STUDY DESIGN: Retrospective prognostic study. OBJECTIVE: To evaluate the safety and efficacy of cervical arthrodesis for cervical instability in patients with skeletal dysplasia. SUMMARY OF BACKGROUND DATA: Individuals with certain skeletal dysplasias have a high incidence of cervical instability, which can lead to compression of the spinal cord and subsequent severe spinal cord symptoms, progressive neurologic decline, quadriplegia, and death. MATERIALS AND METHODS: The charts of 25 patients with skeletal dysplasia (spondyloepiphyseal dysplasia, spondyloepimetaphyseal dysplasia, pseudoachondroplasia, Morquio, or Kniest) who had undergone cervical arthrodesis to treat instability were reviewed for evidence of fusion, neurologic improvement, and complications. RESULTS: Of the 25 patients, 23 (92%) achieved a solid bony fusion, and 5 (20%) experienced surgery-related complications. One of the two patients who did not achieve fusion had a stable pseudarthrosis without neurologic complications, and additional surgical intervention was unnecessary; the second patient is contemplating revision surgery. Of 16 patients with preoperative neurologic manifestations, 14 (88%) experienced improvement. CONCLUSION: Cervical arthrodesis can be a safe and effective treatment for patients with skeletal dysplasia and cervical instability, despite the inherent complications associated with a dysplastic skeleton. The procedure can preserve and/or improve neurologic function while minimizing the risk of neurologic injury from spinal cord compression.
STUDY DESIGN: Retrospective prognostic study. OBJECTIVE: To evaluate the safety and efficacy of cervical arthrodesis for cervical instability in patients with skeletal dysplasia. SUMMARY OF BACKGROUND DATA: Individuals with certain skeletal dysplasias have a high incidence of cervical instability, which can lead to compression of the spinal cord and subsequent severe spinal cord symptoms, progressive neurologic decline, quadriplegia, and death. MATERIALS AND METHODS: The charts of 25 patients with skeletal dysplasia (spondyloepiphyseal dysplasia, spondyloepimetaphyseal dysplasia, pseudoachondroplasia, Morquio, or Kniest) who had undergone cervical arthrodesis to treat instability were reviewed for evidence of fusion, neurologic improvement, and complications. RESULTS: Of the 25 patients, 23 (92%) achieved a solid bony fusion, and 5 (20%) experienced surgery-related complications. One of the two patients who did not achieve fusion had a stable pseudarthrosis without neurologic complications, and additional surgical intervention was unnecessary; the second patient is contemplating revision surgery. Of 16 patients with preoperative neurologic manifestations, 14 (88%) experienced improvement. CONCLUSION:Cervical arthrodesis can be a safe and effective treatment for patients with skeletal dysplasia and cervical instability, despite the inherent complications associated with a dysplastic skeleton. The procedure can preserve and/or improve neurologic function while minimizing the risk of neurologic injury from spinal cord compression.
Authors: Shaukat Khan; Carlos J Alméciga-Díaz; Kazuki Sawamoto; William G Mackenzie; Mary C Theroux; Christian Pizarro; Robert W Mason; Tadao Orii; Shunji Tomatsu Journal: Mol Genet Metab Date: 2016-11-29 Impact factor: 4.797
Authors: A Broomfield; K Zuberi; J Mercer; G Moss; N Finnegan; P Hensman; R Walker; S Bukhari; N B Wright; F Stewart; S A Jones; R Ramirez Journal: Childs Nerv Syst Date: 2018-06-26 Impact factor: 1.475
Authors: Eriko Yasuda; Yasuyuki Suzuki; Tsutomu Shimada; Kazuki Sawamoto; William G Mackenzie; Mary C Theroux; Christian Pizarro; Li Xie; Freeman Miller; Tariq Rahman; Heidi H Kecskemethy; Kyoko Nagao; Thierry Morlet; Thomas H Shaffer; Yasutsugu Chinen; Hiromasa Yabe; Akemi Tanaka; Haruo Shintaku; Kenji E Orii; Koji O Orii; Robert W Mason; Adriana M Montaño; Toshiyuki Fukao; Tadao Orii; Shunji Tomatsu Journal: Mol Genet Metab Date: 2016-04-25 Impact factor: 4.797
Authors: Christian Möllmann; Christian G Lampe; Wibke Müller-Forell; Maurizio Scarpa; Paul Harmatz; Manfred Schwarz; Michael Beck; Christina Lampe Journal: JIMD Rep Date: 2013-04-12
Authors: Guirish A Solanki; Kenneth W Martin; Mary C Theroux; Christina Lampe; Klane K White; Renée Shediac; Christian G Lampe; Michael Beck; William G Mackenzie; Christian J Hendriksz; Paul R Harmatz Journal: J Inherit Metab Dis Date: 2013-02-06 Impact factor: 4.982