Craniocervical decompression for cervicomedullary compression in pediatric patients with achondroplasia

Abstract

The congenital osseous abnormalities associated with achondroplasia include stenosis of the foramen magnum and the upper cervical spinal canal. In the pediatric achondroplastic patient, such stenosis may lead to cervicomedullary compression with serious sequelae, including paresis, hypertonia, delayed motor mile-stones, 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.

Dynamic cervicomedullary cord compression and alterations in cerebrospinal fluid dynamics in children with achondroplasia: review of an 11-year surgical case series

“…To ensure an adequate lateral decompression, care was taken to extend the craniectomy out laterally and anteriorly to the point where the dura of the cervicomedullary junction would curve anteriorly. As has previously been described, 1 the posterior rim of the foramen magnum was thickened and in many cases was found to curve in the cephalad direction. To minimize the risk of a dural tear, we first would thin out the suboccipital bone to a thin shelf posterior to the rim of the foramen magnum while leaving the lip of foramen magnum intact.…”

“…Suboccipital craniectomy for decompression of the foramen magnum has been previously reported. 1,4 We used a modified procedure whereby the dura was not opened surgically.…”

A Case of Achondroplasia Associated with Cervicomedullary-Junction Compression

“…It is known that infants with achondroplasia are at risk of cervical cord compression (2-4). Reported neurological s y m p t o m s in t he s e c o n d i t i o n s in c l u d e quadriparesis, paraparesis, feeding problem dysphasia, poor head control, hypotonia, and delayed motor developmental milestones (2)(3)(4). Especially, apneic spells may cause sudden death (4-9).…”

“…A variety of s keletal malformations are observed and generally diagnosed at birth. Generalized spinal stenosis with spinal cord compression occurs, often requiring surgical decompression (2,3). The anatomical stenosis at the craniocervical junction and upper cervical vertebral canal results in cervical cord or brainstem compression and may be accompanied by edema, gliosis, cystic myelomalacia and syringomyelic cavities (2-4).…”

“…Clinically, upper cervical myelopathy may manifest with recurrent apnea, extreme muscular hypotonia, developmental delay, and spastic diplegia with increased reflexes (2)(3)(4). Especially, interference with the respiratory control centers creates a risk of sudden death in early life (4-9).…”