phlebology vol8 / no1€¦ · • factor v leiden – not detected past medical records in view of...

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.


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.



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


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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