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Tongue–lip adhesion has been a mainstay of
treatment for airway obstruction in children with
Robin Sequence. In this procedure, the undersurface
of the tongue is secured to the mucosa and muscle of
the lower lip, often with a retention sure to remove
tension on the wound while healing. In general, it
seems to be more effective in the child without a
syndrome. Sedaghat
et al.
[8] reviewed a small num-
ber of children with tongue–lip adhesion and found
that most were benefited, but that only 38% had
complete resolution based on polysomnography.
Abramowicz
et al.
[9] felt that one could more accu-
rately predict the success of tongue–lip adhesion
with using a GILLS score of less than 2. This takes
into consideration gastroesophageal reflux, pre-
operative intubation, low birth weight, syndromic
diagnosis and late surgical intervention. Certainly,
not all are benefited by this particular intervention
as some would promote but may be considered in
the decision for treatment.
Much attention has been focused on bilateral
mandibular osteotomy with distraction osteogen-
esis for children with micrognathia with or without
Robin Sequence [6
&&
]. It makes sense that as the jaw
is distracted anteriorly, the tongue will also be
pulled forward, opening the posterior airway. It is
usually very successful for improving the airway as
well as feeding. This has been done both with
internal and external distraction devices. Internal
devices usually offer only linear distraction that may
leave the child with an open bite. The multivector
external distractors have the advantage of allowing
differential distraction based on the observed
relation to the maxillary alveolus. This may include
the closure of the open bite with a rotational dis-
traction as well decreasing the resistance in linear
distraction with varus–valgus adjustments. Scott
et al.
[6
&&
] looked at 18 children under 3 months
with early distraction and felt the procedure to be
both well tolerated and effective as seen from a
3-year follow-up. Though this procedure seems
effective for airway and feeding, there are significant
risks including facial nerve injury (9%), tooth loss
(16%) and a 5.2% need for additional distraction as
the child aged.
Tonsillectomy and partial adenoidectomy
For most otolaryngologists, the understanding of
the benefit of tonsillectomy and adenoidectomy
in children with sleep apnea is apparent. The cleft
population is a concern because of the risk of exacer-
bating VPI if the adenoids are removed. Some even
refuse the use of adenoidectomy in children with
cleft palate. Shapiro [10] initially discussed partial
(superior) adenoidectomy as a way to reduce this
risk. Since then, there have been a number of reports
on techniques to improve the partial adenoidec-
tomy. It has been promoted for all children with
palatal abnormalities undergoing adenoidectomy.
Removing the superior and leaving the inferior
rim of adenoid tissue should improve airway but
allow the palate to contact the residual adenoid
tissue for speech. Some also promote this for chil-
dren with Down syndrome.
In a study by Muntz
et al.
[2], tonsillectomy and
partial adenoidectomy were the initial intervention
for most of the cleft children with obstructive
sleep apnea. Though there was a significant overall
improvement in the sleep, many of the children
continued to have sleep apnea. It is very important
to follow these children to make certain there is not
a significant obstructive sleep issue even after ton-
sillectomy and partial adenoidectomy.
Midface hypoplasia
Midface hypoplasia is often associated with cranio-
facial syndromes and cleft palate. Though often
blamed on early hard palate repair, this is frequently
seen regardless of the timing of palatal repair. The
bony hypoplasia sets back the hard palate pushing
the soft tissue of the soft palate posterior as well.
This results in a decreased airway and as such can
increase the likelihood of obstructive sleep apnea.
Occasionally, we also see midface hypoplasia as a
result of chronic CPAP use. Smatt and Ferri [11] and
Ronchi
et al.
[12] both suggest there is a significant
improvement in obstructive sleep apnea with man-
dibular and maxillary advancement. This has also
been documented in children with craniofacial syn-
dromes such as achondroplasia [13]. As many of the
children will need the distraction or advancement
for occlusion and aesthetics, the more important
issue of airway may be corrected at that same time.
Midface advancement may result in VPI if the upper
jaw is displaced forward interfering with the closure
of the child’s velopharyngeal port.
Obstruction postsurgical correction of
velopharyngeal insufficiency
The treatment of VPI includes surgical management
either with further palatal surgery or the creation of
a velopharyngeal obstruction to allow appropriate
oral pressure for speech. Classically, pharyngeal flap
and sphincter pharyngoplasty have been used to
correct the VPI. Additionally, multiple methods of
velopharyngeal augmentation have been used. If a
surgery has been done to improve the speech and
sleep apnea results, one must balance the issues of
airway and speech production [14,15]. Many of
Pediatric otolaryngology
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