Treatment of lymphatic malformations in children: 10 years of experience

Benzar, I.M.; Levytskyi, A.F.; Diehtiarova, D.S.; Godik, O.S.; Dubrovin, О.G.

doi:10.15574/ps.2022.75.5

Cited by 2 publications

(5 citation statements)

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“…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].…”

Section: B аcontrasting

confidence: 56%

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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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confidence: 99%

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

Diehtiarova

2022

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Lymphatic malformations (LMs) are rare congenital benign malformations of the lymphatic system, consisting of fluid‑filled cysts or channels with unusual growth. The low incidence of LMs leads to a challenging differential diagnosis and a complicated choice of further treatment strategies. In this study, we analyzed our experience in the treatment of abdominal and retroperitoneal cystic LMs and proposed an anatomy‑based treatment strategy that could improve the treatment outcomes. Objective — to prove that the anatomical localization of LM influences treatment effectiveness and should be taken into account when making treatment decisions. Materials and methods. Out of 240 pediatric patients who underwent treatment for cystic LMs at a single center from December 2012 to December 2020, 43 (19.1 %) were diagnosed with abdominal and retroperitoneal LMs. The follow‑up period was 3.50 ± 2.16 years. Results. Surgical resection of abdominal LMs without evident connection with cisterna chyli does not lead to a recurrence. Sclerotherapy is the best treatment option for retroperitoneal LMs. Diffuse mesentery affection can be successfully treated by sirolimus systemic therapy. According to a logistic regression model, initial choice of LM treatment without considering anatomical localization influences the risks of LM treatment failure (p = 0.000503). All patients in our study group received the following treatments: laparoscopic resections (n = 10, 23.2 %), videoassisted resections (n = 11, 25.5 %), laparotomy resections (n = 9, 20.9 %), sclerotherapy (n = 4, 9.3 %), sclerotherapy and surgery combination (n = 1, 2.3 %), sirolimus systemic therapy (n = 2, 4.6 %), and splenectomy (n = 1, 2.3 %). Six (13.4 %) patients are under dynamic observation. The recommended technique for treating abdominal LMs produced excellent outcomes in 35 (81.4 %) patients, good outcomes in 5 (11.6 %) patients, satisfactory outcomes in 2 (4.7 %) patients, and unsatisfactory outcomes in 1 (2.3 %) patient. Conclusions. Treatment strategies for abdominal and retroperitoneal LMs should be based on their anatomical localization. Retroperitoneal localization indicates a high risk of surgical treatment failure (p = 0.0006).

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Section: B аcontrasting

confidence: 56%

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confidence: 99%

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

Diehtiarova

2022

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“…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].…”

Section: обговоренняunclassified

“…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.…”

Section: обговоренняunclassified

“…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.…”

Section: обговоренняunclassified

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

Diehtiarova¹,

Benzar²,

Rusyn³

et al. 2023

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Lymphatic malformations (LMs) - are congenital vascular malformations caused by abnormal embryologic development of the lymphatic system with variable clinical manifestation. Abdominal LMs account approximately 5% of all LMs. The main treatment option for abdominal LMs is surgical. Purpose - to analyze our experience in intestinal and omental LMs diagnosis and treatment, to reveal factors that could influence the complications occurrence risks, that could improve abdominal LMs treatment results in children. Materials and methods. Among 240 pediatric patients, who underwent cystic LMs treatment in a period from December 2012 to July 2022, 43 (19.1%) were diagnosed abdominal and retroperitoneal LMs. 18 (41.8%) patients with intestinal and mesentery affection were included into the study group. Median follow up was 20.7 (95% CI: 12-27) months. Results. Male patients predominated in the study group. Median age at admission was 2.5 (95% CI: 0.75-5) years. 12 (66.6%) had an emergency admission. In 7 (38.8%) children LMs were complicated by intestinal obstruction (n=5; 71.4%), intracystic bleeding and LM cyst infection were observed in n=1, 14.3%) cases both. Surgical treatment was applied in 16 (88.8%) patients, among which 7 (43.7%) underwent laparoscopy. In 4 (25%) cases conversion to laparotomy took place, 5 (31.3%) underwent laparotomic surgeries. No recurrences after surgical treatment were observed. Systemic therapy with mTOR-inhibitors was prescribed in a child with symptomatic total mesentery affection, observational tactics was chosen for asymptomatic child. Conclusions. Intestinal and mesenterial LMs can be asymptomatic or manifest complications, which were observed in 7 (38.8%) children of our group. Macrocystic intestinal LMs are of higher risks of complications development (р=0.0236). Surgical treatment is effective for local intestinal and mesentery affection, excellent treatment result was achieved in 94.1% of patients. In cases with total mesentery affection mTOR-inhibitors systemic therapy is the treatment of choice. 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 the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

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Treatment of lymphatic malformations in children: 10 years of experience

Cited by 2 publications

References 20 publications

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

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

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

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