Treatment of lymphatic malformations in children: 10 years of experience

Abstract

Purpose is to identify the factors that determine the clinical features of the LMs, their complications and the choice of treatment options. Materials and methods. 225 children with LMs who underwent treatment since 2011 to 2020 were enrolled. They were grouped according to ISSVA 2018 classification. Clinical presentation, treatment options, complications and outcomes were analyzed. One-variant analysis using Pearson’s χ2 test was used to evaluate qualitative data. Results. 214 (95.1%) patients had cystic LMs, 11 (4.9%) had combined LMs. 129 (57.3%) LMs were cervicofacial, 17 (11.9%) patients developed airways compression and 6 (4.2%) required tracheostomy. 8 (3.6%) patients developed 10 lymphatic leaks episodes, 3 were lethal. 112 (86.8%) patients with cervicofacial, 5 (11.6%) abdominal, 10 (84.6%) axillar, and 12 (92.2%) extremities LMs underwent sclerotherapy. Surgical resections prevailed in abdominal LMs, 32 (74.5%), versus 4 (3.1%) cervicofacial LMs. 10 (4.4%) patients with combined and complicated cystic LMs underwent systemic sirolimus therapy. Results of treatment were excellent in 116 (51.6%) patients, good in 82 (36.4%), satisfactory in 23 (10.2%), and unsatisfactory in 4 (1.8%), with 4 mortalities caused by sepsis (n=1) and by massive lymph leakage (n=3). Conclusions. The most life-threatening LMs complications are airways compression and uncontrollable lymphatic leakages. Several approaches were used, including surgery, sclerotherapy, and target therapy. Surgery is optional for abdominal LMs but potentially harmful in cervicofacial. Treatment of combined LM is continuous and requires a combination of different methods. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institutions. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors. Keywords: lymphatic malformations, children, sclerotherapy, target therapy.

Anatomy-based treatment strategy for abdominal and retroperitoneal cystic lymphatic malformations in children

“…Retroperitoneal LMs are even less common [15]. Earlier studies [5,13] stated that LMs in children affected the bowel and mesentery only, while later publications included the omentum, mesocolon [1,11,12], mesentery root [3], and retroperitoneum [1,12]. 19.1 % of patients in our study group had abdominal LMs, as opposed to previous studies claiming that they account for no more than 10 % [14].…”

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Оригінальні дослідженняLymphatic malformations (LMs) are congenital benign malformations of the lymphatic system, consisting of disorganized spongy fluid-filled cysts or fluid-filled channels with unusual growth. According to the ISSVA 2018 classification, cystic LMs can be macrocystic, microcystic, or mixed type [1, 7]. Abdominal LMs have a relatively rare incidence [17], amounting to up to 5 % [9], or less than 10 % [14] of all LMs.

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Intestinal and mesenterial cystic lymphatic malformations in children: clinical course features and treatment tactics

“…ЛМ складаються з розширених лімфатичних каналів, які утворюють кістоподібні структури з фіброзними перетинками. Внутрішні стінки ЛМ вистелені зрілим ендотелієм без клітинної атипії, тому ЛМ не є новоутвореннями [1,2,3,18].…”

“…Більшість ЛМ діагностується в перші кілька років життя [1,8,10,12,15]. Пік проявів абдомінальних ЛМ припадає на вік 2-5 років [5,9,[12][13][14], зазвичай через клінічні прояви гострого живота.…”

“…Сполуку, що інгібує mTOR, сіролімус, схвалено для лікування судинних мальформацій, оскільки вона викликає зниження фактора росту ендотелію судин, який є відомим ключовим регулятором у лімфангіогенезі та ангіогенезі. Про успішне застосування mTOR-інгібіторів при обширних мезентеріальних ураженнях у контексті складних лімфатичних аномалій, при яких хірургічне лікування має обмежений успіх, повідомлено в різних джерелах [1,4,7,19]. У нашій досліджуваній групі консервативну терапію mTORінгібіторами призначено дитині із симптомним тотальним ураженням брижі, і за час спостереження в пацієнта отримано добрий результат лікування.…”