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Cervical kyphosis in patients who have Larsen syndrome
C E Johnston1, J G Birch, J L Daniels
1Texas Scottish Rite Hospital for Children, Dallas, Texas 75219, USA.
Insights
Posterior cervical arthrodesis in infants with Larsen syndrome and cervical kyphosis can stabilize or correct the deformity. Early surgical intervention may prevent neurological deficits in these patients.
Area of Science:
- Orthopedics
- Genetics
- Pediatric Surgery
Background:
- Larsen syndrome is a rare genetic disorder associated with skeletal abnormalities.
- Cervical kyphosis can occur in Larsen syndrome patients, potentially leading to neurological complications.
- Operative management of cervical kyphosis in infants requires careful consideration of surgical techniques and outcomes.
Observation:
- Four infants with Larsen syndrome and cervical kyphosis underwent posterior cervical arthrodesis.
- Preoperative kyphosis ranged from 35 to 65 degrees.
- Follow-up averaged seventy months.
Findings:
- Three of four patients achieved stabilization or correction of kyphosis into lordosis following posterior cervical arthrodesis.
- Gradual correction occurred due to continued anterior growth with a solid posterior fusion.
- One patient developed pseudarthrosis, leading to progressive kyphosis and requiring anterior decompression and arthrodesis for a neurological deficit.
Implications:
- Cervical kyphosis may be an underdiagnosed feature of Larsen syndrome.
- Early diagnosis and operative stabilization are crucial for preventing neurological deficits.
- Posterior cervical arthrodesis in infancy offers a viable strategy for managing cervical kyphosis in Larsen syndrome, promoting deformity correction through natural growth processes.
Abstract:
Four patients who had Larsen syndrome and cervical kyphosis were managed operatively and followed for an average of seventy months (range, forty to ninety-two months). The preoperative cervical kyphosis ranged from 35 to 65 degrees. The patients had had a posterior cervical arthrodesis alone when they were infants, at an average age fo fourteen months (range, ten to sixteen months). In three infants, the kyphosis either stabilized (one patient) or reversed into lordosis (two patients). Thus, the kyphosis corrected gradually by continued anterior growth in the presence of a solid posterior fusion. In the fourth infant, the kyphosis progressed to 110 degrees because of pseudarthrosis. This child had anterior decompression and arthrodesis for an acute neurological deficit. We believe that cervical kyphosis is sometimes present but not diagnosed in patients who have Larsen syndrome. Early diagnosis followed by operative stabilization should help such patients avoid neurological deficits. Posterior cervical arthrodesis alone, performed in infancy, provided stability and the opportunity for the gradual correction of the deformity by continued anterior growth in three of our four patients.