Byung-Boong Lee

Terminology and Classification of Congenital Vascular Malformations

Venous malformation (VM) is a congenital vascular malformation (CVM) that develops along the venous system through the various stages of embryogenesis. Older terminology and classification were often misleading and confusing. A newer classification system has emerged that is based on advances in the study of these conditions and is useful in contemporary management. The Hamburg classification was introduced after reappraisal of older terminology and has become the standard system for contemporary classification, which is based on anatomical, pathological and embryological criteria.

Predictors of response to percutaneous ethanol sclerotherapy (PES) in patients with venous malformations: Analysis of patient self-assessment and imaging

BACKGROUNDnPercutaneous ethanol sclerotherapy (PES) is the primary tool in the treatment of venous malformations (VM). However, PES has known serious complications. This study is aimed at identifying predictors of good response to PES in patients with VM to improve patient selection.nnnMETHODSnWe performed a retrospective, cross-sectional study of 158 VM patients (mean age, 14.3 years, male 42%) who underwent ethanol sclerotherapy at a specialized vascular malformation center. For clinical result assessment, patients or parents in pediatric patients answered questions on symptomatic, functional, and cosmetic improvement after PES. In each category, the possible choices were markedly improved, moderately improved, no change, moderately worse, or markedly worse compared with pretreatment status. A good response was defined as one or more areas of marked improvement on the self-assessment in conjunction with marked improvement on post-treatment images (> or =30% decrease in maximal diameter of VM on magnetic resonance imaging [MRI] or > or =50% decrease in abnormal blood pool ratio on whole body blood pool scintigraphy [WBBPS] compared with pretreatment images). To determine predictors of a good response to PES, uni- and multivariate analysis were conducted on demographics (age, gender), clinical features of VM (location, size, depth of involved tissue, presence of associated lymphatic malformation, MRI findings; well-defined vs ill-defined margin, characteristics of venous drainage during PES) and treatment variables (number of PES sessions, maximal concentration and dosage of ethanol used in PES, adjuvant therapy).nnnRESULTSnSymptomatic, functional, and cosmetic improvement was 28%, 27%, and 34%, respectively, based on patient questionnaires. Based on imaging studies, 42 patients (27%) had markedly improvement. Composite outcome combining questionnaire results and imaging study showed that 16% of patients had a good response. On multivariate analysis, female gender (odds ratio [OR]: 4.49, 95% confidence interval [CI]: 1.24-16.28), no or delayed visualization of drainage vein (OR: 9.22, 95% CI: 1.79-47.51), and a well-defined margin on MRI (OR: 13.38, 95% CI: 2.84-63.12) were independent predictors of good response to PES.nnnCONCLUSIONSnPES should be performed in selected patients in order to obtain the best outcomes and minimize complications. No or delayed visualization of drainage vein on initial direct puncture venogram, a well-defined margin on MRI, and female gender were statistically significant predictors of a good response to PES and may be useful in selecting patients.

Congenital vascular malformations: general treatment principles

The era where surgical excision alone was the sole treatment of venous malformation (VM) is now over. A multidisciplinary approach that utilizes both traditional surgical therapy and endovascular therapy is now the standard of care. Endovascular therapy utilizing primarily both embolization therapy and sclerotherapy is the treatment of choice for surgically ‘inaccessible VM lesions. Surgical therapy of VM lesions has been shown to be more effective when combined with supplemental endovascular therapy.

Congenital Vascular Malformations: General Diagnostic Principles:

Venous malformation (VM) is the most common congenital vascular malformation (CVM), which usually presents as a single lesion in the majority of cases. It also presents as a mixed lesion combined with other CVMs (e.g. lymphatic malformation and arteriovenous malformation [AVM]). Therefore, the diagnosis of VM should include an appropriate work-up, to not only confirm and characterize the VM as either extratruncular or truncular but also to diagnose or exclude the presence of other CVMs. The diagnosis of VM can be made safely using non-invasive to minimally invasive studies, which can also distinguish VM from infantile haemangioma. Invasive studies, such as venography and arteriography, are generally reserved for therapeutic planning and diagnosis of more virulent CVMs (e.g. AVM). The work-up of VM should include a complete assessment of the extent and severity of the primary VM lesion. In addition, its embryologic origin, as well as its haemodynamic characteristics and secondary effects should also be determined.

Arterio-venous malformation: how much do we know?

Arterio-venous malformation (AVM) is a congenital vascular malformation that is neither a venous malformation nor a haemangioma. An AVM is a potentially life-threatening and limb-threatening lesion, especially the ‘fistulous’ truncular form due to its unique embryological and haemodynamic characteristics. AVM treatment requires an early aggressive approach, one that is careful and based on a thorough assessment of the risks and benefits associated with the treatment plan. A successful treatment strategy requires an accurate assessment of the AVM taking into account the extent, severity and progression of the lesion. This is critical in order to minimize the morbidity associated with the currently available therapies. A multidisiciplinary approach that integrates endovascular and surgical therapy can substantially improve the treatment results seen in patients with AVMs. Preliminary treatment of a fistulous AVM with coil embolization is essential in order to minimize associated morbidity and to alter the lesion haemodynamics from a high-flow lesion to a low-flow lesion that is more amenable to subsequent, definitive management with ethanol or NBCA glue embolo/sclerotherapy.

Endovascular Management of Takayasu Arteritis: Is It a Durable Option?:

Interim outcome of endovascular management of Takayasu arteritis (TA) was determined retrospectively to assess the efficacy of angioplasty and/or stenting in 24 patients with 35 lesions in the chronic inactive stage. The renal (n = 16), subclavian/innominate (n = 11), and carotid (n = 5) arteries and abdominal aorta (n = 3) were treated. Twenty-six lesions achieved excellent to good target lesion revascularization with no residual or only minimal residual stenosis, whereas five had a moderate result. Thirty lesions achieved satisfactory hemodynamic correction. Restenosis was observed in 8 lesions treated with angioplasty alone (n = 18) and in 3 lesions treated with angioplasty and stenting (n = 17). All recurrent stenoses underwent successful reintervention without significant complication. Treatment of inactive stage TA lesions with angioplasty alone or with angioplasty and stenting results in excellent to good clinical improvement in the majority of patients (follow-up at 46.8 months). Endovascular therapy is a durable treatment option in patients with chronic inactive stage TA.

Primary Lymphoedema and Lymphatic Malformation: Are they the Two Sides of the Same Coin?

OBJECTIVESnTo clear the confusion regarding the relationship between the primary lymphoedema and (truncular) lymphatic malformation (LM); the latter is one of congenital vascular malformations.nnnMATERIALS & METHODSnA literature review was carried out on the primary lymphoedema either existing as an independent LM lesion or as a component of the Klippel-Trenaunay syndrome.nnnRESULTSnThe review was able to provide a contemporary guide/conclusion on the definition and classification, clinical evaluation and clinical management regarding conservative (physical) therapy, reconstructive surgical therapy and ablative/excisional surgical therapy, for the primary lymphoedema as an LM.nnnCONCLUSIONSnPrimary lymphoedema can be considered as congenital since its majority represents a clinical manifestation of the truncular type of LM arising during the later stages of lymphangiogenesis. Such embryological staging information of the LM is critical for proper management of the primary lymphoedema when it exists with other congenital vascular malformations (Klippel-Trenaunay syndrome). 2. Basic non-invasive to minimally invasive tests will provide an adequate diagnosis and lead to the correct multidisciplinary, specifically targeted and sequenced treatment strategy. 3. The mainstay of current management of the primary lymphoedema/truncular LM is complex decongestive therapy; and the reconstructive as well as ablative surgical therapy remain adjunctive therapies at best.

Treatment of Lymphatic Malformations

The development of the Hamburg Classification, along with a better understanding of vascular malformations, has allowed a more concise description of lymphatic malformation (LM). The management of LM has become clear: “lymphangioma” shou no longer be considered a single LM lesion, but rather one of two different LM lesions belonging to an extratruncular lesion, even though the management of lymphangioma is different from its “truncular” lesion counterpart that clinically presents as primary lymphedema. Although they often present as discrete clinical entities, the lesions can occur together, making clinical management confusing.

Changing Concept on Vascular Malformation: No Longer Enigma

Old Concept nThere have been numerous documentations on vascular malformations in the medical literatures for centuries, describing only general/natural appearance of this inborn error.1) But the vast majority were simple reports of anecdotal experiences with limited information on the basis of poor investigational methods then; its complicated nature has never been properly explained and often described as a name-based ‘syndrome’ (e.g. Klippel-Trenaunay Syndrome) to make it look more enigmatic.2, 3) n nFollowing the turn of the last century, however, a good many clinicians challenged to such unique condition of the vascular defects seriously in the middle of much confusion to define its nature. But they were only able to catch a glimpse of thousands faces of this birth defect involving various locations of the vascular system in various conditions, extents, and severities. And it was not possible till lately to draw whole picture with a birds-eye view due to the limited resource and technology. n nIts extreme variety of clinical appearance and unpredictable behavior in general with a stigma of recurrence gave such notorious reputation to entire group of vascular malformations as an enigma in modern medicine. n nThis once condemned and abandoned disease4) is now newly defined as congenital vascular malformation (CVM) and this new terminology began to clear much confusion on this unique vascular disorder as a universal language replacing many nosologies.

Reconstructive surgery for chronic lymphedema: a viable option, but.

The aim of the paper is to assess the efficacy of reconstructive lymphatic surgery in the treatment of chronic lymphedema via retrospective analysis. Lymphovenous anastomotic surgery (LVAS) or free lymph node transplant surgery (FLTS) was performed on 32 patients who failed to respond to complex decongestive therapy (CDT) alone for a minimum of a one-year period. In LVAS, three patients with good compliance among 19 were able to maintain initial improvement through the four-year follow-up period. All three had secondary lymphedema in clinical stage II. In FLTS, among 13 patients, three compliant patients with the secondary lymphedema in clinical stage II kept initial improvement through the four-year follow-up. In conclusion, reconstructive lymphatic surgery (LVAS and FLTS) appears to be more effective in secondary lymphedema versus primary lymphedema when performed in the early stages. Patient compliance to maintain CDT postoperatively remains the most critical factor in maintaining durable long-term results. FLTS seems to have an additional risk involved to the donor lymph node harvest and a limited role compared to LVAS. Further extended study on FLTS is required to demonstrate its efficacy compared with LVAS.