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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