Klippel-Trenaunay Syndrome and Left Iliac Vein Agenesis
Article Outline
This case report describes a patient with Klippel-Trenaunay syndrome (KTS) and a rare deep venous anomaly, agenesis of the left iliac veins. A dilated suprapubic vein was confirmed by duplex ultrasound assessment and by phlebography to be draining his left lower limb into his right iliac veins. The management options and implications are discussed.
Keywords: Klippel-Trenaunay syndrome, Left iliac vein agenesis
Introduction
Klippel-Trenaunay Syndrome (KTS) comprises congenital varicose veins, capillary haemangioma and hemihypertrophy of bone and soft tissue in an affected limb. Defects of the deep venous system although not originally described by Klippel and Trenaunay are often encountered.
This case report describes a patient with KTS presenting with agenesis of the left iliac veins and discusses the role of operative intervention in KTS.
Case Report
A 22-year old male presented to vascular outpatients complaining of painful varicosities in his left lower limb, especially around a large dilated suprapubic vein. The varicosities appeared in early childhood, but had worsened recently.
Examination revealed prominent varicose veins in his left thigh and leg, with a large dilated superficial vein crossing his suprapubic area. There was a capillary haemangioma staining the left side of his trunk extending to the left arm, but no evidence of limb hypertrophy.
The patient had been referred by his General Practitioner for consideration of great saphenous surgery for pain and itching related to the varicosities. Due to the likelihood of KTS and the abnormal suprapubic vein a duplex scan of his left leg was arranged. The scan demonstrated competence of the great saphenous vein at the sapheno-femoral and sapheno-popliteal junctions with incompetent tributaries of the great saphenous vein on the medial aspect of the thigh and leg. The femoral and popliteal veins were patent and interestingly, the suprapubic vein was described as a large incompetent vein crossing from the left to right sapheno-femoral junction (SFJ).
Suspicions of a higher deep venous anomaly were raised and a left common femoral phlebogram was performed. The phlebogram demonstrated complete lack of contrast in the region of the left iliac veins suggesting agenesis of the these veins. Contrast was seen to be flowing in the distribution of the suprapubic vein crossing from the left to right common femoral vein at the SFJ (Fig. 1). This suggests the suprapubic vein was therefore the only venous drainage of the left leg bypassing the absent left iliac veins. Due to the positive findings of the duplex and the venogram, a Computerised Tomography scan was not necessary to confirm left iliac vein agenesis. The patient's main complaint was pain relating to his varicose veins especially during long periods of sitting down necessary for his office job.
Fig. 1
Left common femoral venogram showing complete agenesis of the left iliac veins and the suprapubic crossover vein.
Discussion
In 1900 Klippel and Trenaunay described a congenital syndrome of capillary haemangioma, varicose veins and bone and soft tissue hypertrophy affecting one or more limbs.1
It is accepted a patient may have just two features of KTS for diagnosis.2, 3 The patient described in this case has varicose veins with a deep venous anomaly- often seen in KTS, capillary haemangioma but no limb hypertrophy.
Abnormalities of the deep veins in KTS are common with an overall incidence of 40%.1
Servelle demonstrated by ligating the deep hind leg veins in a canine model limb length subsequently increased by 2.6–7.6%.4 The limb hypertrophy seen in humans with KTS was therefore attributed to deep vein entrapment by fibrous bands. Forty-eight children underwent operation to free the deep vein in the longer limb and ligate the popliteal vein in the shorter limb, the discrepancy in limb length is subsequently described as reduced or even absent in adult life.5
Baskerville et al. report a series of 88 operations on 38 patients with KTS, most operations involved ligation and stripping of varicose veins. Patients initially experienced relief of local symptoms but high rates of recurrence occurred in subsequent years, with symptoms persisting in 90%. Conservative management including graduated compression stockings and elevation, with surgery reserved for excision of localized symptomatic abnormalities was recommended.1
One study recruiting 47 children with KTS reported that conservative measures such as those mentioned above successfully managed symptoms of chronic venous insufficiency. Four children underwent resection of varicose veins and only one child reportedly benefited.6
Noel et al. describes 20 patients with KTS selected for surgery due to severe symptoms of chronic venous insufficiency. All 20 patients underwent preoperative investigation of the deep venous system with duplex scanning, contrast phlebography and magnetic resonance imaging. This is essential prior to KTS surgery as persistent embryonic veins, agenesis, hypoplasia and aneurysms of the deep veins all occur.
All 20 underwent stripping of large lateral veins, avulsion and excision of varicosities. One patient had a hypoplastic femoral vein and underwent a popliteal-saphenous bypass graft. Another patient had bilateral entrapped popliteal veins which were surgically released. After a mean follow up time of 63.6 months 50% experienced recurrence of varicosities, however, the clinical aetiological anatomic and pathophysiological (CEAP) clinical scoring for chronic venous disease of lower limbs, showed overall improvement from 4.3 standard deviation (SD) 2.2 prior to surgery to 3.1 S.D 2.3 after surgery (P
=0.03).3
In conclusion the patient described in this case report may initially try graduated compression stockings and elevation. If symptoms persist, resection of some symptomatic lower limb varicosities could be offered after counselling the patient on the high risk of recurrence. With regard to the suprapubic vein, in the words of Servelle;
‘suprapubic varicose veins should never be resected as they serve as substitute channels for atretic iliac vessels.’5
References
- . The Klippel-Trenaunay syndrome: clinical, radiological and haemodynamic features and management. Br J Surg. 1985;72:232–236
- . Klippel-Trenaunay syndrome: spectrum and management. Mayo Clin Proc. 1998;73(1):28–36
- . Surgical treatment of venous malformations in Klippel-Trenaunay syndrome. J Vasc Surg. 2000 Nov;32(5):840–847
- . Stase veineuse et croissance osseuse. Bull Acad Natl Med. 1948;132:471–474
- . Klippel and Trenaunay's Syndrome 768 operated cases. Ann Surg. 1985;201:365–373
- . Klippel-Trenauany Syndrome: clinical features, complications and management in children. Br J Surg. 1995;82:757–761
PII: S1533-3167(06)00095-1
doi:10.1016/j.ejvsextra.2006.11.006
© 2006 Elsevier Ltd. All rights reserved.
Refers to article:
- Klippel-Trenaunay Syndrome and Left Iliac Vein Agenesis , 16 January 2007
