rospedРоссийский педиатрический журнал имени М.Я. СтуденикинаM.Ya. Studenikin Russian Pediatric JournalФГАУ «НМИЦ здоровья детей» Минздрава России10.46563/1560-9561-2024-27-6-429-434ejbmqsrosped-1188Research ArticleОБЗОРЫREVIEWSОсобенности диагностики и лечения мекониевого илеуса у новорождённых детейDiagnostics and treatment of meconium ileus in newbornshttps://orcid.org/0009-0009-9397-4384ПилоянФеликс СамсоновичPiloyanFelix S.

Аспирант хирургического отделения новорождённых и детей грудного возраста ФГАУ «НМИЦ здоровья детей» Минздрава России

e-mail: fpiloyan@bk.ru

Postgraduate student of the surgical department of newborns and infants, National Medical Research Center for Children’s Health, Moscow, 119991, Russian Federation

e-mail: fpiloyan@bk.ru

fpiloyan@bk.ruhttps://orcid.org/0000-0001-8663-2698ГурскаяАлександра СергеевнаGurskayaAleksandra S.

Канд. мед. наук, зав. хирургическим отделением новорождённых и детей грудного возраста ФГАУ «НМИЦ здоровья детей» Минздрава России, доцент каф. детской хирургии с курсом анестезиологии и реаниматологии Института подготовки медицинских кадров ФГАУ «НМИЦ здоровья детей» Минздрава России

e-mail: aldra_gur@mail.ru

aldra_gur@mail.ruhttps://orcid.org/0000-0002-8563-6002ДьяконоваЕлена ЮрьевнаDyakonovaElena Yu.

Доктор мед. наук, зав. отделением общей и плановой хирургии НИИ детской хирургии ФГАУ «НМИЦ здоровья детей» Минздрава России, зав. каф. детской хирургии с курсом анестезиологии и реаниматологии Института подготовки медицинских кадров ФГАУ «НМИЦ здоровья детей» Минздрава России

e-mail: rytella@mail.ru

rytella@mail.ruФГАУ «Национальный медицинский исследовательский центр здоровья детей» Минздрава РоссииNational Medical Research Center for Children’s Health202424122024276429434Copyright © Пилоян Ф.С., Гурская А.С., Дьяконова Е.Ю., 20242024Пилоян Ф.С., Гурская А.С., Дьяконова Е.Ю.Piloyan F.S., Gurskaya A.S., Dyakonova E.Y.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.rosped.ru/jour/article/view/1188Актуальность

Актуальность. Мекониевый илеус (МИ) — заболевание, характеризующееся формированием густого, вязкого мекония в просвете тонкой кишки с развитием низкой обтурационной кишечной непроходимости у новорождённых детей. Нарушение формирования мекония у детей с муковисцидозом связано с мутацией гена CFTR, развитием апикальной дегидратации секреторного эпителия стенки кишки и экскреторной дисфункцией поджелудочной железы.

Цель обзора

Цель обзора: определить ведущие механизмы формирования МИ у новорождённых детей для оптимизации его лечения. Проведён обзор литературы по теме: МИ. У недоношенных новорождённых развитие обтурационной кишечной непроходимости ассоциировано не только с наличием плотного мекония, но и с незрелостью моторной функции желудочно-кишечного тракта. Гиперэхогенные массы в просвете кишки, кальцификаты в брюшной полости, расширение кишечных петель и свободная жидкость в брюшной полости по данным пренатального ультразвукового исследования указывают на высокую вероятность муковисцидоза у плода, что требует проведения генетического консультирования родителей. Одним из лечебно-диагностических методов при МИ является постановка гипертонической клизмы с водорастворимым гиперосмолярным контрастным веществом. Эта тактика допустима к применению при простых формах МИ в качестве консервативной терапии и является эффективной в 25% случаев. Осложнённые формы МИ характеризуются наличием вторичной атрезии, изолированного заворота тонкой кишки, выраженной ишемии или некрозом кишки. В тяжёлых случаях одновременно с перфорацией кишечной стенки развивается мекониевый перитонит. Хирургические методы лечения простых и осложнённых форм МИ включают отмывание мекония из кишечника с наложением первичного анастомоза, альтернативные малотравматичные операции (энтеротомии), а также различные варианты стомирующих операций. Однако пока ещё не разработан протокол лечения МИ (в том числе у больных муковисцидозом). Сохраняются трудности в выборе способа хирургической коррекции МИ недоношенных.

Участие авторов

Участие авторов: Гурская А.С., Дьяконова Е.Ю. — концепция и дизайн работы, редактирование; Пилоян Ф.С., Гурская А.С. — сбор и обработка материала; Пилоян Ф.С. — написание текста. Все соавторы — утверждение окончательного варианта статьи, ответственность за целостность всех частей статьи.

Финансирование

Финансирование. Работа не имела спонсорской поддержки.

Конфликт интересов

Конфликт интересов. Авторы заявляют об отсутствии конфликта интересов.

Поступила 08

Поступила 08.11.2024Принята к печати 06.12.2024Опубликована 25.12.2024

Introduction

Introduction. Meconium ileus is a disease characterized by the formation of thick, viscous meconium in the lumen of the small intestine with the development of low obstructive intestinal obstruction in newborns. Impaired meconium formation in children with cystic fibrosis is associated with a mutation of the CFTR gene, the development of apical dehydration of the secretory epithelium of the intestinal wall and excretory dysfunction of the pancreas.

The aim of the review

The aim of the review: to identify the leading mechanisms of formation of meconium ileus in newborn children to optimize its treatment. A review of the literature on the topic meconium ileus. In premature newborns, the development of obstructive intestinal obstruction is associated not only with the presence of dense meconium, but also with immaturity of the motor function of the gastrointestinal tract. Hyperechogenic masses in the intestinal lumen, calcifications in the abdominal cavity, dilation of intestinal loops, and free fluid in the abdominal cavity according to prenatal ultrasound indicate a high probability of cystic fibrosis in the fetus, which requires genetic counselling parents. One of the therapeutic and diagnostic methods for meconium ileus is the setting of a hypertensive enema with a water-soluble hyperosmolar contrast agent. This tactic being acceptable for use in simple forms of meconium ileus as a conservative therapy is effective in 25% of cases. Complicated forms of meconium ileus are characterized by the presence of secondary atresia, isolated inversion of the small intestine, severe ischemia or intestinal necrosis. In severe cases, meconium peritonitis develops simultaneously with perforation of the intestinal wall. Surgical methods for the treatment of simple and complicated forms of meconium ileus include washing meconium from the intestine with the imposition of primary anastomosis, alternative low-traumatic operations (enterotomy), as well as various options for stoma operations. However, a protocol for the treatment of meconium ileus has not yet been developed (including patients with cystic fibrosis). Difficulties remain in choosing the method of surgical correction of the meconium ileus of premature infants.

Contribution

Contribution: Gurskaya A.S., Dyakonova E.Yu. — concept and design of the work; Piloyan F.S., Gurskaya A.S. — collection and processing of the material; Piloyan F.S. — writing the text; Gurskaya A.S., Dyakonova E.Yu. — editing the text. All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment

Acknowledgment. The study had no sponsorship.

Conflict of interest

Conflict of interest. The authors declare no conflict of interest.

Received

Received:  November 08, 2024Accepted: December 06, 2024Published: December 25, 2024

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The authors declare that there are no conflicts of interest present.