Craniocervical decompression for cervicomedullary compression in pediatric patients with achondroplasia

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✓ 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 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.

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Address reprint requests to: John Aryanpur, M.D., Department of Neurosurgery, Meyer 7-109, The Johns Hopkins Hospital, 600 North Wolfe Street, Baltimore, Maryland 21205.

© AANS, except where prohibited by US copyright law.

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    Left Pair: Computerized tomography scans in one patient. Preoperative intrathecally enhanced scan through the foramen magnum (upper) revealing effacement of the subarachnoid space posteriorly and compression of the cervicomedullary junction secondary to a small, misshapen foramen magnum. Postoperative unenhanced scan (lower) showing the extent of bone decompression. Right Pair: Magnetic resonance images in another patient. Preoperative midsagittal image through the cervicomedullary junction (spin-echo sequence, TR 600 msec, TE 20 msec, upper) demonstrating indentation of the cervicomedullary junction by a prominent posterior lip of the foramen magnum. Postoperative midsagittal image (spin-echo sequence, TR 600 msec, TE 20 msec, lower) showing no further indentation of the neuraxis. An area of low signal intensity within the upper cervical cord, possibly representing a syrinx, is incidentally noted.

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