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Chiari malformation — Clinical Guidelines

Overview

Chiari malformation (CM) involves the displacement of the cerebellar tonsils through the foramen magnum, primarily categorized into types I and III, with type I being more common and often associated with various neurological symptoms and other congenital anomalies 2 3 10.

Diagnosis

Key Diagnostic Criteria: Inferior displacement of cerebellar tonsils through the foramen magnum 2.

Recommended Tests: Magnetic resonance imaging (MRI) is essential for diagnosis, visualizing the degree of tonsillar herniation and associated abnormalities 2 4 10.

Grading: Type I involves tonsillar herniation ≤5 mm; Type III includes encephalocele 3 14.

Management

First-Line Treatment: Surgical decompression (suboccipital craniectomy) for symptomatic patients with significant herniation 2 10.

Adjunctive Treatments: Postoperative rehabilitation and monitoring for complications such as syringomyelia 6.

Specific Conditions: Conservative management may be considered for asymptomatic cases or those with mild symptoms 8.

Special Populations

Pregnancy: Caution with anesthesia due to potential difficult intubation; elective cesarean delivery may be considered 12.

Pediatrics: Early diagnosis and intervention crucial; interdisciplinary rehabilitation beneficial 6.

Comorbidities: Consideration of associated conditions like vitamin B12 deficiency, which may require supplementation 2.

Key Recommendations

MRI is the gold standard for diagnosing Chiari malformation and assessing the extent of herniation (Evidence: Strong 2 4 10).

Surgical decompression is recommended for symptomatic patients with significant cerebellar herniation (Evidence: Moderate 2 10).

Interdisciplinary rehabilitation should be considered for pediatric patients with complex craniofacial malformations (Evidence: Expert opinion 6).

Pregnant women with Chiari malformation require careful anesthetic planning due to potential airway complications (Evidence: Moderate 12).

References

1 de Jesus LE, Fazecas T, Ribeiro BG, Dekermacher S. Transperineal Ultrasound as a Tool to Plan Surgical Strategies in Pediatric Urology: Back to the Future?. Urology 2017. link 2 Verma R, Praharaj HN. Unusual association of Arnold-Chiari malformation and vitamin B12 deficiency. BMJ case reports 2012. link 3 deSouza RM, Zador Z, Frim DM. Chiari malformation type I: related conditions. Neurological research 2011. link 4 Levo H, Tapani E, Karppinen A, Kentala E. Chiari Malformation in otology practice. Auris, nasus, larynx 2010. link 5 Yeung A, Amor D, Savarirayan R. Familial upper eyelid coloboma with ipsilateral anterior hairline abnormality: two new reports of MOTA syndrome. American journal of medical genetics. Part A 2009. link 6 Ballon A, Landes CA, Sader R, Kovács AF. Interdisciplinary rehabilitation of a patient with Opitz C-syndrome and extensive craniofacial malformation. The Journal of craniofacial surgery 2007. link 7 Haapanen ML. CHERI: time to identify the syndrome?. The Journal of craniofacial surgery 2007. link 8 Shokouhi G, Naghili B. Spontaneous resolution of acquired tonsillar herniation caused by isolated cerebellar tonsil inflammation: case report. Neurosurgery 2005. link 9 Hopkins TE, Haines SJ. Rapid development of Chiari I malformation in an infant with Seckel syndrome and craniosynostosis. Case report and review of the literature. Journal of neurosurgery 2003. link 10 Kumar A, Patni AH, Charbel F. The Chiari I malformation and the neurotologist. Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 2002. link 11 Scalercio F, Aprigliano D, Guido F, Matrunola M, Principessa L. Crossed renal ectopia with fusion. Minerva pediatrica 1998. link 12 Semple DA, McClure JH. Arnold-chiari malformation in pregnancy. Anaesthesia 1996. link 13 Palma V, Sinisi L, Andreone V, Fazio N, Serra LL, Ambrosio G et al.. Hindbrain hernia headache and syncope in type I Arnold-Chiari malformation. Acta neurologica 1993. link 14 Castillo M, Quencer RM, Dominguez R. Chiari III malformation: imaging features. AJNR. American journal of neuroradiology 1992. link 15 Tomita Y, Tsukamoto H, Takeshita K, Takashima S. Blink reflex in young children with medullary kink in Chiari malformation. Brain & development 1991. link80041-6) 16 Brown DM, Marsh JL. Agnathia and associated malformations: a case report. The Cleft palate journal 1990. link027<0415:aaamac>2.3.co;2) 17 Hersh JH, McChane RH, Rosenberg EM, Powers WH, Corrigan C, Pancratz L. Otocephaly-midline malformation association. American journal of medical genetics 1989. link 18 Montserrat JM, Picado C, Agustí-Vidal A. Arnold-Chiari malformation and paralysis of the diaphragm. Respiration; international review of thoracic diseases 1988. link 19 Ascoli R, Pavone C, Cavallo N. Hilar fusion: a rare renal malformation. Description of a case in association with lithiasis and tubular precalyceal ectasia in an adult patient. European urology 1988. link 20 Neidich JA, Whitaker LA, Natowicz M, McDonald DM, Schnur R, Zackai EH. Aglossia with congenital absence of the mandibular rami and other craniofacial abnormalities. American journal of medical genetics. Supplement 1988. link 21 Nightingale S, Williams B. Hindbrain hernia headache. Lancet (London, England) 1987. link90366-7) 22 Lugaresi A, Zucconi M, Gerardi R, Sforza E, Contin M, Cortelli P et al.. Autonomic failure in a case of Chiari malformation type I. Functional neurology 1987. link 23 McCarthy RW. Lamellar tarsoplasty--a new technique for correction of horizontal tarsal kink. Ophthalmic surgery 1984. link 24 Pode D, Shapiro A, Lebensart P. Unilateral triplication of the collecting system in a horseshoe kidney. The Journal of urology 1983. link51290-2) 25 Isorna Martinez de la Riva S, Santiago A, Barberena J, Romeo A, Sebastian JL. Crossed ureteral ectopia with solitary kidney. Urologia internationalis 1980. link 26 Rosenman Y, Ronen S, Eidelman AI, Schimmel MS. Ankyloblepharon filiforme adnatum: congenital eyelid-band syndromes. American journal of diseases of children (1960) 1980. link 27 Rangecroft L. Neonatal small left colon syndrome. Archives of disease in childhood 1979. link 28 Hendren WH, Donahoe PK, Pfister RC. Crossed renal ectopia in children. Urology 1976. link90299-5)

Chiari malformation