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Introduction

Congenital central hypoventilation syndrome (CCHS) is a disorder in which the major feature is an inability to breathe correctly, mainly during sleep. This inability may be less or more severe – the child may need a ventilator only while sleeping or all the time. The ventilator will guarantee the child’s breathing, in order to maintain adequate levels of blood gases (oxygen and carbon dioxide). The ventilator is connected to the patient by a tube and an interface – a tracheostomy canula or a mask.

Most patients need ventilation support since birth.

mask 1 

mask 2  Choosing Interface

Some authors recommend ventilation by tracheostomy during the first years of life, while others advocate mask ventilation. If ventilation is needed for 24 hours, tracheostomy is preferred. Feeding will be easier and blood gases more stable.

If hypoventilation is less severe, non-invasive ventilation with a mask may be an option. The decision will depend on the clinical picture and on the experience of your doctor.

Masks come in different shapes, materials and sizes, although for children there is less variety. The mask that fits best must be thoroughly looked for, so that less problems arise

Pros and Cons

Masks may be nasal (covers the nose) or oro-nasal (covers the nose and mouth). Facial masks are avoided as long as possible in children due to the potential risk of aspiration.

mask 3

mask 4 Some advantages of mask ventilation
  • less invasive (more normal)

  • allows speech and language development

  • less suction needs

  • less respiratory infections

mask 5
Complications to be aware:

  • conjunctivitis

  • mouth dryness

  • gastric distension

  • skin sores on the face

  • easy mask displacement and air leaks (if the child moves a lot during sleep)

  • mid-face hypoplasia, owing to daily pressure of the mask on the developing bone structure.

Size of the mask

In some cases, mask ventilation has been started very early (two weeks of age). Mid-face hypoplasia may be minimized by alternating between nasal and oronasal masks every three days

mask 6 CCHS child with nasal mask and three days later with facial mask. mask 7
Facial mask may also prevent air leakage through the mouth when it opens during sleep

Caring of the mask

Tubes and mask need to be changed periodically to be sure they are in good condition and the size is adequate.

The mask must be kept proper, washing it every dayDoing well with mask ventilation

Following all indications and reporting all problems to the health team will help for a good outcome.

update: 19/12/2011


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