2015 HSC Section 1 Book of Articles

Management of sleep apnea in the cleft population Muntz

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

greater in syndromic children ( P < 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 ( P < 0.001) and were more likely to have a sleep study ( P < 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 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.

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