III. Abnormal Swallowing

1
III. Abnormal Swallowing

• Etiology and Conditions Children

2
Neurological Disorders

• Pediatric feeding disorders are common.
• 25 of children are reported to present with some
  form of feeding disorder.
• They can occur in children who are healthy, in
  children who have gastrointestinal disorders, and
  in those with special needs.
• Indeed 80 of developmentally delayed children
  present with some type of feeding problem
  (Manikam Perman, 2000).

3
Neurological Disorders

• Children with developmental disabilities
  frequently develop problems with feeding that can
  lead to malnutrition and respiratory symptoms.
• Developmental delayed children may present with
  problems similar to those eating difficulties in
  normally developing children although the level
  of skills is often lower and the severity of the
  problem greater

4
Neurological Disorders

• Difficulties with food intake (sucking,
  swallowing, chewing)
• Disruptive or messy eating habits
• Eating too quickly or too slowly
• Eating too much or too little
• Having nutritional deficiencies
• Lacking independent feeding skills (using a spoon
  and fork)
• Having bizarre food habits (eating nonfood
  items)
• Refusing certain food types or textures.

5
Neurological Disorders

• In general, feeding disorders can be severe
  enough to contribute to growth failure,
  susceptibility to chronic illness, and even
  death.
• Obesity is more commonly reported in children
  with Down syndrome, myelomeningocele, and spastic
  cerebral palsy.

6
Neurological Disorders

• Being underweight is more frequent in children
  with athetoid cerebral palsy, autism, and
  congenital anomalies.
• However, the prevalence of feeding problems
  appears to vary somewhat according to the type
  and overall severity of the developmental
  disability.

7
Neurological Disorders

• In one study conducted on 377 parents of
  children with neurological impairment aged 4 to
  13 years on the Oxford Register of Early
  Childhood Impairments, the following prevalence
  was found
• 93 of respondent children had cerebral palsy
  47 were unable to walk 78 had speech
  difficulty and 28 drooled saliva continuously.

8
Neurological Disorders

• Gastrointestinal problems were commonly
  encountered 59 were constipated 22 had
  significant problems with vomiting and 31 had
  experienced at least one chest infection in the
  previous 6 months.
• Feeding problems were prevalent 89 needed help
  with feeding and 56 choked with food 20 of
  parents described feeding as stressful and
  unenjoyable.

9
Neurological Disorders

• Prolonged feeding times (3h/day) were reported by
  28.
• Only 8 received caloric supplements and 8 were
  fed via gastrostomy tube.
• (Sullivan, Lambert, Rose, Ford-Adams, Johnson,
  Griffiths, 2001).

10
Neurological Disorders

• In another study on 349 children referred to an
  intensive feeding program, feeding problems were
  classified into five groups and then correlated
  with medical and developmental conditions.
• The five feeding categories were refusal,
  selectivity by texture, selectivity by type, oral
  motor, and dysphagia.

11
Neurological Disorders

• Results showed that 64 of the children had
  developmental disabilities, confirming that
  children with special needs were at higher risk
  of acquiring feeding problems.
• 62 of the children with autism spectrum disorder
  exhibited food selectivity by type (at only white
  foods or a particular brand of French fries).

12
Neurological Disorders

• Constipation was also common in children with
  feeding disorders and may have resulted from
  their poor diet, which reinforced the feeding
  problem.
• Children with Downs syndrome most often showed
  selectivity by texture, preferring pureed/low
  textured foods and refusing to chew.

13
Neurological Disorders

• Children with CP often displayed moderate-severe
  oral motor impairment.
• Those with food refusal and dysphagia had a
  higher incidence of GER.
• Many of the feeding problems demonstrated by the
  children were thought to be the result of learned
  aversions.

14
Neurological Disorders

• Children with more severe medical and
  developmental conditions often had more severe
  feeding problems because of their exposure to
  aversive feeding experiences.
• 21 of the children in the study had aversive
  reactions to foodvomiting, diarrhea, abdominal
  pain, and poor interest in eating.
• (Field, Garland Williams, 2003)

15
Neurological Disorders

• A study by Rommel, De Meyer, Feenstra, and
  Veerman-Wauters (2003) identified the feeding
  etiology in 700 children referred to their clinic
  for assessment of severe feeding difficulty.
• The authors categorized feeding difficulty into
  three groups medical, oral, and behaviorally
  based.

16
Neurological Disorders

• GER was the most frequently identified underlying
  medical condition of patients with feeding
  problems.
• They also found a significant relationship
  between prematurity and the development of
  feeding disorders.
• Infants born before 34 weeks gestation had more
  gastrointestinal and oral sensory problems than
  those born later.

17
Neurological Disorders

• In general, children with feeding disorders had a
  significantly lower birth weight for gestational
  age.
• Behavioral components of feeding disorder
  occurred more frequently in children older than 2
  years of age.
• Children with GI problems had a variety of oral
  deficits.

18
Neurological Disorders

• Children with heart disease exhibited abnormal
  tactile responses.
• Hypersensitivity may be caused by general sensory
  overload, traumatic oral and facial experiences,
  or oral sensorimotor deprivation.

19
Neurological Disorders

• Tube feeding in children with oncologic and heart
  disease appeared to be temporary as opposed to
  children with neurologic disorders who often need
  permanent feeding with a g-tube.
• Children with oral motor based feeding problems
  more often needed g-tube feedings because of the
  severe oral motor dysfunction related to their
  neurological impairment.

20
Neurological Disorders

• Cerebral palsy, muscular dystrophy,
  myelodysplasia, or damage caused by TBI may be
  manifested in problems of oral-motor function,
  including
• Inefficient suck
• Persistence of primitive reflexes (e.g., rooting,
  suck-swallow)
• Presence of atypical reflexes (e.g., tonic bite)
  
• Reduced ROM of the jaw, tongue, and lips (e.g.,
  drooling)

21
Neurological Disorders

• Tongue thrust
• Swallowing disorders
• Gagging or
• Vomiting.
• Regardless of etiology, children with severe to
  profound mental retardation typically have
  neuromotor coordination impairments that
  interfered with the acquisition of oral-motor
  feeding skills.

22
Neurological Disorders

• These impairments are related to the persistence
  of primitive oral reflexes that interfere with
  mature feeding patterns.
• Additionally, these children may have impairments
  in neuromotor structures and integration,
  difficulties with imitative and receptive
  language abilities involved in learning, or other
  concomitant factors.

23
Cerebral Palsy

• Children with cerebral palsy are a heterogeneous
  group, but, in general, they show three major
  abnormalities, including
• Poor lingual function
• Delayed swallow production and
• Poor pharyngeal peristalsis.
• Other common problems include tongue thrust,
  prolonged and exaggerated bite reflex, abnormally
  strong gag reflex, tactile hypersensitivity in
  the oral area, and drooling.

24
Cerebral Palsy

• Children with CP or other neuromuscular
  difficulties often appear unable to chew.
• Instead of chewing, these children make tongue
  thrusting motions that push the food back out of
  the mouth.
• They sometimes hold the tongue tightly to the
  hard palate, resulting in the food getting
  squashed but not chewed.

25
Cerebral Palsy

• When the food gets to the back of the oral
  cavity, this ineffective bolus may cause gagging
  or choking.
• When the oral phase is characterized by
  incoordination and delay, the childs potential
  for aspiration and choking is greater with thin
  liquids and strained or pureed foods, than it is
  with thickened, semi-solid foods and thickened
  liquids, such as fruit nectars.

26
Cerebral Palsy

• In contrast to oral phase incoordination,
  children with reduced pharyngeal peristalsis and
  persistent residue after a swallow are more
  likely to aspirate on paste-like food.
• These foods are stickier and are harder to clear
  from the pharynx with subsequent swallows.

27
Cerebral Palsy

• Children may also experience considerable
  irritation and discomfort, leading to food
  refusal and behavior problems related to feeding.
  
• The feeding problems and adaptations of a
  school-aged child with athetosis or
  choreoathetosis are similar to adults who have
  these disorders.

28
Cerebral Palsy

• In school-age and pubertal children with spastic
  forms of CP, dysphagia may increase with
  transitional feeding stages and/or mealtime
  demands.
• Hypertonus of the cervical neck and lower
  jaw/tongue muscles may handicap oral and
  pharyngeal feeding actions by a secondary
  malposition of the oral and pharyngeal
  structures.

29
Head Injury

• Children with head injury appear to have similar
  physiologic disorders of feeding and swallowing
  as adults with HI.
• The most common problems include reduced tongue
  control and bolus manipulation, delayed
  production of the pharyngeal swallow, and
  inefficient transport of through through the
  mouth and pharynx.

30
Head Injury

• Gastroesophageal reflux (GER) is also common in
  children with HI or other neurologic impairment
  following placement of a gastrostomy tube
  (G-tube).
• In a recent study by Morgan, Ward, Murdoch, and
  Bilbie (2002), of moderate-severe children
  post-CHI, who ranged in age from 11 months
  through 15 years, common oral preparatory phase
  problems included

31
Head Injury

• Impaired bolus formation
• Tongue extension-retraction
• Piecemeal deglutition
• Impaired mastication
• Anterior spillage
• Use of a primitive suckle pattern and
• Hesitancy of tongue movement.

32
Head Injury

• Oral phase problems were characterized by
• Repetitive tongue pumping
• Impaired oral transit
• Excessive oral residue after swallow
• Premature spillage into the valleculae
• Multiple swallows to clear oral cavity
• Delayed swallow initiation
• Hesitancy of tongue movement
• Premature spillage into pyriform sinuses and
• Poor lingual-palatal contact.

33
Head Injury

• Pharyngeal phase problems were less frequent, but
  included
• Vallecular and pyriform sinus residue
• Epiglottic undercoating leading to laryngeal
  penetration and
• Delayed pharyngeal transit times, especially with
  pureed consistencies.
• Delays in swallow response increase risk of
  aspiration.

34
Duchennes Muscular Dystrophy

• Duchennes muscular dystrophy is the most common
  childhood form of muscular dystrophy.
• Usual age of onset is 2 to 6 years.
• Because it is x-linked, only males are affected.
• Virtually all Duchennes patients have severe
  dysphagia by 12 years of age.
• Episodes of aspiration pneumonia are common by
  age 18 years.

35
Duchennes Muscular Dystrophy

• Deficits of the oral preparatory and oral phases
  include weakness of the lip and cheek muscles,
  weakness of the masticatory muscles, and weakness
  of the tongue elevator muscles.
• Pharyngeal impairment is associated with weakness
  of the superior constrictor muscles and delayed
  swallow initiation, because of impaired elevation
  and retraction of the tongue.

36
Duchennes Muscular Dystrophy

• Aspiration of food and saliva, weight loss, and
  pulmonary complications occur as dysphagia
  progresses.
• Acute cardiorespiratory failure is a significant
  clinical problem and is often the cause of death
  before the age of 20 years.

37
Rett Syndrome

• Rett syndrome is a progressive neurological
  disorder that affects primarily female children.
• These children appear to be developmentally
  normal until 6 to 18 months of age, when they
  begin to lose both motor and cognitive skills.
• They develop inappropriate social interactions,
  decelerated head growth, and severe language
  deficits.

38
Rett Syndrome

• Abnormal chewing associated with tongue thrusting
  and involuntary, undulating tongue movements are
  common.
• In later stages of the disease, apraxia, motor
  problems, and seizures are prominent.
• The last stage of the disease is characterized by
  reduced mobility.

39
Rett Syndrome

• Muscle weakness, rigidity (stiffness),
  spasticity, dystonia (increased muscle tone with
  abnormal posturing of extremity or trunk), and
  scoliosis (curvature of the spine) are prominent
  features.
• Feeding abilities will be affected by these
  changes, but nutrition can generally be
  maintained by infant-patterned suckle feeding
  with pureed or soft foods.

40
Familial Dysautonomia/Riley-Day Syndrome

• Familial dysautonomia is a form of progressive
  neuropathy that affects the offspring of Eastern
  European jews, the Ashkenazi.
• Familial dysautonomia affects the autonomic
  nervous system, the part of the nervous system
  that controls the auto-pilot functions of
  breathing, swallowing, heart rate changes, blood
  pressure, and temperature regulation.

41
Riley-Day Syndrome

• It also affects the sensory nervous system, the
  part of the nervous system that you use to feel
  pain and temperature.
• The most distinctive clinical feature is absence
  of overflow tears with emotional crying although
  it can be normal for a child not to have tearing
  until 7 months of age.

42
Riley-Day Syndrome

• It is identified by the absence of fungiform
  papillae on the tongue.

43
Riley-Day Syndrome

• Other signs of the disorder can be present from
  birth such as a high prevalence of breech
  presentation, weak or absent suck and poor tone.
  Difficulty feeding is observed in 60 of infants
  in the neonatal period.
• Poor suck and misdirected swallows often persist
  and put the patient at risk for aspiration
  pneumonia.

44
Riley-Day Syndrome

• If gastroesophageal reflux is present, the risk
  for aspiration increases.
• Later in infancy, and during transitional and
  mature feeding, feeding impairment may result
  from deficiency of saliva and other secretions.
• Feeding is also affected by small jaw size with
  overcrowding of teeth, that combined with poor
  salivary secretion, may lead to early tooth loss.
  

45
Riley-Day Syndrome

• Gastrointestinally, these children experience
  uncoordinated suck and swallow, episodes of
  cyclic vomiting, reflux, esophageal achalasia,
  and motility problems (constipation, dumping
  syndrome).

46
Structural Disorders

• The simplest impairments, in terms of detection
  and clinical management, are those of the tongue
  and palate.
• Infants who have hypoplasia of the tongue may
  achieve adequate suckle feeding by compensatory
  actions of the constrictor wall, pharyngeal
  palate, palatine folds, and hyoid suspensory
  muscles.

47
Structural Disorders

• The mandible and palate, as well as other
  portions of the midfacial skeleton are adapted in
  shape to form functionally appropriate chambers
  about the hypoplastic tongue.
• Similarly, the infant with cleft palate, but who
  has normal feeding incentive and normal sensory
  inputs and central representations of suckle and
  swallow, may demonstrate remarkable motor
  compensations for this anatomic defect.

48
Structural Disorders

• In contrast, the infant who has palate
  hypoplasia, evidenced by cleft of the uvula or by
  a palate that is visibly short or lax under
  fingertip palpation, may have great problems of
  pharyngeal dysphagia, including nasal
  regurgitation.

49
Structural Disorders

• Most children who have structural problems of the
  oral and pharyngeal areas are expected to
  continue to develop as long as there is adequate
  neurologic mechanisms for postural, respiratory,
  and feeding functions.
• Anatomic adaptations of the facial skeleton will
  also continue during childhood and pubertal
  growth.

50
Structural Disorders

• Typical manifestations of abnormal esophageal
  function in the child are eating difficulties,
  pain, or regurgitation.
• Since the esophagus is composed of both striated
  and smooth muscle, abnormalities of esophageal
  motility can involve either muscle type or both.
• Disorders of esophageal function can be either
  primary or secondary to systemic disease.

51
Structural Disorders

• The symptoms and signs that most commonly suggest
  a disorder of swallowing or esophageal function
  in children are dysphagia (including food
  refusal), abnormally slow eating, persistent
  drooling, posturing during swallow, chest pain or
  odynophagia, recurrent aspiration, and recurrent
  food impaction.

52
Structural Disorders

• Achalasia (incompetently relaxing or non-relaxing
  LES) and chronic intestinal pseudo-obstruction
  are the most common primary esophageal motility
  disorders in children, but both occur
  infrequently.
• Childhood achalasia appears to be more common in
  boys, and familial cases are rare.

53
Structural Disorders

• One study indicates that regurgitation of food
  and dysphagia are the most frequent symptoms in
  affected children.
• Eighteen percent of patients experienced onset of
  symptoms during infancy, but only 6 of the
  patients were identified as having achalasia
  during infancy.

54
Structural Disorders

• Pediatric gastroesophageal reflux (GER) and
  laryngopharyngeal reflux (LPR) have gained better
  recognition over the past few years.
• GER and LPR usually present as regurgitation,
  emesis, epigastric pain, failure to thrive,
  esophagitis, or stricture.
• Many children experience respiratory disorders
  associated with reflux.

55
Structural Disorders

• Gastroesophageal reflux disease (GERD) is a
  common problem in children that is sometimes
  associated with dysphagia.
• Choking, food refusal, and food "getting stuck"
  are nonspecific symptoms that may arise
  consequent to reflux and esophagitis.
• Esophageal motility may also be abnormal
  secondary to reflux esophagitis and
  tracheo-esophageal fistula.