Turkish neurosurgery · 2020
Atlantoaxial Instability Treated with Posterior Atlantoaxial Fixation in Two Siblings with Dyggve-Melchior-Clausen Syndrome.
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Shah Abhidha, Bhambere Sagar, Ranjan Shashi, Dandpat Saswat, Goel Atul
Аннотация
AIM: To discuss the rare association of atlantoaxial instability in patients with the Dyggve-Melchior-Clausen syndrome, a rare autosomal recessive disease characterized by progressive spondyloepimetaphyseal dysplasia and mild to severe mental retardation.
MATERIAL AND METHODS: We report an uncommon association of two siblings with Dyggve-Melchior-Clausen syndrome, odontoid hypoplasia and atlantoaxial instability. Both the patients were treated with Goel?s atlantoaxial fixation procedure.
RESULTS: The patients had a remarkable neurological recovery following the stabilization procedure.
CONCLUSION: Atlantoaxial instability is a potentially life-threatening condition in patients with this syndrome and should be treated early with atlantoaxial stabilization. Recognition and treatment of atlantoaxial instability in patients with Dyggve-Melchior- Clausen syndrome can give gratifying results.
MATERIAL AND METHODS: We report an uncommon association of two siblings with Dyggve-Melchior-Clausen syndrome, odontoid hypoplasia and atlantoaxial instability. Both the patients were treated with Goel?s atlantoaxial fixation procedure.
RESULTS: The patients had a remarkable neurological recovery following the stabilization procedure.
CONCLUSION: Atlantoaxial instability is a potentially life-threatening condition in patients with this syndrome and should be treated early with atlantoaxial stabilization. Recognition and treatment of atlantoaxial instability in patients with Dyggve-Melchior- Clausen syndrome can give gratifying results.