The instinctual drive to gain nourishment can become complicated by structural differences, physiologic instability and environmental influences. Infants with craniofacial anomalies may experience significant feeding and swallowing difficulties related to the type and severity of the anomalies present as well as social-emotional interactions with caregivers. Typical outcome measures and feeding goals are discussed. Details regarding clinical and instrumental evaluation, including fiberoptic endoscopic evaluation of swallowing and modified barium swallow study, as well as management techniques are reported.
Key points
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Feeding and swallowing abilities in infants born with craniofacial anomalies show great variability.
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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.
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Feeding difficulties with cleft lip and/or palate can be more complicated in the presence of an associated syndrome or sequence.
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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 regurgitation, and attainment of physiologic stability accomplished through various management strategies.
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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 swallow function and to devise strategies to promote safe feeding and swallowing.
Video content accompanies this article at http://www.facialplastic.theclinics.com .
Introduction
Feeding abilities in all children gain prompt attention and demand vigilance from parents immediately after birth. Whether receiving a prenatal diagnosis of a craniofacial anomaly by fetal ultrasound or receiving the diagnosis at birth, the anxiety and worry can be overwhelming. The sooner that caregivers are educated regarding feeding management, the sooner they feel empowered to adequately feed their infant. Referrals to cleft and craniofacial teams for prenatal consultations after identification of a cleft on fetal ultrasound have been found to be both informative and anxiety-reducing to the parents. Specifically, Davalbhatka and Hall (2000), reported that antenatal counseling prepared parents for the birth of an infant with a cleft in 85% of respondents; 89% of parents felt they benefitted from knowing the diagnosis ahead of time. Although feeding and swallowing issues persist beyond infancy, this section focuses on typical development of infant feeding and swallowing skills, feeding difficulties and their causes, evaluation, and management.
Typical Feeding and Swallowing Development
In a healthy, typically developing newborn, feeding is reflexive. The rooting reflex encourages the newborn to find the nipple and the suck reflex to pull milk from the nipple. Milk is extracted from the nipple by both positive and negative pressure, otherwise known as compression and suction. Milk extraction results from the coordinated movements of the following oral structures.
Jaw
The supportive structure of the jaw moves in a vertical dimension. Its inferior movement assists with creating suction.
Lips
The lips assist with the anterior seal around the nipple and support stabilization of the nipple within the oral cavity.
Tongue
The tongue tip compresses the nipple. The posterior aspect of the tongue seals the oral cavity against the soft palate. When the tongue drops, it enlarges the oral cavity creating suction. The tongue also forms a midline groove for the transfer of the liquid bolus from the mouth to the oropharynx.
Cheeks
The cheeks provide stability. The greater the fat pads, the greater the stability.
Hard palate
The hard palate assists the tongue with nipple compression and stability.
Soft palate
The soft palate assists the tongue in closing off the oral cavity posteriorly. During the swallow, it rises to seal off the nasal cavity, prevent nasal regurgitation and create suction.
Sucking
These oral movements coordinate with respiration and swallowing to produce a suck-swallow-breathe sequence ideally in a 1:1 suck-swallow ratio. Suckling is the first phase of the suck to emerge. The tongue moves in an anterior-to-posterior dimension and, according to Arvedson and Brodsky (2002), “liquid is drawn into the mouth through a rhythmic licking action of the tongue, combined with pronounced opening and closing of the jaw. Lips are loosely approximated.” This pattern changes by the sixth month of life with onset of differentiation between the jaw, tongue, and lips, including increased lip closure and more vertical excursion of the tongue.
Swallowing
Swallowing, or deglutition, is not a synonymous term to pair with feeding. Swallowing is a complex process of both volitional and reflexive behaviors involving the action of coordinated, sequential motor movements from the mouth to the esophagus. Hence, there are 4 stages to swallowing that are generally accepted: the oral preparatory phase, the oral phase, the pharyngeal phase, and the esophageal phase. The oral preparatory phase involves the suckling or sucking, biting, and chewing actions that bring the food into the mouth and form the bolus. The oral phase consists of bolus transit to the oropharynx by the tongue. The pharyngeal phase involves the closure of the nasopharyngeal port, anterior and vertical excursion of the larynx, inverse movement of the epiglottis, vocal fold closure at midline and relaxation of the upper esophageal sphincter (UES), and inverse movement of the epiglottis with coverage of but not a tight seal for the laryngeal vestibule. The pharyngeal muscle constriction triggers pharyngeal peristalsis. As the bolus moves from the hypopharynx through the open UES, the esophageal phase begins. The involuntary peristaltic action of the esophagus propels the bolus through the relaxed lower esophageal sphincter to allow the bolus to enter the stomach.
Progression
Reaching oral sensorimotor milestones allows the typically developing infant to progress to solid foods. According to Arvedson and Brodsky (2002), spoon-feeding skills emerge between 4 to 6 months of age. At about 6 months, munching with vertical jaw movements emerges. Infants become ready for thicker textures. Rotary jaw action begins at about 7 months and is refined by 12 months of age. New textures should be gradually introduced in order for the child to gain competence in chewing ability and to prevent choking.
Feeding Difficulties: the Mechanics
Parents of infants with clefts often make the following observations regarding limited feeding ability: “My baby isn’t sucking well. My baby doesn’t want to feed. He pushes the nipple out of his mouth. My baby is lazy. My baby isn’t swallowing correctly. It comes out of his nose. My baby is always hungry. My baby acts hungry but then falls asleep as soon as he starts feeding.” These symptoms are often influenced by the mechanical issues that emerge due to the cleft lip and/or palate. In simple terms, the lip latches to the nipple and the palate maintains the latch. Given lip and palatal deficiencies, the latch and its maintenance are adversely affected. The type and severity of the cleft directly influence the intensity of the modifications needed for feeding success. Clefts are classified as unilateral or bilateral and complete versus incomplete. Peterson-Falzone and colleagues (2001) remind us that “clefts vary in three other dimensions: anterior to posterior, width and ‘depth.’” These dimensions are important when deciding on the most efficacious nipple placement within the mouth. Infants with cleft lip and palate innately exhibit a sucking reflex similar to typically developing infants; however, they have limited ability to create suction efficiently and consistently.
Cleft lip
Cleft lip causes a limited labial seal around the nipple of the breast or bottle that may decrease negative pressure to a minor degree and contribute to limited stability of the nipple within the oral cavity. These issues are usually very mild with cleft lip only. The nipple of the breast or bottle will often fill the space of the cleft to create a seal and stability of the nipple. Nutritive breastfeeding is typically preserved in this population.
Cleft lip and alveolus
With significant involvement of the alveolus, it becomes more difficult for the infant to anchor the nipple between supporting structures to achieve a good latch and seal of the oral cavity (ie, lips, tongue, primary palate) and may limit negative pressure generation. The infant has less ability to compress the nipple in a vertical plane (ie, tongue and primary palate). Infants often show a greater extent of vertical jaw excursion to improve compression contributing to fatigue. Nutritive breastfeeding is typically achieved given assistance with milk delivery.
Cleft palate
With compromise of the hard palate comes decreased stability of the nipple within the oral cavity and decreased compression of the nipple. These infants are more reliant on suction for milk extraction. With compromise of the soft palate comes decreased intraoral pressure, decreased suction, and limited milk transfer. This results in decreased sucking efficiency and lengthy feeding times with limited intake. Excessive air intake occurs due to the poor seal between the oral and nasal cavities. As the tongue drops in preparation for the swallow, air is pulled into the oropharynx and hypopharynx from the nasal cavity. This can lead to aerophagia, a false sense of satiation, excessive spit up, and emesis. This also provides a conduit for milk to propel from the mouth into the nasopharyngeal region, otherwise known as nasal regurgitation, before or during the swallow. This leads to nasal congestion and trouble breathing while feeding given that infants are obligate nasal breathers. With a small cleft of the soft palate, the tongue often occludes the cleft for at least part of the transition from suck to swallow, thereby reducing effect on negative pressure generation. With a submucous cleft palate, there may be mild reduction in negative pressure due to the dysfunction of the velopharyngeal valve. Nutritive breastfeeding is most likely possible with a small cleft of the soft palate or submucous cleft palate; however, nutritive breastfeeding is rarely successful in an infant with a large cleft palate.
Cleft lip and palate
The combination of cleft lip and palate will result in the aforementioned feeding issues but likely to a greater degree. Nutritive breastfeeding is rarely successful in an infant with complete cleft lip and palate.
Poor longitudinal feeding outcomes may include general fatigue with limited alertness and interaction, refusal behaviors, limited urinary and fecal output, and limited weight gain. These unfortunate circumstances may negatively affect infant and caregiver interaction over time. Masarei and colleagues (2007) compared feeding skills in infants with unrepaired clefts to healthy infants without clefts. All comparisons were statistically significant in demonstrating the decreased sucking efficiency in infants with unrepaired isolated cleft palate and unilateral cleft lip and palate. The infants with unrepaired clefts demonstrated shorter lengths of individual sucks and shorter lengths of sucking bursts (number of suck-swallow sequences) while rate of sucking, suck-swallow ratio, and positive pressure generation were greater ( Table 1 ).
| Infants with Unilateral Cleft Lip and Palate or Isolated Cleft Palate | Infants without Clefts | |
|---|---|---|
| Length of sucking bursts | 8.97 s | 13.28 s |
| Rate of sucking | 109.26 sucks/min | 75.07 sucks/min |
| Length of individual sucks | 0.57 s | 0.87 s |
| Positive pressure generation | 71.68% | 25.71% |
| Suck-swallow ratio | 2.97:1 | 1.20:1 |
Reid and colleagues (2007) examined the suction capabilities of 40 2-week-old infants with clefts. All 8 infants with cleft lip demonstrated suction. One out of 10 infants with cleft lip and palate demonstrated suction. Thirteen out of 22 infants with cleft palate demonstrated suction. Of the 13 infants, 10 had clefts of the soft palate only and only 3 maintained regular pressure changes over time. The investigators found significant between-group differences, concluding that infants with cleft lip or minor clefts of the soft palate were more likely to generate normal levels of suction and compression than infants with larger clefts.
Special Populations
Pierre Robin sequence
Sucking in infants with Pierre Robin sequence is negatively affected by the limitations in positive pressure generation given the maxillary-mandibular discrepancy of the micrognathia, the misaligned forces of compression by the dental arches, and the lack of contact to the superior surface of the nipple secondary to the typically wide U-shaped cleft palate. The tongue often sits retracted in the mouth (glossoptosis) with minimal ability to produce an anterior tongue carriage to compress the nipple effectively. If the infant is able to produce an anterior tongue carriage to secure the nipple, airway patency typically improves. Suction ability depends on the size of the cleft palate. There is variability in the phenotypic expression of Pierre Robin sequence and there may be absence of a cleft palate in some cases. With less pharyngeal space for airway patency and swallowing mechanics due to micrognathia and glossoptosis, these infants may experience significant chest retractions, nasal flaring, snorting, stertor, and stridor at baseline and while feeding. These infants tend to pause more frequently and tolerate only short sucking bursts. Longer sucking bursts often tax efforts at coordination and lead to extended swallow apneas, gasping, gagging, and possibly aspiration. Furthermore, airway issues may also mask underlying neuromotor dysfunction.
Hemifacial microsomia
This broad group of first and second branchial arch malformations results in mandibular hypoplasia and facial weakness of varying degrees. According to Peterson-Falzone and colleagues (2001), both sides of the face may be involved but typically one side is more affected than the other. Feeding and swallowing difficulties may result from structural anomalies of the jaw, tongue, face, and pharynx, or from neurologic dysfunction and/or congenital heart defects.
22q deletion syndrome
These infants may experience feeding and swallowing problems of multiple causes given the great variability in phenotypic expression. Airway anomalies and cardiac defects can negatively affect the suck-swallow-breathe cycle, induce significant fatigue, and lead to aspiration from an antegrade process. Cardiac defects can cause extrinsic compression of the trachea or of the esophagus, and obstruct the passing of food and liquid. Gastrointestinal tract dysfunction can occur due to hypotonia with reflux, slowed motility, and structural anomalies. These issues can lead to limited caloric intake, feeding refusal, poor weight gain, and failure to thrive. It is important to keep in mind the linear growth trajectory predicted for these infants and the tendency to exhibit slower growth than their same-age peers in early childhood.
Treacher Collins syndrome
These infants often experience poor airway patency and respiratory difficulties. Tracheotomies are frequently required. Surgical repair of a cleft palate may lead to greater risk of airway compromise. Conservative oral feeding measures may be needed with supplemental or alternate enteral feedings to meet nutritional needs but, certainly, variability is present.
Introduction
Feeding abilities in all children gain prompt attention and demand vigilance from parents immediately after birth. Whether receiving a prenatal diagnosis of a craniofacial anomaly by fetal ultrasound or receiving the diagnosis at birth, the anxiety and worry can be overwhelming. The sooner that caregivers are educated regarding feeding management, the sooner they feel empowered to adequately feed their infant. Referrals to cleft and craniofacial teams for prenatal consultations after identification of a cleft on fetal ultrasound have been found to be both informative and anxiety-reducing to the parents. Specifically, Davalbhatka and Hall (2000), reported that antenatal counseling prepared parents for the birth of an infant with a cleft in 85% of respondents; 89% of parents felt they benefitted from knowing the diagnosis ahead of time. Although feeding and swallowing issues persist beyond infancy, this section focuses on typical development of infant feeding and swallowing skills, feeding difficulties and their causes, evaluation, and management.
Typical Feeding and Swallowing Development
In a healthy, typically developing newborn, feeding is reflexive. The rooting reflex encourages the newborn to find the nipple and the suck reflex to pull milk from the nipple. Milk is extracted from the nipple by both positive and negative pressure, otherwise known as compression and suction. Milk extraction results from the coordinated movements of the following oral structures.
Jaw
The supportive structure of the jaw moves in a vertical dimension. Its inferior movement assists with creating suction.
Lips
The lips assist with the anterior seal around the nipple and support stabilization of the nipple within the oral cavity.
Tongue
The tongue tip compresses the nipple. The posterior aspect of the tongue seals the oral cavity against the soft palate. When the tongue drops, it enlarges the oral cavity creating suction. The tongue also forms a midline groove for the transfer of the liquid bolus from the mouth to the oropharynx.
Cheeks
The cheeks provide stability. The greater the fat pads, the greater the stability.
Hard palate
The hard palate assists the tongue with nipple compression and stability.
Soft palate
The soft palate assists the tongue in closing off the oral cavity posteriorly. During the swallow, it rises to seal off the nasal cavity, prevent nasal regurgitation and create suction.
Sucking
These oral movements coordinate with respiration and swallowing to produce a suck-swallow-breathe sequence ideally in a 1:1 suck-swallow ratio. Suckling is the first phase of the suck to emerge. The tongue moves in an anterior-to-posterior dimension and, according to Arvedson and Brodsky (2002), “liquid is drawn into the mouth through a rhythmic licking action of the tongue, combined with pronounced opening and closing of the jaw. Lips are loosely approximated.” This pattern changes by the sixth month of life with onset of differentiation between the jaw, tongue, and lips, including increased lip closure and more vertical excursion of the tongue.
Swallowing
Swallowing, or deglutition, is not a synonymous term to pair with feeding. Swallowing is a complex process of both volitional and reflexive behaviors involving the action of coordinated, sequential motor movements from the mouth to the esophagus. Hence, there are 4 stages to swallowing that are generally accepted: the oral preparatory phase, the oral phase, the pharyngeal phase, and the esophageal phase. The oral preparatory phase involves the suckling or sucking, biting, and chewing actions that bring the food into the mouth and form the bolus. The oral phase consists of bolus transit to the oropharynx by the tongue. The pharyngeal phase involves the closure of the nasopharyngeal port, anterior and vertical excursion of the larynx, inverse movement of the epiglottis, vocal fold closure at midline and relaxation of the upper esophageal sphincter (UES), and inverse movement of the epiglottis with coverage of but not a tight seal for the laryngeal vestibule. The pharyngeal muscle constriction triggers pharyngeal peristalsis. As the bolus moves from the hypopharynx through the open UES, the esophageal phase begins. The involuntary peristaltic action of the esophagus propels the bolus through the relaxed lower esophageal sphincter to allow the bolus to enter the stomach.
Progression
Reaching oral sensorimotor milestones allows the typically developing infant to progress to solid foods. According to Arvedson and Brodsky (2002), spoon-feeding skills emerge between 4 to 6 months of age. At about 6 months, munching with vertical jaw movements emerges. Infants become ready for thicker textures. Rotary jaw action begins at about 7 months and is refined by 12 months of age. New textures should be gradually introduced in order for the child to gain competence in chewing ability and to prevent choking.
Feeding Difficulties: the Mechanics
Parents of infants with clefts often make the following observations regarding limited feeding ability: “My baby isn’t sucking well. My baby doesn’t want to feed. He pushes the nipple out of his mouth. My baby is lazy. My baby isn’t swallowing correctly. It comes out of his nose. My baby is always hungry. My baby acts hungry but then falls asleep as soon as he starts feeding.” These symptoms are often influenced by the mechanical issues that emerge due to the cleft lip and/or palate. In simple terms, the lip latches to the nipple and the palate maintains the latch. Given lip and palatal deficiencies, the latch and its maintenance are adversely affected. The type and severity of the cleft directly influence the intensity of the modifications needed for feeding success. Clefts are classified as unilateral or bilateral and complete versus incomplete. Peterson-Falzone and colleagues (2001) remind us that “clefts vary in three other dimensions: anterior to posterior, width and ‘depth.’” These dimensions are important when deciding on the most efficacious nipple placement within the mouth. Infants with cleft lip and palate innately exhibit a sucking reflex similar to typically developing infants; however, they have limited ability to create suction efficiently and consistently.
Cleft lip
Cleft lip causes a limited labial seal around the nipple of the breast or bottle that may decrease negative pressure to a minor degree and contribute to limited stability of the nipple within the oral cavity. These issues are usually very mild with cleft lip only. The nipple of the breast or bottle will often fill the space of the cleft to create a seal and stability of the nipple. Nutritive breastfeeding is typically preserved in this population.
Cleft lip and alveolus
With significant involvement of the alveolus, it becomes more difficult for the infant to anchor the nipple between supporting structures to achieve a good latch and seal of the oral cavity (ie, lips, tongue, primary palate) and may limit negative pressure generation. The infant has less ability to compress the nipple in a vertical plane (ie, tongue and primary palate). Infants often show a greater extent of vertical jaw excursion to improve compression contributing to fatigue. Nutritive breastfeeding is typically achieved given assistance with milk delivery.
Cleft palate
With compromise of the hard palate comes decreased stability of the nipple within the oral cavity and decreased compression of the nipple. These infants are more reliant on suction for milk extraction. With compromise of the soft palate comes decreased intraoral pressure, decreased suction, and limited milk transfer. This results in decreased sucking efficiency and lengthy feeding times with limited intake. Excessive air intake occurs due to the poor seal between the oral and nasal cavities. As the tongue drops in preparation for the swallow, air is pulled into the oropharynx and hypopharynx from the nasal cavity. This can lead to aerophagia, a false sense of satiation, excessive spit up, and emesis. This also provides a conduit for milk to propel from the mouth into the nasopharyngeal region, otherwise known as nasal regurgitation, before or during the swallow. This leads to nasal congestion and trouble breathing while feeding given that infants are obligate nasal breathers. With a small cleft of the soft palate, the tongue often occludes the cleft for at least part of the transition from suck to swallow, thereby reducing effect on negative pressure generation. With a submucous cleft palate, there may be mild reduction in negative pressure due to the dysfunction of the velopharyngeal valve. Nutritive breastfeeding is most likely possible with a small cleft of the soft palate or submucous cleft palate; however, nutritive breastfeeding is rarely successful in an infant with a large cleft palate.
Cleft lip and palate
The combination of cleft lip and palate will result in the aforementioned feeding issues but likely to a greater degree. Nutritive breastfeeding is rarely successful in an infant with complete cleft lip and palate.
Poor longitudinal feeding outcomes may include general fatigue with limited alertness and interaction, refusal behaviors, limited urinary and fecal output, and limited weight gain. These unfortunate circumstances may negatively affect infant and caregiver interaction over time. Masarei and colleagues (2007) compared feeding skills in infants with unrepaired clefts to healthy infants without clefts. All comparisons were statistically significant in demonstrating the decreased sucking efficiency in infants with unrepaired isolated cleft palate and unilateral cleft lip and palate. The infants with unrepaired clefts demonstrated shorter lengths of individual sucks and shorter lengths of sucking bursts (number of suck-swallow sequences) while rate of sucking, suck-swallow ratio, and positive pressure generation were greater ( Table 1 ).
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