Top

Published in:

01-06-2008 | Original Article

Feeding Difficulties in Children with CHARGE Syndrome: Prevalence, Risk Factors, and Prognosis

Authors: Cindy Dobbelsteyn, Sean D. Peacocke, Kim Blake, William Crist, Mohsin Rashid

Published in: Dysphagia | Issue 2/2008

Abstract

Children with CHARGE syndrome frequently experience long-term and complex feeding difficulties. This study investigated the prevalence and nature of feeding difficulties in a population of 39 children with CHARGE syndrome and explored the relationship between the clinical characteristics of the syndrome and feeding development. Information was collected via a survey (two questionnaires) completed by the parents. One questionnaire, the Pediatric Assessment Scale for Severe Feeding Problems, provided an objective measure of the current severity of feeding difficulties. Results of the survey indicated a high prevalence of long-term feeding issues in this population. Approximately 90% of the children had received tube feeding at some point in time. In contrast to previous literature, choanal atresia and heart malformations were not found to be significantly related to a higher severity of feeding difficulty or associated with long-term tube feeding. Cranial nerve dysfunction was found to be the primary clinical feature of CHARGE syndrome impacting feeding development, reflected in a high prevalence of weak sucking/chewing, swallowing difficulty, gastroesophageal reflux, and aspiration. The presence of these conditions in infancy suggests the likelihood of long-term feeding difficulty and warrants the ongoing attention of feeding specialists.

Available only for authorised users

Hall B. Choanal atresia and associated multiple anomalies. J Pediatr 1979;95(3):395–398.PubMedCrossRef

Pagon RA, Graham JM, Zonana J, Yong SL. Coloboma, congenital heart disease and choanal atresia with multiple anomalies: CHARGE association. J Pediatr 1981;99(2):223–237.PubMedCrossRef

Blake KD, Davenport SL, Hall BD, Hefner MA, Pagon RA, Williams MS, Lin AE, Graham JM. CHARGE association: An update and review for the primary pediatrician. Clin Pediatr (Phila) 1998;37:159–174.

Amiel J, Attiee-Bitach T, Marianowski R, Cormier-Daire V, Abadie V, Bonnet D, Gonzales M, Chemouny S, Brunelle F, Munnich A, Manach Y, Lyonnet S. Temporal bone anomaly proposed as a major criteria for diagnosis of CHARGE syndrome. Am J Med Genet 2001;99(2):124–127.PubMedCrossRef

Chalouhi C, Faulcon P, Le Bihan C, Hertz-Pannier L, Bonfils P, Abadie V. Olfactory evaluation in children: application to the CHARGE syndrome. Pediatrics 2005;116(1):81–88.CrossRef

Tellier AL, Cormier-Daire V, Abadie V, Amile J, Sigaudy S, et al. CHARGE syndrome: report of 47 cases and review. Am J Med Genet 1998;76(5):402–409.PubMedCrossRef

Smith IM, Nichols SL, Issekutz K, Blake K. Canadian Paediatric Surveillance Program, Behavioral profiles and symptoms of autism in CHARGE syndrome: Preliminary Canadian epidemiological data. Am J Med Genet A 2005;133A(3):248–256.PubMedCrossRef

Blake KD, Russell-Eggitt IM, Morgan DW, Ratcliffe JM, Wyse RK. Who’s in CHARGE? Multidisciplinary management of patients with CHARGE association. Arch Dis Child 1990;65:217–223.PubMed

Harvey AS, Leaper PM, Bankier A. CHARGE association: clinical manifestations and developmental outcome. Am J Med Genet 1991;39(1):48–55.PubMedCrossRef

10.

Daoud MA, Gradstein J, Blake KD. CHARGE into the adolescent and adult decade. Pediatr Child Health 2002;7(Suppl A):27A.

11.

Issekutz KA, Graham JM Jr, Prasad C, Smith IM, Blake KD. An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A 2005;133(3):309–317.

12.

Blake KD, Salem-Hartshorne N, Daoud MA, Gradstein J. Adolescent and adult issues in CHARGE syndrome. Clin Pediatr (Phila) 2005;44(2):151–159.CrossRef

13.

Burklow KA, Phelps AN, Schultz JR. MacConnell K, Rudolph C. Classifying complex pediatric feeding disorders. J Pediatr Gastroenterol Nutr 1998;27(2):143–147.PubMedCrossRef

14.

Kosko JR, Moser JD, Erhart N, Tunkel DE. Dysphagia in children, adults and geriatrics. Otolaryngol Clin North Am 1998;31(3):435–451.PubMedCrossRef

15.

O’Neil KH, Purdy M, Falk J, Gallo L. The Dysphagia Outcome and Severity Scale. Dysphagia 1999;14:139–145.PubMedCrossRef

16.

Hall KD. Pediatric Dysphagia: resource guide. San Diego, CA: Singular, 2001.

17.

Arvedson JC, Brodsky L. Pediatric swallowing and feeding: Assessment and management, 2nd ed. Albany, NY: Singular, 2002.

18.

Bhatnager SC. Neuroscience: For the study of communicative disorders, 2nd ed. Baltimore, MD: Lippincott Williams & Wilkins, 2002.

19.

Blake KD, Graham JM, Prasad C, et al. CHARGE association/syndrome. Canadian Paediatric Surveillance Program 2004 results. Ottawa: Canadian Paediatric Society, Health Canada, 2004. pp 27–31.

20.

Crist W, Dobbelsteyn C, Brousseau AM, Napier-Phillips A. Pediatric assessment scale for severe feeding problems: validity and reliability of a new scale for tube-fed children. Nutr Clin Pract 2004;19(4):403–408.PubMedCrossRef

21.

Dobbelsteyn C, Marche DM, Blake K, Rashid M. Early oral sensory experiences and feeding development in CHARGE syndrome: a report of five cases. Dysphagia 2005;20(2):89–100.PubMedCrossRef

22.

Strudwick S. Gastro-oesophageal reflux and feeding: the speech and language therapist’s perspective. Int J Otorhinolaryngol 2003;7(S1):S101–S102.CrossRef

23.

Eicher PS, McDonald-McGinn DM, Fox CA, Driscoll DA, Emanuel BS, Zachai EH. Dysphagia in children with a 22q11.2 deletion: Unusual pattern found on modified barium swallow. J Pediatr 2000;137(2):158–165.PubMedCrossRef

24.

Bazyk S. Factors associated with the transition to oral feeding in infants fed by nasogastric tubes. Am J Occup Ther 1990;44(12):1070–1078.PubMed

25.

Kimber C, Kiely EM, Spitz L. The failure rate of surgery for gastro-oesophageal reflux. J Pediatr Surg 1998;33(1):65–67.

26.

Lawland CMD, Blake KD, Prasad C, et al. The cranial nerve anomalies of CHARGE association/syndrome (A/S). Pediatr Child Health 2003;38(Suppl 8):26B.

27.

Rudolph CD, Link DT. Feeding disorders in infants and children. Pediatr Clin North Am 2002;49(1):97–112.PubMedCrossRef

28.

Davenport SLH, Hefner MA, Mitchell JA. The spectrum of clinical features in CHARGE syndrome. Clin Genet 1986;29:298–310.PubMedCrossRef

29.

Blake KD, Brown D. CHARGE association looking at the future — the voice of a family support group. Child Care Health Dev 1993;19:395–409.PubMedCrossRef

30.

White DR, Giambra BK, Hopkin RJ, Daines CL, Rutter MJ. Aspiration in children with CHARGE syndrome. Int J Pediatr Otorhinolaryngol 2005;69:1205–1209.PubMedCrossRef

Title: Feeding Difficulties in Children with CHARGE Syndrome: Prevalence, Risk Factors, and Prognosis
Authors: Cindy Dobbelsteyn
Sean D. Peacocke
Kim Blake
William Crist
Mohsin Rashid
Publication date: 01-06-2008
Publisher: Springer-Verlag
Published in: Dysphagia / Issue 2/2008
Print ISSN: 0179-051X
Electronic ISSN: 1432-0460
DOI: https://doi.org/10.1007/s00455-007-9111-6

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Feeding Difficulties in Children with CHARGE Syndrome: Prevalence, Risk Factors, and Prognosis

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 2/2008

Rhabdomyoma of the Parapharyngeal Space Presenting with Dysphagia

Evaluation of Swallowing in Children with Vomiting After Feeding

A Question of Rheological Control

Pneumatic Balloon Dilation Therapy Is as Effective as Esophagomyotomy for Achalasia

The Coordination of Breathing and Swallowing in Parkinson’s Disease

Rigid Spine Syndrome: A Radiologic and Manometric Study of the Pharynx and Esophagus