Russian Journal of Pediatric Surgery, Anesthesia and Intensive CareRussian Journal of Pediatric Surgery, Anesthesia and Intensive CareРоссийский вестник детской хирургии, анестезиологии и реаниматологии2219-40612587-6554Eco-Vector95810.17816/psaic958ReviewsОбзорыReview ArticleSpina bifida: a multidisciplinary problem (a literature review)Spina bifida: мультидисциплинарная проблема (обзор литературы)https://orcid.org/0000-0002-2187-39731447-6213IvanovStanislav V.ИвановСтанислав Вячеславович
Russian Federation

Cand. Sci. (Med.)

кандидат медицинских наук, руководитель отделения №5 детского церебрального паралича

ortostas@mail.ruhttps://orcid.org/0000-0002-7651-84855597-8832KenisVladimir M.КенисВладимир Маркович
Russian Federation

Dr. Sci. (Med.)

доктор мед. наук, доцент

kenis@mail.ruhttps://orcid.org/0000-0002-6754-987X7784-8024ShchedrinaAnna Y.ЩедринаАнна Юрьевна
Russian Federation

urologist

врач-уролог

annashchedrina@list.ru1946-6731OnufriichukOleg N.ОнуфрийчукОлег Николаевич
Russian Federation

Dr. Sci. (Med.), ophthalmologis

кандидат медицинских наук, врач-офтальмолог

oon@inbox.ruhttps://orcid.org/0000-0002-2772-67473348-8038KhodorovskayaAlina M.ХодоровскаяАлина Михайловна
Russian Federation

scientific associate

научный сотрудник

alinamyh@gmail.comhttps://orcid.org/0000-0002-1170-3436OsipovIgor B.ОсиповИгорь Борисович
Russian Federation
osipovib@mail.ruSarychevSergei A.СарычевСергей Александрович
Russian Federation

Cand. Sci. (Med.), urologist

кандидат медицинских наук, врач-уролог

serg_sarychev@mail.ruH. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma SurgeryНациональный медицинский исследовательский центр детской травматологии и ортопедии им. Г.И. ТурнераSaint Petersburg State Pediatric Medical UniversityСанкт-Петербургский государственный педиатрический медицинский университетSt. Petersburg State Pediatric Medical UniversityСанкт-Петербургский государственный педиатрический медицинский университет070720211122012130504202112052021Copyright ©; 2021, Ivanov S.V., Kenis V.M., Shchedrina A.Y., Onufriichuk O.N., Khodorovskaya A.M., Osipov I.B., Sarychev S.A.Copyright ©; 2021, Иванов С.В., Кенис В.М., Щедрина А.Ю., Онуфрийчук О.Н., Ходоровская А.М., Осипов И.Б., Сарычев С.А.2021Ivanov S.V., Kenis V.M., Shchedrina A.Y., Onufriichuk O.N., Khodorovskaya A.M., Osipov I.B., Sarychev S.A.Иванов С.В., Кенис В.М., Щедрина А.Ю., Онуфрийчук О.Н., Ходоровская А.М., Осипов И.Б., Сарычев С.А.https://creativecommons.org/licenses/by-nc-nd/4.0https://rps-journal.ru/jour/article/view/958

BACKGROUND: Congenital malformations of the spine and spinal cord can be combined with various clinical manifestations of the spine, spinal cord, and lower extremities. Children with these neurological disorders often lack sensitivity and motor activity of their lower extremities and, in most cases, have bladder infections and incontinence (lack of bladder and bowel control).

AIM: This study aims to analyze publications with the diagnostic and treatment results of patients with neurological, orthopedic, neurological, and ophthalmological problems with spina bifida.

MATERIALS AND METHODS: We searched PubMed, Web of Science, Scopus, MEDLINE, eLibrary, and RSCI databases and found about 2000 references and 374 articles. We selected 60 articles for review in orthopedics, neurosurgery, urology, and ophthalmology.

RESULTS: Neural tube defects are a wide range of congenital malformations, including skull defects and open or closed spinal dysraphism. The incidence of spine and spinal cord malformations in different countries is quite broad and amounts to 0.3–199.4 cases per 10,000 births worldwide. Spinal cord malformations often occur in combination with bladder infections and incontinence, limb deformities, and other central nervous system developmental anomalies. Among the orthopedic problems leading to impaired support function, the most common are foot deformities and hip joint instability. Orthopedic monitoring of a patient with spina bifida consists of mainly preventing or correcting deformities according to the rehabilitation potential of the child. The timely completion of treatment allows the child to maintain mobility and independence of movement daily activities. At the same time, such treatment must pursue realistic goals according to the potential motor level of the child. In addition to neurosurgical and orthopedic problems, most children with spina bifida (88%–94%) suffer from pelvic disorders. A urologist should observe a patient with spina bifida to perform ultrasound and laboratory monitoring of both the lower and upper urinary tract conditions from an early age. Timely procedures to eliminate urinary retention and sanitation can maintain normal kidney function and contribute to the adequate conduct of motor and neurological rehabilitation of the child. The most common complication of spina bifida is the Chiari II malformation, which is manifested by damage to brain stem structures and internal occlusal hydrocephalus with various symptoms, including neuroophthalmological signs.

CONCLUSIONS: A multidisciplinary team of specialists comprising a neurologist, neurosurgeon, urologist, orthopedic surgeon, ophthalmologist, orthosis specialist, and psychologist should be involved in treating the children with the above presented problems. The use of an integrated approach to treat this group is absolutely justified and enabled the maximum rehabilitation potential of the child to be achieved.

Введение. Врожденные пороки развития позвоночника и спинного мозга могут сопровождаться разнообразными клиническими проявлениями со стороны позвоночника, спинного мозга и нижних конечностей. У детей с данной патологией очень часто выявляются неврологические нарушения в виде отсутствия чувствительности и двигательной активности нижних конечностей, у большинства из них отмечаются нарушения функции тазовых органов.

Цель — проанализировать публикации, в которых представлены результаты диагностики и лечения пациентов с неврологическими, ортопедическими, урологическими и офтальмологическими проблемами на фоне врожденных пороков развития позвоночника и спинного мозга.

Материалы и методы. Проанализировано около 2000 ссылок в базах данных PubMed, Web of Science, Scopus, MEDLINE, eLibrary, РИНЦ, просмотрено 374 статьи, отобрано в обзор 60 — по ортопедии, нейрохирургии, урологии, офтальмологии.

Результаты. Дефекты нервной трубки представляют собой широкий спектр врожденных аномалий, которые включают дефекты черепа, открытый или закрытый спинальный дизрафизм. Частота встречаемости пороков развития позвоночника и спинного мозга в разных странах достаточно широка и составляет 0,3–199,4 случая на 10 000 родов в мире. Пороки развития спинного мозга часто сочетаются с нарушениями формирования органов малого таза, деформациями конечностей, а также другими аномалиями развития центральной нервной системы. Среди ортопедических проблем, которые приводят к нарушению функции опоры, наиболее часто встречаются деформации стоп и нестабильность тазобедренного сустава. Ортопедическое наблюдение за пациентом со спинномозговой грыжей (лат. spina bifida) состоит, главным образом, в предотвращении или исправлении деформаций согласно реабилитационному потенциалу ребенка. Вовремя выполненное лечение позволяет сохранить подвижность и независимость передвижения ребенка в окружающей среде. В то же время такое лечение должно оставаться в рамках реалистичных ожиданий и в границах потенциального двигательного уровня ребенка. Помимо вышеуказанных нейрохирургических и ортопедических проблем подавляющее большинство спинальных детей (88–94 %) страдает от тазовых нарушений. Наблюдение уролога за пациентом со spina bifida подразумевает ультразвуковой и лабораторный мониторинг состояния как нижних, так и верхних мочевыводящих путей с раннего возраста. Своевременные процедуры по устранению задержки и санации мочи позволяют сохранить нормальную функцию почек и способствуют адекватному проведению двигательной и неврологической реабилитации ребенка. Наиболее частым осложнением spina bifida является аномалия Киари II, проявляющаяся поражением стволовых структур мозга и внутренней окклюзионной гидроцефалией с разнообразной, в том числе нейроофтальмологической, симптоматикой.

Заключение. Решением представленных в статье проблем у пациентов со spina bifida должна заниматься мультидисциплинарная команда специалистов в составе невролога, нейрохирурга, уролога, ортопеда, офтальмолога, ортезиста, психолога. Использование комплексного подхода в лечении этой группы пациентов абсолютно обоснованно и позволяет достичь максимального реабилитационного потенциала ребенка.

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