2015 HSC Section 1 Book of Articles

greater in syndromic children (

0.001) than non-

syndromic cleft, though in a multivariate analysis

MacLean

et al.

[3] felt that intervention for speech

with a pharyngoplasty was of a greater concern.

Muntz

et al.

[2] suggested that a greater number of

syndromic children had symptoms of sleep apnea

(

0.001) and were more likely to have a sleep

study (

0.05).

Robin Sequence is classically micrognathia caus-

ing posterior tongue placement preventing the

closure of the palate. This is usually seen with a

wide U-shaped cleft palate. The posterior tongue

placement causes airway obstruction as a neonate.

Though this can vary in severity, years ago many

children had tracheotomy for this. Because of feed-

ing issues, gastrostomy tube was common as well.

Even with a normal mandible, the child with a

cleft and cleft repair is at greater risk for airway

obstruction. Nasal septal deviation is a standard

finding in children with any unilateral cleft lip.

Scott

et al

. [5] described some of the issues that could

cause obstruction in the normal repairs of the cleft

palate. The use of the nasal septal mucoperichon-

drial flaps for closure of the nasal layer of the palate

may reduce the airway at the floor of the nose.

Furlow palatoplasty (the double opposing Z-plasty)

has been shown to both lengthen and thicken the

palate. Though this is great for speech, there should

be some tendency to decrease the airway. Tonsil and

adenoid hypertrophy are common in all children

and the cleft population is not exempt. Midface

hypoplasia is sometimes seen in children with cleft

palate. This causes obstruction at the posterior naso-

pharynx and may be a cause of airway obstruction

and sleep disordered breathing. As many as 13% of

the cleft palate population are at risk of

velopharyngeal insufficiency (VPI) and need surgi-

cal correction. Acute airway obstruction in pharyng-

eal flap and sphincter pharyngoplasty has been well

documented. The chronic obstruction can cause

both nasal airway compromise and sleep disordered

breathing or obstructive sleep apnea. The balance

between good speech and breathing is most critical

in this group.

Continuous positive airway pressure (CPAP) is a

standard approach for the treatment of obstructive

sleep apnea in the adult and pediatric population.

CPAP may be tolerated by children, but just as in the

adult many children may refuse to wear the device

or because of the anatomy have difficulty with fit-

ting the mask. Though CPAP is a treatment, it seems

that if the anatomical obstruction can be remedied

surgically the patient would be better served. The

surgical management of sleep disordered breathing

and obstructive sleep apnea in the cleft population

requires an intimate understanding of the anatom-

ical reasons for the obstruction. Careful planning

must be done to reduce the risk and yet open the

airway. As treatments are often not successful,

diligence is required to ask the right questions

and to study the sleep after intervention.

MANAGEMENT OF OBSTRUCTIVE SLEEP

APNEA

The following is a review of the multiple manage-

ment strategies available for the treatment of prim-

arily obstructive sleep apnea in the cleft population.

Robin Sequence

Robin Sequence is the classic cleft palate related

obstruction [6

]. Many children will do very well

with positioning alone (prone or side). As they grow

and the neuromuscular tone improves, tongue pos-

ition can come forward making further intervention

unnecessary. A significant segment of the Robin

population though will not be successfully treated

in this way. The child managed this way may do well

until the baby is more mobile and then the airway

may be obstructed if on the back.

The Seattle Children’s Hospital Team has pro-

moted the use of nasopharyngeal airways in the

treatment of children with Robin Sequence. Over

one-third of the children in the initial study had

Robin Sequence and an additional 11% had other

mandibular abnormalities. The median duration of

the nasopharyngeal airway was 8 weeks before the

airway was secure [7

]. This offers a nonsurgical

approach to the airway and in selected children

may be an alternative. It may also be used with high

flow in the infant.

KEY POINTS

Obstructive sleep apnea is common in all children with

cleft, especially those with a syndrome, and the

clinician should make certain that the right questions

about sleep are asked.

Surgical management of obstructive sleep apnea in

children is not always successful, and postoperative

sleep assessment should be strongly considered.

The use of nasopharyngeal airway should be

considered in those children with Robin Sequence.

Children who have had surgical management of

velopharyngeal insufficiency are at greater risk for

obstructive sleep apnea.

Clear definition of the location of the obstruction should

allow improved outcomes in the surgical management

of sleep apnea in the cleft population.

Management of sleep apnea in the cleft population

Muntz

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