VENO-LYMPHATIC ANGIODYSPLASIAS
CONGENITAL VASCULAR
MALFORMATION
Lee Boong
Byung,
Seoul, Korea
Congenital vascular malformation (CVM)
has been known through centuries as one of enigma in medicine and still remains
as most difficult and confusing diagnostic and therapeutic dilemma, presenting
wide range of clinical manifestation from asymptomatic portwine stains to extensive
lesion of arteriovenous shunting anomaly, which is not only threaten the limb
with extensive ischemia and severe venous hypertension but also the life with
cardiac failure secondary to massive shunting of blood.1,2 Through this century
many attempts were made to control this ever-challenging problem mostly by surgeon
but they all experienced disastrous results mainly due to their cavalier approach
with poor understanding of the disease and subsequent ill-planned overaggressive
approach by surgeon alone.3,4
However, recently better understanding of anatomo-pathophysiology of this congenital
vascular malformation became possible on the basis of new knowledge of pathogenesis
through the advanced and logical classification of CVM.5 That is, depending on
what stage of embryonal life developmental arrest should occur during the organogenesis,
different outcome (e.g. truncular or extratruncular) and/or different combination
of various vascular anomaly (e.g. predominantly venous or venolymphatic) can occur.6,7
Also advanced medical technology during the last two decades provided various
kinds of non to less-invasive tests for more accurate and safer diagnosis and
subsequently improved management of this disease.8,9
Eventhough, surgeons of North America and Europe lately started to recover from
such a disastrous fallout effect of self-inflicted (?) wound through overaggressive
surgical approach in earlier decades, there are still significant wrong prejudice
due to painful memory of earlier mishap with subsequent unfounded backlash effect
of overconservative attitude.
Since 1980 decade, surgeons and physicians of this specialty on both side of Atlantic
looked into for possible collaboration to prepare better strategy with new attitude
on this regard and, through two worldly recognized CVM workshops (Hamburg in 1988
and at Denver in 1992) the urgent necessity to accept new approach through multidisciplinary
coordination was fully confirmed and the new classification of this wide spectrum
of the disease (Hamburg Consensus) properly introduced in order to reduce the
significant confusion especially on terminology between two different camps across
Atlantic.5,10
Though there are still significant differences remained in the concept of vascular
malformation between two camps across Atlantic, very important and fundamental
issues of the necessity for the better communication on the basis of common language
(e.g. same classification) was once again shared together through the first Samsung
International Symposium for Congenital Vascular Malformation on December 1996
at Seoul, Korea.11,12 This presentation was prepared particularly aiming at the
correction of misunderstanding with wrong prejudice to venous malformation which
is not only important as majority of this wide spectrum of congenital vascular
malformation but also is closely related to the proper management of venolymphatic
disease and without proper knowledge on this venous malformation in particular,
the care of venolymphatic disease also will be incomplete, as well.
On the basis of our experiences on 392 patients registered at Congenital Vascular
Malformation Clinic of Vascular Center, Samsung Medical Center this predominantly
venous malformation was confirmed to be the most common form of vascular defect
only second to predominantly lymphatic defect (primary lymphedema) and much more
common than arterio-venous (AV) shunting malformation in Korea, which is compatible
to European and American data of prevalence in general.
Further assessment of this venous malformation in our patient population confirmed
minimal involvement of combined AV shunting defect and hence, venous defect among
Korean is quite rare to combine macro- or micro-AV shunting defect as its component
which is extremely important for the proper management and also for the proper
forecast on its prognosis. Accordingly, most of venous malformation in early pediatric
age group seems to be much safer to be handled conservatively and definite treatment
may be deferred until child grows enough to tolerate extensive diagnosis and treatment
procedures in general comparing to other type of vascular defects unless the venous
malformation has AV shunting defects combined or bone has been involved to CVM.2,9
And therefore, most important part of the management of this venous malformation
is rather precise differential diagnosis to rule out other combined component
of vascular defect especially AV shunting defect which directly and indirectly
alarms for strong potential evolutibility of residual mesenchymal cells in malformed
embryonal tissue. This angioblasts of AV shunting defect is easily awaken by minimal
change of milieu or stimulation like minor trauma including surgery, to grow explosively
like volcano out of control. And therefore more irrationally aggressive the surgeon
charges to awakened this sleeping giant with omnipotential evolutibility, more
explosively this AV shunting defect will react to grow with subsequently disastrous
results of treatment.6,8
The final diagnosis should aim at the proper assessment of the severity, location,
extent of involvement to surrounding structures and nature of this venous malformation
(truncular and/or extratruncular) besides this careful differential diagnosis
to rule out AV shunting defects combined. Baseline study also should include the
proper investigation for the coexistence of other vascular malformation elsewhere
throughout the body before final treatment strategy is established.
For the diagnosis only, non-invasive tests are usually sufficient especially for
the predominantly venous malformation, and the invasive tests may be reserved
as the final diagnostic tool for the therapeutic purpose as road map.2
The non or less-invasive tests for venous malformation in our laboratory include
Magnetic Resonance Image MRI (MRA & MRV)), CT scan, Bone X-ray, Arterial and Color
Doppler Image (CDI) and/or power doppler study of Duplex scan, Aeroplethysmography,
Photoplethysmography, Lymphoscintigraphy, Whole Body Blood Pool Scan (WBBPS) utilizing
radioisotope tagged red blood cell and lung scan with radioisotope tagged microsphere
albumin.
Invasive tests are Arteriography (standard, selective and superselective) and
Venography (ascending, descending segmental with or without direct puncture technic)
as gold standard. However, the most of necessary information of arteriography
only for the diagnosis of venous malformation may be fulfilled and replaced with
the combined informations of MRI and Duplex scan and Transarterial lung scan.
Combined results of these three non-invasive tests can provide sufficient information
to rule out not only macro-AV shunting defect combined with venous defects but
also can rule out even micro-AV shunting component even arteriography can often
miss. However, arteriography still remains as gold standard especially for the
therapeutic purpose as road map especially for the AV shunting defect or arterial
defect and also for the complicated venous malformation. The role of segmental
venography with direct puncture technic is most essential for the final confirmation
of detailed information of venous defect to set up the proper treatment strategy
as road-map and also standard ascending venography to confirm intact deep venous
system is extremely important before the decision for the proper handling of marginal
or embryonal vein, and non-invasive tests like Duplex scan alone cannot handle
this problem quite sufficiently, especially on therapeutic point of view.8,12,13
The new increasing role of MRI (MRV & MRA) for the congenital vascular malformation
in general cannot be overemphasized for the proper diagnosis including differential
diagnosis among various kinds of malformation especially with AV shunting defects.
T1 and T2 weighted images of standard MRI can differentiate high flow (e.g. AV
shunting defect) and low flow (e.g. venous defect) CVM lesion so accurately that
it provides not only crucial information for the severity of disease but also
for the nature of the disease (e.g. truncular and/or extratruncular type) and
the extent of the disease with clear relationship to surrounding structure (infiltrating
and/or localized type) as well which is extremely important to the clinician to
choose right combination of treatment modalities. Recently adopted WBBPS (Whole
Body Blood Pool Scan) as screening test for the initial assessment of CVM has
shown its extra value as the practical follow-up parameter for the interim treatment
results assessment during multistaged therapy and further refinement of the technic
lately can provide reliable quantitative information enough to become practical
and cost effective guideline of multistage therapy and long term follow up assessment
of CVM management together with MRI and Doppler scan replacing classical role
of angiographic tests for this purpose.
Treatment should be well planned on the basis of most precise diagnosis and assessment
of the anatomic locations, depth and extension of the lesions and also the degree
of involvement to the surrounding structures like muscle, tendon, bone, nerve,
organs, etc. through the multidisciplinary approach.9,12 Eventhough there are
not much urgency of the treatment for the asymptomatic predominantly venous malformation
in general in contrast to AV shunting defects, there is significant urgency for
the treatment if this venous defects should be combined with AV shunting defects
with significant hemodynamic effect or if the bone pathology is involved to this
venous defects so that the growth discrepancy of the limb should become obvious
especially in earlier age of rapid bone growth.14,15 Also, if this venous malformation
should exist at the life threatening location like adjacent to airway with bleeding
or compression risk, it should be handled as urgent issue for life threatening
problem. However, the limb threatening location like adjacent to joint especially
knee or foot or hand with high risk of bleeding may be considered as relatively
urgent issue and has to be handled as soon as possible, comparing to other non-hemodynamically
significant lesion, elsewhere.13
Surgery for venous malformation as essential part of treatment should be prepared
as well coordinated and also well harmonized operations between ablative surgery
(e.g. removal of vascular defects) and/or reconstructive general and vascular
surgery (e.g. venorrhaphy; venous bypass). Anyhow, it should aim at the reduction
of hemodynamic abnormality first through the multistep non-radical hemodynamic
operations and then non-hemodynamic operation should be considered to improve
over-all quality of life including cosmetic improvement though they are mostly
non-curable, unfortunately.16,17,18
However, only one well orchestrated and fully integrated treatment strategy can
enhance mutually complimentary effects of multifaced multistep therapy with surgery
and vaso-occlusive angiotherapy (embolo-sclerotherapy).
Non-surgical treatment based on vaso-occlusive angiotherapy known as embolosclerotherapy
should be well integrated with surgical treatment to maximize their mutually complimentary
effect and especially new concept of the sclerotherapy using absolute alcohol
by direct puncture technic especially for venous malforamation are now carefully
welcomed by a few wordly known CVM centers, even though it is not yet truly proven
to be practical and safe for general use. Though W. Yakes et al of Denver, CO.,
USA has been advocating this technic with absolutely successful long-term results
with no recurrence since early 1980,19 in contrast to many conventional scleroagent
with extremely high recurrence rate, it has been known with high risks of various
local and systemic complications including life threatening complication.13 However,
our limited experiences through more than 200 sessions of absolute alcohol sclerotherapy
by direct puncture technic for over 80 patients of our CVM Clinic at Samsung Medical
Center are quite acceptable under the controlled circumstances in spite of skepticism
due to high costs and the time consumed for these multistep procedures. This absolute
alcohol sclerotherapy has to be carried on under general anesthesia, high tech
monitoring and high tech imaging system in general in order to reduce the potential
risk of life threatening pulmonary embolism and/or pulmonary spasm besides high
risk of deep vein thrombosis development during the procedures. There is always
significant risk of pulmonary spasm due to accidentally shunted alcohol from the
injection site into pulmonary bed in spite of all the proper regimen to prevent
the leakage of alcohol into systemic circulation from the lesion to be treated
besides extension of blood clot.
However, initial results of this new sclerotherapy using absolute alcohol against
conventional scleroagent seem to provide extraordinary permanent obliteration
of vessel without late recanalization for over one year following after completion
of therapy and especially for the diffuse infiltrating, extratruncular type of
venous malformation, this particular sclerotherapy has special role for the surgeon
to minimize the surgery related morbidity if not, to exert its unique role to
control the lesion without surgery, especially when the lesion should locate at
the surgically inaccessible or too risky area.13
Eventhough there is significant risk to use absolute alcohol as scleroagent, there
is no doubt about its new commanding role especially on diffuse infiltrating extratruncular
type defect, since this therapy definitely can reduce the morbidity related to
the conventional surgical therapy alone and furthermore there has been no recurrence
following therapy. As part of multidisciplinary approach with fully integrated
strategy to combine surgical and non-surgical treatment, alcohol sclerotherapy
allowed us extraordinary control of previously unchallengeable lesion with minimum
morbidity and especially its new role not only as independent regimen but also
as pre- and postoperative regimen to compliment surgical therapy maximized surgical
control of AV shunting defects with much reduced morbidity, together with other
scleroagent (e.g. N-butyl cyanoacrylote and/or PCBanil).
After all, this complex therapy regimen through multidisciplinary approach, has
to be carefully tailored to suit best to each individual patient and also at the
same time, the life time commitment by the patient oneself and its family and
management team altogether will become absolutely mandatory precondition before
mutual full commitment. It may be carried on through the new clinic system with
maximal efficacy and this new clinic system with new concept of multidisciplinary
approach will become essential to the clinical team to give sense of accomplishment
rather than humiliating defeat, through the relentless challenge as team to this
ever difficult congenital vascular malformation.
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