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The role of colour Doppler sonography in the diagnosis of lower limb Klippel–Trénaunay syndrome
Clinical Radiology 68 (2013) 716e720
Contents lists available at SciVerse ScienceDirect
Clinical Radiology journal homepage: www.clinicalradiologyonline.net
The role of colour Doppler sonography in the diagnosis of naunay syndrome lower limb KlippeleTre H.T. Qi a, X.M. Wang a, *, X.D. Zhang a, M.H. Zhang b, C.M. Li a, S.G. Bao a, H. Yuan c a
Shandong Medical Imaging Research Institute, Shandong Provincial Key Laboratory of Diagnosis and Treatment of Cardio-cerebral Vascular Diseases, Shandong University, Jinan, Shandong, China b Ultrasound Department, Qingdao Municipal Hospital, Qingdao, China c Vascular surgery, Provincial Hospital affiliated to Shandong University, Jinan, Shandong, China
article in formation Article history: Received 21 October 2012 Received in revised form 7 February 2013 Accepted 19 February 2013
AIM: To investigate the accuracy of colour Doppler sonography as compared to phlebography naunay syndrome (KTS). in patients with Klippel-Tre MATERIALS AND METHODS: From September 2004 to May 2012, 59 consecutive patients seen in Shandong medical imaging research institute with a clinical suggestion of KTS were included. Thirty-four were female and 25 were male, with a mean age of 28.4 years. Colour Doppler sonography was used to assess the lower limb veins. The main sonographic criteria for a positive diagnosis were visualization of the lateral vein or sciatic vein, capillary haemangioma, and abnormality of the deep veins. These data were compared with phlebography findings. The k statistic was used to determine the level of agreement. The sensitivity, specificity, positive and negative predictive values, and accuracy of colour Doppler sonography as a diagnostic test were assessed. RESULTS: Colour Doppler sonography findings were positive in 21 of 59 patients with a clinical suggestion of KTS. The diagnosis was confirmed using phlebography in 22 patients. There were two false-positive results and one false-negative result by colour Doppler sonography. The k-value was 0.892. Sensitivity, specificity, positive and negative predictive values, and accuracy for colour Doppler sonography were 95.4, 94.6, 91.3, 97.2, and 94.9%, respectively. CONCLUSION: Colour Doppler sonography is an accurate, reliable, and non-invasive investigation in the assessment of patients with suspected KTS. Ó 2013 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.
Introduction naunay syndrome (KTS) is a rare The KlippeleTre vascular malformation, which was first described by Klippel naunay in 1900. KTS is a clinical syndrome with and Tre * Guarantor and correspondent: X.M. Wang, Shandong Medical Imaging Research Institute, Shandong Provincial Key Laboratory of Diagnosis and Treatment of Cardio-cerebral Vascular Diseases. No. 324 Jingwu Road, Jinan, Shandong 250021, China. Tel.: þ86 531 68776723; fax: þ86 531 87938550. E-mail address: [email protected] (X.M. Wang).
three major features: (1) hypertrophy of the soft tissue and bone overgrowth; (2) capillary haemangioma; and (3) underlying diffuse venous and lymphatic malformations. Two of these three symptoms are sufficient for a diagnosis of KTS.1 In KTS patients, the superficial veins tend to be located along the lateral aspect of the lower limb with the so-called lateral veins or sciatic veins,2 and some combine with abnormity of the deep veins (hypoplasia or absence). All three symptoms are present in 63% of KTS patients.3 Cutaneous vascular lesions usually involve the affected limb. Unilateral involvements occur in 85% of patients; 95% of
0009-9260/$ e see front matter Ó 2013 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.crad.2013.02.004
H.T. Qi et al. / Clinical Radiology 68 (2013) 716e720
717
Table 1 naunay syndrome. The colour Doppler sonography examination results in low limb KlippeleTre Patient no./sex/age (years)
Hypertrophy of soft tissue or bone overgrowth
Capillary haemangioma/ lymphoedema
Characteristic vein (lateral vein or sciatic vein)
Deep vein abnormality
1/M/10 2/F/24 3/M/2.5 4/F/7 5/F/45 6/F/31a 7/M/20 8/F/16 9/F/1.5 10/F/43 11/M/21a 12/F/24 13/M/17 14/F/35 15/F/46
þ þ þ þ þ þ þ þ þ þ þ þ þ þ þ
Capillary haemangioma e Capillary haemangioma Capillary haemangioma Capillary haemangioma Capillary haemangioma Lymphedema Capillary haemangioma Capillary haemangioma Capillary haemangioma Capillary haemangioma Capillary haemangioma Lymphedema Capillary haemangioma e
Lateral vein Lateral vein Lateral vein Lateral vein Sciatic vein Lateral vein Lateral vein e Lateral vein Sciatic vein Sciatic vein Lateral vein Lateral vein Sciatic vein Sciatic vein
Hypoplastic right superficial femoral vein
16/M/5 17/F/4 18/F/11 19/M/17 20/F/2.5 21/M/28
þ þ þ þ þ þ
Capillary Capillary Capillary e Capillary Capillary
Lateral vein e e Sciatic vein Lateral vein Lateral vein
a
haemangioma haemangioma haemangioma haemangioma haemangioma
e e Absent left superficial femoral vein e e Hypoplastic left popliteal vein e Hypoplastic right superficial femoral vein e e e e Absent popliteal vein and hypoplastic femoral vein e e e e Left superficial femoral vein hypoplastic e
naunayeWeber syndrome by arteriography. The patients were tested for KlippeleTre
patients have lower extremity involvement.4 Patients usually come to medical attention during infancy. There are many imaging examination techniques that can be used to assess KTS, such as computed tomography (CT) venography,5 magnetic resonance (MR) venography,6 and phlebography, but colour Doppler sonography is a non-invasive diagnostic method provide immediate confirmation. The reliability of colour Doppler sonography to study blood vessels is generally accepted. The objective of the present study was to investigate the accuracy of colour Doppler sonography as compared to phlebography in patients with KTS.
Materials and methods From September 2004 to May 2012, 59 consecutive patients admitted to the Vascular Surgery Department of our hospital with clinically suspected KTS were included. Thirty-four (57.6%) were female and 25 (42.4%) were male, with a mean age of 28.4 years (range 2.5e47 years). Institutional ethics committee approval had been obtained. All ultrasound examinations were performed by an
Table 2 Comparison of results: colour Doppler sonography and conventional venography. Conventional venography
Colour Doppler Positive Negative Total
Positive
Negative
21 1 22
2 35 37
Total
23 36 59
experienced cardiovascular ultrasound expert, who had over 7 years of experience in cardiovascular ultrasound. The results from vascular sonography were accessed by two cardiovascular ultrasound experts, who had 10 and 7 years of experience in cardiovascular ultrasound imaging. Both the assessors were blinded to the results from phlebography. The GE Vivid seven-dimensional sonography system (GE Healthcare, Holten Norway) with a linear array transducer (9e14 MHz) and 3.5 MHz for pelvic vessels was used. The length of the lower limb and the colour of the skin of the lower limb were first examined, and then the vein system of the low extremity was examined with particular attention to the lateral vein (or sciatic vein) and its drainage path. A standing position was adopted for the examination of the superficial vein; and the deep veins were examined in the supine, decubitus, and sitting positions without the use of tourniquets. The drainage paths of the superficial veins were recorded, i.e., draining into the femoral vein, iliac vein, or great saphenous vein. The diameter of the deep veins were also recorded, especially the popliteal and femoral vein. Hypoplasia was defined as veins with a smaller than normal diameter (<50% reduction in calibre).7 Regurgitation of the
Table 3 k statistics between conventional venography and colour Doppler sonography. Parameter
Value
Asymptomatic SE
Value/asymptomatic SE
Pa
k
0.892 59
0.061
6.858
0.000
No. of valid cases a
Approximate significance.
718
H.T. Qi et al. / Clinical Radiology 68 (2013) 716e720
Figure 1 A 24-year-old woman with KTS. (a) The ultrasound image shows a section of the thigh lateral vein (arrow). M, medial; L, lateral; P, proximal; D, distal. (b) The ultrasound image shows the lateral vein draining into the femoral vein on the left side. The great saphenous (GSV) vein is located superior to the lateral vein (LV). Colour Doppler sonography shows lateral vein regurgitation. (c) Phlebography shows that the lateral vein of the left limb extremity drains into the femoral vein. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
valves of the deep veins was evaluated according to the reflux time of the Valsalva manoeuvre (<0.5 s ¼ normal, 0.5e1 s borderline, >1 s ¼ definitely abnormal).8 If necessary, a control study of the bilateral deep veins was performed to determine whether the suspected deep veins were normal or not. The arteries of the lower limb were scrutinized, particularly to investigate whether an arteriovenous fistula was present, to exclude Klippele naunayeWeber syndrome. Tre Phlebography was performed by cannulating a dorsal pedal vein with a 21 G needle and rapidly injecting 50e100 ml (300 mg iodine/ml) of iodinated contrast medium (Iohexol; GE Healthcare Ireland Cork, Ireland) with the patient supine and the table tipped 30 feet downward. A tourniquet was applied above the ankle. One anteroposterior and two oblique views of the deep calf and popliteal veins were obtained. Views of the femoral and iliac veins were then obtained. All phlebography examinations were performed by a senior vascular technician, who had 6 years of experience in phlebography. All phlebography examinations were assessed independently by two senior radiologists, who had 8 and 9 years of experience in angiography. Both the assessors were blinded to the colour Doppler sonographic findings. The SPSS program (version 13.0, SPSS, Chicago, IL, USA) was used for statistical analysis. The level of agreement between colour Doppler sonography and phlebography was determined by k-test. Sensitivity, specificity, and positive and negative predictive values were obtained.
false-negative result using colour Doppler sonography. Detailed colour Doppler sonography findings in each patient are shown in Table 1. Comparison of the results obtained using phlebography and colour Doppler sonography are shown in Table 2. All patients with a negative diagnosis using colour Doppler sonography had negative findings on phlebography except one patient of lymphoedema, for whom colour Doppler sonography did not display the deep vein clearly, because the sound beam could not penetrate the hyperplastic soft tissue, and the hypoplastic superficial femoral vein was not found, which was testified by phlebography. Two patients (6 and 11 in Table 1) with positive colour Doppler sonography diagnosis were finally proved to have naunayeWeber syndrome, but not KTS. PhleKlippeleTre bography showed an increased regional venous flow velocity in patients with small arteriovenous fistula, and this was confirmed using arteriography. Analysis of the internal validity parameters of colour Doppler sonography as a diagnostic test for KTS showed a sensitivity of 95.4%, specificity of 94.6%, a positive predictive value of 91.3%, and a negative predictive value of 97.2%. The overall accuracy was 94.9%. The k level of agreement between phlebography and colour Doppler sonography for KTS was 0.892 (high level; Table 3).
Results In the 59 patients with a clinical suspicion of KTS, phlebography, which was the established standard for the diagnosis of KTS, results were positive for KTS in 20 patients and negative in 39 patients, two patients had bilateral lower extremity involvement, and 19 patients had the unilateral involvement. Colour Doppler sonography diagnosis was positive in 21 and negative in 38. Compared with phlebography, there were two false-positive results and one
Figure 2 A 31-year-old woman with KTS. The photo shows the capillary haemangioma on the lower extremity laterally.
H.T. Qi et al. / Clinical Radiology 68 (2013) 716e720
719
Figure 3 A 10-year-old boy with KTS. (a) The axial ultrasound image shows a hypoplastic right superficial femoral vein (RSFV). The diameter of the RSFV is significantly less than the right superficial femoral artery (RSFA). (b) Colour Doppler sonography shows the blood flow of the hypoplastic RSFV. (c) The hypoplastic RSFV is confirmed by phlebography. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
Discussion KTS is a rare congenital vessel abnormality, with few case reports in the recent literature.5,6,9 Tian et al.10 reported that a vessel gene VG5Q mutation of the short arm of chromosome 5 can induce KTS. The buttocks, trunk, skull, and lymphatic vessels can be involved. The diagnosis of the disease is often established by clinical symptoms and imaging examinations; phlebography is usually required for confirmation. Various imaging techniques can be used to assess KTS, such as CT venography, MR venography, etc., but CT venography examination is invasive and requires the administration of contrast medium. Contrast medium is not required in MR venography,11 but it is a costly and complicated examination. Colour Doppler sonography is a noninvasive diagnostic test from which confirmation can be obtained immediately. In 1994, Howlett et al.12 first reported the use of colour Doppler sonography in the diagnosis of KTS. The main feature described was the presence of the lateral vein (Fig 1), which originated from the lateral foot, located in the lower extremity lateral, and drained in the femoral vein or pelvic vein. In 1996, Cherry et al.13 reported the other characteristic feature was the persistent sciatic vein (PSV), which connected the popliteal vein and internal iliac vein, located in the lateral thigh. Other findings, such as the presence of capillary haemangioma (Fig 2) or lymphoedema, were combined with abnormality of the deep veins, especially a hypoplastic or absence of the superficial femoral vein (Fig 3), helped to establish the prognosis of the disease. Colour Doppler sonography can display the lateral vein or sciatic vein in real time and highlight the drainage position of the lateral vein or sciatic vein, whereas phlebography can display only one part of the lateral vein or sciatic vein on a single image. Hypoplasia or absence of the deep veins can be evaluated accurately according using the Valsalva manoeuvre or pressing the leg. Colour Doppler sonography is a non-invasive examination, and thus has no
restrictions to its usage, whereas phlebography is invasive. Radiation exposure and the use of contrast media are the main limitations of phlebography, so it is not appropriate for all patients. Nevertheless, colour Doppler sonography does have some limitations. When the soft tissue of the lower extremity is noticeably hyperplastic, such as in cases of lymphoedema, the acoustic attenuation is increased and the penetration of the beam is reduced, especially for the femoral veins, because of their deep location; this may cause the femoral vein to be unclear at imaging. Moreover, arteriovenous fistula, especially small arteriovenous fistula, can be easily missed at vascular ultrasound. The present study has shown that colour Doppler sonography of the lower limb veins is useful in the diagnosis of KTS because it has high accuracy as a diagnostic test (94.9%), compared with the accuracy of phlebography, and there is a high level of agreement between both tests (k ¼ 0.892). Therefore, in patients with typical symptoms and positive colour Doppler sonography findings, diagnosis can be reached without phlebography. In conclusion, colour Doppler sonography may provide sufficient information for clinical treatment. It is an accurate, reliable, and non-invasive investigation in the assessment of patients with suspected KTS.
Acknowledgements The authors thank Dr Jiuhong Chen, Siemens Healthcare, for her contribution regarding manuscript editing.
References naunay syn1. Jacob AG, Driscoll DJ, Shaughnessy WJ, et al. KlippeleTre drome: its spectrum and management. Mayo Clin Proc 1998;73:28e36. naunay syndrome: clinical features, 2. Samuel M, Spitz L. KlippeleTre complications and management in children. Br J Surg 1995;82:757e61. 3. Baskerville PA, Ackroyd JS, Browse NL. The aetiology of the Klipnaunay syndrome. Ann Surg 1985;202:624e7. peleTre
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naunay syn4. Gloviczki P, Atanson AW, Stickler GB, et al. KlippeleTre drome: the risks and benefits of vascular interventions. Surgery 1991;110:469e79. 5. Mavili Ertugrul, Ozturk Mehmet, Akcali Yigit, et al. Direct CT Venography for evaluation of the lower extremity venous anomalies of Klipnaunay syndrome. AJR Am J Roentgenol 2009;192:311e6. peleTre 6. Lohrmann C, Bartholoma JP, Foeldi E, et al. Magnetic resonance lymphannaunay syndrome. Br J Radiol 2007;80:188e92. giography in KlippeleTre € cker M. Nomenclature of the veins of the lower 7. Reich-Schupke S, Stu limbsdcurrent standards. J Dtsch Dermatol Ges 2011;9:189e94. 8. Demirpolat G, Ozbek SS, Cinar C, et al. Doppler ultrasound diagnosis of lower extremity deep vein insufficiency: valsalva maneuvre or pneumatic cuff? Tani Girisim Radyol 2004;10:162e6.
9. Rebarber A, Roman AS, Roshan D, et al. Obstetric management of Klipnaunay syndrome. Obstetrics Gynecol 2004;104:1205e8. peleTre 10. Tian XL, Kadaba R, You SA, et al. Identification of an angiogenic factor naunay synthat when mutated causes susceptibility to KlippeleTre drome. Nature 2004;427:640e5. naunay syndrome: 11. Murray AD, Kincaid W, Smith FW. MRI of KlippeleTre use of the short tau inversion recovery (STIR) sequence. Clin Radiol 1996;51:813e5. 12. Howlett DC, Roebuck DJ, Christopher K, et al. The use of ultrasound in naunay syndrome. Eur the venous assessment of lower limb KlippeleTre J Radiol 1994;18:224e6. 13. Cherry KJ, Gloviczki P, Stanson AW. Persistent sciatic vein: diagnosis and treatment of a rare condition. J Vasc Surg 1996;23:490e7.
Contents lists available at SciVerse ScienceDirect
Clinical Radiology journal homepage: www.clinicalradiologyonline.net
The role of colour Doppler sonography in the diagnosis of naunay syndrome lower limb KlippeleTre H.T. Qi a, X.M. Wang a, *, X.D. Zhang a, M.H. Zhang b, C.M. Li a, S.G. Bao a, H. Yuan c a
Shandong Medical Imaging Research Institute, Shandong Provincial Key Laboratory of Diagnosis and Treatment of Cardio-cerebral Vascular Diseases, Shandong University, Jinan, Shandong, China b Ultrasound Department, Qingdao Municipal Hospital, Qingdao, China c Vascular surgery, Provincial Hospital affiliated to Shandong University, Jinan, Shandong, China
article in formation Article history: Received 21 October 2012 Received in revised form 7 February 2013 Accepted 19 February 2013
AIM: To investigate the accuracy of colour Doppler sonography as compared to phlebography naunay syndrome (KTS). in patients with Klippel-Tre MATERIALS AND METHODS: From September 2004 to May 2012, 59 consecutive patients seen in Shandong medical imaging research institute with a clinical suggestion of KTS were included. Thirty-four were female and 25 were male, with a mean age of 28.4 years. Colour Doppler sonography was used to assess the lower limb veins. The main sonographic criteria for a positive diagnosis were visualization of the lateral vein or sciatic vein, capillary haemangioma, and abnormality of the deep veins. These data were compared with phlebography findings. The k statistic was used to determine the level of agreement. The sensitivity, specificity, positive and negative predictive values, and accuracy of colour Doppler sonography as a diagnostic test were assessed. RESULTS: Colour Doppler sonography findings were positive in 21 of 59 patients with a clinical suggestion of KTS. The diagnosis was confirmed using phlebography in 22 patients. There were two false-positive results and one false-negative result by colour Doppler sonography. The k-value was 0.892. Sensitivity, specificity, positive and negative predictive values, and accuracy for colour Doppler sonography were 95.4, 94.6, 91.3, 97.2, and 94.9%, respectively. CONCLUSION: Colour Doppler sonography is an accurate, reliable, and non-invasive investigation in the assessment of patients with suspected KTS. Ó 2013 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.
Introduction naunay syndrome (KTS) is a rare The KlippeleTre vascular malformation, which was first described by Klippel naunay in 1900. KTS is a clinical syndrome with and Tre * Guarantor and correspondent: X.M. Wang, Shandong Medical Imaging Research Institute, Shandong Provincial Key Laboratory of Diagnosis and Treatment of Cardio-cerebral Vascular Diseases. No. 324 Jingwu Road, Jinan, Shandong 250021, China. Tel.: þ86 531 68776723; fax: þ86 531 87938550. E-mail address: [email protected] (X.M. Wang).
three major features: (1) hypertrophy of the soft tissue and bone overgrowth; (2) capillary haemangioma; and (3) underlying diffuse venous and lymphatic malformations. Two of these three symptoms are sufficient for a diagnosis of KTS.1 In KTS patients, the superficial veins tend to be located along the lateral aspect of the lower limb with the so-called lateral veins or sciatic veins,2 and some combine with abnormity of the deep veins (hypoplasia or absence). All three symptoms are present in 63% of KTS patients.3 Cutaneous vascular lesions usually involve the affected limb. Unilateral involvements occur in 85% of patients; 95% of
0009-9260/$ e see front matter Ó 2013 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.crad.2013.02.004
H.T. Qi et al. / Clinical Radiology 68 (2013) 716e720
717
Table 1 naunay syndrome. The colour Doppler sonography examination results in low limb KlippeleTre Patient no./sex/age (years)
Hypertrophy of soft tissue or bone overgrowth
Capillary haemangioma/ lymphoedema
Characteristic vein (lateral vein or sciatic vein)
Deep vein abnormality
1/M/10 2/F/24 3/M/2.5 4/F/7 5/F/45 6/F/31a 7/M/20 8/F/16 9/F/1.5 10/F/43 11/M/21a 12/F/24 13/M/17 14/F/35 15/F/46
þ þ þ þ þ þ þ þ þ þ þ þ þ þ þ
Capillary haemangioma e Capillary haemangioma Capillary haemangioma Capillary haemangioma Capillary haemangioma Lymphedema Capillary haemangioma Capillary haemangioma Capillary haemangioma Capillary haemangioma Capillary haemangioma Lymphedema Capillary haemangioma e
Lateral vein Lateral vein Lateral vein Lateral vein Sciatic vein Lateral vein Lateral vein e Lateral vein Sciatic vein Sciatic vein Lateral vein Lateral vein Sciatic vein Sciatic vein
Hypoplastic right superficial femoral vein
16/M/5 17/F/4 18/F/11 19/M/17 20/F/2.5 21/M/28
þ þ þ þ þ þ
Capillary Capillary Capillary e Capillary Capillary
Lateral vein e e Sciatic vein Lateral vein Lateral vein
a
haemangioma haemangioma haemangioma haemangioma haemangioma
e e Absent left superficial femoral vein e e Hypoplastic left popliteal vein e Hypoplastic right superficial femoral vein e e e e Absent popliteal vein and hypoplastic femoral vein e e e e Left superficial femoral vein hypoplastic e
naunayeWeber syndrome by arteriography. The patients were tested for KlippeleTre
patients have lower extremity involvement.4 Patients usually come to medical attention during infancy. There are many imaging examination techniques that can be used to assess KTS, such as computed tomography (CT) venography,5 magnetic resonance (MR) venography,6 and phlebography, but colour Doppler sonography is a non-invasive diagnostic method provide immediate confirmation. The reliability of colour Doppler sonography to study blood vessels is generally accepted. The objective of the present study was to investigate the accuracy of colour Doppler sonography as compared to phlebography in patients with KTS.
Materials and methods From September 2004 to May 2012, 59 consecutive patients admitted to the Vascular Surgery Department of our hospital with clinically suspected KTS were included. Thirty-four (57.6%) were female and 25 (42.4%) were male, with a mean age of 28.4 years (range 2.5e47 years). Institutional ethics committee approval had been obtained. All ultrasound examinations were performed by an
Table 2 Comparison of results: colour Doppler sonography and conventional venography. Conventional venography
Colour Doppler Positive Negative Total
Positive
Negative
21 1 22
2 35 37
Total
23 36 59
experienced cardiovascular ultrasound expert, who had over 7 years of experience in cardiovascular ultrasound. The results from vascular sonography were accessed by two cardiovascular ultrasound experts, who had 10 and 7 years of experience in cardiovascular ultrasound imaging. Both the assessors were blinded to the results from phlebography. The GE Vivid seven-dimensional sonography system (GE Healthcare, Holten Norway) with a linear array transducer (9e14 MHz) and 3.5 MHz for pelvic vessels was used. The length of the lower limb and the colour of the skin of the lower limb were first examined, and then the vein system of the low extremity was examined with particular attention to the lateral vein (or sciatic vein) and its drainage path. A standing position was adopted for the examination of the superficial vein; and the deep veins were examined in the supine, decubitus, and sitting positions without the use of tourniquets. The drainage paths of the superficial veins were recorded, i.e., draining into the femoral vein, iliac vein, or great saphenous vein. The diameter of the deep veins were also recorded, especially the popliteal and femoral vein. Hypoplasia was defined as veins with a smaller than normal diameter (<50% reduction in calibre).7 Regurgitation of the
Table 3 k statistics between conventional venography and colour Doppler sonography. Parameter
Value
Asymptomatic SE
Value/asymptomatic SE
Pa
k
0.892 59
0.061
6.858
0.000
No. of valid cases a
Approximate significance.
718
H.T. Qi et al. / Clinical Radiology 68 (2013) 716e720
Figure 1 A 24-year-old woman with KTS. (a) The ultrasound image shows a section of the thigh lateral vein (arrow). M, medial; L, lateral; P, proximal; D, distal. (b) The ultrasound image shows the lateral vein draining into the femoral vein on the left side. The great saphenous (GSV) vein is located superior to the lateral vein (LV). Colour Doppler sonography shows lateral vein regurgitation. (c) Phlebography shows that the lateral vein of the left limb extremity drains into the femoral vein. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
valves of the deep veins was evaluated according to the reflux time of the Valsalva manoeuvre (<0.5 s ¼ normal, 0.5e1 s borderline, >1 s ¼ definitely abnormal).8 If necessary, a control study of the bilateral deep veins was performed to determine whether the suspected deep veins were normal or not. The arteries of the lower limb were scrutinized, particularly to investigate whether an arteriovenous fistula was present, to exclude Klippele naunayeWeber syndrome. Tre Phlebography was performed by cannulating a dorsal pedal vein with a 21 G needle and rapidly injecting 50e100 ml (300 mg iodine/ml) of iodinated contrast medium (Iohexol; GE Healthcare Ireland Cork, Ireland) with the patient supine and the table tipped 30 feet downward. A tourniquet was applied above the ankle. One anteroposterior and two oblique views of the deep calf and popliteal veins were obtained. Views of the femoral and iliac veins were then obtained. All phlebography examinations were performed by a senior vascular technician, who had 6 years of experience in phlebography. All phlebography examinations were assessed independently by two senior radiologists, who had 8 and 9 years of experience in angiography. Both the assessors were blinded to the colour Doppler sonographic findings. The SPSS program (version 13.0, SPSS, Chicago, IL, USA) was used for statistical analysis. The level of agreement between colour Doppler sonography and phlebography was determined by k-test. Sensitivity, specificity, and positive and negative predictive values were obtained.
false-negative result using colour Doppler sonography. Detailed colour Doppler sonography findings in each patient are shown in Table 1. Comparison of the results obtained using phlebography and colour Doppler sonography are shown in Table 2. All patients with a negative diagnosis using colour Doppler sonography had negative findings on phlebography except one patient of lymphoedema, for whom colour Doppler sonography did not display the deep vein clearly, because the sound beam could not penetrate the hyperplastic soft tissue, and the hypoplastic superficial femoral vein was not found, which was testified by phlebography. Two patients (6 and 11 in Table 1) with positive colour Doppler sonography diagnosis were finally proved to have naunayeWeber syndrome, but not KTS. PhleKlippeleTre bography showed an increased regional venous flow velocity in patients with small arteriovenous fistula, and this was confirmed using arteriography. Analysis of the internal validity parameters of colour Doppler sonography as a diagnostic test for KTS showed a sensitivity of 95.4%, specificity of 94.6%, a positive predictive value of 91.3%, and a negative predictive value of 97.2%. The overall accuracy was 94.9%. The k level of agreement between phlebography and colour Doppler sonography for KTS was 0.892 (high level; Table 3).
Results In the 59 patients with a clinical suspicion of KTS, phlebography, which was the established standard for the diagnosis of KTS, results were positive for KTS in 20 patients and negative in 39 patients, two patients had bilateral lower extremity involvement, and 19 patients had the unilateral involvement. Colour Doppler sonography diagnosis was positive in 21 and negative in 38. Compared with phlebography, there were two false-positive results and one
Figure 2 A 31-year-old woman with KTS. The photo shows the capillary haemangioma on the lower extremity laterally.
H.T. Qi et al. / Clinical Radiology 68 (2013) 716e720
719
Figure 3 A 10-year-old boy with KTS. (a) The axial ultrasound image shows a hypoplastic right superficial femoral vein (RSFV). The diameter of the RSFV is significantly less than the right superficial femoral artery (RSFA). (b) Colour Doppler sonography shows the blood flow of the hypoplastic RSFV. (c) The hypoplastic RSFV is confirmed by phlebography. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
Discussion KTS is a rare congenital vessel abnormality, with few case reports in the recent literature.5,6,9 Tian et al.10 reported that a vessel gene VG5Q mutation of the short arm of chromosome 5 can induce KTS. The buttocks, trunk, skull, and lymphatic vessels can be involved. The diagnosis of the disease is often established by clinical symptoms and imaging examinations; phlebography is usually required for confirmation. Various imaging techniques can be used to assess KTS, such as CT venography, MR venography, etc., but CT venography examination is invasive and requires the administration of contrast medium. Contrast medium is not required in MR venography,11 but it is a costly and complicated examination. Colour Doppler sonography is a noninvasive diagnostic test from which confirmation can be obtained immediately. In 1994, Howlett et al.12 first reported the use of colour Doppler sonography in the diagnosis of KTS. The main feature described was the presence of the lateral vein (Fig 1), which originated from the lateral foot, located in the lower extremity lateral, and drained in the femoral vein or pelvic vein. In 1996, Cherry et al.13 reported the other characteristic feature was the persistent sciatic vein (PSV), which connected the popliteal vein and internal iliac vein, located in the lateral thigh. Other findings, such as the presence of capillary haemangioma (Fig 2) or lymphoedema, were combined with abnormality of the deep veins, especially a hypoplastic or absence of the superficial femoral vein (Fig 3), helped to establish the prognosis of the disease. Colour Doppler sonography can display the lateral vein or sciatic vein in real time and highlight the drainage position of the lateral vein or sciatic vein, whereas phlebography can display only one part of the lateral vein or sciatic vein on a single image. Hypoplasia or absence of the deep veins can be evaluated accurately according using the Valsalva manoeuvre or pressing the leg. Colour Doppler sonography is a non-invasive examination, and thus has no
restrictions to its usage, whereas phlebography is invasive. Radiation exposure and the use of contrast media are the main limitations of phlebography, so it is not appropriate for all patients. Nevertheless, colour Doppler sonography does have some limitations. When the soft tissue of the lower extremity is noticeably hyperplastic, such as in cases of lymphoedema, the acoustic attenuation is increased and the penetration of the beam is reduced, especially for the femoral veins, because of their deep location; this may cause the femoral vein to be unclear at imaging. Moreover, arteriovenous fistula, especially small arteriovenous fistula, can be easily missed at vascular ultrasound. The present study has shown that colour Doppler sonography of the lower limb veins is useful in the diagnosis of KTS because it has high accuracy as a diagnostic test (94.9%), compared with the accuracy of phlebography, and there is a high level of agreement between both tests (k ¼ 0.892). Therefore, in patients with typical symptoms and positive colour Doppler sonography findings, diagnosis can be reached without phlebography. In conclusion, colour Doppler sonography may provide sufficient information for clinical treatment. It is an accurate, reliable, and non-invasive investigation in the assessment of patients with suspected KTS.
Acknowledgements The authors thank Dr Jiuhong Chen, Siemens Healthcare, for her contribution regarding manuscript editing.
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