Literature DB >> 26702938

Dental treatment of a child with congenital central hypoventilation syndrome.

V Boka1,2, A Lefkelidou3, E Athanasiadou3.   

Abstract

BACKGROUND: Congenital Central Hypoventilation Syndrome (CCHS) is a rare condition combining respiratory hypoventilation with symptoms of autonomic dysregulation. Management of patients requires both medical and dental expertise to achieve a successful outcome. The aim of this paper is to present the dental management of a child diagnosed with CCHS, without pharmacological measures and in cooperation with medical expertise. CASE REPORT: A 7-year-old girl was referred to a private dental practice with the chief complaints being pain and poor aesthetics. The child had been diagnosed with CCHS since infancy and had undergone several courses of medication. Although the patient was uncooperative, the paediatric pulmonologist advised against general anaesthesia. As a result, she was treated in the operating theater (OT) without sedatives, being monitored throughout the entire procedure. A total of eight primary teeth needed dental treatment (3 were restored, 4 were extracted and 1 was restored with a preformed metal crown). A mandibular lingual holding arch was placed, two weeks later. FOLLOW-UP: The patient was seen after 6 months and 1 year. Her oral hygiene had improved significantly and her mother reported that the child ate better, brushed her teeth on a daily basis and was careful with dietary habits.
CONCLUSION: The collaboration between medical experts and a paediatric dentist was of crucial importance. The use of basic behaviour management techniques in conjunction with monitoring the patient's vital signs led to a successful outcome and an improvement in the behaviour of the patient.

Entities:  

Keywords:  Congenital central hypoventilation syndrome; Dental treatment; Ondine’s curse

Mesh:

Year:  2015        PMID: 26702938     DOI: 10.1007/s40368-015-0213-9

Source DB:  PubMed          Journal:  Eur Arch Paediatr Dent        ISSN: 1818-6300


  15 in total

Review 1.  Idiopathic congenital central hypoventilation syndrome: diagnosis and management. American Thoracic Society.

Authors: 
Journal:  Am J Respir Crit Care Med       Date:  1999-07       Impact factor: 21.405

2.  Adult identified with congenital central hypoventilation syndrome--mutation in PHOX2b gene and late-onset CHS.

Authors:  Debra E Weese-Mayer; Elizabeth M Berry-Kravis; Lili Zhou
Journal:  Am J Respir Crit Care Med       Date:  2005-01-01       Impact factor: 21.405

3.  Facial phenotype in children and young adults with PHOX2B-determined congenital central hypoventilation syndrome: quantitative pattern of dysmorphology.

Authors:  Emily S Todd; Seth M Weinberg; Elizabeth M Berry-Kravis; Jean M Silvestri; Anna S Kenny; Casey M Rand; Lili Zhou; Brion S Maher; Mary L Marazita; Debra E Weese-Mayer
Journal:  Pediatr Res       Date:  2005-12-02       Impact factor: 3.756

4.  Failure of automatic control of ventilation (Ondine's curse). Report of an infant born with this syndrome and review of the literature.

Authors:  R B Mellins; H H Balfour; G M Turino; R W Winters
Journal:  Medicine (Baltimore)       Date:  1970-11       Impact factor: 1.889

5.  An official ATS clinical policy statement: Congenital central hypoventilation syndrome: genetic basis, diagnosis, and management.

Authors:  Debra E Weese-Mayer; Elizabeth M Berry-Kravis; Isabella Ceccherini; Thomas G Keens; Darius A Loghmanee; Ha Trang
Journal:  Am J Respir Crit Care Med       Date:  2010-03-15       Impact factor: 21.405

6.  Respiratory sensations in subjects who lack a ventilatory response to CO2.

Authors:  S A Shea; L P Andres; D C Shannon; A Guz; R B Banzett
Journal:  Respir Physiol       Date:  1993-08

7.  Congenital central hypoventilation syndrome: diagnosis, management, and long-term outcome in thirty-two children.

Authors:  D E Weese-Mayer; J M Silvestri; L J Menzies; A S Morrow-Kenny; C E Hunt; S A Hauptman
Journal:  J Pediatr       Date:  1992-03       Impact factor: 4.406

8.  Neuropsychologic abnormalities in children with congenital central hypoventilation syndrome.

Authors:  J M Silvestri; D E Weese-Mayer; M N Nelson
Journal:  J Pediatr       Date:  1992-03       Impact factor: 4.406

Review 9.  Dental anxiety and behaviour management problems in paediatric dentistry--a review of background factors and diagnostics.

Authors:  G Klinberg
Journal:  Eur Arch Paediatr Dent       Date:  2008-02

10.  Expression and interactions of the two closely related homeobox genes Phox2a and Phox2b during neurogenesis.

Authors:  A Pattyn; X Morin; H Cremer; C Goridis; J F Brunet
Journal:  Development       Date:  1997-10       Impact factor: 6.868

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