) drains into right saphenous vein (curved arrow).
Arrowheads indicate collateral veins.
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The venous abnormalities of the deep venous system that occur in KTS
include aneurysmal dilatation (Fig.
2A,
2B), duplication
(Fig. 3), aplasia (Figs.
4A,
4B and
5A,
5B,
5C), and hypoplasia
[8,
14,
15] (Figs.
2A,
2B and
6A,
6B,
6C). Although all deep veins
of the lower extremities can be affected, it has been reported
[8,
14] that the popliteal vein is
most commonly involved. Hypoplasia, aplasia, compression, and fenestration are
the most commonly encountered popliteal vein anomalies (Fig.
2A,
2B). The femoral vein is second
most commonly affected. Hypoplasia, compression, and fenestration are the most
common anomalies (Fig. 3)
[8,
14]. Abnormalities of the
iliac vein and inferior vena cava are rare. Abnormalities of the iliac vein
are agenesis and hypoplasia (Figs.
3 and
4A,
4B) and have been found in
3–6% of patients [9,
16]. Abnormalities of the
infrarenal inferior vena cava are found in only 0.7% of patients with KTS
[8].
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Fig. 2A —13-year-old girl with Klippel-Trénaunay syndrome, type
2. Volume-rendered 3D image shows extrinsic compression on popliteal vein
(long arrow). Popliteal vein (short arrow) distal to this
area is dilated, and femoral vein (arrowhead) is hypoplastic. Great
saphenous vein (curved arrow) also is evident.
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Fig. 3 —3-month-old girl with Klippel-Trénaunay syndrome, type
1. Oblique volume-rendered image shows duplication of femoral vein
(arrows). Venous malformation (arrowhead) extends between
duplicated femoral veins.
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Fig. 4A —11-year-old girl with Klippel-Trénaunay syndrome, type
1. Anteroposterior volume-rendered 3D image shows aplastic deep veins on right
side and venous drainage through sciatic vein (thick long arrow),
which passes through obturator foramina and joins internal iliac veins. Common
iliac veins and inferior vena cava also are aplastic, so veins drain into
ascending paravertebral veins (arrowheads). Lateral vein drains
directly into ascending paravertebral veins (curved arrow). Great
saphenous vein (thin short arrow) is evident at knee level, but upper
part is aplastic, and collateral veins (thick short arrow) are
present. Left femoral vein (thin long arrows) also is duplicated.
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Fig. 4B —11-year-old girl with Klippel-Trénaunay syndrome, type
1. Lateral volume-rendered 3D image shows sciatic vein (long arrow),
collateral veins (short arrows), and lateral vein (curved
arrow).
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Fig. 5A —44-year-old man with Klippel-Trénaunay syndrome, type
1. Volume-rendered 3D images show aplastic left proximal femoral vein and
iliac vein. Numerous varicose veins (short arrows) join and
constitute large vein that drains into contralateral femoral vein through
suprapubic vein (arrowhead). Long arrow indicates right saphenous
vein.
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Fig. 5B —44-year-old man with Klippel-Trénaunay syndrome, type
1. Volume-rendered 3D images show aplastic left proximal femoral vein and
iliac vein. Numerous varicose veins (short arrows) join and
constitute large vein that drains into contralateral femoral vein through
suprapubic vein (arrowhead). Long arrow indicates right saphenous
vein.
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Fig. 5C —44-year-old man with Klippel-Trénaunay syndrome, type
1. Surface-shaded image shows suprapubic vein (arrowhead),
superficial varicose veins (short arrows), and saphenous vein
(long arrow).
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Fig. 6A —10-year-old girl with Klippel-Trénaunay syndrome, type
1. Volume-rendered 3D image shows no deep vein accompanies artery (long
arrow) on left side. Great saphenous vein (thick short arrow) is
dilated below knee; at level of knee it gives rise to lateral vein (thin
short arrow), which drains into femoral vein (arrowhead).
Duplication of right saphenous vein (curved arrow) is evident.
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If deep venous aplasia or hypoplasia is present, collateral venous
structures develop. Common collateral flow patterns of iliac and femoral
aplasia and hypoplasia include the ascending lumbar veins, anterior abdominal
veins, and the internal paravertebral venous plexus
[16] (Figs.
1A,
1B,
1C,
4A,
4B, and
5A,
5B,
5C). The sciatic vein and
lateral veins (Figs. 4A,
4B and
6A,
6B,
6C) of the lower extremity
also serve as compensatory venous pathways. The sciatic vein is a rare
embryonic vein often associated with KTS. It has three anatomic variations: a
complete persistent sciatic vein, which originates from the popliteal vein and
ends in the internal venous system; an upper persistent sciatic vein, which
originates in the upper thigh and ends in the pelvis; and a lower persistent
sciatic vein, which is present in the distal or mid portion of the thigh.
Anomalous lateral veins that originate near the ankle and extend to the
infrainguinal or pelvic deep venous system also are found frequently
[15].
Conventional venography and CT venography are useful for imaging veins
filled with contrast material. The injected contrast material, however,
follows the path of least resistance, and the intact deep venous system may
fill slowly, incompletely, or not at all
[4,
17]. Therefore, MDCT is more
useful than conventional venography because it yields transverse images, which
show whether a vein accompanies an artery and whether the vein is completely
filled.
Radiation exposure and the use of contrast material are the main
limitations of CT venography. The amount of contrast material used, however,
is smaller than for conventional venography, and CT venography is more
comfortable for patients. MR venography may yield the same information as CT
venography without radiation exposure and contrast administration, but it is
time-consuming and the spatial resolution is low. Doppler ultrasound can be
used, but it is operator dependent, duplications can be overlooked,
visualization of the deep veins can be difficult, and visualization of all of
the veins in their full length, which is important for surgical planning, is
not possible. We use CT venography to obtain detailed anatomic information and
for surgical planning, but for follow-up, we use Doppler ultrasound.
MDCT is helpful for visualizing the full length of venous structures and
for evaluating extremity length and thickness on one image. Although
underfilling can occur, careful evaluation of the source images helps to
overcome this problem.
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Abstract
Introduction
