Symptomatic Atlantoaxial Instability Associated With Medical and Rehabilitative Procedures in Children With Down Syndrome

PEDIATRICS ◽  
1990 ◽  
Vol 85 (3) ◽  
pp. 447-449
Author(s):  
Michael E. Msall ◽  
Mary E. Reese ◽  
Kathleen DiGaudio ◽  
Kim Griswold ◽  
Carl V. Granger ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Case Series ◽  
Medical Genetics ◽  
Atlantoaxial Instability ◽  
Children With Down Syndrome ◽  
Radiographic Screening

The Canadian College of Medical Genetics recently questioned the rationale for radiographic screening for atlantoaxial instability in Down syndrome. We report a case series of four children with Down syndrome with symptomatic atlantoaxial instability and followed for more than 5 years. In all cases a medical or rehabilitational procedure was associated with significant apnea, cardiopulmonary compromise, or severe neurological impairments. None of these patients were screened radiographically. The need for protocols to detect atlantoaxial instability in children with Down syndrome undergoing medical and rehabilitational procedures is highlighted by these cases.

Accuracy of Parental Perception of Nighttime Breathing in Children with Down Syndrome

2017 ◽  
Vol 158 (2) ◽  
pp. 364-367 ◽  
Author(s):  
Norman R. Friedman ◽  
Amanda G. Ruiz ◽  
Dexiang Gao ◽  
David G. Ingram
Keyword(s):  
Down Syndrome ◽  
American Academy ◽  
Tertiary Care ◽  
Case Series ◽  
Neck Surgery ◽  
Parental Perception ◽  
Breathing Patterns ◽  
Children With Down Syndrome ◽  
American Academy Of Pediatrics ◽  
Obstructive Sleep

Objective In 2011, the American Academy of Pediatrics published a guideline for children with Down syndrome (DS), recommending a polysomnogram (PSG) by age 4 years regardless of symptoms. Their rationale was based on 2 publications with small cohorts, where at least 50% of the children had no obstructive sleep apnea (OSA) symptoms but their PSG results were abnormal. The American Academy of Otolaryngology—Head and Neck Surgery Foundation published a clinical practice guideline recommending PSG prior to adenotonsillectomy for these children. This study aimed to assess parents’ accuracy of their children’s breathing patterns as compared with PSGs in a larger cohort of children with DS. Study Design Case series with chart review. Setting Tertiary care academic pediatric hospital. Subjects and Methods Sleep intake forms assessing frequency of parent-observed apnea, snoring, and restless sleep were analyzed. None of the children had a previous tonsillectomy. Two groups were analyzed according to symptoms: infrequent (<3 nights per week on all questions answered) and frequent (≥6 nights per week on at least 1 question). OSA severity was categorized as follows: normal, <2 events per hour; mild, 2 to 4.9; moderate, 5 to 9.9; and severe, ≥10. Results A total of 113 children met inclusion criteria: 34% (n = 38) had infrequent symptoms, and 66% (n = 75) had frequent symptoms. Parents were unable to predict the presence or absence of OSA by nighttime symptoms ( P = .60). The risk of OSA for children with frequent symptoms versus those with infrequent symptoms was 1.04 (95% CI, 0.89-1.3). Conclusion Parents of DS children are unable to predict the presence or absence of OSA by nighttime symptoms, nor are they able to determine its severity.

scholarly journals Obstructive Sleep Apnea in Children with Down Syndrome: Demographic, Clinical, and Polysomnographic Features

2018 ◽  
Vol 160 (1) ◽  
pp. 150-157 ◽  
Author(s):  
Bahir H. Chamseddin ◽  
Romaine F. Johnson ◽  
Ron B. Mitchell
Keyword(s):  
Obstructive Sleep Apnea ◽  
Down Syndrome ◽  
Sleep Apnea ◽  
Odds Ratio ◽  
Medical Center ◽  
Case Series ◽  
Apnea Hypopnea Index ◽  
Severe Obstructive Sleep Apnea ◽  
Children With Down Syndrome ◽  
Obstructive Sleep

Objectives To evaluate demographic, clinical, and polysomnographic features of children with Down syndrome suspected of having obstructive sleep apnea. To identify factors that predict severe obstructive sleep apnea among children with Down syndrome. Study Design Case series with chart review. Setting Children’s Medical Center Dallas / University of Texas Southwestern Medical Center. Subject and Methods Demographic, clinical, and polysomnographic data were collected for children with Down syndrome aged 2 to 18 years. Simple and multivariable regression models were used to study predictors of severe obstructive sleep apnea (apnea-hypopnea index ≥10). P≤ .05 was considered significant. Results A total of 106 children with Down syndrome were included, with 89 (84%) <12 years old, 56 (53%) male, 72 (68%) Hispanic, 15 (14%) African American, and 14 (13%) Caucasian. Ninety percent of children had ≥1 medical comorbidities; 95 (90%) patients had obstructive sleep apnea; and 46 (44%) had severe obstructive sleep apnea. The mean SaO2 nadir was lower among obese than nonobese children (80% vs 85%, P = .02). Obese versus nonobese patients had a higher prevalence of severe obstructive sleep apnea (56% vs 35%, P = .03). Severe OSA was associated with heavier weight (odds ratio = 1.0, 95% CI: 1.0-1.1, P = .002) and age ≥12 years (odds ratio = 1.2, 95% CI: 0.2-2.5, P = .02). The multivariable model showed that severe obstructive sleep apnea was associated only with weight (odds ratio = 1.1, 95% CI: 1.0-1.1, P = .02). Conclusion Obese children with DS are at a high risk for severe OSA, with weight as the sole risk factor. The results of this study show the importance of monitoring the weight of children with DS and counseling parents of children with DS about weight loss.

Evaluation of Pediatrician Adherence to the American Academy of Pediatrics Health Supervision Guidelines for Down Syndrome

2018 ◽  
Vol 123 (5) ◽  
pp. 387-398 ◽  
Author(s):  
Meghan E. O'Neill ◽  
Alexandra Ryan ◽  
Soyang Kwon ◽  
Helen J. Binns
Keyword(s):  
Primary Care ◽  
Down Syndrome ◽  
American Academy ◽  
Medical Records ◽  
Atlantoaxial Instability ◽  
Anticipatory Guidance ◽  
Comorbid Conditions ◽  
Children With Down Syndrome ◽  
Health Supervision ◽  
American Academy Of Pediatrics

Abstract The American Academy of Pediatrics's guideline on health supervision for children with Down syndrome (DS) offers pediatricians guidance to improve detection of comorbid conditions. Pediatrician adherence has not yet been comprehensively evaluated. Medical records of 31 children with DS who received primary care at two urban academic clinic sites from 2008–2012 were reviewed. Data was extracted on adherence to age-specific individual guideline components for each subject by year-of-life (total 84 years-of-life). Overall adherence across all components was 83% (2001 guideline) and 67% (2011 guideline). Adherence to thyroid, hearing, vision, and developmental components was &gt;85%, and anticipatory guidance regarding atlantoaxial instability and sexuality was &lt;35%. Overall adherence was higher when a subject was younger and when a provider was an attending-level pediatrician.

New radiological parameters for the assessment of atlantoaxial instability in children with Down syndrome

2016 ◽  
Vol 98-B (12) ◽  
pp. 1704-1710 ◽  
Author(s):  
N. Nakamura ◽  
Y. Inaba ◽  
Y. Aota ◽  
M. Oba ◽  
J. Machida ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Atlantoaxial Instability ◽  
Children With Down Syndrome ◽  
Radiological Parameters

Craniovertebral Junction Abnormalities in Down Syndrome

Neurosurgery ◽  
2010 ◽  
Vol 66 (suppl_3) ◽  
pp. A32-A38 ◽  
Author(s):  
Todd C. Hankinson ◽  
Richard C.E. Anderson
Keyword(s):  
Down Syndrome ◽  
Cervical Spine ◽  
Craniovertebral Junction ◽  
Atlantoaxial Instability ◽  
Special Olympics ◽  
Cervical Instability ◽  
Neurologic Injury ◽  
Current Opinion ◽  
Children With Down Syndrome ◽  
Upper Cervical

Abstract CHILDREN WITH DOWN syndrome may have occipitocervical and atlantoaxial instability. To prevent neurologic injury during athletic competitions, such as the Special Olympics, radiographic cervical spine screening was established in 1983 as a prerequisite for participation in some events. This review discusses the biomechanics underlying upper cervical instability in children with Down syndrome, the evolution of cervical spine screening protocols, and current opinion regarding management for children with Down syndrome and upper cervical instability.

Novel 2 Radiographical Measurements for Atlantoaxial Instability in Children With Down Syndrome

Spine ◽  
2014 ◽  
Vol 39 (26) ◽  
pp. E1566-E1574 ◽  
Author(s):  
Naoyuki Nakamura ◽  
Yutaka Inaba ◽  
Masatoshi Oba ◽  
Yoichi Aota ◽  
Yogen Morikawa ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Atlantoaxial Instability ◽  
Children With Down Syndrome
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