Chiari I malformation associated with syringomyelia and scoliosis. 1997

I B Ghanem, and C Londono, and O Delalande, and J F Dubousset
Département de Chirurgie Orthopédique, Hôpital Hotel-Dieu de France, Paris, France.

METHODS A retrospective review of a series of 12 children who underwent suboccipital foraminotomy and duroplasty for Chiari I malformation. OBJECTIVE To assess the effects of this surgery on associated syringomyelia and scoliosis. BACKGROUND Suboccipital foraminotomy for the treatment of syringomyelia associated with Chiari I malformation was greatly stimulated by Gardner's hydrodynamic theory, and its results proved to be encouraging. However, several authors reported improvement or stabilization of associated scoliosis after this surgery. METHODS A retrospective review was conducted on 12 patients who underwent suboccipital foraminotomy for Chiari I malformation associated with syringomyelia. Neurologic Impairment, extent of syringomyelia, and severity of associated spinal deformity were assessed preoperatively and at a 4.5-year average follow-up (range, 2.1-12 years). Anomaly of superficial abdominal reflexes was found in all cases, and para or tetraparesis in three cases. Syringomyelia was of variable localization and extent. Scoliosis was present in 7 cases (greater than 40 degrees in 5 cases). RESULTS Diminution or complete disappearance of syringomyelia was observed in 11 cases, 3 months to 1 year after surgery. Superficial abdominal reflexes anomaly improved in four cases. Minimal neurologic deficit persisted in one case. Scoliosis improved in one case, remained unchanged in one case, and progressed in the five cases with preoperative severe deformity, requiring instrumentation and fusion. CONCLUSIONS Improvement of syringomyelia and neurologic deficit, observed with suboccipital foraminotomy, supports the theory that abnormal hydrodynamics of the cerebral spinal fluid is most likely to cause these deficits.

UI MeSH Term Description Entries
D007796 Laminectomy A surgical procedure that entails removing all (laminectomy) or part (laminotomy) of selected vertebral lamina to relieve pressure on the SPINAL CORD and/or SPINAL NERVE ROOTS. Vertebral lamina is the thin flattened posterior wall of vertebral arch that forms the vertebral foramen through which pass the spinal cord and nerve roots. Laminotomy,Laminectomies,Laminotomies
D008297 Male Males
D002574 Cervical Vertebrae The first seven VERTEBRAE of the SPINAL COLUMN, which correspond to the VERTEBRAE of the NECK. Cervical Spine,Cervical Spines,Spine, Cervical,Vertebrae, Cervical
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D005260 Female Females
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
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

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