Clinical case of Arnold-Chiari syndrome in combination with dextrocardia in a child

Arnold-Chiari syndrome is a rare congenital disease characterized by a decrease in the volume of the cranial cavity and a change in the anatomy of the posterior cranial fossa. Arnold-Chiari syndrome has several types (from 1 to 4), each of which is characterized by a different degree of compression of the brain and hydrocephalus, and its new forms have recently been added (type 0 and 1.5). the quality of life of patients. The disease is most common in children and young people. According to experts, its prevalence ranges from 3.2 to 8.4 cases per 100,000 people worldwide. The incidence and severity of Arnold-Chiari syndrome have a statistically significant tendency to increase in the female sex. The etiology of Arnold-Chiari syndrome has not yet been fully studied, but it is assumed that genetic factors and effects on the fetus during pregnancy may play a dominant role in its development. Clinically, the syndrome is manifested by headache, vomiting, swallowing disorders, various motor disorders, impaired coordination and long-lasting Babinsky reflex, weakness and numbness in the extremities, sleep problems and other symptoms. Patients may also develop hydrocephalus, which increases the risk of serious complications and requires surgical interventions. The article presents data on the prevalence, clinical manifestations and treatment of Arnold-Chiari syndrome, as well as our own clinical observation of the course of this disease, which is combined with dextrocardia and heterotaxia – the mirror position of internal organs in a 10-year-old patient. Arnold-Chiari syndrome today refers to severe congenital diseases of the nervous system that are difficult to diagnose at an early age, therefore it is important for a pediatrician to understand the mechanism of occurrence of this disease in order to be able not only to eliminate, but also to prevent the undesirable consequences of this disease