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374 Vascular Surgery
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great deal of pain. In extreme instances of aortic or common iliac occlusion, tissue
necrosis may demand a hip disarticulation, which can usually be performed without
difficulty, although the inherent severity of the disease brings a high morbidity and
mortality. In the upper limb, the level of amputation is determined more easily on
clinical grounds, and prosthetic advice should be obtained where possible before
the amputation to secure the optimum level and type of amputation for the available prosthesis.
In our patient, the conflict from the amputation point of view was between a
transtibial amputation, which would be decisive and lead to acceptable prosthetic
use at an early stage without the need for healing, but which might have compromised the bypass graft and required revision. It was therefore decided to proceed to
the distal amputation, accepting that time would be required to heal the ulcerated
skin in the lower leg following revascularisation. This has had a penalty of hospitalisation time and illustrates the many factors that may lead to the selection of a particular amputation level.
The vascular amputee, by nature of the extent and severity of their atheromatous
disease, has been shown to have a diminished life expectation and an appreciable
risk of a further amputation of the contralateral limb [10]. Therefore, there is considerable emphasis on utilising the patient’s remaining lifespan to achieve a reasonable quality of life – if possible, independence – in their normal environment. This
objective may not be achieved if there is delayed healing of the amputation stump,
or problems with the amputation stump, which delay prosthetic fitting and use.
There may be factors that cannot be avoided that lead to the patient needing a
wheelchair or institutional care [11]. It is particularly important that the amputation level selection is accurate as the principal cause of delayed healing or breakdown of an amputation stump is inadequate blood supply. This will result in the
stump being painful and prone to infection, which may precipitate the breakdown.
[Q7] More general factors may contribute, in particular tobacco smoking and nutritional deficiency with a low total protein and albumin level, as in our patient [12].
The presence of renal insufficiency, liver dysfunction, advanced age and poorly controlled diabetes could contribute to wound breakdown in the amputation stump.
Whatever the cause, the need for secondary surgery, in particular re-amputation,
will contribute significantly to the morbidity and mortality of the procedure,
delay the rehabilitation of the patient, and prejudice the degree of rehabilitation
that the patient achieves. A further factor that is particularly difficult to assess
but that is often significant in limiting the ability of the patients to use the prosthesis is their cognitive state and ability to learn. One advantage of management
by a multidisciplinary team is that the patient who is unable to use a prosthesis
will be identified at an early stage and rehabilitated to wheelchair independence.
If this selection is not made, then the provision of prosthesis and a failure to use
it will be a disappointment to the patient at considerably unnecessary expense
[13].
With this exception, a positive and enthusiastic attitude of a multidisciplinary
team to the amputee suffering from vascular disease should make the patient feel
that the turning point has been reached and a return to independent activity is
now possible. The amputation is not a failure but a means to restoration of
function.
Amputation is now performed less frequently by the vascular surgeon [3, 4]. The
improvement in limb salvage is diminishing the practical experience of amputation
technique. It is more important now than ever before that the surgeon realises that
Amputation in an Ischaemic Limb 375
amputation is not just cutting off the diseased part, but also making an amputation
stump that the patient will rely on for the rest of their life so that they can expect
optimum function [14]. The vascular surgeon must therefore be fully competent to
produce an amputation stump that will function well with the available prosthesis
and will allow them to return to an independent way of life without unnecessary
delay after the operation.
References
1. Robinson KP. Disarticulation at the ankle using an anterior flap. J Bone Joint Surg Br
1999;81:617–20.
2. Ubbin DT, Spincemaille GH, Reneman RS, Jacobs MJHM. Prediction of imminent amputation in
patients with non-re-constructable leg ischaemia by means of microcirculatory investigations. J Vasc
Surg 1999;30:114–21.
3. Karlstrom L, Bergqvist D. Effects of vascular surgery on amputation rates and mortality. Eur J Vasc
Endovasc Surg 1997;14:273–83
4. Quigley FG, Ling J, Avramovic J. Impact of femorodistal bypass on major lower limb amputation
rate. Aust N Z J Surg 1998;68:35–7.
5. Eskov LB, Hindso K, Holstein P. Level of amputation following failed arterial reconstruction compared to primary Amputation. Eur J Vasc Endovasc Surg 1999;17:35–40.
6. Tsang GMK, Crowson MC, Hickey NC, Simms M. Failed femorocrural reconstruction does not prejudice amputation level. Br J Surg 1991;78:1479–81.
7. Sarin S, Shami S, Shields DA, Scurr JH, Smith PD. Selection of amputation level: a review. Eur J Vasc
Surg 1991;5:611–20.
8. Clyne CA. Selection of level for lower limb amputations with severe peripheral vascular disease. Ann
R Coll Surg Engl 1991;73:148–51.
9. Greant P, Van den Brande P. Amputation in elderly and high risk vascular patients. Ann Vasc Surg
1990;4:288–90.
10. Stewart CPU, Jain AS, Ogston SA. Lower limb amputee survival. Prosthet Orthot Int 1992;
16:11–18.
11. Campbell WB, St Johnston JA, Kernick VF, Rutler EA. Lower limb amputation: striking the balance.
Ann R Coll Surg Engl 1994;76:205–9.
12. Eneroth M, Apelqvist J, Larsson J, Persson BM. Improved wound healing in transtibial amputation
receiving supplementary nutrition. Int Orthop 1997;21:104–8.
13. Hanspal RS, Fisher K. Assessment of cognitive and psychomotor function and rehabilitation of
elderly people with prosthesis. BMJ 1991;302:940.
14. Bowker JH. Surgical technique for conserving tissue and function in lower limb amputation for
trauma, infection and vascular disease. Instr Course Lect 1990;39:355–60.
41. Congenital Vascular Malformation
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Byung-Boong Lee
A 10-year-old girl presented with a history of recurrent painful swelling of the left
knee with mild ecchymosis. The latest episode of tender swelling of soft tissue
along the left knee was preceded by a direct blow to the area during a ball game.
In addition, she has had an abnormally grown left lower limb with scattered multiple soft tissue masses throughout the limb since birth.
Physical examination revealed diffuse swelling of the entire left limb, which
was longer and larger than the opposite limb and more pronounced along the
foot and lower leg. The swollen limb had slightly increased firmness on palpation
throughout its entire length except for the soft tissue mass areas.
Multiple soft tissue masses were easily compressible and scattered from the
dorsum of foot to the upper thigh; their diameters varied between 2 and 8 cm.
Similar lesions were also noticed at the left perineum, left labia, left lower
abdomen, and left flank. Diffuse swelling along the medial side of left foot collapsed spontaneously when the foot was elevated.
Further evaluation of the skeletal system revealed the left lower extremity to be
5.0 cm longer – 3.0 cm longer in the tibia and 2.0 cm longer in the femur – in
total length than the right lower extremity, accompanied by pelvic tilt and compensatory scoliosis of the lower spine.
However, the patient had minimal limitation of her daily activities except for
moderate limping.
Family history and past history were unremarkable except for a vague history
of cellulitis along the affected limb.
Question 1
What is the most fundamental problem on which clinician should focus in order to
establish the proper diagnosis and treatment of this condition?
A. Scoliosis with pelvic tilt.
B. Abnormal long-bone growth with length discrepancy.
377
378 Vascular Surgery
C. Abnormal swelling of lower limb with scattered soft tissue tumors.
D. Mechanical problem of knee joint with symptoms.
Question 2
What is the most basic laboratory test required to verify the nature of the problem?
A. Lumbosacral spine assessment.
B. Radiologic assessment of bone length discrepancy.
C. Duplex ultrasonography for the hemodynamic assessment.
D. Locomotive test including gait evaluation.
Question 3
Which of the following non-invasive studies could be most useful in the clinical
diagnosis of the disease complex in our patient?
A. Volumetric assessment of limb size.
B. Special radiologic study of epiphyseal plate of abnormally long bone.
C. Magnetic resonance imaging (MRI) study of soft tissue masses.
D. Transarterial lung perfusion scintigraphy.
E. Bone scan.
Question 4
Which of the following non-invasive tests is not appropriate to assist in the differential diagnosis for the extremity lesions in our patient?
A. Whole-body blood-pool scintigraphy (WBBPS).
B. Computed tomography (CT) scan.
C. Radionuclide lymphoscintigraphy.
D. Transarterial lung perfusion scintigraphy (TLPS).
E. Lymphangiography (lymphography).
Clinical Evaluation
This patient underwent a thorough investigation of the nature and extent of the
congenital vascular malformation (CVM) involved.
A combination of various non- to minimally- invasive studies were performed to
confirm the clinical impression of venolymphatic malformation (VLM): duplex
Congenital Vascular Malformation 379
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ultrasonography, whole-body blood-pool scintigraphy (WBBPS), magnetic resonance image (MRI) study, transarterial lung perfusion scintigraphy (TLPS), and/or
radionuclide lymphoscintigraphy.
The primary hemodynamic impact and the secondary musculoskeletal impact of
the venous malformation (VM) were assessed as the main CVM lesion in addition
to the extent/degree of each component of the VM, truncular (T) and extratruncular
(ET) form, involved in the extremity.
A thorough skeletal evaluation of the long-bone growth discrepancy of the lower
extremity and the degree of pelvic tilt with its compensatory scoliosis was also made
with conventional bone X-rays.
The TLPS assessment was performed substituting arteriographic investigation of
the lower extremity for the possible hidden micro-arteriovenous malformation
(AVM) lesion, which was marginally indicated due to an unusually increased
venous flow by the isolated VM lesion alone on the duplex scan under the normally
developed and functioning deep vein system.
An ascending phlebography was also performed together with the percutaneous
direct-puncture phlebography as a therapeutic guide; mandatory confirmation of
the presence of a normal deep vein system of the lower extremity was made before
starting the treatment to the infiltrating ET-form lesion of the VM.
The final diagnosis confirmed extensive involvement of the VM as an infiltrating
type of the ET form causing serious clinical impact directly to the venous system
hemodynamically as well as to the skeletal system to induce abnormal long-bone
growth of the left lower extremity. A moderate degree of venectasia as a T form of
VM along the left femoral-popliteal vein segment was also found, by WBBPS, MRI
and duplex scan, and subsequently confirmed by separate ascending phlebography.
A venectasia of the femoral vein was assessed to have a limited clinical
significance at this stage in comparison to the ET-form lesions of the VM.
The lymphatic malformation (LM) component which is mixed with the ET form
of VM, was confirmed as the ET form, giving minimum and limited clinical
impact so that a conservative management/observation was instituted for this LM
component.
Therefore, the ET-form lesions of VM along the knee region were selected for
active treatment as a priority; this was followed by the ankle and foot lesions.
The primary indication to initiate the treatment immediately was that these
lesions were potentially limb-threatening (e.g. hemarthrosis) due to their proximity
to the joints with increased vulnerability to repeated trauma, especially as a cause of
her knee symptoms.
The treatment was further indicated to arrest/slow down their impact on abnormal long-bone growth.
Multiple infiltrating ET lesions of the VM along the knee region, which is surgically not amenable, were selected for ethanol sclerotherapy as independent
therapy. Multisession ethanol sclerotherapy was given using 100–80 per cent
absolute ethanol in calculated dosage – not exceeding 1.0 mg per kg of body
weight as maximum dose per session – by direct puncture technique under
general anesthesia. Close cardiopulmonary monitoring during the procedure was
ensured to control and/or prevent transient pulmonary hypertension by the
unavoidable spillage of ethanol into the systemic circulation from the lesion
during treatment.
The symptomatic lesions along the knee with recurrent painful swelling following
minor injuries were controlled well without complication/morbidity and substan-
380 Vascular Surgery
tially reduced the risk of intra-articular bleeding and subsequent hemarthrosis.
Subsequently, the ET-form VM lesions at the foot and ankle underwent surgical
excision following preoperative multisession ethanol and N-butyl cyanoacrylic glue
embolosclerotherapy with much reduced perioperative morbidity to improve foot
function.
Following successful control of multiple VM lesions along the knee, ankle, and
foot with priority as a potentially limb-threatening condition, other VM lesions,
scattered throughout the lower extremity, were also treated with absolute ethanol to
assist further attempts to arrest the abnormal long-bone growth of the lower
extremity. The abnormal long-bone growth is attributed to these VM lesions
scattered within the muscular structure of the lower extremity in the extensive
infiltrating type of ET, with significant impact on the venous circulation along the
epiphyseal plate.
In addition to the multisession embolosclerotherapy as independent and/or
adjunct perioperative therapy to the VM lesions, the conservative supportive measures to improve and/or maintain overall venous function have been supplemented
with the use of a graded compression above-knee stocking to prevent chronic
venous insufficiency.
The final decision for the T-form lesion was left femoral-popliteal venectasia, but
it was decided to defer treatment until urgent treatment of the ET form of the VM
was finished, but to keep it under close observation. It might eventually require
treatment (e.g. venorrhaphy, venous bypass) to prevent development of venous
thromboembolism when significant venous flow/volume reduction should occur
following successful control of the ET form of VM lesions. The hemodynamic consequences of the treatment of such extensive ET-form lesions directly affect total
venous blood volume through the deep vein system.
The LM component in this patient was treated only with complex decongestive
therapy (CDT) in order to prevent full development of lymphedema. The infiltrating
ET form of LM detected together with the ET form of VM has been shown to put
extra burden on the marginally normal lymph-conducting system on lymphoscintigraphic evaluation. Therefore, continuous surveillance for aggressive preventive
measurement of local to systemic cellulitis along this ET form of LM lesions is
mandated.
This patient will continue to be managed by the multidisciplinary team of the
CVM Clinic at regular intervals for her entire lifetime, through periodical followup assessment of the treatment results and the natural course of the untreated
lesions.
Question 5
What is the first priority in the management of this patient?
A. Correction of scoliosis.
B. Correction of bone length discrepancy.
C. Control of abnormal hemodynamic status of lower extremity by vascular
lesions.
D. Correction of gait with physical therapy and shoe adjustment.
E. Biopsy of the soft tissue mass.
Congenital Vascular Malformation 381
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Question 6
Which of the following is not an indication for the treatment of venous malformation?
A. Lesion located near to the limb threatening region.
B. Life threatening lesion.
C. Symptomatic lesion.
D. Lesion with complication.
E. All the lesions regardless of their condition.
Question 7
What is the International Society for the Study of Vascular Anomaly (ISSVA) recommended and most popular strategy with respect to limb length discrepancy?
A. Immediate surgical intervention to the epiphyseal plate to arrest further abnor-
mal growth of the affected bone.
B. Conservative treatment of limb length discrepancy only with physical therapy
and shoe adjustment.
C. Hemodynamic control of venous malformation as a priority whenever possible.
D. Corrective surgery of bone for length discrepancy with the unaffected limb as a
priority.
E. None of the above.
Question 8
What is the current trend of therapeutic strategy for venous malformation lesions in
the lower extremity?
A. Surgical excision of the vascular lesions and related procedure only.
B. Transarterial embolotherapy only.
C. Transvenous sclerotherapy only.
D. Multidisciplinary approach with surgical therapy and embolosclerotherapy.
E. Percutaneous direct puncture sclerotherapy only.
Question 9
What is the general consensus on invasive investigations (e.g. arteriography;
phlebography) for venous malformation?
A. There is no indication for invasive investigation for the diagnosis and treatment
of venous malformation.
382 Vascular Surgery
B. Invasive investigations are indicated in every suspected case of venous malfor-
mation for the confirmation of the diagnosis.
C. Invasive investigation can be reserved for the therapeutic regimen as a road map
and/or occasional differential diagnosis.
D. Invasive investigation should be used only for the follow-up assessment.
E. None of the above.
Question 10
What is the most important precondition for the treatment of venous malformation
in the lower extremity?
A. History of deep vein thrombosis.
B. Combined lymphatic malformation.
C. Vascular-bone syndrome: length discrepancy of the long bone.
D. Existence of deep vein system.
E. Skin lesion with ulcer and necrosis.
Question 11
What has to be included in the differential diagnosis of venous malformation?
A. Lymphatic malformation.
B. AV shunting malformation.
C. Infantile hemangioma.
D. Capillary malformation.
E. All of the above.
Commentary
Congenital vascular malformation (CVM) is regarded as one of the most difficult
diagnostic and therapeutic enigmas in the practice of medicine. Vascular surgeons
often take this vascular malformation quite casually without any specific knowledge, and this cavalier approach can end in failure. Clinical presentations of the
CVMs are extremely variable, ranging from an asymptomatic birthmark to a lifethreatening condition. This variability in the clinical presentation has been a major
challenge to even the most experienced clinicians [1, 2]. Many attempts to control
this ever-challenging problem, especially in the twentieth century, were led by surgeons alone, but with mostly disastrous results because of poorly planned and overaggressive surgical treatment carried out on the basis of limited knowledge [3, 4].
Recently, a multidisciplinary approach was introduced with a new concept based on
Congenital Vascular Malformation 383
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Table 41.1. Hamburg classification of congenital vascular malformation: 1988 consensus with modification
Species Anatomical form
Predominantly: Truncular forms:
Arterial defects Aplasia or obstruction
Predominantly: Truncular forms:
Venous defects Aplasia or obstruction
Predominantly: Truncular forms:
Arteriovenous (AV) shunting defects Deep AV fistula
Combined: Truncular forms:
Vascular defects Arterial and venous
Predominantly: Truncular forms:
Lymphatic defects Aplasia or obstruction
Dilatation
Extratruncular forms:
Infiltrating
Limited
Dilatation
Extratruncular forms:
Infiltrating
Limited
Superficial AV fistula
Extratruncular forms
Infiltrating
Limited
Hemolymphatic
Extratruncular forms
Infiltrating hemolymphatic
Limited hemolymphatic
Dilatation
Extratruncular forms:
Infiltrating
Limited
Hamburg classification [5, 6]. The Hamburg classification gives excellent clinical
applicability with minimum confusion because the new terminology itself provides
substantial information on the anatomico-pathophysiological status of vascular
malformation; it has become the most fundamental rationale for the advanced
concept of vascular malformation [7–9] (Table 41.1). It classifies complex groups of
various vascular malformations based on the predominant type: VM, LM, AVM,
and combined form which is mostly hemolymphatic malformation (HLM). The VM
is the most common type of CVM together with LM and they often combine
together to make the clinical condition quite complicated.
When this HLM consists of only two components, that is, VM and LM, it is
grouped separately as VLM which is almost equivalent to Klippel–Trenaunay syndrome, where our patient belongs.
The new Hamburg classification provides critical information relating to recurrence based on precise information of embryonal stage when the developmental
arrest has occurred [9, 10].
384 Vascular Surgery
When this developmental arrest occurs in an early stage of embryonal life, it
remains with mesenchymal cell characteristics so it is grouped as ET form; when it
occurs in the later stage of embryogenesis, it is grouped as T form with lack of mesenchymal cell characteristics, which is extremely crucial for the clinical management.
This patient presented with the most common clinical manifestation of CVM,
with various findings related to the venous malformation (VM) as primary lesion as
well as its secondary phenomena since birth (Fig. 41.1). Among many clinical
findings, this patient presented with multiple, scattered, soft tissue mass lesions
along the lower extremity, extending from the toe to flank, which provide the necessary clues to initiate proper investigation of VM as the etiology of this condition
[11, 12]. [Q1: C]
Relatively firm diffuse swelling of the entire left lower extremity, in addition to
the abnormal long-bone growth with length discrepancy, may give further clues to
the investigation on the combined nature of VM and LM as the cause of the vascular-bone syndrome [13, 14].
Fig. 41.1.
Clinical appearance of the patient, with extensive VM lesions scattered along the left lower extremity
from toe to thigh, with extension to the perineum, labia, lower abdomen and flank, left.
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