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Paraplegia resulting from thoracolumbar stenosis in a seven-month-old achondroplastic dwarf
Y S Hahn1, H H Engelhard, T Naidich
1Pediatric Neurosurgery, Loyola University of Chicago, Ill.
Insights
A young achondroplastic child recovered from paraplegia after surgery for spinal cord compression caused by a rare dural fold. This case highlights a unique cause of neurological issues in achondroplasia.
Area of Science:
- Pediatric Neurology
- Skeletal Dysplasias
- Neurosurgery
Background:
- Achondroplasia commonly presents with neurological issues like macrocephaly and spinal stenosis.
- Thoracolumbar kyphosis can lead to neurological deficits in achondroplastic individuals, typically later in childhood.
- Spinal cord compression in achondroplasia is usually attributed to bony abnormalities.
Observation:
- A 7-month-old achondroplastic infant developed paraplegia.
- Spinal cord compression was identified as the cause, but not due to bony stenosis.
- A unique, dural layer of fibrous tissue was constricting the spinal canal.
Findings:
- Histological examination confirmed the fibrous tissue was identical to the dura.
- Surgical intervention involving decompressive laminectomy and resection of the fibrous tissue was performed.
- The infant experienced a complete recovery of motor function.
Implications:
- This case reveals a previously undescribed cause of spinal cord compression in achondroplasia.
- Early diagnosis and surgical decompression of this dural anomaly can lead to full neurological recovery.
- Highlights the importance of considering non-bony causes of spinal compromise in pediatric achondroplasia.
Abstract:
In young achondroplastic children, neurological manifestations have been found to include macrocephaly, hydrocephalus or ventriculomegaly and cervicomedullary compression. Occasionally in the second decade, lumbar radiculopathy or paraparesis resulting from severe thoracolumbar kyphosis develops. In this paper, we report the unique case of an achondroplastic dwarf who developed paraplegia due to thoracolumbar spinal cord compression at the age of 7 months. Compromise of the spinal canal was found to be due not to bony stenosis, but to a second layer of fibrous tissue, histologically identical to the dura. Treatment consisting of decompressive laminectomy and resection of the constricting tissue allowed the child to recover completely. Clinical, radiographic and pathological findings are discussed.