Current Concepts in Lymphatic Malformation

Abstract

A lymphatic malformation (LM) is the most common form of congenital vascular malformation (CVM). The new Hamburg classification of CVM distinguishes the truncular (T) form from the extratruncular (ET) form of LMs. Both are consequences of a developmental arrest at the different stages of lymphangiogenesis as a result of defective genes. The purpose of this review was to evaluate the current management results of both forms of LMs. A retrospective review of the clinical data of 315 patients with a diagnosis of LMs treated between September 1994 and December 2001 was performed. Lymphoscintigraphy was the most frequent diagnostic test. The patients with the ET form were treated with sclerotherapy with OK-432 and/or ethanol. Combinations of CDP (complex decongestive physiotherapy) and/or compressotherapy were used to treat all the T-form patients. In addition, surgery, either reconstructive or ablative, was offered to patients with the T form who failed to respond to the proper CDP. A multidisciplinary team performed the management of LM, and the results were evaluated every 6 months. Among 797 patients with CVM, 315 were confirmed to have LMs, either as the T form (226) or the ET form (89). Another 66 LMs were diagnosed with hemolymphatic malformations (HLM). Most of the ET forms (89/315) were the cystic type (70/89), while the T forms included aplasia and/or an obstruction (204/226). The ET form was most frequent in the head, neck, and thorax (69/89). The T form was located most frequently to the extremities (202/226), mostly to the lower limb (180/202). Two hundred and twenty-six T forms belonged to the various clinical stages: stages I-32, II-104, III-48, IV-18, and an unclear stage-24. The ET form was treated with sclerotherapy using OK-432 (108/120) and absolute ethanol (12/120). Among the 11 patients with the multiple ET form, 7 patients underwent perioperative sclerotherapy with OK-432 and a subsequent surgical excision. The clinical response of the T form at the extremity to CDP was excellent to good in a majority of clinical stages I to II (121/136) but decreased to a good to fair degree in stages III to IV (31/66). The additional surgical therapy, either reconstructive (10/19) or ablative (9/19), provided limited success in improving CDP efficacy, owing mainly to poor compliance. The long-term outcome of the initial success through self-motivated home-maintenance care during the follow-up period of up to 48 months was totally dependent on patient compliance. OK-432 sclerotherapy to 51 ET forms has shown excellent results on 88.9% of the cystic type (40/45) and 50% (3/6) of the cavernous type (minimum follow-up for 24 months). Seventeen ET forms in 7 patients were treated with a preoperative OK-432 sclerotherapy and a subsequent surgical excision, which provided good to excellent results in 14 for a minimum of 24 months. Primary lymphedema, which is the T form of LMs, can be managed safely by a combination of CDP with compressotherapy. Patients with good compliance can benefit from additional surgical thera...

Changing Concept on Vascular Malformation: No Longer Enigma

“…leakage, sepsis). 34,35) However, when these three different CVMs: AVM, VM, and LM should coexist as a combined form of CVMs: hemolymphatic malformation (HLM), its clinical management becomes much complicated due to their interwound hemodynamics. Additional precaution is mandated when the treatment is required to one of the coexisting CVMs since it often affects to other remaining CVM component negatively to worsen the condition.…”

“…OK 432). 34,55) However, once this ethanol is indicated, it should be given in multi-sessions with minimally possible amount for each session to reduce potential risk of acute/chronic morbidity and complication.…”

Malformaciones linfáticas: tratamiento percutáneo con bleomicina

“…1 Existen publicaciones que sugieren una conducta expectante para las ML asintomáticas, 2 pero la mayoría de los autores concuerda en que se requiere algún tipo de tratamiento, ya que en su evolución pueden aumentar de tamaño y comprimir órganos vecinos, producir deformaciones óseas o cosméticas, o sufrir infección o sangrado en su interior. [3][4][5][6][7][8][9] Tradicionalmente, la exéresis quirúrgica era considerada la terapia de elección para estas malformaciones; 5,[10][11][12] pero su popularidad ha ido menguando con el tiempo, pues no está exenta de complicaciones, es frecuente la recurrencia de la lesión (10-53%) e, incluso, existe una mortalidad de 2-6%. 3 Más recientemente, dos agentes esclerosantes, la bleomicina 13 y el OK-432, 14 han ganado popularidad mundial en el tratamiento de estas lesiones, debido a su elevada eficacia terapéutica y mínimos efectos adversos.…”

“…[3][4][5][6][7][8][9] Tradicionalmente, la exéresis quirúrgica era considerada la terapia de elección para estas malformaciones; 5,[10][11][12] pero su popularidad ha ido menguando con el tiempo, pues no está exenta de complicaciones, es frecuente la recurrencia de la lesión (10-53%) e, incluso, existe una mortalidad de 2-6%. Más recientemente, dos agentes esclerosantes, la bleomicina 13 y el OK-432, 14 han ganado popularidad mundial en el tratamiento de estas lesiones, debido a su elevada eficacia terapéutica y mínimos efectos adversos.…”

Isolation and characterization of lymphatic endothelial cells from human glossal lymphangioma

“…The limitations of surgical treatment have awoken the interests in other therapeutic methods. The administration of sclerosing agents such as bleomycin and hypotonic salt solutions provokes an inflammation of the vascular endothelium leading to total or partial remission of human lymphangioma (7)(8)(9)(10)(11). These substances may extend beyond the limits of the original lymphangioma, unsatisfactory aesthetic results and increased difficulties for future surgery.…”