Chiari Malformasyonunda Epidemiyoloji, Konservatif Tedaviler ve Gebelikte Hasta Yönetimi
Özet
Chiari malformasyonları (CM), serebellumun, beyin sapının ve kraniyoservikal bağlantının anatomik anomalileriyle tanımlanan, serebellumun tek başına veya alt medulla ile birlikte spinal kanala doğru aşağı doğru yer değiştirmesiyle tanımlanan heterojen bir bozukluk grubudur (1). Bu hastalığa ek olarak sirengomiyeli (SM), hidrosefali, ensefalosel veya spinal disrafizm gibi intra ve ekstrakraniyal anomaliler eşlik edebilir. CM’nin yaygınlığı halen tam olarak bilinmeyen bu hastalık asemptomatik klinikten şiddetli baş ağrısı, boyun ağrısı, baş dönmesi dengesizlik, motor ve duyu kayıpları geniş bir klinik tablo oluşturabilir (2).
CM ilk olarak 1883'te John Cleland tarafından tanımlanmıştır (3,4). 1891 ve 1894 yılında Avusturyalı Patolok Hans Chiari tarafından dört alt tipi tanımlamıştır (1,5). 1894'te, Alman Julius Arnold serebellum hernisi olan birlikte hidrosefali olmayan yeni doğanda bir miyelodisplastik bebeği rapor etmiş. Yenidoğanlarda görülen hastalığa Arnold Chiari terimi başlık olarak kullanılırken, erişkinlerde görülenlere ise Chiari malformasyonu başlığı tercih edilmektedir (5).
Referanslar
Sarnat HB. Disorders of segmentation of the neural tube: Chiari malformations. Handb Clin Neurol 2008; 87:89.
Hidalgo JA, Tork CA, Varacallo MA. Arnold-Chiari Malformation. [Updated 2023 Sep 4]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK431076/
Carmel PW, Markesbery WR. Early descriptions of the Arnold-Chiari malformation. The contribution of John Cleland. J Neurosurg 1972; 37:543.
Pearce JM. Arnold chiari, or "Cruveilhier cleland Chiari" malformation. J Neurol Neurosurg Psychiatry 2000; 68:13.
Schijman E. History, anatomic forms, and pathogenesis of Chiari I malformations. Childs Nerv Syst 2004; 20:323.
Arnautovic A, Splavski B, Boop FA, Arnautovic KI. Pediatric and adult Chiari malformation Type I surgical series 1965-2013: a review of demographics, operative treatment, and outcomes. J Neurosurg Pediatr. 2015 Feb;15(2):161-77. [PubMed]
Langridge B, Phillips E, Choi D. Chiari Malformation Type 1: A Systematic Review of Natural History and Conservative Management. World Neurosurg. 2017 Aug;104:213-219. [PubMed]
Rodríguez-Blanque R, Almazán-Soto C, Piqueras-Sola B, Sánchez-García JC, Reinoso-Cobo A, Menor-Rodríguez MJ, Cortés-Martín J. Chiari Syndrome: Advances in Epidemiology and Pathogenesis: A Systematic Review. J Clin Med. 2023 Oct 23;12(20):6694. doi: 10.3390/jcm12206694. PMID: 37892831; PMCID: PMC10607306.
Iskandar BJ, Hedlund GL, Grabb PA, Oakes WJ: The resolution of syringohydromyelia without hindbrain herniation after posterior fossa decompression. J Neurosurg 89:212- 216, 1998
Tubbs RS, Iskandar BJ, Bartolucci AA, Oakes WJ. A critical analysis of the Chiari 1.5 malformation. J Neurosurg. 2004;101(2 Suppl):179-183.
Tubbs RS, Muhleman M, Loukas M, Oakes WJ: A new form of herniation: The Chiari V malformation. Childs Nerv Syst 28:305-307, 2012
Holste KG, Muraszko KM, Maher CO. Epidemiology of Chiari I Malformation and Syringomyelia. Neurosurg Clin N Am. 2023 Jan;34(1):9-15. doi: 10.1016/j.nec.2022.08.001. Epub 2022 Nov 3. PMID: 36424068.
Vernooij MW, Ikram MA, Tanghe HL, et al. Incidental findings on brain MRI in the general population. N Engl J Med 2007;357(18):1821–8.
Morris Z, Whiteley WN, Longstreth WT Jr, et al. Incidental findings on brain magnetic resonance imaging: systematic review and meta-analysis. Bmj 2009;339:b3016.
Aitken LA, Lindan CE, Sidney S, et al. Chiari type I malformation in a pediatric population. Pediatr Neurol 2009;40(6):449–54.
Strahle J, Muraszko KM, Kapurch J, et al. Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr 2011;8(2):205–13.
Ciaramitaro P, Garbossa D, Peretta P, et al. Syringomyelia and Chiari Syndrome Registry: advances in epidemiology, clinical phenotypes and natural history based on a North Western Italy cohort. Ann Ist Super Sanita 2020;56(1):48–58.
Bogdanov EI, Faizutdinova AT, Mendelevich EG, et al. Epidemiology of Symptomatic Chiari Malformation in Tataristan: Regional and Ethnic Differences in Prevalence. Neurosurgery 2019;84(5):1090–7.
Horn SR, Shepard N, Vasquez-Montes D, et al. Chiari malformation clusters describe differing presence of concurrent anomalies based on Chiari type. Clin Neurosci 2018;58:165–71.
Oldfield EH, Muraszko K, Shawker TH, et al. Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg 1994;80(1):3–15.
Meadows J, Kraut M, Guarnieri M, et al. Asymptomatic Chiari Type I malformations identified on magnetic resonance imaging. J Neurosurg 2000;92(6): 920–6.
Mikulis DJ, Diaz O, Egglin TK, et al. Variance of the position of the cerebellar tonsils with age: preliminary report. Radiology 1992;183(3):725–8.
Smith BW, Strahle J, Bapuraj JR, et al. Distribution of cerebellar tonsil position: implications for understanding Chiari malformation. J Neurosurg 2013; 119(3):812–9.
Abdallah A, C¸ınar I_, Gu¨ndag Papaker M, et al. The factors affecting the outcomes of conservative and surgical treatment of chiari i adult patients: a comparative retrospective study. Neurol Res 2022; 44(2):165–76.
Wilkinson DA, Johnson K, Garton HJ, et al. Trends in surgical treatment of Chiari malformation Type I in the UnitedStates. J NeurosurgPediatr 2017;19(2):208–16. 17. Weber F, Knopf H. Cranial MRI as a screening tool: findings in 1,772 military pilot applicants. Aviat Space Environ Med 2004;75(2):158–61
Greenberg JK, Olsen MA, Yarbrough CK, et al. Chiari malformation Type I surgery in pediatric patients. Part 2: complications and the influence of comorbid disease in California, Florida, and New York. Neurosurg Pediatr 2016;17(5):525–32.
Krucoff MO, Cook S, Adogwa O, et al. Racial, Socioeconomic, and Gender Disparities in the Presentation, Treatment, and Outcomes of Adult Chiari I Malformations. World Neurosurg 2017;97:431–7.
Vedantam A, Mayer RR, Staggers KA, et al. Thirtyday outcomes for posterior fossa decompression in children with Chiari type 1 malformation from the US NSQIP-Pediatric database. Childs Nerv Syst 2016;32(11):2165–71.
Sakushima K, Tsuboi S, Yabe I, et al. Nationwide survey on the epidemiology of syringomyelia in Japan. Neurol Sci 2012;313(1–2):147–52
Brickell KL, Anderson NE, Charleston AJ, et al. Ethnic differences in syringomyelia in New Zealand. Neurol Neurosurg Psychiatr 2006;77(8):989–91.
Heiss JD, Oldfield EH, Zhuang Z, Lubensky I,Patronas N, Smith R, et al. Genetic analysis of theChiari I malformation; 2000. Available from: http://clinicaltrials.gov/ct2/show/NCT00004738?term=chiari+AND+genetics&rank=2.
Krucoff MO, Cook S, Adogwa O, Moreno J, Yang S, Xie J, et al. Racial, socioeconomic, and gender disparities in the presentation, treatment, and outcomes of adult Chiari I malformations. World Neurosurg. 2017;97:431–7.
Killeen A, Roguski M, Chavez A, Heilman C,Hwang S. Non-operative outcomes in Chiari Imalformation patients. J Clin Neurosci. 2015;22:133-138.
Avellaneda Fernández A, Isla Guerrero A, Izquierdo Martínez M, et al. Malformations of the craniocervical junction (Chiari type I and syringomyelia: classification, diagnosis and treatment). BMC Musculoskelet Disord. 2009;10:S1.
Abdallah A, Rakip U. Conservative Treatment of Chiari Malformation Type I Based on the Phase-Contrast Magnetic Resonance Imaging: A Retrospective Study. World Neurosurg. 2022 Jul;163:e323-e334. doi: 10.1016/j.wneu.2022.03.126. Epub 2022 Apr 1. PMID: 35367644.
Fabritius ML, Strøm C, Koyuncu S, et al. Benefit and harm of pregabalin in acute pain treatment: a systematic review with meta-analyses and trial sequential analyses. Br J Anaesth. 2017;119:775-791.
Chalaupka FD. Therapeutic effectiveness of acetazolamide in hindbrain hernia headache. Neurol Sci. 2000 Apr;21(2):117-9. doi: 10.1007/s100720070107. PMID: 10938192.
Karimy JK, Duran D, Hu JK, Gavankar C, Gaillard JR, Bayri Y, Rice H, DiLuna ML, Gerzanich V, Marc Simard J, Kahle KT. Cerebrospinal fluid hypersecretion in pediatric hydrocephalus. Neurosurg Focus. 2016 Nov;41(5):E10. doi: 10.3171/2016.8.FOCUS16278. PMID: 27798982.
Garcia MA, Allen PA, Li X, Houston JR, Loth F, Labuda R, Delahanty DL. An examination of pain, disability, and the psychological correlates of Chiari Malformation pre- and post-surgical correction. Disabil Health J. 2019 Oct;12(4):649-656. doi: 10.1016/j.dhjo.2019.05.004. Epub 2019 May 21. PMID: 31147250
Cuthbert S, Blum C. Symptomatic Arnold-Chiari malformation and cranial nerve dysfunction: a case study of applied kinesiology cranial evaluation and treatment. J Manipulative Physiol Ther. 2005 May;28(4):e1-6. doi: 10.1016/j.jmpt.2005.03.001. PMID: 15883570.
Khoury, Chaouki. Chiari malformations. UpToDate. Wolters Kluwer Health, Philadelphia, 2015
Mueller DM, Oro' J. Chiari I malformation with or without syringomyelia and pregnancy: case studies and review of the literature. Am J Perinatol. 2005 Feb;22(2):67-70. doi: 10.1055/s-2005-837271. PMID: 15731983.)
Janjua MB, Haynie AE, Bansal V, Bhattacharia S, Grant T, McQuillan D, Passias PG, Ozturk AK, Hwang SW. Determinants of Chiari I progression in pregnancy. J Clin Neurosci. 2020 Jul;77:1-7. doi: 10.1016/j.jocn.2020.05.026. Epub 2020 May 7. PMID: 32414621.
Vig S, Kumar KR, Poudel D. Acute exacerbation of Chiari malformation: A rare cause for non-awakening from anaesthesia. Indian J Anaesth. 2018 Mar;62(3):238-239. doi: 10.4103/ija.IJA_760_17. PMID: 29643564; PMCID: PMC5881332.
Gruffi TR et al. Anesthetic management of parturients with Arnold Chiari malformation-I: a multicenter retrospective study. Int J Obstet Anesthesia 2019;37:52–6. https://doi.org/10.1016/j.ijoa.2018.10.002 .
Wilkinson DA et al. Obstetric management and maternal outcomes of childbirth among patients with Chiari malformation type I. Neurosurgery 2020;87(1):45–52. https://doi.org/10.1093/neuros/nyz341 .
Semple DA, McClure JH. Arnold-Chiari malforma tion in pregnancy. Anaesthesia. 1996;51(6):580–2.
Bolognese PA, Kula RW, Onesti ST. Chiari I malfor mation and delivery. Surg Neurol Int. 2017;8:12.
Chantigian RC, Koehn MA, Ramin KD, Warner MA. Chiari I malformation in parturients. J Clin Anesth. 2002;14(3):201–5.
Parker JD, Broberg JC, Napolitano PG. Maternal Arnold-Chiari type I malformation and syringomy elia: a labor management dilemma. Am J Perinatol. 2002;19(8):445–50.
Choi CK, Tyagaraj K. Combined spinal-epidural anal gesia for laboring parturient with Arnold-Chiari type I malformation: a case report and a review of the litera ture. Case Rep Anesthesiol. 2013;2013:512915.
Rosalind L. Jeffree, Marcus A. Stoodley, Management of Chiari in pregnancy, Journal of Clinical Neuroscience, Volume 83, 2021, Pages 153-154, ISSN 0967-5868,https://doi.org/10.1016/j.jocn.2020.10.025. (https://www.sciencedirect.com/science/article/pii/S0967586820315770)
Roelofse JA, Shipton EA, Nell AC. Anaesthesia for caesarean section in a patient with syringomyelia: a case report. S Afr Med J 1984;65(18):736–7.
Ghaly RF, Tverdohleb T, Candido KD, Knezevic NN. Management of parturients in active labor with Arnold Chiari malformation, tonsillar herniation, and syringomyelia. Surg Neurol Int. 2017;8:10.
Garvey GP, Wasade VS, Murphy KE, Balki M. Anesthetic and obstetric Management of syringo myelia during Labor and delivery: a case series and sys tematic review. Anesth Analg. 2017;125(3):913–24.
Roper JC, Al Wattar BH, Silva AHD, Samarasekera S, Flint G, Pirie AM. Management and birth outcomes of pregnant women with Chiari malformations: a 14 years retrospective case series. Eur J Obstet Gynecol Reprod Biol. 2018;230:1–5
Sicuranza GB, Steinberg P, Figueroa R. Arnold-Chiari malformation in a pregnant woman. Obstet Gynecol. 2003;102(5 Pt 2):1191–4.
Tew J., McMahon N. Mayfield Chiari Center | Chiari I Overview |. Mayfieldchiaricenter.com. http://mayfieldchiaricenter.com. Published 2019. Accessed October 3, 2019
Orth T, Gerkovich M, Babbar S, Porter B, Lu G. 716: maternal and pregnancy complications among women with Arnold Chiari malformation: a national database review. Am J Obstet Gynecol. 2015;212(1):S349.
Puissant L, Deckers J, Soetens F. Chiari malformation unmasked by accidental dural puncture. Eur J Anaesthesiol. 2014 Nov;31(11):646-8. doi: 10.1097/EJA.0000000000000097. PMID: 24879071.