Achondroplasia and Cervicomedullary Compression: Prospective Evaluation and Surgical Treatment
| Autor: | Glenn L. Keiper, Kerry R. Crone, Bernadette Koch |
|---|---|
| Rok vydání: | 1999 |
| Předmět: |
Male
musculoskeletal diseases medicine.medical_specialty Decompression Sudden death Achondroplasia Death Sudden Spinal cord compression medicine Humans Prospective Studies Foramen magnum business.industry Infant Newborn Occipital bone Infant General Medicine medicine.disease Magnetic Resonance Imaging Cervicomedullary Junction Surgery medicine.anatomical_structure Occipital Bone Pediatrics Perinatology and Child Health Cervical Vertebrae Female Neurology (clinical) business Spinal Cord Compression Ventriculomegaly |
| Zdroj: | Pediatric Neurosurgery. 31:78-83 |
| ISSN: | 1423-0305 1016-2291 |
| DOI: | 10.1159/000028838 |
| Popis: | The association between sudden death and cervicomedullary compression in infants with achondroplasia has been well described. Prospective clinical and imaging evaluations have been recommended to identify those infants with achondroplasia who are at risk of dying suddenly from respiratory arrest secondary to unrecognized cervicomedullary compression. Since 1988, we have prospectively evaluated 11 infants (average age 13 weeks) with achondroplasia who were asymptomatic for cervicomedullary compression on initial clinical evaluation. Craniocervical magnetic resonance imaging (MRI) findings included narrowing of the foramen magnum, effacement of the subarachnoid spaces at the cervicomedullary junction, abnormal intrinsic cord signal intensity and mild to moderate ventriculomegaly. Two patients with severe cord compression underwent immediate decompression. Two patients developed opisthotonic posturing within 3 months of evaluation and underwent foramen magnum decompression, including suboccipital craniectomy and atlantal laminectomy. Surgery in all cases revealed forward extension of the squamous portion of the occipital bone, thickened posterior rim of the foramen magnum and a dense fibrotic epidural band. There were no complications from surgery. Seven patients did not require surgery and were followed closely. All 11 patients remain asymptomatic at follow-up (mean 4.6 years; range 16 months to 7.3 years), and no patient has required a diversionary shunt procedure. The results of this prospective study confirm that early clinical and MRI evaluations are necessary to determine whether infants with achondroplasia have cervicomedullary compression. With early recognition, an immediate decompression can be performed safely to avoid serious complications associated with cervicomedullary compression, including sudden death. |
| Databáze: | OpenAIRE |
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