Feedingmanagementin Infantswithcraniofacial Anomalies: Jill M. Merrow
Feedingmanagementin Infantswithcraniofacial Anomalies: Jill M. Merrow
I n f a n t s w i t h Cr a n i o f a c i a l
Anomalies
Jill M. Merrow, MA, CCC-SLP
KEYWORDS
Cleft Craniofacial Feeding management Dysphagia Breastfeeding
Fiberoptic endoscopic evaluation of swallowing Modified barium swallow study
Video swallow study
KEY POINTS
Feeding and swallowing abilities in infants born with craniofacial anomalies show great variability.
Difficulties with feeding mechanics in infants with cleft lip and/or palate include limited labial seal
and stability of the nipple within the oral cavity, suboptimal intraoral pressure, suction, and milk
transfer.
Feeding difficulties with cleft lip and/or palate can be more complicated in the presence of an asso-
ciated syndrome or sequence.
Common feeding goals for infants with craniofacial anomalies include improvement of milk flow to
meet caloric intake requirements, prevention of excessive air intake, minimalization of nasal regur-
gitation, and attainment of physiologic stability accomplished through various management
strategies.
Instrumental assessment via fiberoptic endoscopic evaluation of swallowing and/or modified
barium swallow study can be used when appropriate to gain further objective data regarding swal-
low function and to devise strategies to promote safe feeding and swallowing.
ferrals to cleft and craniofacial teams for prena- ficulties and their causes, evaluation, and
tal consultations after identification of a cleft on management.
of the modifications needed for feeding success. breathing while feeding given that infants are obli-
Clefts are classified as unilateral or bilateral and gate nasal breathers. With a small cleft of the soft
complete versus incomplete. Peterson-Falzone palate, the tongue often occludes the cleft for at
and colleagues5 (2001) remind us that “clefts least part of the transition from suck to swallow,
vary in three other dimensions: anterior to poste- thereby reducing effect on negative pressure gen-
rior, width and ‘depth.’” These dimensions are eration. With a submucous cleft palate, there may
important when deciding on the most efficacious be mild reduction in negative pressure due to the
nipple placement within the mouth. Infants with dysfunction of the velopharyngeal valve. Nutritive
cleft lip and palate innately exhibit a sucking reflex breastfeeding is most likely possible with a small
similar to typically developing infants; however, cleft of the soft palate or submucous cleft palate;
they have limited ability to create suction efficiently however, nutritive breastfeeding is rarely success-
and consistently. ful in an infant with a large cleft palate.
Table 1
According to Peterson-Falzone and colleagues5
Sucking differences in infants with clefts (2001), both sides of the face may be involved
but typically one side is more affected than the
Infants with other. Feeding and swallowing difficulties may
Unilateral Cleft result from structural anomalies of the jaw, tongue,
Lip and Palate Infants face, and pharynx, or from neurologic dysfunction
or Isolated without and/or congenital heart defects.
Cleft Palate Clefts
22q deletion syndrome
Length of 8.97 s 13.28 s
sucking
These infants may experience feeding and swal-
bursts lowing problems of multiple causes given the great
variability in phenotypic expression. Airway anom-
Rate of 109.26 75.07
sucking sucks/min sucks/min alies and cardiac defects can negatively affect the
suck-swallow-breathe cycle, induce significant fa-
Length of 0.57 s 0.87 s
individual
tigue, and lead to aspiration from an antegrade
sucks process. Cardiac defects can cause extrinsic
compression of the trachea or of the esophagus,
Positive 71.68% 25.71%
pressure and obstruct the passing of food and liquid.
generation Gastrointestinal tract dysfunction can occur due
Suck-swallow 2.97:1 1.20:1
to hypotonia with reflux, slowed motility, and
ratio structural anomalies. These issues can lead to
limited caloric intake, feeding refusal, poor weight
Data from Masarei AG, Sell D, Habel A, et al. The nature of gain, and failure to thrive. It is important to keep in
feeding in infants with unrepaired cleft lip and/or palate
compared with healthy noncleft infants. Cleft Palate mind the linear growth trajectory predicted for
Craniofac J 2007;44:321–8. these infants and the tendency to exhibit slower
growth than their same-age peers in early
childhood.
misaligned forces of compression by the dental Treacher Collins syndrome
arches, and the lack of contact to the superior sur- These infants often experience poor airway
face of the nipple secondary to the typically wide patency and respiratory difficulties. Tracheot-
U-shaped cleft palate. The tongue often sits omies are frequently required.5 Surgical repair of
retracted in the mouth (glossoptosis) with minimal a cleft palate may lead to greater risk of airway
ability to produce an anterior tongue carriage to compromise. Conservative oral feeding measures
compress the nipple effectively. If the infant is may be needed with supplemental or alternate
able to produce an anterior tongue carriage to enteral feedings to meet nutritional needs but,
secure the nipple, airway patency typically im- certainly, variability is present.
proves. Suction ability depends on the size of the
cleft palate. There is variability in the phenotypic SOCIAL–EMOTIONAL INTERACTIONS
expression of Pierre Robin sequence and there
may be absence of a cleft palate in some cases. The technical aspects of feeding infants with
With less pharyngeal space for airway patency clefts and other craniofacial anomalies are of
and swallowing mechanics due to micrognathia utmost importance to ensure adequate growth;
and glossoptosis, these infants may experience however, attention should also be paid to the
significant chest retractions, nasal flaring, snort- feeding interaction between parent and child.
ing, stertor, and stridor at baseline and while Speltz and colleagues8 (1994) assessed 3-
feeding. These infants tend to pause more month-old infants with clefts and their mothers.
frequently and tolerate only short sucking bursts. The infants were divided into 3 groups: cleft lip
Longer sucking bursts often tax efforts at coordi- and palate; cleft palate only; and healthy, typical
nation and lead to extended swallow apneas, infants. Infants’ temperament and mothers’
gasping, gagging, and possibly aspiration. emotional and attitudinal factors were critiqued.
Furthermore, airway issues may also mask under- Infants in the typical group rated higher than
lying neuromotor dysfunction. both cleft groups on the clarity of cues scale. In-
fants with cleft lip and palate were less likely to
Hemifacial microsomia signal readiness to eat and less likely to display
This broad group of first and second branchial changes in tension at the onset of the feeding
arch malformations results in mandibular hypopla- and shortly thereafter. Both cleft groups were
sia and facial weakness of varying degrees.4 less likely than the typical infants to smile or laugh
Feeding Management in Infants with Craniofacial Anomalies 441
with infants feeding from a bottle or a neurologi- Occlusion of the cleft palate: Obturators are not
cally impaired child who has difficulty with self- necessary and used infrequently.
pacing, are better evaluated by MBSS; otherwise, Nipple modifications: Wide base and shaft for
the view from the endoscope is obstructed by the better occlusion of the cleft, length depends
repetitive peristaltic movement of the bolus on size of the mouth and cleft, softness for
through the hypopharynx. Globus complaints, easier compression, and variable hole size
possible cricopharyngeal dysfunction, and to adjust the flow rate of milk.
nonspecific complaints are better evaluated by Infant-directed, assisted milk flow by bottle
MBSS.19 If a patient would have poor tolerance (Fig. 1): Use a soft-sided bottle and pliable
of endoscopy or refusal thereof, an MBSS would nipple or a one-way flow valve.
be indicated. A prospective study by Weir and col- Feeder-directed, assisted milk flow by bottle: Use
leagues20 (2011) supported the use of MBSS to a soft-sided bottle and pliable nipple, angle the
identify silent aspiration in children, including nipple to contact a portion of bone for better
those with aspiration lung disease and neurologic positive pressure generation, squeeze the bot-
impairment. MBSS is also a helpful assessment tle when the infant sucks to synchronize posi-
tool when diagnosing functional effects of asym- tive pressure application with the infant’s
metric movement given the lateral, anteroposte- suck-swallow-breathe pattern (see Fig. 1).
rior, and Towne views available. Other: Burp frequently, irrigate the nose only if
needed.
Breastfeeding (if applicable depending on the
FEEDING MANAGEMENT type and severity of the cleft): Nurse through
let down, use manual expression, use assis-
If an infant is showing feeding difficulty, modifica-
ted milk flow at the breast (eg, a supplemental
tions should be trialed during the evaluative ses-
nursing system), and close monitoring of
sion, including the following:
growth.
Positioning of the feeder: Supported position to Breastfeeding (if the infant is not a good candi-
maintain stability throughout the feeding. date for nutritive feeding at the breast): Put
Positioning of the infant: Upright or elevated side the infant to breast briefly either at the onset
lying to minimize the effects of nasal regurgi- of the feeding or after nutritive feeding from
tation and glossoptosis (if applicable). the bottle, encourage infant to breast for
Occlusion of the cleft lip and alveolus: With the skin-to-skin contact and stimulation of milk
breast or a wide nipple. Stabilize the jaw and production, elicit assistance from a lactation
cheeks for better oral closure, being mindful consultant for milk production strategies.
that the infant may rely on oral versus nasal Compensatory strategies for poor feeding: Add
breathing if airway patency issues are present. supplement to the breastmilk, increase caloric
concentration of the formula, provide tempo- 7. Reid J, Reilly S, Kilpatrick N. Sucking performance
rary enteral feedings if necessary. of babies with cleft conditions. Cleft Palate Cranio-
Monitoring: Weight checks with the pediatrician, fac J 2007;44(3):312–20.
follow-up with clinician, referral to other 8. Speltz M, Goodell EW, Endriga MC, et al. Feed-
specialists as needed, hospital admission for ing interactions of infants with unrepaired cleft
failure to thrive or if physiologic stability is lip and/or palate. Infant Behav Dev 1994;17:
significantly compromised. 131–40.
Postoperative Feeding: Recommendations 9. Growth charts. Center for Disease Control and Pre-
based on surgeon’s preferences, practice vention Web site. Page reviewed September 9,
preoperatively. 2010. Available at: http://www.cdc.gov/growth-
charts/index.htm. Accessed February 1, 2016.
SUMMARY 10. Darrow DH, Harley CM. Evaluation of swallowing
disorders in children. Otolaryngol Clin North Am
Feeding and swallowing abilities in infants born 1998;31(3):405–18.
with craniofacial anomalies show great variability. 11. Wolf LS, Glass RP. Feeding and swallowing
Those infants with the same medical diagnosis or disorders in infancy: assessment and manage-
craniofacial anomaly may present very differently ment. San Antonio (TX): Therapy Skill Builders;
in their intrinsic management of food and liquid 1992.
consumption. The instinctual drive to gain nourish- 12. Leder SB, Karas DE. Fiberoptic endoscopic evalua-
ment can become complicated by structural differ- tion of swallowing in the pediatric population. Laryn-
ences, physiologic instability, and environmental goscope 2000;110(7):1132–6.
influences. These factors assessed individually 13. DaSilva AP, Lubianco Neto J, Santoro PP. Compari-
and in combination will assist in producing the son between videofluoroscopy and endoscopic
most favorable feeding outcomes possible with evaluation of swallowing for the diagnosis of
overall goals of providing adequate nutrition and dysphagia in children. Otolaryngol Head Neck
hydration for brain development and growth, and Surg 2010;143:204–9.
facilitating the most positive feeding experience 14. Willging JP, Miller CK, Thompson Link D, et al. Use
for both infant and caregiver. of FEES to assess and manage pediatric patients.
In: Langmore SE, editor. Endoscopic evaluation
SUPPLEMENTARY DATA and treatment of swallowing disorders. New York:
Supplementary data related to this article can Thieme; 2001. p. 213–34.
be found at http://dx.doi.org/10.1016/j.fsc.2016. 15. Link DT, Willging JP, Miller CK, et al. Pediatric laryng-
06.004. opharyngeal sensory testing during flexible endo-
scopic evaluation of swallowing: feasible and
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