136 -10 (60) 2023 — Gurskaya A.S., Sulavko M.A. — DIAGNOSIS AND TREATMENT OF LYMPHATIC ANOMALIES IN THE SURGICAL DEPARTMENT OF NEWBORNS AND INFANTS OF THE FGAU «NATIONAL MEDICAL RESEARCH CENTER FOR CHILDREN’S HEALTH» OF THE MINISTRY OF HEALTH OF THE RUSSIAN FEDERATION

DIAGNOSIS AND TREATMENT OF LYMPHATIC ANOMALIES IN THE SURGICAL DEPARTMENT OF NEWBORNS AND INFANTS OF THE FGAU “NATIONAL MEDICAL RESEARCH CENTER FOR CHILDREN’S HEALTH” OF THE MINISTRY OF HEALTH OF THE RUSSIAN FEDERATION

Gurskaya A.S., Federal State Budgetary Institution “National Medical Research Center for Children’s Health” of the Ministry of Health of Russia, Moscow

Sulavko M.A., Federal State Budgetary Institution “National Medical Research Center for Children’s Health” of the Ministry of Health of Russia, Moscow

Dyakonova E.Yu., Federal State Budgetary Institution “National Medical Research Center for Children’s Health” of the Ministry of Health of Russia, Moscow

Bayazitov R.R., Federal State Budgetary Institution “National Medical Research Center for Children’s Health” of the Ministry of Health of Russia, Moscow

Nakovkin O.N., Federal State Budgetary Institution “National Medical Research Center for Children’s Health” of the Ministry of Health of Russia, Moscow

Ekimovskaya E.V., Federal State Budgetary Institution “National Medical Research Center for Children’s Health” of the Ministry of Health of Russia, Moscow

Karnuta I.V., Federal State Budgetary Institution “National Medical Research Center for Children’s Health” of the Ministry of Health of Russia, Moscow

Klepikova A.A., Federal State Budgetary Institution “National Medical Research Center for Children’s Health” of the Ministry of Health of Russia, Moscow

Xagurov R.A., GBUZ Children’s City Clinical Hospital named after N.F. Filatov Department of Health of the City of Moscow, Moscow

Sagoyan G.B. Federal State Budgetary Institution “National Medical Center of Oncology named after N.N. Blokhin” Ministry of Health of Russia, Moscow

Resume

The purpose of the study was to analyze the results of treatment of various types of LM in newborns and infants. The authors analyzed the medical histories of children who were in the surgical department of newborns and infants of the Federal State Institution “National Medical Research Center for Children’s Health” of the Ministry of Health of Russia, for the period from 2017 to 2023. with lymphatic malformations of various locations, chylous effusions in the abdominal and thoracic cavities. A total of 19 case histories of children with confirmed LM of various types. Of these, 16 patients had a cystic type of LM, 1 patient had a combined type of vascular malformation, and 2 patients had vascular malformations associated with other anomalies.

According to the authors’ conclusion, further multicenter studies are required to develop clinical recommendations for the diagnosis and treatment of various types of LM, given the rarity of this pathology. In order to study the etiology, all patients with LM should undergo genetic testing for a spectrum of overgrowth syndromes.

Key words: diagnosis and treatment of lymphatic abnormalities, surgical departments for newborns and infants, Russia, CIS countries.

First page

786

Last page

791

For citaton: Gurskaya A.S., Sulavko M.A., Dyakonova E.Yu., Bayazitov R.R., Nakovkin O.N., Ekimovskaya E.V., Karnuta I.V., Klepikova A.A., Xagurov R.A., Sagoyan G.B. – DIAGNOSIS AND TREATMENT OF LYMPHATIC ANOMALIES IN THE SURGICAL DEPARTMENT OF NEWBORNS AND INFANTS OF THE FGAU “NATIONAL MEDICAL RESEARCH CENTER FOR CHILDREN’S HEALTH” OF THE MINISTRY OF HEALTH OF THE RUSSIAN FEDERATION //New day in Medicine 2023 10(60): 786-791 https://newdaymedicine.com/index.php/2023/12/27/l-695/

LIST OF REFERENCES:

  1. Amodeo I., Colnaghi M., Raffaeli G., Cavallaro G., Ciralli F., Gangi S., et al. The use of sirolimus in the treatment of giantcystic lymphangioma. Four case reports and update of medical therapy. // Medicine 2017;96:51(e8871) DOI:10.1097/MD.0000000000008871
  2. Olive G., Kipnis J., Randolph G.J., Harvey N.L.The Lymphatic Vasculature in the 21st Century:Novel Functional Roles in Homeostasis and Disease. // Cell 2020 Jul 23;182(2):270-296. doi: 10.1016/j.cell.2020.06.039.
  3. Byrne A.B., Pascal Brouillard P., Sutton D.L., Kazenwadel J, Montazaribarforoushi S, Secker G.A. Pathogenic variants in MDFIC cause recessive central conducting lymphatic anomaly with lymphedema. // Science Translation Medicine, 2022 Mar 2;14(634):eabm4869 DOI:10.1126/scitranslmed.abm4869
  4. Сагоян Г.Б, Клецкая И.С., Имянитов Е.Н., Мареева Ю.М., Жуков Н.В., Хагуров Р.А., Сулейманова А.М. Спектр синдромов избыточного роста, связанных с мутацией PIK3CA. Обзор литературы. // Российский журнал детской гематологии и онкологии. 2022;9(1):29-44
  5. ISSVA Classifi cation of Vascular Anomalies©. 2018 International Society for the Study of Vascular Anomalies Accessed. [Electronic resource]: http://www.issva.org/classifi cation.
  6. Кучеров Ю.И., Холоднова Н.В., Адлейба С.Р., Белая А.Л., Макарова Л.М., Овсянникова М.А. и др. Хилоперитонеум у новорожденных: этиология, патогенез, диагностика и лечение. // Детская хирургия. 2019; 23(3):139-142. DOI:10.18821/1560-9510-2019-23-3-139-142
  7. Ayman A., El-Asmar Khaled M., Mohamed M., Sameh A. Surgical management of congenital chylous ascites. Annals of Pediatric Surgery 2018;14(2):56-59. DOI:10.1097/01.XPS.0000525972.33509.0
  8. Донюш Е.К., Кондрашова З.А., Поляев Ю.А., Гарбузов Р.В. Опыт использования сиролимуса в лечении детей с сосудистыми аномалиями. // Российский журнал детской гематологии и онкологии. 2020;7(3):22-31 DOI: 10.21682/2311-1267-2020-7-3-22-31
  9. Saziye K., Gino G., Afksendiyos K. Somatostatin treatment of a persistent chyloperitoneum following abdominal aortic Surgery. Journal of vascular surgery. 2012;56(5):1409-1412. DOI:10.1016/j.jvs.2012.05.004
  10. Roehr Charles C, Jung A., Proquitté H., Blankenstein O., Hammer H.,Lakhoo K. et al. Somatostatin or octreotide as treatment options for chylothorax in young children: a systematic review. // Intensive Care Med 32, 2006;650-657 DOI:10.1007/s00134-006-0114-9
  11. Mizuno T, Fukuda T, Emoto C, Mobberley-Schuman PS, Hammill AM, Adams DM, and Vinks AA.Developmental pharmacokinetics of sirolimus: Implications for precision dosing in neonates and infants with complicated vascular anomalies. // Pediatr Blood Cancer. 2017;00:e26458. doi: 10.1002/pbc.26470.

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