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DOI: 10.1055/s-0030-1261883
Bleomycin Sclerotherapy for Extensive Lympho-Venous Malformation. A Retrospective Analysis of A Patient's Death
Publikationsverlauf
Publikationsdatum:
28. Juli 2010 (online)
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Introduction
Lympho-venous malformations (LVM) in children may be localized everywhere in a child's body as limited or extensive malformations, including a few anatomical regions representing errors in vascular and lymphatic development. LVM are classified as combined malformations [1]. A radical surgical excision of the malformation reported to be the best treatment, however in extensive LVM complete resection of the malformation is impossible. Incomplete resection of the malformation leads to recurrence and over-resection leads to tissue and organ damage [2] [3] [4]. Thus, the search for a non-surgical form of treatment for LVM continues. Direct injection sclerotherapy has been proposed with few side-effects, but the effectiveness of direct sclerotherapy was poor.
Yura and co-workers in 1997 reported good results with bleomycin sclerotherapy of lymphatic malformations (LM) after unsuccessful surgical treatment [5]. Other centers have been skeptical of this treatment due to the unclear therapeutic mechanism of bleomycin and high risk of post therapeutic pulmonary complications [6] [7].
An early toxic effect on pulmonary tissue was observed in 10% of all oncologic patients who received i. v. bleomycin therapy and presented with fever, dry cough, dyspnea at rest and cyanosis. Permanent damage to pulmonary tissue causing pulmonary fibrosis after bleomycin therapy was described as occurring some weeks or even months after treatment. In patients who underwent bleomycin therapy, spirometric restrictive changes were described. Vital capacity (VC), total lung capacity (TLC) and diffusion lung capacity for carbon monoxide (DLCO) have been shown to decrease. A DLCO below 40% of initial values is a simple contraindication to bleomycin therapy. Early as well as late pulmonary toxicity of bleomycin with progressive pulmonary fibrosis causes death in 1–2% of patients [8] [9].
A combined treatment of a patient with extensive LVM who died at the age of 20 years is presented.
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Correspondence
Dr. Piotr Wojciechowski
Jagiellonian University
Pediatric Surgery
Wielicka 265
PL-60-663 Kraków
Poland
Telefon: +48 60 2634 722
Fax: +48 12 6581 325
eMail: piwojr@vp.pl