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Clinical diagnosis-part I: what is really caused by Chiari I.
Childs Nerv Syst. 2019 10; 35(10):1673-1679.CN

Abstract

PURPOSE

Chiari malformation is a group of congenital malformations involving the brainstem, cerebellum, and upper spinal cord, frequently identified in both young adults and in children. Chiari I malformation (CM1), classically defined as a caudal displacement of the cerebellar tonsils through the foramen magnum into the spinal cord, is the most common clinical type. A syringomyelia can be associated at the time of the diagnosis or appear secondarily and manifest with medullary symptoms. The aim of this paper is to update the knowledge on clinical manifestations specifically related to Chiari I malformation with or without syringomyelia in the pediatric population.

METHODS

Current literature with focus on relevant clinical pediatric issues is reviewed and discussed, comparing with those related to adults; we include the results of a 10-year single-center experience on 600 CM1 patients.

RESULTS AND CONCLUSIONS

Herniation of the cerebellar tonsils may lead to significant clinical symptoms, including neck and cervical pain, short-lasting occipital "cough" headache, dizziness, and gait impairment; in children younger than 3 years, oropharyngeal symptoms are prevalent (sleep apnea, feeding problems) whereas in those older than 3 years, a higher incidence of cough headache and scoliosis is reported. CM1 clinical features, both in children and in adults, have in common the presence of anatomical deformities of the brainstem and cerebellum. Clinical myelopathy (sensory/autonomic disorders, motor weakness) can result from direct compression of the cervical spinal cord by the herniated cerebellar tonsils or can be due to the presence of a syrinx, reported in association with Chiari I between 35 and 75% of pediatric patients. Similarly, in our series (440 females, 160 males, 98% > 18 years), syringomyelia associated with Chiari I was ranging from 40 to 60% (respectively in asymptomatic and symptomatic groups); headache was reported in 65%. Sensory disturbances (48%), cranial nerve deficits (45%), motor weakness (32%), and autonomic disorders (35%) were the most frequent neurological signs in our cohort. In Chiari I malformation, cervical pain and occipital cough headache are the most characteristic presenting symptoms, both in old children and in adults; however, headache is often multifactorial, and CM1 patients can report a wide variety of non-specific symptoms and signs. Clinical diagnostic CM1 criteria, shared at the national and international level, are recommended with the aim to avoid consequent controversies on diagnosis and on surgical decision making.

Authors+Show Affiliations

Interregional Centre of Expertise for Syringomyelia and Chiari, Neuroscience Department, AOU Città della Salute e della Scienza di Torino, Torino, Italy. palma.ciaramitaro@gmail.com.Diagnostic Imaging Department, AOU Citta' della Salute e della Scienza di Torino, Torino, Italy.Neurosurgery Unit, Neuroscience Department, University of Torino, Torino, Italy.Neurosurgery Unit, Neuroscience Department, University of Torino, Torino, Italy.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

31161267

Citation

Ciaramitaro, Palma, et al. "Clinical Diagnosis-part I: what Is Really Caused By Chiari I." Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery, vol. 35, no. 10, 2019, pp. 1673-1679.
Ciaramitaro P, Ferraris M, Massaro F, et al. Clinical diagnosis-part I: what is really caused by Chiari I. Childs Nerv Syst. 2019;35(10):1673-1679.
Ciaramitaro, P., Ferraris, M., Massaro, F., & Garbossa, D. (2019). Clinical diagnosis-part I: what is really caused by Chiari I. Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery, 35(10), 1673-1679. https://doi.org/10.1007/s00381-019-04206-z
Ciaramitaro P, et al. Clinical Diagnosis-part I: what Is Really Caused By Chiari I. Childs Nerv Syst. 2019;35(10):1673-1679. PubMed PMID: 31161267.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clinical diagnosis-part I: what is really caused by Chiari I. AU - Ciaramitaro,Palma, AU - Ferraris,Marilena, AU - Massaro,Fulvio, AU - Garbossa,Diego, Y1 - 2019/06/03/ PY - 2019/04/18/received PY - 2019/05/09/accepted PY - 2019/6/5/pubmed PY - 2020/10/24/medline PY - 2019/6/5/entrez KW - Chiari I malformation KW - Chiari syndrome KW - Clinical diagnosis KW - Hindbrain hernia KW - Syringomyelia SP - 1673 EP - 1679 JF - Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery JO - Childs Nerv Syst VL - 35 IS - 10 N2 - PURPOSE: Chiari malformation is a group of congenital malformations involving the brainstem, cerebellum, and upper spinal cord, frequently identified in both young adults and in children. Chiari I malformation (CM1), classically defined as a caudal displacement of the cerebellar tonsils through the foramen magnum into the spinal cord, is the most common clinical type. A syringomyelia can be associated at the time of the diagnosis or appear secondarily and manifest with medullary symptoms. The aim of this paper is to update the knowledge on clinical manifestations specifically related to Chiari I malformation with or without syringomyelia in the pediatric population. METHODS: Current literature with focus on relevant clinical pediatric issues is reviewed and discussed, comparing with those related to adults; we include the results of a 10-year single-center experience on 600 CM1 patients. RESULTS AND CONCLUSIONS: Herniation of the cerebellar tonsils may lead to significant clinical symptoms, including neck and cervical pain, short-lasting occipital "cough" headache, dizziness, and gait impairment; in children younger than 3 years, oropharyngeal symptoms are prevalent (sleep apnea, feeding problems) whereas in those older than 3 years, a higher incidence of cough headache and scoliosis is reported. CM1 clinical features, both in children and in adults, have in common the presence of anatomical deformities of the brainstem and cerebellum. Clinical myelopathy (sensory/autonomic disorders, motor weakness) can result from direct compression of the cervical spinal cord by the herniated cerebellar tonsils or can be due to the presence of a syrinx, reported in association with Chiari I between 35 and 75% of pediatric patients. Similarly, in our series (440 females, 160 males, 98% > 18 years), syringomyelia associated with Chiari I was ranging from 40 to 60% (respectively in asymptomatic and symptomatic groups); headache was reported in 65%. Sensory disturbances (48%), cranial nerve deficits (45%), motor weakness (32%), and autonomic disorders (35%) were the most frequent neurological signs in our cohort. In Chiari I malformation, cervical pain and occipital cough headache are the most characteristic presenting symptoms, both in old children and in adults; however, headache is often multifactorial, and CM1 patients can report a wide variety of non-specific symptoms and signs. Clinical diagnostic CM1 criteria, shared at the national and international level, are recommended with the aim to avoid consequent controversies on diagnosis and on surgical decision making. SN - 1433-0350 UR - https://www.unboundmedicine.com/medline/citation/31161267/Clinical_diagnosis_part_I:_what_is_really_caused_by_Chiari_I_ L2 - https://doi.org/10.1007/s00381-019-04206-z DB - PRIME DP - Unbound Medicine ER -