morphoМорфологические ведомостиMorphological newsletter1812-31712686-8741Private Medical University REAVIZ10.20340/mv-mn.2024.32(1).818morpho-818Research ArticleОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯRESEARCH ARTICLESОСОБЕННОСТИ МОРФОЛОГИЧЕСКОЙ ДИАГНОСТИКИ БОЛЕЗНИ ГИРШПРУНГАFEATURES OF THE MORPHOLOGICAL DIAGNOSTICS OF HIRSCHSPRUNG'S DISEASEhttps://orcid.org/0000-0002-1605-7031ГоворухинаОльга АлексеевнаGovorukhinaOl'ga A.

кандидат медицинских наук, доцент, заведующая хирургическим отделением № 2

Docent, Candidate of Medical Sciences, Head of the 2nd Surgical Department

govorukhina@mail.ruhttps://orcid.org/0000-0002-1347-6847ПрокопеняНаталья СергеевнаProkopenyaNatal'ya S.

детский хирург

Pediatric Surgeon

6666917@gmail.comХаритончикВасилий АлександровичKharitonchikVasily A.

детский хирург

Pediatric Surgeon

harivas@mail.ruНоваковскаяСветлана АлексеевнаNovakovskayaSvetlana A.

кандидат медицинских наук, ведущий научный сотрудник

Candidate of Medical Sciences, Leading Researcher

novakovskaya@tut.byКузнецоваТатьяна ЕвгеньевнаKuznetsovaTat'yana E.

кандидат биологических наук, ведущий научный сотрудник

Candidate of Вiological Sciences, Leading Researcher

tania_k@mail.ruКлецкийСемен КивовичKletskySemyon K.

доцент, кандидат медицинских наук, патологоанатом

Pathologist, Docent, Candidate of Medical Sciences

s.kletski@mail.ruРеспубликанский научно-практический центр детской хирургии, МинскБеларусьRepublican Scientific and Practical Center fof Children's Surgery, MinskBelarusРеспубликанский научно-практический центр детской хирургии, МинскБеларусьRepublican Scientific and Practical Center of Children's Surgery, MinskBelarusИнститут физиологии Национальной академии наук Республики Беларусь, МинскБеларусьInstitute of Physiology of the National Academy of Sciences of Belarus Republic, MinskBelarusГородское клиническое патологоанатомическое бюро, МинскБеларусьCity Clinical Pathology Bureau, MinskBelarus202404072024321ID-818Copyright © Говорухина О.А., Прокопеня Н.С., Харитончик В.А., Новаковская С.А., Кузнецова Т.Е., Клецкий С.К., 20242024Говорухина О.А., Прокопеня Н.С., Харитончик В.А., Новаковская С.А., Кузнецова Т.Е., Клецкий С.К.Govorukhina O.A., Prokopenya N.S., Kharitonchik V.A., Novakovskaya S.A., Kuznetsova T.E., Kletsky S.K.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.morpholetter.com/jour/article/view/818

Болезнь Гиршпрунга – сложное хирургическое заболевание, верификация которого возможна только гистологическими методами. Это заболевание относится к группе нейрокристопатий, которая включает не только аганглиоз толстой кишки, но и другие виды нарушений ее структурной организации, лечебно-диагностическая тактика при которых остается до сих пор спорной. Цель исследования – выявить морфологические изменения в стенке толстой кишки различными методами исследования, необходимыми для диагностики, хирургической коррекции и прогнозирования результатов лечения болезни Гиршпрунга. Настоящее исследование выполнено на материалах 119 пациентов детского возраста для проведения проспективного анализа результатов их лечения. Среди них был 91 пациент мужского пола и 28 женского пола. Пациентам были выполнены гистохимическое, иммуногистохимическое, электронно-микроскопическое исследования толстой кишки, световая микроскопия нативных препаратов для экспресс-биопсии и постоперационного материала с окраской гематоксилином и эозином. Морфологическое исследование биоптатов толстой кишки свидетельствовало о наличии дегенеративных процессов во всех оболочках толстой кишки на развитие которых влияет не только полное отсутствие интрамуральных нейронов в зоне аганглиоза, но и гипоплазия интрамуральных нервных волокон и сплетений на всем протяжении толстой кишки. Полученные данные об изменениях в оболочках толстой кишки проксимальнее зоны аганглиоза свидетельствуют как о первичной гипоплазии структур нервной, эндокринной и иммунной систем в оболочках кишки, так и вторичных дегенеративных изменениях, развивающихся у пациентов с возрастом. Выявленные изменения в структурах ганглиев толстого кишечника при болезни Гиршпрунга не всегда могут объяснить функциональные результаты, связанные с моторикой кишечника, даже после успешного хирургического лечения. Указанные изменения также объясняют возникновение энтероколитов как в до-, так и в послеоперационном периоде. Нарушения в структурах иммунной системы являются причиной возникновения рецидивирующих энтероколитов. Анализ собственных результатов позволяет утверждать, что для каждой формы болезни Гиршпрунга и вида биопсии необходимы соответствующие методы морфологического исследования.

Hirschsprung's disease is a complex surgical disorder that requires histological methods to be verified. This disease is part of the neurocristopathies group, which encompasses colon aganglionosis and other structural disorders, about their treatment and diagnostic tactics are still unresolved. The purpose of the study is to identify morphological changes in the colon wall by using a variety of research methods necessary for diagnosing, surgical correction, and predicting treatment results for Hirschsprung's disease. A prospective analysis of their treatment results was performed on the materials of 119 pediatric patients in this study (91 – boys, 28 – girls). Histochemical, immune-histochemical, and electron microscopic methods were performed on the patients' bioptats colon, along with light microscopy for express biopsy and postoperative material stained with hematoxylin and eosin. Morphological analysis of colon biopsy specimens revealed the presence of degenerative processes in all colon shits, which led to the changes of its structures. Intramural nerve fibers and plexuses throughout the colon were hypoplastic and the complete absence of intramural neurons in the aganglionosis zone is what causes this changes. The findings on the changes in the colon shits close to the aganglionosis zone suggest that the neuromuscular, endocrine, and immune system structures in the intestinal membranes are undergoing primary hypoplased. Secondary degenerative changes also occur in patients with age. Despite successful surgical treatment, functional results associated with intestinal motility in Hirschsprung's disease may not always be satisfactory, as evidenced by changes in the structures of intestinal ganglia. The occurrence of enterocolitis is also explained by these changes, both during and after surgery. Our own results allow us to state that diverse morphological examination methods are necessary for different forms of Hirschsprung's disease and biopsy types.

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