Biomaterial Database

Chiari I malformations with syringomyelia: long-term results of neurosurgical decompression. 2020

Bram P Verhofste, and Eric A Davis, and Patricia E Miller, and Michael T Hresko, and John B Emans, and Lawrence I Karlin, and Daniel J Hedequist, and Brian D Snyder, and Edward R Smith, and Mark R Proctor, and Michael P Glotzbecker

Department of Orthopaedic Surgery, Boston Children's Hospital (Harvard Teaching Hospital), Boston, MA, USA.

Retrospective case series. The objective was to assess the long-term outcomes on scoliosis following Chiari-I (CM-I) decompression in patients with CM-I and syringomyelia (SM). A secondary objective was to identify risk factors of scoliosis progression. The association between CM-I with SM and scoliosis is recognized, but it remains unclear if CM-I decompression alters the long-term evolution of scoliosis in patients with associated syringomyelia. A retrospective review of children with scoliosis, CM-I, and SM during 1997-2015 was performed. Congenital, syndromic, and neuromuscular scoliosis were excluded. Clinical and radiographic characteristics were recorded at presentation, pre-decompression, after 1-year, and latest follow-up. A scale to measure syringomyelia area on MRI was used to evaluate SM changes post-decompression. 65 children with CM-I, SM, and scoliosis and a mean age of 8.9 years (range 0.7-15.8) were identified. Mean follow-up was 6.9 years (range 2.0-20.4). Atypical curves were present in 28 (43%) children. Thirty-eight patients (58%) underwent decompression before 10 years. Syringomyelia size reduced a mean of 70% after decompression (p < 0.001). Scoliosis improved in 26 (40%), stabilized in 17 (26%), and progressed in 22 (34%) cases. Early spinal fusion was required in 7 (11%) patients after a mean of 0.5 ± 0.37 years and delayed fusion in 16 (25%) patients after 6.0 ± 3.24 years. The remaining 42 (65%) patients were followed for a median of 6.1 years (range 2.0-12.3) without spine instrumentation or fusion. Fusion patients experienced less improvement in curve magnitude 1-year post-decompression (p < 0.001) and had larger curves at presentation (43° vs. 34°; p = 0.004). Syringomyelia size decreased by 70% after CM-I decompression and scoliosis stabilized or improved in two-thirds of patients. Greater curve improvement within the first year post-decompression and smaller curves at presentation decreased the risk of spinal fusion. Neurosurgical decompression is recommended in children with CM-I, SM, and scoliosis with the potential to treat all three conditions. Level IV.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008297	Male		Males
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D001139	Arnold-Chiari Malformation	A group of congenital malformations involving the brainstem, cerebellum, upper spinal cord, and surrounding bony structures. Type II is the most common, and features compression of the medulla and cerebellar tonsils into the upper cervical spinal canal and an associated MENINGOMYELOCELE. Type I features similar, but less severe malformations and is without an associated meningomyelocele. Type III has the features of type II with an additional herniation of the entire cerebellum through the bony defect involving the foramen magnum, forming an ENCEPHALOCELE. Type IV is a form a cerebellar hypoplasia. Clinical manifestations of types I-III include TORTICOLLIS; opisthotonus; HEADACHE; VERTIGO; VOCAL CORD PARALYSIS; APNEA; NYSTAGMUS, CONGENITAL; swallowing difficulties; and ATAXIA. (From Menkes, Textbook of Child Neurology, 5th ed, p261; Davis, Textbook of Neuropathology, 2nd ed, pp236-46)	Arnold-Chiari Deformity,Arnold-Chiari Malformation, Type 1,Arnold-Chiari Malformation, Type 2,Arnold-Chiari Malformation, Type 3,Arnold-Chiari Malformation, Type 4,Arnold-Chiari Malformation, Type I,Arnold-Chiari Malformation, Type II,Arnold-Chiari Malformation, Type III,Arnold-Chiari Malformation, Type IV,Arnold-Chiari Syndrome,Chiari Malformation Type 2,Chiari Malformation Type I,Chiari Malformation Type II,Malformation, Arnold-Chiari,Type I Arnold-Chiari Malformation,Type II Arnold-Chiari Malformation,Type III Arnold-Chiari Malformation,Type IV Arnold-Chiari Malformation,Arnold Chiari Deformity,Arnold Chiari Malformation,Arnold Chiari Malformation, Type 1,Arnold Chiari Malformation, Type 2,Arnold Chiari Malformation, Type 3,Arnold Chiari Malformation, Type 4,Arnold Chiari Malformation, Type I,Arnold Chiari Malformation, Type II,Arnold Chiari Malformation, Type III,Arnold Chiari Malformation, Type IV,Arnold Chiari Syndrome,Deformity, Arnold-Chiari,Malformation, Arnold Chiari,Syndrome, Arnold-Chiari,Type I Arnold Chiari Malformation,Type II Arnold Chiari Malformation,Type III Arnold Chiari Malformation,Type IV Arnold Chiari Malformation
D012189	Retrospective Studies	Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons.	Retrospective Study,Studies, Retrospective,Study, Retrospective
D012306	Risk	The probability that an event will occur. It encompasses a variety of measures of the probability of a generally unfavorable outcome.	Relative Risk,Relative Risks,Risk, Relative,Risks,Risks, Relative