Advanced management of congenital vascular malformations (CVM).

INTRODUCTION: Congenital vascular malformations (CVM) still remain one of the most difficult diagnostic and therapeutic enigmas in modern medicine due to its extremely variable clinical presentation ranging from asymptomatic birthmarks to life threatening conditions. Extreme rarity also made it quite difficult for the clinician to make a proper diagnosis and institute optimum treatment. Poor understanding of the nature of the disease and subsequent ill-planned cavalier approach mostly by the surgeon alone only resulted in often disastrous outcomes due to prohibitively high complication and recurrence. Recently more logical attitude with multidisciplinary approach based on Hamburg classification achieved better understanding of the anatomy and pathophysiology and also advanced medical technology was able to provide the improved management in general. CLINICAL ASSESSMENT: The diagnosis itself is now feasible mostly with various combination of basic laboratory tests of non- to less- invasive nature. Selective invasive study now can be reserved as road map for the treatment in general. CLINICAL MANAGEMENT: Treatment strategy has to be set up separately for the "primary malformation" itself first from their "secondary disorders" along the vascular system and/or skeleton and soft tissue. In addition to various absolute indications of the treatment, the relative indication includes various conditions to affect the quality of life significantly besides the lesions with potentially high risk of complication, vascular-bone syndrome and cosmetically severe deformity. Various combinations of the embolo/sclerotherapy with conventional surgical therapy is implemented properly to each different malformation: absolute ethanol sclerotherapy to the venous malformation, absolute ethanol and/or N-butyl cyanoacrylate and/or coil, etc. to the AV malformation, and OK-432 to lymphatic malformation, as major embolo/scleroagents. Surgical therapy as part of total care management of CVM on the basis of multidisciplinary approach has been fully integrated to the embolo/sclerotherapy and active incorporation of the preoperative and/or postoperative embolo/sclerotherapy provides substantial improvement on the overall results of the therapy. Non- to poor surgical candidate should be considered for embolo/sclerotherapy as the primary mode of therapy. Extreme lesion with completely non-functional status of the limb should be considered for the early amputation and subsequent proper rehabilitation, instead of continuation of the aimless embolo/sclerotherapies.
CONCLUSIONS: Multidisciplinary approach to congenital vascular malformations allows proper diagnosis and optimum treatment with selective combination of wide variety of therapy strategy by the integration of embolo/sclerotherapy and surgical therapy providing excellent results of the treatment even in case of complex lesions.