British Journal of Radiology (2007) 80, e188-e192
© 2007 British Institute of Radiology
doi: 10.1259/bjr/46840541
Magnetic resonance lymphangiography in Klippel–Trénaunay syndrome
C Lohrmann, MD
1
J-P Bartholomä, MD
1
E Foeldi, MD
2
O Speck, PhD
1 and
M Langer, MD
1
1 Department of Radiology, University Hospital of Freiburg, Hugstetter Strasse 55, D-79106, Freiburg, 2 Foeldi Clinic for Lymphology, Hinterzarten, Rößlehofweg 2-6, D-79856, Hinterzarten, Germany
Correspondence: Christian Lohrmann, Department of Radiology, University Hospital of Freiburg, Hugstetter Strasse 55, 79106 Freiburg i. Br., Germany. E-mail: lohrmann{at}mrs1.ukl.uni-freiburg.de
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Abstract
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To date, lymphoscintigraphy and conventional, direct lymphography have been the favoured imaging modalities in assessing the lymphatic system in patients with Klippel–Trénaunay syndrome. We report on the first patient suffering from Klippel–Trénaunay syndrome whose lymphatic vasculature of the lower limbs was evaluated with MR lymphangiography.
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Introduction
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The combination of capillary malformations, soft tissue or bone hypertrophy, and varicose veins or venous malformations corresponds to Klippel–Trénaunay syndrome (KTS) [1–3]. It is possible to diagnose KTS if any two of the three features exist [1, 2]. The lower extremities are involved in 95% and the upper extremities in 5% of patients suffering from KTS [4]. To a lesser degree, intra-abdominal and thoracic structures are affected [2, 4–7]. The prognosis is usually benign and the management is primarily supportive unless symptoms of haematuria, haematochezia or oesophageal varices occur. Associated pathologies of the lymphatic system are also not uncommon [4, 5, 8, 9]. Aplasia, hypoplasia and reduction of the lymphatic vessels and nodes are described. Clinical sequelae of the pathological lymphatic components can be extremely disabling and include lymphangitis, lymphoedema, lymphfistulae with lymphorrhoea, and mass effects from lymphatic malformations [10, 11]. To date, mainly direct conventional lymphography and lymphoscintigraphy have been used to delineate lymphangiodysplasias in patients with KTS [4, 5, 8, 9].
Magnetic resonance lymphangiography (MRL) with intradermal injection of an extracelllular, paramagnetic contrast agent is a new diagnostic imaging tool for the detection of pathologically modified lymphatic pathways [12, 13]. The technique has proved to be safe and technically feasible in patients suffering from primary and secondary lymphoedema [12, 13]. To our knowledge, this is the first report on evaluating the lymphatic vasculature in a patient suffering from KTS with MRL.
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Case report
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A 19-year-old man with KTS of the lower extremities was referred by the Foeldi Clinic for Lymphology for MRL. The indication for performing MRL was the evaluation of associated abnormalities of the lymphatic vasculature. The study had been approved by the local ethics committee and the patient had given informed consent before the examination.
MRL technique
MRL was performed with a 1.5 T system (Avanto; Siemens Medical Systems, Erlangen, Germany) equipped with high-performance gradients. Three stations were examined: first, the lower leg and foot region; second, the upper leg and the knee region; and third, the pelvic region and the proximal upper leg. A phased array body coil was used to image the pelvic region, and a dedicated peripheral surface coil was used to image the upper and lower leg. Before MRL was performed, the extent and distribution of the lymphoedema were evaluated using a heavily T2 weighted 3D-turbo spin echo (TSE) sequence (repetition time (TR)/echo time (TE) 2000/694; flip angle 180°; matrix 256x256, bandwidth 247 Hz pixel–1; 6/8 rectangular field of view 480 mm; slices 96; voxel size 2.0 mmx1.9 mmx1.7 mm; acquisition time 4 min 04 s). For MRL a 3D spoiled gradient-echo sequence (volumetric interpolated breath hold examination, VIBE) with the following parameters was used: TR/TE 3.4/1.47; flip angle 25°; matrix 448x448, bandwidth 490 Hz pixel–1; 6/8 rectangular field of view with a maximum dimension of 500 mm; slices 128; voxel size 2.2 mmx1.1 mmx1.5 mm; acquisition time 0 min 44 s. The three stations were first imaged without contrast material and subsequently repeated 5 min, 15 min, 25 min, 35 min, 45 min and 55 min after intracutaneous application of gadodiamide (Omniscan; GE Healthcare, UMich, Germany). To highlight the gadodiamide-containing structures, baseline images were subtracted before three-dimensional (3D) maximum intensity projection (MIP) reconstructions were calculated. A total contrast material dose of 18 ml and 2 ml of mepivacainhydrochloride 1% were subdivided into 10 portions. Four portions were injected intradermally into the dorsal aspect of each foot in the region of the four interdigital webs; one portion was injected medial to both first proximal phalanges.
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Clinical history
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Clinical examination revealed capillary malformations and varicose veins of the left lower leg and foot. The left lower leg was swollen with oedematous changes. Furthermore, the patient had suffered from recurrent erysipelas at the level of the left lower leg for a period of 3 years. An outside performed duplex venogram and conventional venography revealed ectatic superficial veins with valvular incompetence of the left lower leg. The deep venous system of both legs and the superficial venous system of the right leg were unremarkable with no signs of thrombosis. Additionally, no arteriovenous malformations could be observed by an outside conducted conventional arteriography. No vascular surgery was performed prior to presentation.
MRL findings
MRL detected concomitant venous enhancement in the lower and upper leg 5 min after contrast material application (
Figures 1 and 2) The lymphatic vessels of the right lower and upper leg were unremarkable with the best delineation at 25 min and 35 min, respectively (Figure 3). MRL images of the left lower leg revealed delayed lymphatic flow with several dilated lymphatic vessels up to 5 mm with the best demarcation at 45 min (Figure 1). At the level of the left upper leg just one distally located, dilatated lymphatic vessel of 5 mm was detected (Figure 2). An enhancement of the left-sided inguinal lymph nodes was not visible during the examination. The lymphoedema at the level of the left lower leg demonstrated an epifascial distribution with a high signal intensity on T2 weighted images (Figure 4). An area of dermal back-flow was not detected. The patient described the pain at the time of gadodiamide application as mild, and he was able to walk well and without discomfort after the examination. No complications were observed after the examination.
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Figure 1. A 19-year-old man with Klippel–Trénaunay syndrome (KTS). (a) Frontal 3D spoiled gradient-echo magnetic resonance lymphangiography maximum intensity projection (MIP) image, obtained 45 min after gadodiamide injection, clearly delineates several enlarged lymphatic vessels in the left lower leg with typical nodular appearance (arrows). Note the concomitantly enhanced superficial vein, which shows a lower signal intensity (arrowheads). (b,c) Angled 3D spoiled gradient-echo magnetic resonance lymphangiography MIP images, obtained 45 min after gadodiamide injection, clearly delineate several enlarged lymphatic vessels in the left lower leg with typical nodular appearance (arrows). Note the concomitantly enhanced superficial vein, which shows a lower signal intensity (arrowheads).
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Figure 2. A 19-year-old man with Klippel–Trénaunay syndrome. Angled 3D spoiled gradient-echo magnetic resonance lymphangiography image, obtained 55 min after gadodiamide injection, clearly delineates an enlarged lymphatic vessel in the distal part of the left upper leg with typical nodular appearance (arrows). No lymphatic collectors were detected in the proximal and middle part of the left upper leg. Note the concomitantly enhanced superficial vein, which shows a lower signal intensity (arrowheads).
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Figure 3. A 19-year-old man with Klippel–Trénaunay syndrome. Frontal 3D spoiled gradient-echo magnetic resonance lymphangiography MIP image, obtained 25 min after gadodiamide injection, clearly delineates unremarkable lymphatic vessels in the right lower leg with typical nodular appearance (arrows). Note the concomitantly enhanced superficial vein (arrowhead).
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Figure 4. A 19-year-old man with Klippel–Trénaunay syndrome. Coronal heavily T2 weighted 3D-turbo spin echo (TSE) source image demonstrates epifascial lymphoedema of the left lower leg (arrows).
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Therapy
For therapy, a conservative approach combining manual lymphoedema treatment and compression stockings was recommended. Furthermore, the patient was educated on the basics of skin hygiene to minimize skin infections.
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Discussion
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KTS is an uncommon disease occurring in all ethnic groups with equal frequency [2]. Although theories have been proposed for pathophysiological characteristics of KTS, the aetiology remains largely unclear. The major associated pathologies seen in KTS are generally divided into vascular, skeletal, cutaneous and lymphatic [2–6]. The various symptoms indicate that KTS is genetically heterogeneous and affected by many factors [6].
Superficial varicose veins are frequently present at birth or may appear in the first 2 weeks of life [2, 4–6, 11]. Anomalous veins may also be observed, e.g. in over two-thirds of patients a characteristic incompetent lateral venous channel arises near the ankle and extends a variable distance up the extremity to the pelvic deep venous system [5]. KTS patients most frequently complain about their painful varicosities. Generally, these are managed supportively with intermittent rest, elevation and repeated reinforcement with graduated compressive stockings [2–6]. The capillary malformation found in KTS seldom causes management problems, which can be camouflaged with make-up and ageing may lead to major fading.
The role of the involvement of the lymphatic system in KTS is controversially discussed [4, 5, 8, 9, 14]. If involvement is suspected, it is indispensable to define the degree of the lymphatic pathway abnormality and pathological drainage to guide optimal therapy.
To date, lymphoscintigraphy and conventional, direct lymphography have been the favoured imaging modalities in assessing the lymphatic system in patients with KTS [4, 5, 8, 9]. In a patient series concerning mixed vascular deformities of the lower limbs published by Kinmonth et al [8], 13 patients with mainly venous deformities, the so-called "Klippel" type, were examined with bipedal lymphography. The lymphatic vessels in 10 patients showed either aplasia or hypoplasia, while the lymphatic pathways of three patients were within normal limits. Kinmonth et al [8] pointed out that the general tendency, therefore, was towards underdevelopment and insufficiency of the lymph pathways of the limbs. In addition, many exhibited vesicles, fistulae and lymph cysts. Patients with arteriovenous fistulae demonstrated large hyperplastic lymph pathways, which were possibly either congenital or a hypertrophic response.
Baskerville et al [5] presented a series of 49 patients with KTS. 15% of the patients had lymphoedema and in 22% cutaneous lymphatic vesicles were observed. Additionally, 55% of limbs showed lymphatic hypoplasia in 14 performed lymphangiograms.
Berry et al [4] performed lymphoscintigraphy in 13 patients with KTS. In nine patients essentially no tracer uptake along major lymphatics of the affected limb over at least a 1 h period was detected. Collins et al [9] examined four patients suffering from KTS with lymphoscintigraphy. In two patients an obstruction of the lymphatic flow was detected, while two others showed normal and enhanced patterns. It was noted that the two patients with decreased flow patterns were older and had suffered from bouts of cellulitis and lymphangitis, while the patients with normal and enhanced flow were young and had no infections of the involved extremity.
In the presented patient suffering from KTS, MRL was able to clearly demonstrate, non-invasively, the pathological aspect of the lymphatic vasculature in the left limb. Thereby, MRL supported the clinical assumption that the oedema attributed to a venous disorder had a significant lymphatic component. In the presented patient no lymphatic vessels could be reliably detected on T2 weighted images. Presumably the velocity of the moving lymphatic fluid was still too high to receive a continuous high signal.
A major hindrance in creating accurate treatment plans in patients with lymphoedema has been the difficulty of imaging the lymphatic system in humans. Direct lymphography provides the greatest concentration of the contrast agent in lymph vessels and nodes. Invasiveness, long examination times, radiation exposure and potential side effects, such as pulmonary embolism and local wound infection, have, therefore, limited its clinical applicability [15, 16]. Lymphoscintigraphy has the disadvantage of ionizing radiation exposure and poor spatial and temporal resolution, limiting its value for accurate assessment of the lymphatic pathology [17].
In contrast, MRL was shown to be capable of visualizing the lymphatic vessels in patients with primary and secondary lymphoedema non-invasively [12, 13].
Furthermore, the lack of radiation exposure in MRL enables it to be safely used to monitor the results of treatment and the clinical course of patients suffering from a lymphostatic component of KTS. The associated lymphatic pathologies should be approached with caution. The lymphostatic component of the syndrome can normally be successfully treated with decongestive therapy, and surgery should only be performed in selected cases.
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Conclusion
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MRL represents a safe, non-invasive imaging modality for assessing the involvement of the lymphatic system in KTS. Consequently, in addition, optimal therapy can be initiated in considering as well the pathological lymphatic components.
Received for publication January 23, 2006.
Revision received April 14, 2006.
Accepted for publication May 15, 2006.
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References
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Abstract
Introduction
