Advanced management of congenital vascular malformations: a multidisciplinary approach

Abstract

Introduction: Management of congenital vascular malformations (CVM) remains a major challenge because treatment carries a substantial risk of morbidity and recurrence of the fundamental problem. The new classification of CVM allows a multidisciplinary approach to the treatment with full integration of the several treatment modalities. The multidisciplinary approach was introduced at our CVM Clinic in 1995 and this report summarizes our results to date. Patients and methods: The CVM Clinic is attended by 15 different specialties. These allow application of advanced diagnosis and treatments. The interdisciplinary consultation allows proper application of the various treatment modalities including embolosclerotherapy and surgical therapy. The embolosclerosants utilized are absolute ethanol and N-butyl cyanoacrylate (NBCA). These are used for venous malformations (VM), arteriovenous shunting malformations (AVM), and hemolymphatic malformations (HLM). These agents are used independently and as preoperative adjuncts. Among the 438 patients treated between September 1995 and September 1999, there were 99 patients treated with combinations of embolosclerotherapy. There were 286 sessions, 252 of which were for ethanol sclerotherapy and 247 of these employed ethanol alone and five were combined with NBCA. Independent embolotherapy with NBCA was instituted more recently and has been used in 28 sessions. Perioperative embolosclerotherapy has been performed in 43 sessions, mostly as preliminary preparation to reduce subsequent surgical morbidity. Followup assessment of immediate and interim results after completion of multisession therapy has been done using combinations of noninvasive diagnostic testing. Results: The immediate success rate of embolosclerotherapy has been 94.7% (271/286 sessions). There has been an immediate 5.2% failure (15/286 sessions). Failures have largely been due to forced abandonment of the sclerosing procedure due to risk of deep venous thrombosis. Interim results though short-term success following completion of multisession therapy, average 3.2 sessions per patient, were satisfactory. Complications, mostly skin damage from embolosclerotherapy were experienced in 31 patients during the 286 sessions performed on 99 patients. These skin complications were discussed and accepted by the multidisciplinary team which recognized unavoidable morbidity accompanying the ethanol therapy when applied to superficially located lesions. The overall morbidity included complication rate per session (14.7%, 42/286) and 31.3% per patient (31/99). Recovery from the skin complications has been mostly spontaneous but one case in which peroneal nerve palsy occurred became permanent. There has been no recurrence of the lesions treated successfully, and this has been confirmed through a battery of noninvasive testing. The average follow-up period after completion of multisession therapy is relatively short with 10.6 months (6.0–32 months) only to meet the condition as interim results. Fourteen patients have undergone surgical ablation after preoperative embolosclerotherapy and the surgical morbidity has been minimal. Conclusion: An accurate diagnosis and multidisciplinary treatment strategy for management of CVMs can improve overall treatment success with a reduced morbidity and recurrence over conventional approaches. This study reviews current trends in contemporary diagnosis and clinical management of congenital vascular malformations (CVM) of the peripheral vascular system emphasizing our new multidisciplinary approach.