TY - JOUR
T1 - Craniocervical decompression for cervicomedullary compression in pediatric patients with achondroplasia
AU - Aryanpur, J.
AU - Hurko, O.
AU - Francomano, C.
AU - Wang, H.
AU - Carson, B.
PY - 1990
Y1 - 1990
N2 - The congenital osseous abnormalities associated with achondroplasia include stenosis of the foramen magnum and the upper cervical spinal canal. In the pediatric achondroplastic patients, such stenosis may lead to cervicomedullary compression with serious sequelae, including paresis, hypertonia, delayed motor milestones, and respiratory compromise. Using a standardized protocol the authors have treated 15 young achondroplastic patients with documented cervicomedullary compression by craniocervical decompression and duroplasty. Following this procedure, significant improvement in presenting neurological or respiratory complaints was noted in all patients. The mortality rate in this series was zero. The major cause of morbidity associated with this procedure was perioperative cerebrospinal fluid (CSF) leakage from the surgical wound, presumably related to coexisting abnormalities of CSF dynamics. This problem was successfully managed by temporary or, when necessary, permanent CSF diversion procedures. It is concluded that craniocervical decompression is an effective and safe treatment for young achondroplastic patients with cervicomedullary compression.
AB - The congenital osseous abnormalities associated with achondroplasia include stenosis of the foramen magnum and the upper cervical spinal canal. In the pediatric achondroplastic patients, such stenosis may lead to cervicomedullary compression with serious sequelae, including paresis, hypertonia, delayed motor milestones, and respiratory compromise. Using a standardized protocol the authors have treated 15 young achondroplastic patients with documented cervicomedullary compression by craniocervical decompression and duroplasty. Following this procedure, significant improvement in presenting neurological or respiratory complaints was noted in all patients. The mortality rate in this series was zero. The major cause of morbidity associated with this procedure was perioperative cerebrospinal fluid (CSF) leakage from the surgical wound, presumably related to coexisting abnormalities of CSF dynamics. This problem was successfully managed by temporary or, when necessary, permanent CSF diversion procedures. It is concluded that craniocervical decompression is an effective and safe treatment for young achondroplastic patients with cervicomedullary compression.
KW - achondroplasia
KW - children
KW - craniocervical decompression
KW - hydrocephalus
KW - spinal compression
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U2 - 10.3171/jns.1990.73.3.0375
DO - 10.3171/jns.1990.73.3.0375
M3 - Article
C2 - 2384775
AN - SCOPUS:0025126601
SN - 0022-3085
VL - 73
SP - 375
EP - 382
JO - Journal of neurosurgery
JF - Journal of neurosurgery
IS - 3
ER -