А.Т. FAYZUTDINOVA^1,2, E.I. BOGDANOV^1,2

¹Republican Clinical Hospital of the MH of RT, 138 Orenburgskiy Trakt, Kazan, Russian Federation, 420064

²Kazan State Medical University, 49 Butlerov Str., Kazan, Russian Federation, 420012 

Fayzutdinova A.T. ― Cand. Med. Sc., Associate Professor of the Department of Neurology and Rehabilitation, neurologist of Clinical Expertise Department, tel. +7-903-306-44-38, e-mail: aisluzab@mail.ru

Bogdanov E.I.― D. Med. Sc., Professor of the Department of Neurology and Rehabilitation, Head of Neurological Clinic, tel. (843) 237-34-72, e-mail: enver_bogdanov@mail.ru 

Severe clinical polymorphism of Chiari Malformation Type 1 (CM1) determines the practical interest to the study of the natural history of the disease, as well as the possibilities of its surgical correction. Analysis of the natural history of CM1 shows that about half of patients with this malformation are clinically asymptomatic. After the clinical manifestation, the majority of patients have a relatively favorable course of the disease. Significant progression of symptoms occurs in 30% of patients. In 12% of patients severe neurological disorders develop. The main method of preventing the progression and development of CM1 and CM1-associated syringomyelia complications is surgical decompression of the posterior cranial fossa. But currently CM1 surgical treatment is performed in 6-69% of cases. The differences in surgical activity indicators may be due to the heterogeneity of populations being compared, and the difference in surgical approaches to the disease. The review discusses algorithms for patient selection for surgical treatment and its effectiveness. To develop ways to improve care for patients with CM1, stages of treating patients with this pathology were determined and analyzed: the stage of identifying patients, the stage of determining the diagnosis and treatment strategies, the stage of treatment and follow-up, and the stage of remote observation and correction of treatment.

Key words: Chiari Malformation Type 1, natural history of the disease, surgical correction, ways to improve care for patients.

REFERENCES

1. Zabbarova A.T., Bogdanov E.I. Syndrome “close” the rear cranial fossa and associated neurological symptom. Kazanskiy meditsinskiy zhurnal, 2011, no. 6, pp. 890-896 (in Russ.).
2. Klekamp J., Samii M. Syringomyelia: diagnosis and treatment. Springer-Verlag, Berlin, 2002. 195 r.
3. Luciano M. Chiari malformation: are children little adults? Neurol. Research, 2011, vol. 33 (3), pp. 272-277.
4. Vannemreddy P., Nourbakhsh A., Willis B., Guthikonda B. Congenital Chiari malformations. Neurol. India, 2010, vol. 58, pp. 6-14.
5. Strahle J., Muraszko K., Kapurch J. et al. Natural history of Chiari malformation Type I following decision for conservative treatment. J. Neurosurg. Pediatr, 2011, vol. 8 (2), pp. 214-221.
6. Chavez A., Roguski M., Killeen A. et al. Comparison of operative and non-operative outcomes based on surgical selection criteria for patients with Chiari I malformations. J. Clin. Neurosci, 2014, vol. 21 (12), pp. 2201-2206.
7. Massimi L., Caldarelli M., Frassanito P., Di Rocco C. Natural history of Chiari type I malformation in children. Neurol. Sci, 2011, vol. 32, suppl. 3, pp. 275-277.
8. Bogdanov E. Epidemiology. Flint G., Rusbridge C. (eds.). Syringomyelia. Springer-Verlag Berlin Heidelberg, 2014. Pp. 11-24.
9. Boman K., Livanainen M. Prognosis of syringomyelia. Acta Neurol. Scand, 1967, vol. 43, pp. 61-68.
10. Brickell K., Anderson N., Charleston A. et al. Ethnic differences in syringomyelia in New Zealand. JNNP, 2006, vol. 77, pp. 989-991.
11. Fayzutdinova A.T. Sindrom vnezapnoy smerti pri mal’formatsii Kiari [The syndrome of sudden death in Chiari malformation]. Sbornik tezisov 16-go Kongressa Ros. obshchestva kholterovskogo monitorirovaniya i neinvazivnoy elektrofiziologii (ROKhMiNE), 8-go Vseros. kongressa “Klinicheskaya elektrokardiologiya”, 1-y Vseros. konf. detskikh kardiologov FMBA Rossii, Kazan, 22-23.04.2015. Kazan: Praktika, 2014. Pp. 32-33.
12. Roohi F., Gropen T. Sudden unexpected nocturnal death in Chiari type 1 malformation and potential role of opioid analgesics. Surg. Neurol. Int, 2014, vol. 5, p. 177.
13. Ferre M.A., Poca M.A., de la Calzada M.D. et al. Sleep disturbance: A forgotten syndrome in patients with Chiari I Malformation. Neurologia, 2014, vol. 29 (5), pp. 294-304.
14. Levy W.J., Mason L., Hahn J.F. Chiari malformation presenting in adults: a surgical experience in 127 cases. Neurosurgery, 1983, vol. 12 (4), pp. 377-390.
15. Stevens J., Serva W., Kendall B. et al. Chiari malformation in adults: relation of morphological aspects to clinical features and operative outcome. JNNP, 1993, vol. 56, pp. 1072-1077.
16. Zuev A.A., Pedyash N.V., Epifanov D.S., Kostenko G.V. Results of surgical treatment of syringomyelia associated with Chiari abnormality of type 1 (analysis of 125 cases). Voprosy neyrokhirurgii im. N.N. Burdenko, 2016, no. 80 (1), pp. 27-34 (in Russ.).
17. Bao Ch., Yang F., Liu L. et al. Surgical treatment of Chiari I malformation complicated with syringomyelia. Experiment. Therapeut. Med, 2013, vol. 5, pp. 333-337.
18. Bao C.S., Liu L., Wang B. et al. Craniocervical decompression with duraplasty and cerebellar tonsillectomy as treatment for Chiari malformation-I complicated with syringomyelia. Genet. Mol. Res, 2015, vol. 3, no. 14 (1), pp. 952-960.
19. Deng X., Yang C., Gan J. et al. Long-term outcomes following small-bone-window posterior fossa decompression and duraplasty in adults with Chiari malformation type I. World Neurosurg, 2015, vol. 84 (4), pp. 998-1004.
20. Greenberg J., Yarbrough Ch., Radmanesh A. et al. The Chiari Severity Index: A Preoperative Grading System for Chiari Malformation Type 1. Neurosurgery, 2015, vol. 76 (3), pp. 279-285.
21. Grinberg M.S. Neyrokhirurgiya [Neurosurgery]. Moscow: MEDpress inform, 2010. 1008 p.
22. Gushcha A.O., Shakhnovich A.R., Kashcheev A.A. et al. The new minimally invasive method of surgical treatment of anomalies Arnold — Chiari:. experimentally-clinical research. Rossiyskiy neyrokhirurgicheskiy zhurnal im. prof. A.L. Polenova, 2010, vol. II, no. 4, pp. 28-38 (in Russ.).
23. Alzate J.C., Kothbauer K.F., Jallo G.I., Epstein F.J. Treatment of Chiari I malformation in patients with and without syringomyelia: a consecutive series of 66 cases. Neurosurg. Focus, 2001, vol. 15, no. 11 (1), E3.
24. Shamji M.F., Ventureyra E.C., Baronia B. et al. Classification of symptomatic Chiari I malformation to guide surgical strategy. Can. J. Neurol. Sci, 2010, vol. 37 (4), pp. 482-487.
25. Quon J.L., Grant R.A., Di Luna M.L. Multimodal evaluation of CSF dynamics following extradural decompression for Chiari malformation Type I. Neurosurg. Spine, 2015, vol. 22 (6), pp. 622-630.
26. Chotai S., Medhkour A. Surgical outcomes after posterior fossa decompression with and without duraplasty in Chiari malformation-I. Clin. Neurol. Neurosurg, 2014, vol. 125, pp. 182-188.
27. Arnautovic A., Splavski B., Boop F., Arnautovic K. Pediatric and adult Chiari malformation Type I surgical series 1965-2013: a review of demographics, operative treatment, and outcomes. J. Neurosurg. Pediatr, 2015, vol. 15 (2), pp. 161-177.
28. Bikmullin T.A., Bariev E.R., Anisimov V.I. Comparative analysis of different methods of surgical treatment of anomalies Arnold — Chiari. Prakticheskaya meditsina, 2015, no. 4 (89), pp. 28-30 (in Russ.).
29. Greenberg J., Ladner T., Olsen M. et al. Complications and Resource Use Associated With Surgery for Chiari Malformation Type 1 in Adults: A Population Perspective. Neurosurgery, 2015, vol. 77 (2), pp. 261-268.
30. McGirt M.J., Attenello F.J., Atiba A. et al. Symptom recurrence after suboccipital decompression for pediatric Chiari I malformation: analysis of 256 consecutive cases. Childs Nerv. Syst, 2008, vol. 24 (11), pp. 1333-1339.
31. Meadows J., Guarnieri M., Miller K. et al. Type I Chiari Malformation: A Review of the Literature. Neurosurg. Quarterly, 2001, vol. 11, no. 3, pp. 220-229.
32. Nishikawa M., Sakamoto H., Kitano S. A clinical study on long-term outcome in patients with syringomyelia associated with Chiari malformation. No To Shinkei, 1999, vol. 51, pp. 318-323.
33. Fayzutdinova A.T., Bogdanov E.I.. Shakurov A.R. et al. Ambulatornaya konsul’tativno-diagnosticheskaya pomoshch’ pri mal’formatsii Kiari i siringomielii [Outpatient consultative-diagnostic help with Chiari malformation and syringomyelia]. Materialy VIII Ros. nauch.-prak. konferentsii s mezhdunarod. uchastiem “Zdorov’e cheloveka v XXI veke”, Kazan, 31.03-01.04.2016. Kazan: Brig, 2016. Pp. 584-591.
34. Zabbarova A.T., Bogdanov E.I. Sravnitel’naya chastota anomaliy zadney cherepnoy yamy sredi vzroslogo naseleniya v rayonakh Respubliki Tatarstan s raznoy ekologicheskoy obstanovkoy [Relative frequency of posterior cranial fossa abnormalities in the adult population in the Republic of Tatarstan regions with different ecological conditions]. Materialy II Ros. nauch.-prakt. konferentsii “Zdorov’e cheloveka v XXI veke”, Kazan, 03.04.2010. Vol. 2. Kazan, 2010. Pp. 40-41.
35. Mendelevich E.G., Mikhaylov M.K., Bogdanov E.I. Siringomieliya i mal’formatsiya Arnol’da — Kiari [Syringomyelia and malformation of Arnold — Chiari]. Kazan: Meditsina, 2002. 236 p.