Persistent sciatic vessels, varicose veins, and lower

limb hypertrophy: An unusual case or discrete

clinical syndrome?
Duncan J. Parry, MBChB, FRCSEd,c Munther I. Aldoori, PhD, FRCSGlasg, FRCSEd, FACS,a
Richard J. Hammond, MBChB, DMRD, FRCR,b David O. Kessel, FRCP, FRCR,d
Mike Weston, MRCP, FRCR,d and David J. A. Scott, MD, FRCS, FRCEd,c Huddersfield and Leeds,
United Kingdom

Persistent sciatic artery is a rare congenital anomaly with a high incidence rate of aneurysmal degeneration and risk of
thromboembolization or rupture. Despite a number of recognized associations, the presence of coexistent venous
anomalies is extremely rare. We present the case of a 27-year-old woman with atypical left-sided varicose veins and soft
tissue hypertrophy. Imaging showed persistence of both sciatic artery and vein. Whether these anomalies are an incidental
finding or represent a discrete clinical syndrome remains unclear. We emphasize that unusual distribution varicose veins
may be associated with underlying persistent sciatic vessels and recommend formal duplex scan assessment for these
anomalies. (J Vasc Surg 2002;36:396-400.)

The axial artery provides the dominant blood supply to
the lower limb bud during early embryologic development.
Failure to involute results in a rare congenital anomaly
known as persistent sciatic artery (PSA). PSA is associated
with other congenital anomalies, including Müllerian and
renal agenesis, arteriovenous fistulae, bone or soft tissue
hypertrophy/hypotrophy, and abnormalities of the lower
limb arteries.1 Coexistent venous anomalies, however, are
extremely rare. Persistence of both sciatic vessels has been
reported as an incidental finding only twice in the English-
language literature.2,3 More recently, an association be-
tween PSA, varicose veins, and limb length discrepancy in
young patients has been described.1,4-6 We present the case
of a 27-year-old woman with gross left-sided telangectasias,
varicose veins, and soft tissue hypertrophy. Imaging
showed a persistence of both sciatic artery and vein with
valvular incompetence. This combination of congenital
anomalies has been previously unreported.
CASE REPORT
A 27-year-old woman was seen in the outpatient department
with left-sided varicose veins. These had become progressively
more prominent over the previous 20 years and ached on standing.
The patient had no family history of varicose veins nor medical
history of deep venous thrombosis, thrombophlebitis, or claudica-
From the Departments of Vascular Surgerya and Radiology,b Huddersfield
Royal Infirmary; and the Departments of Vascular Surgeryc and Vascular
Interventional Radiology,d St James’s University Teaching Hospital.
Competition of interest: nil.
Reprint requests: Duncan J. Parry, MBChB, FRCSEd, Department of
Vascular Surgery, Lincoln Wing, St James’s University Teaching
Hospital, Beckett St, Leeds, United Kingdom, LS9 7TF (e-mail:
DuncsParry@aol.com).
Copyright © 2002 by The Society for Vascular Surgery and The American
Association for Vascular Surgery.
0741-5214/2002/$35.00 ⫹ 0 24/4/125844
doi:10.1067/mva.2002.125844
396
tion. Examination revealed extensive left-sided telangectatic, retic-
ular, and varicose veins. These were atypical in distribution, with
extensive involvement of both the medial and lateral aspects of the
leg (Fig 1). Although mild venous pigmentation was seen in the
lower leg, no port-wine stain was visible. The left thigh and calf
measured 4 cm and 2.5 cm, respectively, more than the right leg,
without discrepancy in length. No clinical lymphoedema was
found. The left femoral pulse was weaker than the right, but the left
popliteal pulse was bounding. All other pulses were of normal
volume and ankle-brachial pressure indices were 1.3 bilaterally.
Hand-held Doppler scan showed an easily audible biphasic arterial
signal in the popliteal fossa and evidence of venous reflux.
Ascending venography showed short saphenous varices with a
venous aneurysm. The short saphenous vein drained into the
popliteal vein, and filling of a single ectatic vein in the thigh was
seen (Fig 2). This vein was lying in the line of the femoral axis at the
level of the hip and was initially presumed to be the superficial
femoral vein (SFV). Duplex scan was performed immediately af-
terwards, however, and showed the SFV to be hypoplastic. The
ectatic thigh vein was seen to course posteriorly, running into the
line of the profunda vein, and transient valvular reflux was found in
the deep veins. These findings were consistent with the presence of
a lower persistent sciatic vein (PSV), draining into the profunda
vein and thence into the common femoral/iliac veins. Finally, the
saphenopopliteal junction (SPJ) was incompetent, with sustained
reflux into a grossly dilated short saphenous vein.
Arterial duplex scan showed a complete PSA, in continuity
with the hypogastric and popliteal arteries. The left common iliac
artery was ectatic, measuring 2 cm in diameter. The axial vessel was
also ectatic, measuring up to 1.5 cm in the thigh, but without focal
aneurysm formation. Magnetic resonance angiography showed
arteriomegaly of the PSA in the thigh with some abnormal collat-
eral vessels (Fig 3). Subsequent intraarterial digital subtraction
angiography showed the typical course of the PSA in the left leg
(Fig 4). Multiple abnormal arterial vessels with an increased capil-
JOURNAL OF VASCULAR SURGERY
Volume 36, Number 2 Parry et al 397

Fig 1. Atypical distribution left-sided telangectatic, reticular, and varicose veins.

Fig 2. Ascending venography. A, Oblique view shows ectatic short saphenous vein with venous aneurysm (small
arrow). B, Oblique view shows short saphenous vein (large arrow) draining into popliteal vein. C, Anteroposterior view
shows filling of single ectatic vein in thigh. This can be seen as in line with femoral axis at level of hip.
 JOURNAL OF VASCULAR SURGERY
398 Parry et al August 2002

Fig 3. Magnetic resonance angiogram shows arteriomegaly of left-sided PSA with some abnormal primitive collateral
vessels (white arrows). Superficial femoral artery is absent, and below-knee anterior tibial and peroneal arteries arise from
common trunk.

Fig 4. Intraarterial digital subtraction angiogram shows typical course of PSA in pelvis and leg (large arrow). Multiple
abnormal arterial vessels (small arrows) with increased capillary stain are seen, but no evidence is found of discrete
arteriovenous fistula.

lary stain were seen, possibly suggestive of shunting at capillary DISCUSSION

level, although no discrete arteriovenous malformation was visible.
The patient’s arterial and venous anomalies are shown in Fig 5.
Although SPJ ligation with multiple stab avulsions may have
improved the patient’s symptoms, we believed that a significant
risk of recurrent varices in the presence of deep venous reflux
existed. Furthermore, this procedure would not improve the ap-
pearance of many of the extensive reticular and telangectatic veins
in the thigh. After discussion of these factors, the patient opted for
conservative treatment in compression hosiery. After 2 years, she
remains well and has undergone serial arterial duplex scan surveil-
lance of PSA without incident.
The axial artery is a dorsal branch of the primitive
umbilical artery and provides the dominant blood supply to
the lower limb bud in the 9-mm embryo. The femoral
artery is a continuation of the external iliac artery and is
visible by the 12-mm stage. As the femoral artery develops,
synchronous involution of the axial system occurs. By the
22-mm stage, the familiar adult vascular tree has fully
developed. Failure of the femoral artery to develop, or
the axial artery to involute, results in a PSA.1,7 Although
the embryologic development of the arterial system of the
lower limb is well described, this is not true of the venous
JOURNAL OF VASCULAR SURGERY
Volume 36, Number 2
Fig 5. Diagram represents arterial and venous embryology. Ec-
tatic ipsilateral hypogastric artery (1) gives rise to PSA, which
passes through sciatic notch. PSA is complete, passing posteriorly
down thigh into popliteal artery (2). Superficial femoral artery is
aplastic (3), although porfunda femoris is patent. Short saphenous
vein can be seen to drain into popliteal vein (6). SFV is hypoplastic
(5). Lower PSV is ectatic and courses posteriorly, emptying into
profunda vein and thence common femoral vein (4).
Parry et al 399
system. Within weeks of the axial artery development, a
peripheral border vein provides venous outflow from the
limb bud. Ultimately, the tibial continuation of this disap-
pears and the fibula portion becomes the anterior tibial,
lesser saphenous, and inferior gluteal veins. The retroperi-
toneal postcardinal vein gives rise to the greater saphenous
vein, which gives off the femoral and posterior tibial veins.8
The axial artery in utero is paired with an axial venous
network, which may provide a source of collateral venous
outflow in the presence of deep venous obstruction.9 With
ingrowth of the femoral vein, the axial vein normally invo-
lutes, with remnants persisting as the sciatic veins of the
glutei and satellite vein of the sciatic nerve. In a manner
analogous to the arterial system, failure of the femoral vein
to develop, or the axial vein to involute, results in a PSV.
Morphologically, the PSA is complete if continuity
between hypogastric and popliteal arteries is maintained.
Arteriomegaly is frequently observed, with focal aneurysm
formation in as many as 46% of patients.7,10 Acute lower
limb ischemia, caused by thromboembolism, rupture, and
“blue toe” syndrome are documented complications.1
Consequently, some investigators advocate duplex scan
surveillance for all PSAs, with surgical intervention in the
presence of aneurysm formation.1,4,7
PSV is most commonly found in association with the
Klippel-Trénaunay syndrome (KTS). Of these patients,
20% to 48% have a demonstrable PSV on magnetic reso-
nance imaging.11,12 PSV may also occur in isolation and is
a rare cause of recurrent posterior varicose veins.9 Although
the sciatic artery and vein are paired in utero, their synchro-
nous persistence is extremely rare. This has been reported
only twice previously in the English-language literature as
an incidental finding during a cadaveric study and work-up
for resection of a malignant femoral nerve schwannoma.2,3
Our patient’s particular combination of congenital anom-
alies (ie, atypical varicosities, persistence of both sciatic
vessels, and soft tissue hypertrophy) has not been previously
reported.
The etiology of vascular anomalies is poorly under-
stood. During embryogenesis, blood islands, containing
angioblasts, are derived from the extraembryonic meso-
derm. Vasculogenesis describes the differentiation of an-
gioblasts into a primitive vascular plexus, and angiogenesis
the remodelling and expansion of this preexisting network.
The molecular basis for vascular morphogenesis is ill-de-
fined but is likely to follow sequential changes in genetic
expression. Regulatory factors implicated in these processes
include vascular endothelial growth factor and its receptors,
the angiopoietic system, and the ephrin-B system.13 The
cause of our patient’s anomalies is not known. We postulate
that a single genetic or environmental insult, occurring
early during vascular morphogenesis, synchronously af-
fected the development of arterial and venous systems of
the lower limb. Similarly, the musculoskeletal system is also
derived from mesoderm and this insult possibly may have
also caused this patient’s soft tissue hypertrophy. Although
the latter may be caused by hemodynamic change, we

400 Parry et al
believe these anomalies reflect a generalized mesodermal
defect.
In a young patient with atypical distribution varicose
veins, diagnostic consideration should be given to other
congenital anomalies, including KTS, Parkes Weber syn-
drome, and vascular malformations. KTS has been defined
as “a rare congenital malformation characterised by a triad
of (i) capillary malformation (usually a port-wine stain) (ii)
atypical varicose veins - often with the persistence of em-
bryological veins (iii) bone/soft tissue hypertrophy.”14 In
addition, abnormalities of the deep venous and lymphatic
systems may be seen. These anomalies are variable, how-
ever, and some physicians will assign a diagnosis of KTS in
the presence of two of these features. With the previous
criteria, one could say that our patient has KTS with an
associated PSA. Nevertheless, the patient lacks a capillary
malformation, by far the most consistent feature of KTS
and present in as many as 98% of patients.14 In addition,
peripheral arterial anomalies are not a recognized feature of
this syndrome. We believe our patient bares a close resem-
blance to the patients described by Madson et al1 with PSA,
varicose veins, and limb length discrepancies. Whether this
combination of mesodermal anomalies is an incidental find-
ing or whether they represent a discrete clinical syndrome
remains unclear. It remains plausible that, depending on
the timing of the insult during embryologic development,
any combination of vascular anomalies is possible. We
simply wish to emphasize that atypical distribution varicose
veins in young patients may be associated with persistence
of both sciatic vessels. Because of the high incidence rate of
sciatic artery aneurysm formation, we recommend formal
duplex scan assessment for these anomalies.
In our patient, valvular incompetence of the deep veins
was the pathogenic cause of the chronic venous hyperten-
sion, with consequent SPJ incompetence and widespread
reticular veins, telangectasias, and venous pigmentation. In
the absence of a history of deep venous thrombosis, incom-
petence seems likely to be primary in origin, although
whether this is the result of intrinsic weakness within the
embryonic vein wall or valve cusp remains obscure. Exci-
sion of incompetent PSVs has been reported10,11 but was
not feasible in this case because a hypoplastic SFV would
not allow adequate collateral venous drainage. Although
superficial venous surgery was not contraindicated, we be-
lieved that the presence of deep venous incompetence
would greatly increase the risk of recurrent varicosities.
Furthermore, SPJ ligation would not improve the appear-
JOURNAL OF VASCULAR SURGERY
August 2002
ance of many of the extensive reticular and telangectatic
veins in the thigh, which were nonshort saphenous in
distribution. In view of these factors, the patient opted for
conservative treatment in compression hosiery and remains
well at 2 years. In the absence of focal aneurysm formation
within the PSA, the patient will continue to undergo annual
duplex scan surveillance.
In conclusion, we believe this case shows that persist-
ence of the sciatic artery and vein may occur synchronously.
We emphasize that atypical distribution varicose veins may
be associated with persistence of the sciatic artery and
recommend formal duplex scan assessment for these anom-
alies.
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Submitted Oct 12, 2001; accepted Feb 27, 2002.