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Hydrosyringomyelia associated with a Chiari I malformation in children and adolescents.
Neurosurgery. 1990 Apr; 26(4):591-6; discussion 596-7.N

Abstract

The clinical presentation, radiological features, and results of surgical treatment were analyzed in 17 cases of hydrosyringomyelia associated with a Chiari malformation, in children and adolescents younger than 20 years of age. The initial symptoms were a skeletal abnormality (71%), such as scoliosis (11 patients) or pes cavus (1 patient), pain or numbness (24%), and motor weakness (6%). Frequently seen signs on admission were sensory deficit (100%), scoliosis (85%), muscle weakness (64%), muscle atrophy (35%), and lower cranial nerve palsy (35%). The characteristic neurological findings were unilateral sensory and motor deficits (65%) with decreased or absent deep tendon reflexes on the same side. The localization of the syrinx on the axial section varied according to the level, even in the same patient. In 11 patients with unilateral sensory disturbances or unilateral sensory and motor deficits, the syrinx was located in the region corresponding to the posterolateral portion on the same side as that of sensory disturbance at the cervical or thoracic level. On the other hand, in 6 patients with bilateral sensory and motor deficits, the syrinx was located in the central portion and extended into the posterolateral portion of the more affected side. A syringosubarachnoid shunt was placed in 16 patients, foramen magnum decompression without closure of the obex was performed in 1 patient, ventriculoperitoneal shunt in 1 patient, terminal syringostomy in 1 patient, and foramen magnum decompression with terminal syringostomy in 1 patient. In 15 of 17 patients (88%), the neurological symptoms improved after an average follow-up of 4 years and 1 month. We think that as a surgical treatment, placement of a syringosubarachnoid shunt is effective.

Authors+Show Affiliations

Department of Neurosurgery, University of Hokkaido School of Medicine, Japan.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

2330080

Citation

Isu, T, et al. "Hydrosyringomyelia Associated With a Chiari I Malformation in Children and Adolescents." Neurosurgery, vol. 26, no. 4, 1990, pp. 591-6; discussion 596-7.
Isu T, Iwasaki Y, Akino M, et al. Hydrosyringomyelia associated with a Chiari I malformation in children and adolescents. Neurosurgery. 1990;26(4):591-6; discussion 596-7.
Isu, T., Iwasaki, Y., Akino, M., & Abe, H. (1990). Hydrosyringomyelia associated with a Chiari I malformation in children and adolescents. Neurosurgery, 26(4), 591-6; discussion 596-7.
Isu T, et al. Hydrosyringomyelia Associated With a Chiari I Malformation in Children and Adolescents. Neurosurgery. 1990;26(4):591-6; discussion 596-7. PubMed PMID: 2330080.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hydrosyringomyelia associated with a Chiari I malformation in children and adolescents. AU - Isu,T, AU - Iwasaki,Y, AU - Akino,M, AU - Abe,H, PY - 1990/4/1/pubmed PY - 1990/4/1/medline PY - 1990/4/1/entrez SP - 591-6; discussion 596-7 JF - Neurosurgery JO - Neurosurgery VL - 26 IS - 4 N2 - The clinical presentation, radiological features, and results of surgical treatment were analyzed in 17 cases of hydrosyringomyelia associated with a Chiari malformation, in children and adolescents younger than 20 years of age. The initial symptoms were a skeletal abnormality (71%), such as scoliosis (11 patients) or pes cavus (1 patient), pain or numbness (24%), and motor weakness (6%). Frequently seen signs on admission were sensory deficit (100%), scoliosis (85%), muscle weakness (64%), muscle atrophy (35%), and lower cranial nerve palsy (35%). The characteristic neurological findings were unilateral sensory and motor deficits (65%) with decreased or absent deep tendon reflexes on the same side. The localization of the syrinx on the axial section varied according to the level, even in the same patient. In 11 patients with unilateral sensory disturbances or unilateral sensory and motor deficits, the syrinx was located in the region corresponding to the posterolateral portion on the same side as that of sensory disturbance at the cervical or thoracic level. On the other hand, in 6 patients with bilateral sensory and motor deficits, the syrinx was located in the central portion and extended into the posterolateral portion of the more affected side. A syringosubarachnoid shunt was placed in 16 patients, foramen magnum decompression without closure of the obex was performed in 1 patient, ventriculoperitoneal shunt in 1 patient, terminal syringostomy in 1 patient, and foramen magnum decompression with terminal syringostomy in 1 patient. In 15 of 17 patients (88%), the neurological symptoms improved after an average follow-up of 4 years and 1 month. We think that as a surgical treatment, placement of a syringosubarachnoid shunt is effective. SN - 0148-396X UR - https://www.unboundmedicine.com/medline/citation/2330080/Hydrosyringomyelia_associated_with_a_Chiari_I_malformation_in_children_and_adolescents_ L2 - https://academic.oup.com/neurosurgery/article-lookup/doi/10.1097/00006123-199004000-00006 DB - PRIME DP - Unbound Medicine ER -