Simultaneous two-stage surgical treatment for Chiari malformation association with occlusive hydrocephalus: a clinical case

   Treatment of patients with the simultaneous presence of Chiari malformation type I (MC I) and hydrocephalus may include both one-stage and two-stage surgical interventions. The article describes the two-stage surgical treatment experience performed for the first time in the Republic of Tajikistan.
   Description of the case. A 50-year-old female patient with a three-year history of bursting headache, dizziness, nausea, and vomiting developed impaired coordination of movements and decreased visual acuity. Computed tomography (CT) revealedsigns of MC I: descent of the cerebellar tonsils 5 mm below the level of the foramen magnum and occlusive triventricular hydrocephalus with symmetrical enlargement of the lateral and third ventricles. The patient underwent surgery – endoscopic third ventriculocisternostomy (ETV) in combination with decompressive suboccipital craniectomy, which was supplemented by CI laminectomy. After the surgery, the symptoms regressed. According to the control CT scan, there was a moderate decrease in the size of the lateral and third ventricles, restoration of the normal anatomical structure of the posterior cranial fossa.

1. Ciaramitaro P., Ferraris M., Massaro F., Garbossa D. Clinical diagnosis-part I: what is really caused by Chiari I. Childs Nerv Syst. 2019 Oct; 35 (10): 1673–1679. doi: 10.1007/s00381-019-04206-z. Epub 2019 Jun 3. PMID: 31161267.

2. Ciaramitaro P., Massimi L., Bertuccio A., et al. Diagnosis and treatment of Chiari malformation and syringomyelia in adults: international consensus document. Neurol Sci. 2022 Feb; 43 (2): 1327–1342. doi: 10.1007/s10072-021-05347-3. Epub 2021 Jun 15. Erratum in: Neurol Sci. 2021 Nov 17. PMID: 34129128.

3. Garner O., Rajasekar S., Ramirez A., Iardino A. Type I Chiari malformation presenting in an adult. BMJ Case Rep. 2018 Jun 12; 2018: bcr2018224889. doi: 10.1136/bcr-2018-224889. PMID: 29895578.

4. Суфианов А. А. Малоинвазивная техника эндоскопической вентрикулоцистерностомии дна III желудочка у детей с окклюзионной гидроцефалией / А. А. Суфианов [и др.] // Вестник Авиценны. – 2019. – 21 (3): 400–407. doi: 10.25005/2074-0581-2019-21-3-400-407. EDN: WZLMLH

5. Kahn E. N., Muraszko K. M., Maher C. O. Prevalence of Chiari I malformation and syringomyelia. Neurosurg Clin N Am. 2015 Oct; 26 (4): 501–507. doi: 10.1016/j.nec.2015.06.006. Epub 2015 Aug 4. PMID: 26408058.

6. Bogdanov E. I., Faizutdinova A. T., Mendelevich E. G., et al. Epidemiology of symptomatic Chiari malformation in Tatarstan: regional and ethnic differences in prevalence. Neurosurgery. 2019 May 1; 84 (5): 1090–1097. doi: 10.1093/neuros/nyy175. PMID: 29788393.

7. Aitken L. A., Lindan C. E., Sidney S., et al. Chiari type I malformation in a pediatric population. Pediatr Neurol. 2009 Jun; 40 (6): 449–454. doi: 10.1016/j.pediatrneurol.2009.01.003. PMID: 19433279.

8. Buell T. J., Heiss J. D., Oldfield E.H. Pathogenesis and cerebrospinal fluid hydrodynamics of the Chiari I malformation. Neurosurg Clin N Am. 2015 Oct; 26 (4): 495–499. doi: 10.1016/j.nec.2015.06.003. Epub 2015 Aug 4. PMID: 26408057.

9. Giannakaki V., Nissen J. Foramen magnum decompression for Chiari malformation type I – UK surgical practice. Br J Neurosurg. 2022 Dec; 36 (6): 737–742. doi: 10.1080/02688697.2022.2107177. Epub 2022 Aug 9. PMID: 35946115.

10. George T. M., Higginbotham N. H. Defining the signs and symptoms of Chiari malformation type I with and without syringomyelia. Neurol Res. 2011 Apr; 33 (3): 240–246. doi: 10.1179/016164111X12962202723760. PMID: 21513644.