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V. Kerkfeld
24.2.2 Pathological Diculties
Infants with craniofacial abnormalities (CLP,
branchial arch diseases, syndromic craniosynostoses, others) suffer from many problems regarding feeding. As mentioned before, lips should
seal the nipple while the palate grants stability
and delivers the foundation for a functional compression of the nipple with the collaboration of
the tongue. Furthermore, it is necessary that
infants are able to breathe during feeding.
Decient lips and/or palates or a compromised
airway (Fig. 24.2) have different occurrences.
Their effects for feeding and sucking need to be
declared individually [14, 15].
24.2.2.1 Clefts
Cleft Lip
Infants with a cleft lip are not able to form a labial
seal; therefore, there is less negative pressure
built up while sucking. However, in general, the
disadvantage is not incisive in the clinical picture
Fig. 24.2 CBCT in a median view
of isolated cleft lips, because the gap is often
sealed by the breast or the bottle [
16].
Cleft Lip andAlveolar Process
Due to combined insufciencies of the lips and
alveolar process, the act of sucking is much more
difcult. The cleft lip decreases the buildup of
negative pressure. In addition, a gap in the osseous structures of the alveolar process prevents a
sufcient compression of the nipple. As a consequence, the infant tries to compensate the physical disabilities by a more distinctive elevation of
the mandible. Due to excessive work during the
act of sucking, the infants are quickly exhausted.
Most of the infants with cleft lip and alveolar process have to be bottle-fed, in addition to the
breast, to guarantee an adequate uptake of calories per day.
Cleft Palate
The clinical patterns of cleft palates can be
divided into isolated cleft hard palates, submucous cleft palates, or clefts including the soft
palate.
In patients with cleft hard palates, the com-
pression and stability of the nipple is restricted.
However, the sealing of the nipple is not affected.
Like the clinical picture of cleft lip and alveolus,
the infant has to afford an increased amount of
physical work to gain milk and suffers from
fatigue.
In patients with submucous cleft palates, the
velopharyngeal valve is incomplete. The submucous gap as well leads to reduced negative pressure while sucking, but in most patients,
breastfeeding does not seem to be limited by the
malformation.
Cleft soft palates are associated with the clini-
cal picture of a remaining gap between the oral
and nasal cavity. During the act of sucking, a
steady airow lls the oral cavity with gas.
Swallowing milk in combination with air leads to
an early satiation, even though the caloric uptake
is not sufcient. The swallowing of air can lead to
massive burping and even emesis. The remaining
gap between the two cavities can also cause a
regurgitation of the milk through the nose. Milk
in the infant’s nose inhibits physiological
24 Feeding andBreathing Aspects inInfants withCraniofacial Malformations
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breathing; in consequence, the infant is not able
to take a breath during the act of swallowing.
Most of the time, the tongue of the infants, in
reection, is able to close minor clefts of the soft
palate while being fed. Depending on the extent
of the soft palate cleft, breastfeeding is possible
most of the time. However, children with major
clefts of the soft palate cannot be breastfed [15].
Cleft Lip andPalate
All the deciencies and their effects on feeding
and sucking convene in the combined pathology
of cleft lip and palate (Fig.24.3). The difculties
mentioned occur at the same time, and therefore,
breastfeeding infants with cleft lip and palate is
most likely not possible [17].
In conclusion, the feeding limitations lead to
fatigue and a limited caloric intake. The impact
of the limitations always depends on the clinical
picture and the extension and type of the cleft.
24.2.2.2 Oropharyngeal Dysphagia
Oral dysphagia arises commonly from diseases
and malformations of the mouth or throat, such as
clefts or craniofacial syndromes. Cricopharyngeal
dysphagia cannot be discriminated clinically from
oropharyngeal dysphagia and is therefore assigned
in the same category.
Infants with oropharyngeal dysphagia tend to
have a leak of saliva or bolus outside of the mouth
and are most likely not skilled in initiating or terminating the act of swallowing. In addition, the
accumulation of liquids in the pharynx leads to
choking, coughing, and postnasal regurgitation.
Affected infants often show symptoms of apnea
and cyanosis.
In children, a slurred speech as well as speech
defects can reveal an insufcient closure of the
soft palate or deciencies of the pharyngeal muscles. Even after a successful operation of the cleft
a speaking defect can remain, but logopedics
assist before and after [
18, 19].
24.2.2.3 Syndromes withCraniofacial
Anomalies
Many infants with syndromes suffer from feeding issues. Some syndromic groups display special features of feeding difculties.
Pierre Robin Sequence (CLP Group)
Infants with Pierre Robin sequence suffer from
micrognathia, which causes glossoptosis accompanied by obstruction of the airway. In addition, many infants appear with cleft palate. All
named characteristics have to be differentiated
separately in the context of breastfeeding
difficulties.
Micrognathia leads to an incongruity between
the upper jaw and the mandible. Therefore, the
infant is not able to compress the nipple appropriately. This drawback is accompanied by a limited
stability of the nipple. A sufcient milk transfer
from the anterior part of the tongue to the oropharynx is mostly not possible due to the glossoptosis. In addition, the cleft palate leads to the
difculties mentioned above, such as an insufcient lip seal and a reduced compression of the
nipple. Furthermore, the upper airway is not only
obstructed due to the nasal regurgitation but also
by the retracted tongue [20].
All these limitations lead to a frustrating feeding process with an early fatigue accompanied by
breathing difculties leading to apneas.
Fig. 24.3 Clefts
Goldenhar Syndrome (Branchial Arch
Disease Group)
The hypoplasia of the craniofacial skeleton and
the masticatory muscles leads to feeding issues
[21]. The prevalence of feeding difculties in
Goldenhar infants is between 42% [22] and 83%
[23]. Especially the sucking efciency is
decreased by a restricted excursion of the
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V. Kerkfeld
mandible arch because of the hypoplasia.
Additionally, the weakness of the N. facialis limits the cheek and lip movements, which play an
important role in the physiological mechanism of
sucking. Malformations of the tongue may disrupt a normal sucking behavior as well. Besides
the clinical picture of the craniofacial malformations, patients with Goldenhar syndrome as well
suffer from gastrointestinal malformations and
heart failures that might aggravate the feeding
issues as well [2]. Feeding difculties in
Goldenhar infants can only be treated with a
nasogastric tube that ensures continuous feeding
[24–26].
Apert Syndrome (Syndromic
Craniosynostosis Group)
Infants with Apert syndrome suffer from multisuture craniosynostosis and a retrusion of the midface. The clinical picture typically appears with
syndactyly of the second to fourth digits as well.
Nearly all infants show a coronal craniosynostosis and a majority also present synostosis of the
sagittal and lambdoidal sutures. The midface is
both retruded and hypoplastic. Some also show
cleft palates.
Feeding difculties are often observed in
infants with Apert syndrome. Besides the cleftrelated problems previously described, a constriction of the posterior nasal aperture or the
nasal meatus may lead to an interrupted sucking
by often breathing through an opened mouth.
This behavior can be mistaken for primary feeding issues.
24.2.3 Social-Emotional Interactions
Besides the technical aspects of feeding infants
with craniofacial malformations, the socialemotional interactions between parents and
infant need to be given attention. Infants with
clefts often show a decreased readiness to be fed.
In comparison to not affected infants, they show
overall fewer positive emotions before and during the feeding, even though there is no difference in the behavior of the mothers.
It is necessary to pay attention on both aspects,
the physiological (feeding technique) and the
psychological (social interactions). Prenatal
medical advices can help parents to learn about
the technical aspects of feeding and the individual anatomic issues, so that they can probably
focus on the social demands and the enjoyment
with their newborn [
feeding often leads parents to a feeling of frustration and anxiety.
17, 27]. Severely impaired
24.2.4 Management
Feeding and swallowing issues occurring congenitally and remaining throughout the whole
life are very common problems in relation to craniofacial malformations. Dysphagia can be
divided into oral and pharyngeal pathologies.
Most clinical cases mentioned before are oral
pathologies caused by craniofacial anomalies.
Craniofacial anomalies are very individual and
lead to different types and extents of feeding
issues. Pharyngeal pathologies can compound
the feeding issues. Some disturbances are even
caused neurologically during pregnancy and lead
to neurological disorders with altered hindbrain.
These malfunctions can also provide craniofacial
malformations and/or altered development of
cranial nerves in addition to the previously
described pathogenesis of the syndromes.
To nd the best support for feeding an affected
infant, it is necessary to obtain a full medical history, a good clinical examination, including
objective physiological skills, and a critical
investigation of the individual feeding process.
To achieve objective outcomes of the swallowing, instrument-based diagnostics can be used.
The ber-optic endoscopic evaluation of swallowing reveals the function of the laryngeal structures. A barium swallow study is able to reveal
even more phases of swallowing: the oral, the
pharyngeal, and the esophageal phase.
In a differential diagnosis approach, besides
the craniofacial malformation due to syndromes,
pediatric dysphagias on the basis of disrupted
hindbrain patterning have to be kept in mind.
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24.2.4.1 Medical History
A detailed medical history delivers many important details about the individual feeding issue.
Therefore, parents need to describe the issues
from their point of view regarding the overall
problems, such as the regularities and amount of
time necessary to feed the infant and the individual amount of milk swallowed. The mother’s
milk and/or the nutritional supplements and its
composition the infant receives have to be examined, to differentiate the uptake and number of
calories and also the structure of the liquid,
regarding the viscosity. It has to be identied how
the infant is regularly fed, if there is only breastor bottle-feeding, or a combination of both.
24.2.4.2 Observation
In addition to the observation of the act of swallowing, it is important to look wisely at the phase
of resting and especially pay attention on the
interaction between the feeder and the infant.
Important subjects are the posture of the infant,
the airway (stridor, phases of apnea, position of
the chest), swallowing of saliva, drooling, and
coughing.
A comparison of the resting phase and the act
of swallowing helps to understand the individual
issues of the infant. It is important to examine
how the physiological parameters change and to
pay attention on the oral, pharyngeal, and esophageal phase of swallowing. Potential obstacles
have to be spotted and eliminated.
24.2.4.3 Examination
Clinical Examination
First of all, vital parameters (pO2, heart rate,
breathing rate) and the oral status need to be
examined. Some minor obstacles can be identied and eliminated directly. Depending on the
situation, it can be helpful to decrease the air
intake during the act of swallowing to correct the
nasal regurgitation. A simple modication of the
position of the infant during feeding can decrease
the unwanted airow. Variances in the viscosities
of the bottle liquid or the amount of the milk/liquid ow and an individual support during milk
intake can enhance the situation.
Instrumental Examination
Instrumental examination can show hidden
obstacles or consolidate present diagnoses. The
ber-optic endoscopic evaluation and the modied barium swallow study can both detect the
swallowing pattern and the individual restrictions. The evaluation of the anatomic and physiological circumstances of the swallowing might
detect possibilities to advance or modify the
feeding process. Modications can be different
postures, other viscosities of the exposed liquids, and a variation of the dosage forms (e.g.,
breastfeeding, bottle-feeding, cup-feeding,
spoon-feeding).
Both clinical methods mentioned above have
advantages and disadvantages; in some cases, the
endoscopic evaluation, in other cases the barium
swallow method, or sometimes a combination of
both techniques can be clinically indicated.
Fiber-Optic Endoscopic Evaluation
ofSwallowing
The endoscopic technique (Fig.
direct view on laryngeal structures and helps to
determine the laryngeal function. It is always
preferable to the barium swallow, because in this
direct technique there is no application of radioactive technology. In addition, the advantage of
the endoscopic method is the practicability. There
is no radiology department needed to execute the
examination [28, 29].
Modied Barium Swallow Study
Video: https://www.shutterstock.com/de/video/clip-
19,831,825-x-rays-esophagus- contrast- barium
The barium swallow study (Fig.24.5) offers
the way to examine the oropharyngeal swallowing pattern. This technique convinces by its
clear view that might be masked in endoscopic
methods by the bolus. Globus sensations, cricopharyngeal malfunctions, and unspecic discomforts can be evaluated better. Another
advantage is the high acceptance rate in infants
that sometimes do not tolerate the endoscopic
procedure. A disadvantage is the application of
radioactive emission and the necessity to cooperate with a specialized radiology department
[30].
24.4) obtains a
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Fig. 24.4 Fiber-optic
investigation. (Reprinted
from: a katz/
Shutterstock.com with
permission)
V. Kerkfeld
24.2.5 Treatment
Some exercises can be adopted to improve the
feeding procedure. First of all, the posture of both
the feeder and the infant should be stabilized in
an upright position.
In cases of cleft lips, the breast and/or nipple
should seal the oral cavity to the outside; sometimes the mother can manage a closure by supporting the breast/nipple in a specic angle to the
infant’s mouth. The mandible can be stabilized as
well so that a sufcient closure of the mouth can
be granted. Most children with cleft palates do
not benet from synthetic obturators.
24.2.6 Conclusion
There is a high variance on feeding and sucking
abilities in infants with craniofacial malformations. Besides the overall medical diagnosis, individual physiological and anatomic structures can
be manifested in different extents. The innate
demand on nutrition can differ to the infant’s
competence to ingest a certain amount of milk.
The feeding issues can be determined by observation and mechanical techniques for an individual support of the feeding procedure. The
overarching goal is to provide an adequate nutri-
tional uptake and to guarantee a seasonable physiological development of the child.
24.3 Breathing
24.3.1 General Aspects
Many infants with craniofacial malformations
appear to have a disordered breathing while
sleeping to a various extent. Sleep disordered
breathing can be divided into obstructive sleep
apnea syndrome (OSAS), central sleep apnea,
and sleep-related hypoventilation. There are
many factors that inuence breathing and lead to
sleep disordered breathing. Midface hypoplasia
comes along with constrictions of the posterior
nasal aperture or the nasal meatus. For example,
infants with Pierre Robin sequence show glossoptosis that obstructs the oropharynx. In conclusion, all disturbances reduce the patency of the
upper airway and may lead to breathing
difculties.
24.3.1.1 Obstructive Sleep Apnea
Syndrome
Most infants with craniofacial malformations
suffer from a diagnosed obstructive sleep apnea
syndrome (OSAS). The prevalence is 67% [31].
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Fig. 24.5 Barium
swallow. (Reprinted
from top: April stock/
Shutterstock.com,
bottom: whitetherock
photo/Shutterstock.com
with permission)
385
The infants tend to have a partial or temporarily
complete obstruction of the upper airway that
affects the breathing as well as the sleeping pattern [32]. In addition, the OSAS causes many diseases such as cardiovascular and neurological
disorders [32–35]. Obstructive sleep apnea is
characterized by partial or complete interruption
of airow resulting in a temporarily decreasing
pO2 [36]. The extent of the malfunction depends
on the patency of the upper airways, which is
often affected in children with craniofacial malformations [37]. Hypoplasia of the midface,
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V. Kerkfeld
especially micrognathia, and glossoptosis are
well-known risk factors for breathing issues.
Besides, the high incidence of tonsillar and adenoidal encroachment is also a form of restriction
for breathing. However, the etiology and pathogenesis are determined by many factors.
24.3.2 Pathological Diculties
24.3.2.1 Craniosynostosis
Many syndromes are accompanied by craniosynostoses such as Pfeiffer, Apert, Crouzon, Muenke,
Saethre-Chotzen, and Carpenter. The prevalence
of those syndrome-affected infants is 68% [38] to
87% [39]. Etiologically a mutation of the FGFR
gene causes the premature fusion of sutures.
Breathing restrictions tend to be very variable
due to the different manifestations of the
malformations.
OSAS inPatients withCraniosynostosis
As mentioned before, OSAS is often caused by
hypoplasia of the midface. Therefore, patients
mostly breathe by mouth and tend to snore, which
may result in sleep apnea symptoms [40, 41].
In addition, craniosynostoses can provoke an
increased intracranial pressure due to cranial disproportions, pathological venous drain, and an
increased amount of brain liquor (hydrocephalus). However, intracranial pressure is also
affected by an increased pCO
that occurs due to
2
obstructive sleep apnea and its effects on the
blood pressure. In consequence, the cerebral perfusion is different [42–45]. It is recommended to
screen children with syndromes on a regular
basis every year by polysomnography to detect
OSAS [46].
Infants with craniosynostosis and OSAS benet from a nasopharyngeal airway that circumvents the obstruction.
Central Apneas inPatients
withCraniosynostosis
Infants with craniosynostosis also suffer from
central apneas. The pathogenesis is not well
known, but pressure on the respiratory center is
discussed. Etiologically a Chiari malformation
can cause this appearance [
47]; therefore, patients
with Crouzon or Pfeiffer syndrome often occur
with this malformation [48]. However, Chiari
malformations are rarely seen in patients with
Apert syndrome [49]. In the end, breathing issues
lead to developmental disturbances, minor quality of life, and behavior problems [50].
24.3.2.2 Clefts
Many syndromes are accompanied by clefts such
as Down, Pierre Robin, and Treacher Collins.
However, 70% of patients occur with isolated
clefts without other comorbidities [51].
There is a higher incidence in children with
clefts to suffer from OSAS.Pharyngeal airways
are smaller and the craniofacial relation differs to
those of healthy infants. Cleft palates affect the
oropharyngeal muscles that aggravate the speech
and the act of swallowing and inuence the
patency of the airway [52]. Sixty-nine percent of
infants with isolated lip and cleft palates suffer
from sleep apnea [53]. Facial dimensions such as
the length of the mandible and the height of the
face are important aspects for the extent of
obstructive sleep apnea [54].
Surgical treatment aims to improve the velopharyngeal function and to restrict the unwanted
nasal airway [55]. In surgery, the intent is to
reduce the space between the soft palate and the
posterior pharynx [56]. The surgical treatment
itself is able to induce obstructive sleep apnea as
a complication that might result to the use of
CPAP [57].
24.3.2.3 Syndromes withCraniofacial
Anomalies
Many syndromes with craniofacial anomalies
result in breathing issues.
Pierre Robin Sequence
Patients with Pierre Robin sequence occur with
the triad of micrognathia, glossoptosis, and
resulting airway obstruction. Pierre Robin himself declared the drop of the base of the tongue as
a disturbance of the nasopharyngeal airway [58].
The sequence is also often accompanied by cleft
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palates. It is commonly assumed that the micrognathia causes a dislocation of the tongue to an
upper and posterior direction medially between
the two parts of the developing palates during
pregnancy. This irregular development results in
a U-shaped cleft [59].
For a long time, practitioners thought of glossoptosis to be responsible for obstructive sleep
apnea in infants with Pierre Robin sequence.
However, endoscopic procedures of the nasopharynx revealed a multifactorial genesis: some
patients show that the base of the tongue presses
the soft palate against the posterior pharynx,
while some suffer from the lateral pharynges
coming close to each other and others occur with
a combination of both, resulting in a circumferential constriction of the pharynx [60]. In addition, maxillary hypoplasia plays another
important part in the genesis of OSAS [61].
Eighty-ve percent of infants with Pierre Robin
sequence occur with OSAS [62].
Children with Pierre Robin sequence should
be screened every year to detect OSAS.This is
very important due to the high incidence of sleep
apnea in patients with Pierre Robin sequence. In
addition, usual symptoms are often veiled, such
as snoring which is not represented in every case
or inadequate motions of the chest and abdomen
during sleep that might be misunderstood by
parents.
Achondroplasia
Patients with achondroplasia occur with macrocephaly and hypoplasia of the midface [63].
Because of the malformation, 54% of affected
children have OSAS [64].
Down Syndrome
Patients with Down syndrome occur with hypoplasia of the midface and are associated to clefts
and obesity [65]. Therefore, a majority of the
affected children (80%) have OSAS [66].
Treacher Collins Syndrome
Infants suffering from Treacher Collins syndrome often show a minor patency of airways
resulting in breathing issues. Many patients need
to be tracheostomized to ensure a steady airow
to grant a sufcient oxygenation. Surgical interventions on the cleft palates might affect the airways and aggravate the breathing issues.
Infants with Treacher Collins occur with
hypoplasia of the viscerocranium, cleft palates,
malformation of the ears, pharyngeal hypoplasia,
and various other symptoms [67]. Fifty-four percent of children with Treacher Collins syndrome
suffer from obstructive sleep apnea [68]. Studies
supported by endoscopic procedure of the nasopharynx show multifactorial genesis by many
different anatomic variations between the nasal
septum and the trachea. However, most obstructive malformations can be found in the oropharynx. The diversity of obstructions of the upper
airway leads to the recommendation to use an
endoscopic technique for diagnosis [69].
Goldenhar Syndrome
Many children suffering from Goldenhar syndrome are at risk of OSAS.The prevalence lays
between 7% and 67% [70, 71]. Primarily, the
hypoplasia of the mandible causes the breathing
issue similar to the pathogenesis of OSAS in children with Pierre Robin sequence and Treacher
Collins syndrome [70, 72]. The hypoplasia
causes minor patency in the oropharynx and
therefore hinders a physiological airow.
24.3.3 Management
The treatment of breathing issues, especially OSAS,
contains CPAP, tracheostomy (Fig.24.6), and surgical intervention. In many cases, surgical treatment
in even more than just one part of the nasopharyngeal airway becomes necessary. However, it is
important to determine which intervention suits the
infant. Some may benet more from CPAP, while
others prot from tracheotomy [73].
All infants with severe craniofacial malformations should be supervised by overnight polysomnography in order to detect (hidden) OSAS
[74]. Pulsoximetry is a number-two choice due to
its lower sensitivity [75]. To evaluate the individual extent of apnea and hypopnea phases, the
apnea-hypopnea index counts the episodes of
those events per hour.
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Fig. 24.6
Tracheostomy
Fig. 24.7 Continuous
positive airway pressure
(CPAP). (Reprinted
from JPC-PROD/
Shutterstock.com with
permission)
V. Kerkfeld
24.3.4 Treatment
There are many ways to handle OSAS, and the
correct treatment needs to be detected individually for every infant. Many practitioners recommend prone position in non-severe extents of
sleep-related breathing difculties. Besides, there
are other nonsurgical treatment approaches like
nasopharyngeal airway tubes or continuous positive airway pressure (CPAP) (Fig.24.7). In addition, another nonsurgical treatment is the use of
Tübingen palatal plate (TPP) (Fig.24.8) in more
severe cases. TPP is an intraoral orthodontic palatal appliance with a posterior extension that
leads the tongue in an anterior direction. This
ensures patency of the upper airway and enhances
breathing. internal treatments may contain in distinct cases weight-loss and anti-inammatory
drugs, while other infants benet from an orthodontic intervention with removable appliances
for a rapid maxillary expansion. Depending on
the situation, a surgical operation can be helpful
24 Feeding andBreathing Aspects inInfants withCraniofacial Malformations
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formed children. The most common breathing
disorder is the OSAS, while central apneas
should not be disregarded especially in population of infants with craniosynostosis. Besides
facial and nasopharyngeal malformations like
hypoplasia of the midface and glossoptosis, decient upper airways due to oropharyngeal dysfunctions can cause sleep apneas. Infants benet
from an abdominal position, nasopharyngeal airway bypassing the obstruction, surgical procedures, and as well CPAP in order to suffer less
from sleep apneas.In infants with therapy-refractory sleep apneas, tracheotomy might be evaluated as an ultima ratio.
24.4 Case Report
The strong interrelation between breathing and
feeding becomes obvious by a closer inspection
of an infant with a syndromic disease. Therefore,
a case report is presented in the following.
389
Fig. 24.8 Tübingen palatal plate (TPP)
to distract the mandible or to eliminate obstructions in the upper airway. Those patients suffer
from hypoplasia of the mandible. Therefore, a
surgical distraction of the mandible can cure or
attenuate the breathing issues.
24.3.5 Conclusion
Every infant with craniofacial malformation
should be screened for sleep-related breathing
disorders due to the high prevalence in mal-
24.4.1 Initial Situation
An infant with Pfeiffer syndrome appears in a
desolate state (Fig.24.9). Overall, he/she is not
able to swallow adequately and struggles for air.
Because of the feeding and breathing issues, it is
dystrophic and underdeveloped. Besides, the
infant occurs with craniosynostosis and hydrocephalus accompanied by eye proptosis
(Fig. 24.10). However, it lacks the common
appearance of syndactyly that is observed in
other patients affected by Pfeiffer syndrome.
24.4.2 Treatment
24.4.2.1 Breathing Management
First of all, proper breathing needed to be ensured.
Initially, an endoscopic examination (Fig.24.11)
was performed due to detect constriction of the
upper airway. A nasal intubation followed
(Fig. 24.12). However, this procedure was not
able to grant breathing sufciently. Therefore, a
modied Tübingen palatal plate with an endotra-
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