Symptomatic atlantoaxial instability in Down syndrome

Nader-Sepahi, Ali; Casey, Adrian; Hayward, Richard; Crockard, H. A.; Thompson, Dominic

doi:10.3171/ped.2005.103.3.0231

Cited by 37 publications

(36 citation statements)

References 33 publications

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“…If no significant radiographic abnormalities are present, flexion and extension radiographs may be obtained before the patient is promptly referred. 23,62,63 • Measure TSH annually or sooner if child has symptoms that could be related to thyroid dysfunction.…”

Section: The Symptomatic Childmentioning

confidence: 99%

“…Current evidence does not support performing routine screening radiographs for assessment of potential atlantoaxial instability in asymptomatic children. [55][56][57][58][59][60][61][62][63][64] Parents should be advised that participation in some sports, including contact sports such as football and soccer and gymnastics (usually at older ages), places children at increased risk of spinal cord injury 65 and that trampoline use should be avoided by all children with or without Down syndrome younger than 6 years and by older children unless under direct professional supervision. 66,67 Special Olympics has specific screening requirements for participation in some sports.…”

Section: The Asymptomatic Childmentioning

confidence: 99%

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Health Supervision for Children With Down Syndrome

2011

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These guidelines are designed to assist the pediatrician in caring for the child in whom a diagnosis of Down syndrome has been confirmed by chromosome analysis. Although a pediatrician's initial contact with the child is usually during infancy, occasionally the pregnant woman who has been given a prenatal diagnosis of Down syndrome will be referred for review of the condition and the genetic counseling provided. Therefore, this report offers guidance for this situation as well.

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Section: The Symptomatic Childmentioning

confidence: 99%

Section: The Asymptomatic Childmentioning

confidence: 99%

Health Supervision for Children With Down Syndrome

2011

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“…3,7,15,24,26,27 The author believes that a dorsal craniocervical fusion requires autograft rather than allograft, which has a high failure rate. Therefore, rib or iliac crest is used, and bone extenders are routinely incorporated such as AlloMatrix (Wright Medical).…”

Section: Operative Proceduresmentioning

confidence: 99%

Craniocervical fusions in children

Menezes¹

2012

PED

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The surgical management of craniovertebral junction (CVJ) instability in pediatric patients presents unique challenges. As compared with the adult patient, the anatomical variations of the CVJ in the pediatric patient are significant, complicate the approach, and limit the use of internal fixation. Diminutive osseous and ligamentous structures and syndromic craniovertebral abnormalities complicate the issue. Advances in imaging analysis and instrumentation have improved the armamentarium for managing the pediatric patient who requires craniocervical stabilization. In this paper, the author's experience of performing more than 850 pediatric CVJ fusions is reviewed. This work includes the indications for atlantoaxial arthrodesis and occipitocervical fusion. Early atlantoaxial fusions were performed using interlaminar rib graft fusion, and more recently using either transarticular screw fixation in the older patient, or lateral mass screws at C-1 and rod fixation with either C-2 pars interarticular screw fixation or pedicle screw fixation. A C-2 translaminar screw fixation is also described. Occipitocervical fusions are performed with rib grafts in patients younger than 6 years of age. Subsequently, above that age, contoured loop fixation was performed, and in the past 8–10 years, screw and rod fixation was used. Abnormal spine growth was not observed in children who underwent craniocervical stabilization below the age of 5 years (clearly the bone grew with the patient). However, no deleterious effects were noted in the children treated with rigid instrumentation. The success rate for bone fusion alone was 98%. The author's success rate with rigid instrumentation is nearly 100%. A detailed review of the technique of fusion is presented, as well as the indications and means of avoidance of complications, their prevention, and management.

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“…Thus, prior to this an incompletely ossified C1 ring may be a [3] and some of the bone dysplasias (such as spondyloepiphyseal dysplasia and Morquio disease). In these instances, there is commonly underlying atlantoaxial instability due to ligamentous laxity, and, rather than being the cause of instability the posterior arch defect is a consequence of that instability, the excessive movement has impeded the normal ossification of the C1 ring.…”

Section: Embryologymentioning

confidence: 99%

Expert’s Comment concerning Grand Rounds Case entitled “Congenital C1 arch deficiency: Grand Round Presentation” (by M. Elmalky, S. Elsayed, G. Arealis, H. Mehdian doi:10.1007/s00586-013-2682-z)

Thompson

2013

Eur Spine J

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Symptomatic atlantoaxial instability in Down syndrome

Cited by 37 publications

References 33 publications

Health Supervision for Children With Down Syndrome

Health Supervision for Children With Down Syndrome

Craniocervical fusions in children

Expert’s Comment concerning Grand Rounds Case entitled “Congenital C1 arch deficiency: Grand Round Presentation” (by M. Elmalky, S. Elsayed, G. Arealis, H. Mehdian doi:10.1007/s00586-013-2682-z)

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