Lymphatic malformations in children: treatment outcomes of sclerotherapy in a large cohort

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ORIGINAL ARTICLE

Lymphatic malformations in children: treatment outcomes of sclerotherapy in a large cohort Frédérique C. M. Bouwman 1,2,4 & Silje S. Kooijman 1 & Bas H. Verhoeven 1,4 & Leo J. Schultze Kool 2,4 & Carine J. M. van der Vleuten 3,4 & Sanne M. B. I. Botden 1,4 & Ivo de Blaauw 1 Received: 24 June 2019 / Revised: 3 August 2020 / Accepted: 14 September 2020 # The Author(s) 2020

Abstract This retrospective study examines the outcomes of sclerotherapy in children with (veno)lymphatic malformations who received sclerotherapy between 2011 and 2016 (116 children, 234 procedures). Complication severity was classified using the Society of Interventional Radiology classification. Clinical response was rated on a scale of 0 (no change) to 3 (good improvement). The sclerosants used were bleomycin (n = 132; 56%), lauromacrogol (n = 42; 18%), doxycycline (n = 15; 6%), ethanol (n = 12; 5%), or a combination (n = 33; 14%). Four major and 25 minor complications occurred without significant differences between the agents. The median response rate per procedure was 2—some improvement—for all sclerosants. However, in pure LMs (67%), bleomycin and a combination of agents resulted in the best clinical response. On patient level, all had some or good clinical response. Mixed macrocystic and microcystic lesions showed a significantly lower clinical response (median 2 versus 3; p = 0.023 and p = 0.036, respectively) and required significantly more procedures (median 2 versus 1; p = 0.043 and p = 0.044, respectively) compared with lesions with one component. Conclusion: Sclerotherapy for (V)LMs in children is safe and effective. Bleomycin is the most frequently used agent in this clinic and seemed most effective for pure LMs. Mixed macrocystic and microcystic lesions are most difficult to treat effectively.

What is Known: • A variety of agents can be used for sclerotherapy of lymphatic malformations in children. • Macrocystic lesions have favorable outcomes compared with microcystic and mixed lesions. What is New: • Bleomycin and a combination of agents seem to be most effective to treat lymphatic malformations in children. • Mixed macrocystic and microcystic lesions are more difficult to treat effectively compared with lesions with either one of these components.

Communicated by Piet Leroy * Frédérique C. M. Bouwman [email protected] Silje S. Kooijman [email protected]

Ivo de Blaauw [email protected] 1

Department of Surgery, Division of Pediatric Surgery, Radboudumc-Amalia Children’s Hospital, Nijmegen, Netherlands

2

Leo J. Schultze Kool [email protected]

Department of Radiology and Nuclear Medicine, Division of Interventional Radiology, Radboud University Medical Center (Radboudumc), Nijmegen, Netherlands

3

Carine J. M. van der Vleuten [email protected]

Department of Dermatology, Radboud University Medical Center (Radboudumc), Nijmegen, Netherlands

4

Center for Vascular Anomalies, Hecovan, Radboud University Medical Center (Radboudumc), VASCERN VASC