Elsevier

The Journal of Pediatrics

Volume 136, Issue 2, February 2000, Pages 225-231
The Journal of Pediatrics

Oropharyngeal dysphagia and aspiration in patients with ataxia-telangiectasia☆,☆☆,★

https://doi.org/10.1016/S0022-3476(00)70106-5Get rights and content

Abstract

Objectives: To determine whether patients with ataxia-telangiectasia exhibit oropharyngeal dysphagia with concomitant aspiration and to examine the relationships among swallowing function, age, and nutritional status. Study design: Seventy patients (mean age, 10.7 years; range, 1.8 to 30 years) had feeding/swallowing and nutritional evaluations. Fifty-one patients, in whom there were concerns about swallowing safety, were examined with a standardized videofluoroscopic swallow study. Results: Fourteen of the 51 patients (27%) with histories suggestive of dysphagia demonstrated aspiration. Of these, silent aspiration (aspiration without a cough) occurred in 10 (71%) patients. Aspirators were significantly older than non-aspirators (mean age, 16.9 vs 10.8 years; P = .002). Advancing age was the strongest factor associated with aspiration during continuous drinking (P = .01). In patients with ataxia-telangiectasia, weight and weight/height were abnormally low at all ages and most compromised in older patients. Patients who aspirated had significantly lower mean weight (P < .002) and weight/height z scores (P < .001) than did patients who did not aspirate. Conclusions: Oropharyngeal dysphagia is common and appears to be progressive in patients with ataxia-telangiectasia. Older patients also have a higher incidence of poorer nutritional status. The relationship between dysphagia and nutritional status deserves further investigation. (J Pediatr 2000;136:225-31)

Section snippets

Subjects

In 1995, the A-T Children’s Project established a national clinical center for A-T at Johns Hopkins Medical Institutions. Between February 1996 and March 1998, 75 consecutive patients participated in multidisciplinary evaluations including a physical examination, a clinical feeding/swallowing evaluation, and a nutritional assessment. Criteria for a diagnosis of A-T included the presence of characteristic neurologic features (gait ataxia, oculomotor dysfunction, dysarthria, and a movement

Patient Demographics and Growth Parameters

Of the 70 patients completing a clinical feeding/swallowing evaluation, 51 also participated in a VFSS procedure. The mean age of patients participating in only the clinical evaluation was significantly younger than the mean age of patients who participated in both the clinical evaluation and VFSS procedure (Table I). At all ages, patients with A-T demonstrated growth parameters that were lower than normative values for age. Height, weight, and weight for height were significantly lower for the

DISCUSSION

In this investigation we examined the oropharyngeal swallow and identified the presence of dysphagia in patients with A-T. This problem appears to be progressive because dysphagia with concomitant airway contamination and complaints of swallowing problems occur more frequently in older patients with A-T. The assumption that swallowing function worsens with age is consistent with the neurodegeneration progression and the bulbar impairments associated with A-T.2, 3

Although this was a

Acknowledgements

We acknowledge the assistance of Ms Donna Dieterich, patient coordinator for the A-T Clinical Center.

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    ☆

    Supported in part by the Ataxia-Telangiectasia Children’s Project, Boca Raton, Florida; the Pediatric General Clinical Research Center, the Johns Hopkins Hospital, Baltimore, Maryland; Grant RR00052, Division of Research Resources National Institutes of Health; and the Hospital for Consumptives of Maryland Eudowood Board.

    ☆☆

    Reprint requests: Maureen A. Lefton-Greif, PhD, The Ataxia-Telangiectasia Clinical Center, Johns Hopkins Children’s Center, CMSC 1102, 600 N Wolfe St, Baltimore, MD 21287-3923.

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