Spontaneous Resolution of Chiari 1-Associated Syringomyelia: A Report of Two Cases

Abstract

Background: Traditional teaching is that Chiari 1 malformations with syrinx should be operated as soon as possible. We present 2 cases of a radiologically proven Chiari 1 malformation with syrinx which were treated nonoperatively and improved radiologically. Methods: Two children with an MRI-proven Chiari 1 malformations were followed up nonoperatively for 7 years (2010–2017). One was a boy aged 8 years and the other a girl aged 9 years at first presentation. Their parents were not interested in a surgical option and so it was decided to adopt a wait-and-watch policy. Both patients were followed up with a regular half-yearly clinical examination and yearly MRI studies (2010–2017). Results: It was found that, in both of them, the initial cough during swallowing and the headache improved. Serial MRI showed that the hind-brain protrusion was reduced and syrinx diminished or resolved well. Conclusions: Some children with Chiari I and syringomyelia may show spontaneous resolution of symptoms and syringomyelia. Large registry studies will be needed to determine how likely it is that Chiari-associated syringes will resolve, and whether there are particular anatomic or neurologic differences between those who require surgery and those who don’t.

Natural History of Chiari 1 Deformity

“…There have been numerous reported cases of spontaneous resolution of Ch1 with or without associated syringomyelia. 2,5,12,[14][15][16][17][18][19] Cases 2 and 3 represent spontaneously resolving Ch1, supporting the role of conservative management in asymptomatic patients. The process of spontaneous resolution of Ch1 is not clearly understood.…”

“…Because there are difficult choices between conservative and surgical management for Ch1, it is of utmost importance to be aware of the variable natural history of the disease as well as the variable surgical outcome. As Ramnarayan et al 18 have advised, it may be useful to maintain a large registry of studies to determine whether there is spontaneous resolution of Ch1 or the factors that can influence such resolution.…”

The significance of occipitocervical dura angulation in selection of surgery procedures for Chiari malformation type I

“…9). However, there is no need for immediate treatment for children with Chiari malformation type I without syringomyelia and hydrocephalus; routine observation and regular follow-up are recommended [15].…”

Benign syringomyelia with the abortive type of the course

“…К настоящему времени описано несколько десятков наблюдений, в которых сделана попытка объяснить механизм спонтанного коллапса полости при сирингомиелии [6]. Наиболее часто коллапс полости был верифицирован в детском возрасте и сопровождался подъемом миндалин мозжечка [8][9][10][11][12]. Предполагается, что это связано с ростом скелета у детей, увеличением объема ЗЧЯ и, как следствие, с поднятием миндалин, восстановлением нормальной циркуляции ЦСЖ в области БЗО и последующим исчезновением сирингомиелической полости [8].…”

“…В 1998 г. Т. Fukutake и T. Hattori [16] опубликовали наблюдение, которое свидетельствовало о том, что исключение физических нагрузок и других вальсальва-подобных маневров, приводящих к усилению поршневого движения миндалин мозжечка [17], может способствовать регрессу сирингомиелии, ассоциированной с МК. Основываясь на данных литературы, можно условно выделить две ведущие группы теорий спонтанного спадения полости: 1) восстановление потока ЦСЖ через БЗО (подъем миндалин мозжечка в связи с ростом ЗЧЯ [8][9][10][11][12]; разрыв арахноидальных спаек на уровне БЗО [14,18]; устранение вальсальва-подобных маневров [16,[19][20][21]; подъем миндалин мозжечка вследствие атрофии мозга [15]); 2) образование дренажа между полостью и спинальным субарахноидальным пространством [13,22,23].…”