**6. Adverse effects of noninvasive ventilation**

In ND, DS, and other diseases, it is frequent to use noninvasive ventilation to help children ventilation. Masks, type bilevel positive airway pressure (BiPAP), continuous positive airway pressure (CPAP), and similar are generally supported on the maxilla. However, if used in the tender bone of young children and for a long time, these appliances (BiPAP, CPAP, or similar) may produce retrusion of the face. The deleterious facial effects of noninvasive ventilation [49] are described in a girl with neuromuscular dystrophy, an example of these rare diseases (**Figure 4**). And the necessity of an interdisciplinary team is essential for successful treatment.

**Figure 4.** *Extraoral traction appliance (face mask) in a child with neuromuscular dystrophy.*

In the same way, RME may be used to restore the compressed maxilla of Carey Fineman Ziter syndrome, a very rare genetic muscular disorder present at birth (congenital myopathy), and ETA may reestablish the correct relationship between maxilla and mandible. In **Figure 4**, we may find maxillary constriction in conjunction with anterior and bilateral posterior crossbites in a child with Carey Fineman Ziter syndrome that used a BiPAP from the age of four months.

Congenital nemaline myopathy is another rare disease that may benefit from pediatric dental treatment, as oral maxillofacial dystrophy is highly dysfunctional (**Figure 5**) and needs intervention and special care from birth.

#### **Figure 5.**

*Maxillary constriction in conjunction with anterior and bilateral posterior crossbites in a child with Carey Fineman Ziter syndrome that used a BiPAP from the age of 4 months.*

#### **Figure 6.**

*Severe dentomaxillofacial pathology in a boy with congenital nemaline myopathy with one of the known mutations, nebulin. In addition to the compressed jaw, the maxilla has enormous retrusion, affecting several essential functions, from breathing to swallowing, chewing, and speaking.*

Anyway, this is just one of the phases of the multidisciplinary treatment that may help oral functions in a moment of life, but that always needs to be complemented by other professionals, including pediatric surgeons, speech therapists, myofunctional physiotherapists, ENT, and others (**Figure 6**).
