Foot or hand malformations related to deep venous system anomalies of the lower limb in Klippel-Trénaunay syndrome.
BACKGROUND
Klippel-Trénaunay syndrome (KTS) is a capillary-lymphatic-venous malformation associated with soft tissue and skeletal hypertrophy of one or more limbs. Deep venous system (DVS) anomalies are reported to be present in 8% to 18% of patients with KTS; approximately 25% of patients with KTS have hand or foot malformations.


OBJECTIVE
We sought to assess whether the presence of hand or foot malformations in KTS is a predictor of DVS anomalies.


METHODS
Retrospective data were collected from 51 consecutive patients with KTS seen in a university hospital between January 2000 and February 2008. Patients with possible Proteus syndrome were not included. The presence and patency of the DVS was studied using conventional venography, multidetector computed tomography, or fast 3-dimensional magnetic resonance imaging venography.


RESULTS
Seventeen hand or foot malformations were present in 9 patients, consisting of: toe macrodactyly in 5 patients (two bilateral and one with plantar expansion); toe microdactyly in one patient; finger macrodactyly in one patient; finger macrodactyly and ectrodactyly in one patient; syndactyly in 4 patients; and clinodactyly with camptodactyly of the hand of one patient with lower limb KTS. Eleven patients had DVS anomalies (one with aplasia of entire DVS; one with duplication of the superficial femoral vein; 7 with hypoplasia of femoral vein; and 7 with aplasia of the popliteal vein). All patients with hand or foot malformations also had DVS anomalies (P < .001).


CONCLUSIONS
Small sample size was a limitation.


CONCLUSIONS
The presence of hand or foot malformations in KTS may predict the presence of DVS anomalies.
Redondo
Pedro
P
Department of Dermatology, Unit of Vascular Malformations, University Clinic of Navarra, Pamplona, Spain. predondo@unav.es
Bastarrika
Gorka
G
Aguado
Leyre
L
Martínez-Cuesta
Antonio
A
Sierra
Alejandro
A
Cabrera
Juan
J
Alonso-Burgos
Alberto
A
D000328:Adult; D002648:Child; D005260:Female; D005268:Femoral Vein / Q000002:abnormalities* / Q000000981:diagnostic imaging; D005532:Foot Deformities, Congenital / Q000473:pathology*; D006228:Hand Deformities, Congenital / Q000473:pathology*; D006801:Humans; D006984:Hypertrophy; D007715:Klippel-Trenaunay-Weber Syndrome / Q000000981:diagnostic imaging / Q000473:pathology*; D008208:Lymphatic System / Q000002:abnormalities; D008297:Male; D010690:Phlebography; D011152:Popliteal Vein / Q000002:abnormalities* / Q000000981:diagnostic imaging; D019339:Port-Wine Stain / Q000473:pathology; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D055815:Young Adult