CLINICAL AND MORPHOLOGICAL CHARACTERISTICS OF RECTAL HYPOGANGLIONOSIS IN CHILDREN

INTRODUCTION. Hypoganglionosis in children remains an insufficiently studied disease, often considered a variant of Hirschsprung's disease, which complicates its morphological diagnosis and choice of treatment tactics. Data on the dynamics of histological and immunohistochemical changes depending on the duration of the disease, as well as on the role of apoptosis, inflammation, and microcirculation disorders, are limited. In this regard, a comprehensive morphological study taking into account the duration of hypoganglion appears to be relevant and allows us to clarify its pathogenesis and nosological independence. 

OBJECTIVE. To study the morphological and immunohistochemical features of hypoga nglionosis in children depending on the duration of the disease.

MATERIALS AND METHODS. Rectal biopsy specimens from 13 children with hypoganglionosis were analyzed. Morphological and immunohistochemical examinations were performed using antibodies to calretinin, vimentin, GFAP, CD95, and IL-6.

RESULTS. The severity of morphological changes was found to correlate with disease duration. Early stages showed reversible vascular and neuronal alterations, while at 2–3 years signs of progression were observed, including edema, sclerosis, and hypertrophy of nerve trunks. In cases exceeding 3 years, pronounced destructive and neurodegenerative changes were noted. Immunohistochemistry revealed CD95 and IL-6 expression in neurons and endothelial cells, indicating activation of apoptosis and hypoxia.

CONCLUSION. Hypoganglionosis in children is characterized by progressive vascular and neurodegenerative changes, the severity of which depends on disease duration. The findings confirm the role of apoptosis and microcirculatory disorders in its pathogenesis and support considering hypoganglionosis as an independent pathological entity

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