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I n t r o d u c t i o n
Variations in lower limb venous anatomy are common.1
However, whilst variations in the superficial venous
anatomy are usually recognised, the possibility of such
variations in the deep venous anatomy can be overlooked.
This case study highlights the need for adequate
investigation of both the superficial and deep venous
systems. Regarding treatment, there is no consensus as to
the single best approach to the treatment of varicose veins.2
This case study also highlights the need for the phlebologist
to consider all available treatment options before deciding
on a course of action.
C a s e P r e s e n t a t i o n
The patient is a twenty-year-old male who presented with
a history of varicosities involving the left calf for five years.
(Figure 1) These were associated with telangiectasia over
the medial malleolus. The patient had experienced bleeding
from the telangiectasia on a weekly basis for the three
months prior to presentation. His poor skin condition in
this area had been contributed to by repeated minor trauma
from hockey and rollerblading. He had suffered from non-
varicose eczema over the lateral aspect of the left foot and
in other areas of the body, but all had reduced in severity
over the past 18 months. His occupation is as a chef.
On examination, there were varicosities and telangiectasia
involving the left medial malleolus, together with
hemosiderin deposits. (Figure 2) There was no evidence of
lipodermatosclerosis and in particular, there was no
swelling of the left lower limb. There were two vertical scars,
extending over two-thirds of the anteromedial aspect of the
thigh (Figure 3) and over a similar distance on the lateral
aspect. (Figure 4) The patient believed these were related to
an operation on his femur at the age of four during which a
“plate” had been inserted. He was unclear on any further
details relating to this procedure at the initial consultation.
There was no past history of varicose vein surgery,
sclerotherapy, deep venous thrombosis, superficial throm-
bophlebitis or ulceration. He denied any family history of
thrombosis.
P R E S E N T A T I O N
INVESTIGATION ANDTREATMENT OPTIONS INACQUIRED DEEP VENOUSHYPOPLASIA -A CASE REPORT
DR JACQUELINE CHIRGWIN MB BS (Hons)
Phlebologist, Newcastle Vein Clinic,Newcastle, NSW, Australia
Case
14 V O L U M E 8 ( 1 ) : D E C E M B E R 2 0 0 4 A U S T R A L I A N & N E W Z E A L A N D J O U R N A L O F P H L E B O L O G Y
pp14 - 19
Case presentation of acquired deep venous hypoplasiain a 19 year-old male following osteomyelitis, fracture ofthe femur and subsequent femoral osteotomy andosteosynthesis aged between 2 and 4 years.Variationsin lower limb venous anatomy are common1 and thiscase study highlights the need for adequateinvestigation of both the superficial and deep venoussystems. It also demonstrates the need for carefulhistory taking and investigation of every patient.
The practical application of the modified Perthes test isdemonstrated. This test can be applied simply andeffectively in an office setting and provides importantinformation regarding the function of the deep venoussystem in the presence of gross abnormalities of thatsystem.
In the past, it has generally been accepted that theremoval of varicose veins in patients with absent orhypoplastic deep veins was contraindicated. In thispaper a range of treatment options is considered,including both sclerotherapy and surgery.
Key Words: Common femoral vein, ambulatoryphlebectomy, ambulatory venous pressure, bleedingtelangectasia, chronic venous hypertension, acquireddeep venous hypoplasia, deep venous system function,duplex ultrasound scanning, femoral osteotomy,modified Perthes test, osteomyelitis, pathologicalfractures femur, perforator, phlebography,photopletysmography, sclerotherapy, subfascialcollaterals, surgery, thrombophilia screen.
ABSTRACT
Address Correspondence to: Dr Jacqueline Chirgwin P.O. Box 429Newcastle, 2300 NSW AUSTRALIA. Telephone: : +61 413 291 188Facsimile: +61 2 4925 2952 E-mail: [email protected]
15A U S T R A L I A N & N E W Z E A L A N D J O U R N A L O F P H L E B O L O G Y V O L U M E 8 ( 1 ) : D E C E M B E R 2 0 0 4
Acquired deep venous hypoplasia - a case report
Figure 1: Left medial calf varicosities. Figure 3: Anteromedial thigh scar. Figure 4: Lateral thigh scar.
Figure 2: Haemosiderin deposits and recently healedbleeding site.
Figure 5: Anteriorduplex venous map.
Figure 6: Posteriorduplex venous map.
16 V O L U M E 8 ( 1 ) : D E C E M B E R 2 0 0 4 A U S T R A L I A N & N E W Z E A L A N D J O U R N A L O F P H L E B O L O G Y
J Chirgwin
I n v e s t i g a t i o n s
C o l o u r D u p l e x U l t r a s o u n d L e f t L e g
The initial investigation was a Colour Duplex Ultrasound
of the left lower limb. (Figures 5 & 6) This revealed a grossly
abnormal deep venous system in addition to the superficial
varicosities. The deep venous findings can be summarised
as follows:
• The external iliac vein was patent but displayed low
venous flow.
• An absent common femoral vein.
• The superficial femoral vein appeared bifid and very
small in calibre throughout the thigh, but displayed
normal flow. (Figures 7 & 8)
• The popliteal, posterior tibial and peroneal veins were
normal.
• There was a small competent communication from the
proximal great saphenous vein to the proximal superficial
femoral vein.
Interestingly, it was noted by the ultrasonographer that
the deep vein walls did not appear to be thickened, and the
venous channels did not appear to be tortuous.
Superficially, there was an incompetent vein from within
the posterior thigh muscle that communicated with the
Giacomini vein, which displayed bi-directional flow in the
posterior thigh. The Giacomini vein communicated with
the proximal great saphenous vein, which then displayed
reflux for a short distance in the thigh. The great saphenous
vein then gave rise to an incompetent vein travelling down
the medial aspect of the leg, with branches over the antero-
medial thigh, proximal antero-medial calf and posterior
calf. There was a 5mm incompetent perforator in the region
of the medial knee. The ultrasonographer did not note any
increased flow through the great saphenous system.
Figure 7: Duplex ultrasound showing small bifid femoralvein in mid-thigh.
Figure 8: Duplex ultrasound showing 2mm diameterfemoral vein in mid-thigh.
Figure 9: Modified Perthe’s test using blood pressure cuffbelow knee.
In light of these abnormal findings, a second Duplex scan
was performed to allow the ultrasound technician more
time to assess in particular, the major source of the deep
venous outflow in the limb.
In addition to the above findings, the second scan revealed
a competent continuation of the great saphenous vein
above the groin and onto the supra-pubic area. The perfo-
rator at the medial knee displayed flow out of the deep
venous system. However, as a result of the bony landmarks
in this area, the actual communication with the deep veins
could not be localized. The popliteal vein communicated
with a competent vein coursing proximal through the
posterior thigh muscle and this could be followed as far as
the buttock.
Subsequently, a vascular physician performed a third
duplex scan. Dr Mark Malouf of Sydney, to whom the
patient was referred, arranged this scan. He considered the
deep venous outflow was still unclear despite the first two
ultrasounds. The findings of this scan confirmed those of
the previous two ultrasound examinations and again noted
a large vein running from the left great saphenous vein,
proximally across the symphysis pubis, to link with the
saphenofemoral junction on the other side.
P a t h o l o g y
A thrombophilia screen was performed with the results as
follows:
• FBC - Normal
• Protein S and C – Normal
• Prothrombin Gene Mutation – Not detected
• Anti-thrombin III – High (low levels associated with
thrombosis)
• Lupus Inhibitor – No evidence
• Cardiolipin Antibodies – Normal (The last two tests act
as screens for Anti-Phospholipid Syndrome)
• Homocysteine – Normal
• Factor V Leiden – Not detected
P a s t M e d i c a l R e c o r d s
In view of the patient’s age at the time of the femoral
plating, with his permission medical records were obtained
from the Royal Newcastle Hospital, his former GP and his
parents. It transpired that at the age of 2 1/2 he had suffered
from acute haematogenous osteomyelitis of the left distal
femur whilst living in Wales. The records surrounding this
episode were not available. However, soon after his arrival
in Australia, he suffered a pathological fracture of the distal
femur and biopsies of the femur were taken. He ultimately
experienced a mal-union of the femoral fracture that was
corrected with a femoral osteotomy and osteosynthesis one
year later. There was no documentation of a deep venous
thrombosis at any stage of his treatment.
M o d i f i e d P e r t h e s Te s t
Perthe’s test traditionally involves using a rubber strip
tourniquet to establish whether the subfascial collaterals are
functioning well. A modification of this test has been used
in a study in the detection of the development of subfascial
collaterals in post-thrombotic deep-vein occlusion cases. 3 It
has also been used in a recently published study by Bihari et
al 4 to establish whether patients with deep vein aplasia or
hypoplasia were suitable for treatment of their superficial
varicosities. A tensiometer or blood pressure cuff is placed
on the limb just below or just above the knee. The cuff is
inflated to 110 mm Hg, and the patients are asked to walk
quickly for 5 minutes. The test is considered positive when
the limb becomes livid and the patient complains of heavy
pain within 1 to 2 minutes. In negative cases, when collateral
channels in the subfascial space are sufficient in number
and diameter to drain the venous blood from the leg, the
patient’s leg is unaffected.
Despite three duplex venous scans, the functional outflow
of the deep venous system was still unclear. The patient was
therefore recalled and this modified Perthe’s test was
performed. (Figure 9) The cuff was placed both above and
below the knee in an attempt to isolate the function of the
5mm perforator found at the knee on duplex scanning.
Initially, the patient experienced some venous
engorgement and pain in the left foot and calf when the cuff
was positioned below the knee. Repeating the test on a
further two occasions produced negative results. That is, the
patient noticed no adverse effects from the cuff. At no stage
did positioning the cuff above the knee result in any
engorgement or pain.
P r o p o s e d Tr e a t m e n t
This patient is young, with long-standing varicosities,
early venous hypertensive changes and has suffered from
numerous episodes of bleeding. His occupation as a chef
involves long hours of standing. It was therefore felt an
active rather than a passive approach to his varicosities was
warranted. The patient was ultimately referred to Dr Mark
Malouf, Sydney, for consideration for ambulatory
phlebectomy. Following further discussion, it was decided
that the patient would benefit from avulsion of the incom-
petent perforator at the knee and varicosity over the medial
calf. It is planned this procedure will be performed in
hospital on a short stay basis.
Acquired deep venous hypoplasia - a case report
17A U S T R A L I A N & N E W Z E A L A N D J O U R N A L O F P H L E B O L O G Y V O L U M E 8 ( 1 ) : D E C E M B E R 2 0 0 4
In addition to the active treatment of the lower limb
varicosities, with the patient’s permission a conference was
held with the patient, his parents and girlfriend. In view of
his age and the nursing background of his mother, it was
agreed that the likelihood of any inadvertent complications
of future treatment to his venous system could be reduced
if more of his family were informed of his condition.
D i s c u s s i o n
In the assessment and treatment of any patient in the field
of medicine, the underlying mantra is always to “do no
harm”. This stresses the importance of the treating practi-
tioner fully assessing the presenting complaint and making
an informed decision regarding not only the efficacy of any
proposed treatment, but its subsequent impact on the
patient.
The patient presented here has gross abnormalities of his
deep venous system, which were most likely acquired as a
result of the surgical interventions to his left femur in his
early years. It is unclear whether the patient suffered from a
single or multiple episodes of deep venous thrombosis or
whether the patient suffered direct damage to the deep veins
either as a result of the pathological fracture or subsequent
operative intervention. There was certainly no documen-
tation in the medical records of a thrombotic episode at the
time, nor was there any familial history of thrombosis or
abnormalities on the patient’s thrombophilia screen.
In the past, studies have suggested the removal of varicose
veins in patients with absent or hypoplastic deep veins was
contraindicated.5-8 However, the agenesia or hypoplasia of a
shorter or longer segment of deep veins is not a contraindi-
cation to radical varicectomy in every patient.4 According
to Comerota,9 obstruction should be viewed in a linear
sense (as a spectrum) rather than “all or none.” Studies have
shown that in some cases of post thrombotic deep venous
occlusion, radical varicectomy did not result in any intra-
operative or postoperative circulatory disturbance.3, 10
The difficulty in cases of deep venous aplasia, hypoplasia
or obstruction, has been to distinguish between those
patients whose overall venous function will be compromised
by treatment of any superficial varicosities, and those who
will obtain some benefit.
In addition to the traditional distinctions of deep and
superficial veins, it is believed there is a system of venous
channels called subfascial collaterals, which lie in and
between the muscles of the lower limb, and which dilate
after an occlusion or in the absence of deep veins. As stated
by the Phlebologist Robert Linton: “While working in this
field (in phlebology for 40 years), it has become obvious to
me that the great veins of the lower part of the body and the
extremities are not absolutely necessary as conduits for the
return of blood to the heart, because there are innumerable
smaller calibre collaterals that actually suffice and gradually
increase in calibre.”11 With time, these collaterals can alone
maintain the venous drainage of the limb.4
Neither phlebography4 nor venous duplex scan can give
useful information about the function of the subfascial
collaterals. Raju concluded the anatomy of the venous
system could not be the sole basis for therapeutic decisions
and that it was the hemodynamic result rather than the
anatomic site and extension of obstruction in post-throm-
botic limbs that determines the outcome. Ambulatory
venous pressure measurements and photopletysmography
are useful in venous reflux disease but are not helpful in
venous obstruction.4
Bihari et al 4 have suggested a modification of Perthe’s test
to assess the function of the subfascial collaterals. This
modified test is more readily standardized and is based on
sound physiological principles. Their cuff pressure test is
calculated to be optimal at 110 mm Hg, as the subfascial
veins can develop a pressure greater than 200 to 300 mm Hg13,14 during walking, but in the muscular compartments, the
pressure is even higher.15 Thus the cuff pressure is high
enough to compress the superficial varices but not higher
than a walking patient's arterial blood pressure in the lower
limb. This test therefore provides the treating practitioner
with a simple, non-invasive method of assessing the
possible outcome of any proposed treatment to the super-
ficial venous system. It can be performed in an office setting
with equipment that is readily available.
Having determined the likely outcome of treatment to the
superficial venous system, the practitioner will then need to
decide on the most appropriate type of treatment. In
general, active treatments can be divided into two broad
categories, being surgery and sclerotherapy. Surgery can be
further divided into ambulatory phlebectomy, and short or
long stay hospital based surgery. Ultimately, the goal of any
varicose vein surgery is to remove reflux and visible varicose
veins with the aim to achieve the most favorable hemody-
namic and cosmetic results. 17 There has been a trend
toward less invasive procedures to reduce the number of
incisions and provide more selective ablation of
varicosities.2 In the situation where the patient is relying on
subfascial collaterals for deep venous drainage, it has been
stated that operations on the superficial veins can be
performed if these pathways are functioning well.16
18 V O L U M E 8 ( 1 ) : D E C E M B E R 2 0 0 4 A U S T R A L I A N & N E W Z E A L A N D J O U R N A L O F P H L E B O L O G Y
J Chirgwin
The procedure of ambulatory phlebectomy, as described
by Muller, is a remarkable esthetic, effective, and cost-
sparing technique for definitive removal of varicose veins.18
Sites particularly appropriate for ambulatory phlebectomy
include incompetent saphenous veins, their major tribu-
taries, perforating, groin pudendal veins, reticular veins and
veins of the ankles and the dorsal venous network of the
foot.19 It effectively removes all varicosities, eliminates the
proximal source of reflux, and disconnects potentially
outflowing perforators, yet leaves in situ undamaged trunk
veins.20
On reviewing the literature, there is very little in the way of
evidence-based medicine to support the safety and efficacy
of sclerotherapy in the presence of absent or hypoplastic
deep veins. In the case of this patient, the presence of the
perforator at the knee was of concern. It measured 5 mm in
diameter and its path could not be traced on duplex
scanning because of the bony prominences. A venogram
was considered but it was felt that while it would provide
anatomical data, it would not provide any further infor-
mation regarding venous function. Additionally, it is an
invasive procedure with the associated complications.
Sclerotherapy is an extremely safe and effective procedure
in the majority of cases. However, in this particular case,
any inadvertent sclerosis of the subfascial collaterals or the
channel travelling from the popliteal vein into the buttock
may have had a severe impact on venous function. For this
reason, it was decided the patient should undergo the
procedure outlined above.
In conclusion, this case demonstrates the practical appli-
cation of the modified Perthes test, which can be applied
simply and effectively in an office setting. This test provides
important information regarding the function of the deep
venous system in the presence of gross abnormalities of
that system.
This case also demonstrates the need for careful history
taking and investigation of every patient. The patient
presented here is young, with obvious varicosities but little
on examination to suggest the extent of the deep venous
abnormalities. It is the opinion of the author that a compre-
hensive duplex ultrasound of both the deep and superficial
venous systems is the minimum requirement in the
assessment of any patient presenting with varicosities, to
determine the anatomy and any other features that may
impact on treatment. Additionally, practitioners should be
prepared to repeat the Duplex Ultrasound if necessary, to
allow adequate time for a thorough examination and
documentation of venous anomalies.
Finally, there is a range of options available for the
treatment of varicose veins. As Phlebologists, it is
important to maintain a working knowledge of these proce-
dures, and consider which is the most appropriate treatment
for each patient.
Editor’s comment: An acceptable alternative treatment inthis case is foam echosclerotherapy.
R e f e r e n c e s
1. Quinlan DJ. Alikhan R. Gishen P. Sidhu PS. Variations in lower limb venousanatomy: implications for US diagnosis of deep vein thrombosis. Radiology 2003;228(2): 443-448.2. Brethauer SA. Murray JD. Hatter DG. Reeves TR. Hemp JR. Bergan JJ.Treatment of varicose veins: proximal saphenofemoral ligation comparingadjunctive varicose phlebectomy with sclerotherapy at a military medical center.Vascular Surgery 2001; 35(1): 51-58.3. Bihari I. Can varicectomy be performed if deep veins are occluded? J DermatolSurg Oncol 1990; 16:806–807.4. Bihari I. Tasnadi G. Bihari P. Importance of subfascial collaterals in deep-veinmalformations. Dermatologic Surgery 2003; 29(2): 146-9.5. Vollmar J, Voss E. Vena marginalis lateralis presistens: die vergessene vene derangiologen. Vasa 1979; 8:192–2026.Eifert S, Villavicencio L, Kao T-C, et al. Prevalence of deep venous anomalies oncongenital vascular malformations of venous predominance. J Vasc Surg 2000;31: 462–471. 7. Gorenstein A, Shifrin E, Gordon RL, et al. Congenital aplasia of the deep veinsof lower extremities in children: the role of ascending functional phlebography.Surgery 1986; 99: 414–419. 8.Schobinger RA, Nachbur B, Senn A. The syndrome of Klippel-Trenaunay, apolyvalent angiodysplasia. J Cardiovasc Surg 1987; 28:531–534. 9. Comerota AJ. Myths, mystique, and misconceptions of venous disease. J VascSurg 2001; 34: 765–773. 10. Raju S, Easterwood L, Fountain T, et al. Saphenectomy in the presence ofchronic venous obstruction. Surgery 1998; 123: 637–44. 11. Linton RR. John Homan's impact on diseases of the veins of the lowerextremity, with special reference to deep thrombophlebitis and the post-thrombotic syndrome with ulceration. Surgery 1977; 81:1–11. 12. Raju S. New approaches to the diagnosis and treatment of venous obstruction.J Vasc Surg 1986; 4:42–54. 13. Browse NL, Burnand KG, Irvine AT, Wilson NM. Diseases of the Veins, 2nd ed.London, Sydney, Auckland: Arnold, 1999. 14. Sumner DS. Hemodynamics and pathophysiology of venous disease. In:Rutherford RB, ed. Vascular Surgery. Philadelphia, London, Toronto, Mexico City,Rio de Janeiro, Sydney, Tokyo: W.B. Saunders, 1984:148–67. 15. Alimi YS, Barthelemy P, Juhan C. Venous pump of the calf: a study of venousand muscular pressures. J Vasc Surg 1994; 20:728–735. 16. Bihari I, Tasnádi G, Bohár L, et al. Varicectomy in deep vein aplasia. PhlebolSuppl 1995; 1:829–83117. Recek C. [Principles of surgical treatment of varicose veins with regard to new findingson venous hemodynamics]. [Czech] Rozhledy V Chirurgii. 2002; 81(9): 484-91.18. Ramelet AA. Complications of ambulatory phlebectomy. [Review] [43 refs]Dermatologic Surgery. 1997; 23(10): 947-54.19. Ramelet AA. Phlebectomy. Technique, indications and complications.[Review] [29 refs] International Angiology 2002; 21(2 Suppl 1): 46-51.20. Goren G. Yellin AE. Ambulatory stab evulsion phlebectomy for truncalvaricose veins. American Journal of Surgery 1991; 162(2): 166-74. �
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Acquired deep venous hypoplasia - a case report