Sucking and Swallowing Difficulty

Suckingand swallowing involve a complex set of actions involving the lips,mouth, pharynx, larynx, esophagus, and neuromuscular system. Inmany cases, the same underlying pathology causes difficulty in bothsucking and swallowing.

Choking while feeding, difficulty inswallowing, and pain during swallowing are common manifestationsof sucking and swallowing disorders.

Principal Causes of Sucking and Swallowing Difficulty

1. Disordersof oropharynx
   1. Anatomic
      1. Cleftlip or palate
      2. Pierre Robin syndrome
      3. Lingual ankyloglossia
      4. Macroglossia
      5. Ankylosis of temporomandibular joint
   2. Infection
      1. Pharyngitis
      2. Tonsillitis
      3. Abscess
   3. Trauma
   4. Foreign body
   5. Neoplasm
2. Disorders of supraglottis and larynx
   1. Anatomic
      1. Laryngomalacia
      2. Cleft larynx
   2. Infection
      1. Supraglottitis
3. Disorders of esophagus
   1. Anatomic
      1. Esophagealatresia
      2. Tracheoesophageal fistula without esophagealatresia
      3. Esophageal stenosis, web, duplication,and stricture
      4. Vascular anomalies
      5. Mediastinal masses
   2. Infection/inflammation
      1. Gastroesophagealreflux disease
      2. Idiopathic eosinophilic esophagitis
      3. Candidiasis
      4. Herpes simplex virus
      5. Cytomegalovirus
      6. Chagas disease
   3. Foreign body
   4. Motility disorders
      1. Cricopharyngealdysfunction
      2. Achalasia
      3. Scleroderma
   5. Neoplasm
4. Disorders affecting suck-swallow-breathingcoordination
   1. Anycause of respiratory distress
5. Disorders affecting neuromuscular coordinationof sucking and swallowing
   1. Brain disorders
      1. Delayed maturation
      2. Mental retardation
      3. Cerebral palsy
      4. Other
   2. Cranial nerve disorders
      1. Infection
         1. Tetanus
         2. Rabies
         3. Poliomyelitis
      2. Moebius syndrome
      3. Isolated palatal paralysis
   3. Anterior horn cell disorders
      1. Spinalmuscle atrophy
      2. Poliomyelitis
   4. Peripheral nerve disorders
      1. Guillain-Barré syndrome
   5. Neuromuscular junction disorders
      1. Myastheniagravis
      2. Botulism
   6. Muscle disorders
      1. Congenitalmuscular dystrophy
      2. Myotonic dystrophy
      3. Dermatomyositis
   7. Autonomic nervous system disorders
      1. Familialdysautonomia (Riley-Day syndrome)
6. Postsurgery complications
7. Psychologic disturbance

Clinical Features and Diagnosis

Disorders of Oropharynx

Anatomic

Cleft Lip or Palate

Cleft lipis due to failure of fusion of embryonic structures surroundingoral cavity, whereas cleft palate is due to failure of fusion ofsecondary palatine plates.

Diagnosis of cleft lip or palate ismade on physical exam.

May occur alone or together.

Pierre Robin Syndrome

Feeding problems are common with Pierre Robinsyndrome, which consists of cleft palate, micrognathia, and glossoptosis.

Lingual Ankyloglossia

Short, tight lingual frenulum between tongueand floor of mouth can prevent normal sucking by limiting excursionof tongue, which helps move bolus along.

Macroglossia

May causeobstruction of oral pharynx and as a consequence, sucking and swallowing problems.

Some causes include congenital hypothyroidism,glycogen storage disease type II, Hurler syndrome, Beckwith-Wiedemannsyndrome, tongue infections, tumors, and idiopathic.

Ankylosis of Temporomandibular Joint

Prevents proper opening of jaw. Pain on jointmovement is common. Plain radiography is diagnostic.

Infection

Oral thrush(Candida infection) is common cause of painful, sore mouth in infancy. Whiteplaques are seen on oral mucosa and tongue. Difficulty in scrapingthese plaques from oral mucosa with tongue blade or occurrence ofbleeding with scraping provides clue to diagnosis, which is usually clinical.

Pharyngitis, tonsillitis, and abscessesinvolving oropharynx and hypopharynx usually cause painful swallowing(see Chap. 61, Sore Throat).

Trauma

Any traumato mouth or pharynx may cause pain and difficulty swallowing. Burnof oral cavity or corrosive ingestion also can produce painful swallowing.

History and physical exam are diagnostic.

Especially with corrosive ingestion,esophagoscopy should be performed to determine if esophagus is involved.

Foreign Body

Foreign body in oropharynx (e.g., fish boneor chicken bone) may cause painful swallowing until it is removed.

Neoplasm

Benign tumorsof oropharynx include hemangioma and lymphangioma.

Most common malignant tumor of oralcavity is rhabdomyosarcoma.

Salivary gland tumors also may occurin oral cavity.

Besides history and physical exam,plain radiography of the pharynx, nasopharyngoscopy, CT, and MRIcan help define location and extent of mass.

Except for hemangioma, which usuallycan be diagnosed clinically, histologic exam confirms diagnosis.

Disorders of Supraglottis and Larynx

Anatomic

Laryngomalacia

Severe laryngomalacia may interfere withswallowing and breathing (see Chap.63, Stertor, Stridor, and Airway Obstruction).

Cleft Larynx

Producesabnormal communication between airway and esophagus.

Attempts at feeding produce chokingand coughing.

Recurrent aspiration leads to pneumoniaand failure to thrive.

Esophagraphy shows contrast materialin trachea with swallowing.

Endoscopy is usually necessary to confirmdiagnosis.

Infection

Supraglottitis

Is an infectionof supraglottic tissues that includes epiglottis.

Occurrence has markedly decreased sinceintroduction of H. influenzae type b vaccine.

Age of onset is usually 1–5yrs of age.

Acute onset of fever, inspiratory stridor,drooling, and painful swallowing are characteristic findings.

Visualization of epiglottis and othersupraglottic tissues confirms diagnosis.

Bacterial culture of supraglottic tissuesor blood culture may reveal specific pathogen (see Chap. 63, Stertor, Stridor, and Airway Obstruction).

Disorders of Esophagus

Anatomic

Esophageal Atresia

Causes chokingand regurgitation of first feedings in newborns.

Nasogastric tube cannot be passed intostomach.

Diagnosis is confirmed by barium swallow.

Tracheoesophageal Fistula without Esophageal Atresia

Tracheoesophagealfistula without esophageal atresia (H-type) is open communicationbetween trachea and esophagus that may produce choking and coughingwith feedings. Choking and coughing disappear with gavage feedingsbut resume with regular feedings.

Recurrent aspiration pneumonia is commonproblem.

Upper GI radiographic series with attentionto esophagus usually demonstrates this lesion.

Esophageal Stenosis, Web, Duplication, and Stricture

All of these lesions cause esophageal obstructionand may be diagnosed by combination of radiographic imaging andesophagoscopy (see Chap. 55,Regurgitation and Vomiting).

Vascular Anomalies

May causeairway and esophageal obstruction. Anomalies include double aorticarch, right aortic arch with retroesophageal left subclavian arteryand left ligamentum arteriosum, right aortic arch with mirror-imagebranching and left ligamentum arteriosum, and anomalous left pulmonaryartery (pulmonary artery sling).

Left aortic arch with retroesophagealright subclavian artery and left ligamentum arteriosum is rarely,if ever, symptomatic (see Chap.63, Stertor, Stridor, and Airway Obstruction).

Mediastinal Masses

Some mediastinal masses may compress esophagusand cause difficulty in swallowing (see Chap. 56, Respiratory Distress and Apnea).

Infection/Inflammation

Gastroesophageal Reflux Disease

May cause esophagitis with difficult or painfulswallowing (see Chap. 55, Regurgitationand Vomiting).

Idiopathic Eosinophilic Esophagitis

Characterizedby eosinophilic infiltration of esophagus in absence of other disorders knownto cause eosinophilic inflammation.

Dysphagia and chest pain are commonsymptoms in childhood.

Diagnosis is confirmed by endoscopywith biopsy.

Candidiasis

Candidainfection of esophagus may occur with cancer chemotherapy, primaryimmune deficiency, hypoparathyroidism, or hypoadrenocorticism.

Positive KOH preparation and fungalculture obtained at esophagoscopy are diagnostic.

Herpes Simplex Virus

May causeesophageal ulceration, resulting in painful swallowing.

Common in immunocompromised hosts butalso may occur in absence of immune deficiency.

Positive viral culture obtained duringesophagoscopy is diagnostic.

Cytomegalovirus

Esophagealinvolvement with cytomegalovirus usually occurs in immunocompromisedindividuals.

Endoscopy with biopsy and culture isdiagnostic.

Chagas Disease

Caused bythe parasite T. cruzi.

Large dilated esophagus and difficultyin swallowing may occur many years after initial phase of the disease.

Diagnosis may be confirmed by serologictests.

Foreign Body

Foreignbodies in esophagus may cause difficulty in swallowing.

Most common are coins.

Chest radiography is useful in locatingtheir position in esophagus.

Motility Disorders

Cricopharyngeal Dysfunction

May causechoking, difficulty in swallowing, and recurrent aspiration. Maybe seen with CNS disorders (e.g., Chiari malformations).

Upper GI series with video playbackcapability is diagnostic.

Achalasia

Rare disorderin which lower esophageal sphincter fails to relax. Difficulty withswallowing and vomiting are most common complaints.

Upper GI series shows dilated esophagusand distal narrowing at esophageal junction. Manometric studiesshow failure of relaxation of lower esophageal sphincter and absenceof normal esophageal peristalsis.

Scleroderma

Connectivetissue disorder of unknown etiology that may cause decreased esophageal motilityand difficulty in swallowing.

Clinical manifestations include skinthickening and edema; joint stiffness of fingers, wrists, knees,and ankles; muscle weakness; and paroxysmal vasospasm of the fingers(Raynaud phenomenon).

Upper GI series or esophageal manometrydemonstrates absence of peristalsis in distal esophagus.

Clinical findings and skin biopsy arediagnostic.

Neoplasm

Althoughtumors of esophagus are rare in pediatric population, can presentwith difficulty swallowing and persistent vomiting.

Compression of trachea by tumor alsomay cause cough, wheezing, respiratory distress, and recurrent pulmonaryinfection.

Benign tumors include hamartoma, lipoma,neurofibroma, and leiomyoma.

Most common malignant tumor is esophagealcarcinoma.

Chest radiography, upper GI series,esophagoscopy, CT, and MRI are useful in locating and defining extentof mass.

Histologic diagnosis is definitive.

Presence of leukemic infiltrates inesophagus also may cause difficulty in swallowing. Upper GI seriesshows diffuse filling defects in this area. Diagnosis of leukemiahas usually been made previously. Esophagoscopy with biopsy is diagnostic.

Disorders Affecting Suck-Swallow-Breathing Coordination

Any Cause of Respiratory Distress

Respiratory distress may cause difficultywith coordination of breathing and feeding. See Chap. 56, Respiratory Distress and Apnea.

Disorders Affecting Neuromuscular Coordination of Suckingand Swallowing

Brain Disorders

Delayed Maturation

Transient palatal or pharyngeal muscle dysfunctionmay occur in preterm infants due to delayed maturation. Chokingduring feeding and difficulty in swallowing with regurgitation offeedings are common findings. This problem gradually improves withage.

Mental Retardation

Severe mental retardation of any cause maybe associated with palatal and pharyngeal muscle dysfunction, whichmay produce difficulty in feeding and swallowing. See Chap. 13, Developmental Delay.

Cerebral Palsy

Often associated with palatal and pharyngealmuscle dysfunction, resulting in feeding and swallowing difficulty.

Other

Hypoxic-ischemic encephalopathy, brain malformations,bacterial meningitis, viral encephalitis, head trauma, kernicterus,and neoplasms may damage motor cortex and neural connections innervous system that control sucking and swallowing. These disordersare discussed in other chapters.

Cranial Nerve Disorders

Disorders that damage cranial nerves V, VII,IX, or X may produce difficulty in sucking and swallowing. Theseinclude infections (tetanus, rabies, poliomyelitis), Moebius syndrome,and isolated palatal paralysis.

Infection

Tetanus

C. tetaniorganisms can enter the body through infected wound or damaged tissue. Sporesproduce neurotoxin that acts on brain and peripheral nerves.

Common in neonates in many poorer countriesof the world because of contamination of umbilical stump.

Onset of trismus and severe musclespasms usually occurs within 1 wk after contamination of wound andmay last several weeks.

Although positive wound or blood cultureconfirms diagnosis, organism is infrequently cultured.

Rabies

Acute generalizedinfection of nervous system produced by rabies virus. Major reservoirsare bats, raccoons, skunks, foxes, and coyotes, who can infect domesticdogs and cats.

After animal bite, virus reaches brainalong peripheral nerves. Usual incubation period is average of 4–6wks but may range from 5 days to 1 yr.

Onset usually begins with headacheand fever, which are followed in several days by lacrimation, salivation,sweating, apprehension, insomnia, photophobia, pupillary dilatation,and increase in muscle tone.

Painful and violent spasms of musclesof deglutition occur during attempts to swallow fluids or food.

Seizures are common, and progressiveparalysis and coma can follow.

Diagnosis of rabies can be made byisolation of virus from saliva or by detection of antibody in serumor spinal fluid of unvaccinated individuals. If affected animalis available, finding pathognomonic cytoplasmic inclusions (Negribodies) or demonstrating virus-specific fluorescent antigen in braintissue is diagnostic.

Poliomyelitis

Although only a few cases of vaccine-relatedpoliomyelitis occur each year in the Western hemisphere, it stilloccurs in other areas of the world (see Chap. 33, Hypotonia and Weakness).

Moebius Syndrome

Consistsof facial nerve weakness, which is nearly always bilateral and severe.There is association with dysfunction of other cranial nerves. Abducensnerve palsy occurs in most cases and is almost always bilateral.Oculomotor nerve involvement as well as bilateral ptosis also mayoccur. Tongue atrophy and weakness secondary to involvement of thehypoglossal nerve are also common. Eyes cannot be closed, face isexpressionless, and lack of a proper seal around the nipple leadsto feeding difficulty.

Associated findings include talipesequinovarus, arthrogryposis, brachydactyly, syndactyly, centralrespiratory dysfunction, and mental retardation.

This disorder is usually sporadic,but a few cases have been familial, and several gene loci have beenmapped.

Isolated Palatal Paralysis

Isolated palatal paralysis caused by involvementof cranial nerve X prevents closure of velopharyngeal space duringphonation and deglutition. Persistent regurgitation of formula mayoccur during feeding. Speech is hypernasal and unintelligible.

Anterior Horn Cell Disorders

In addition to poliomyelitis, spinal muscularatrophies and enteroviral infections affect anterior horn cellsin spinal cord and motor nuclei of brainstem. Progression of diseasecan cause difficulty in sucking and swallowing (see Chap. 33, Hypotonia and Weakness).

Peripheral Nerve Disorders

Guillain-Barré syndrome is prototypeof peripheral nerve disorders that may affect swallowing (see Chap. 33, Hypotonia and Weakness).

Neuromuscular Junction Disorders

Myasthenia gravis and botulism are 2 neuromuscularjunction disorders that also may affect swallowing (see Chap. 33, Hypotonia and Weakness).

Muscle Disorders

Congenital muscular dystrophy, myotonic dystrophy,and dermatomyositis are discussed in Chap.33, Hypotonia and Weakness.

Autonomic Nervous System Disorders

Familial Dysautonomia (Riley-Day Syndrome)

In U.S.,this autosomal-recessive disorder occurs almost exclusively in individualsof Ashkenazi Jewish extraction. Gene locus has been mapped to chromosome9q31.

Affected infants and children havepoor sucking and swallowing, lack of tearing, excessive sweating,decreased taste sensation, relative indifference to pain, episodicvomiting, corneal anesthesia, skin blotching, episodic fever, paroxysmalhypertension, vasomotor instability, and emotional lability.

5 signs useful in diagnosis are lackof tearing, absence of fungiform papillae on tongue, decreased deeptendon reflexes, lack of axon flare after intradermal injectionof histamine, and pupillary miosis after conjunctival instillationof 2.5% methacholine.

Postsurgery Complications

Difficultyin swallowing may develop after tracheostomy, but this usually resolvesin a few days.

Vagotomy also may produce difficultyin swallowing, which usually disappears within several months.

Psychologic Disturbance

Childrenmay become anxious and refuse to swallow solids after choking episode.Severe anxiety (globus hystericus) and conversion reactions maycause individuals to complain that they cannot swallow.

History and physical exam are usuallydiagnostic.

Diagnostic Approach

History,physical exam, and observation of infant or child feeding may bediagnostic.

Age of child, whether swallowing ispainful, and presence of associated findings are helpful. Painfulswallowing is most commonly caused by infection or mechanical obstruction.Difficulty swallowing due to neurologic causes is usually painless.

For swallowing problems, radiographiccontrast study should be performed to investigate any anatomic orstructural abnormalities.

Videofluoroscopy study helps evaluateoropharyngeal and esophageal function during swallowing.

Esophageal manometry can provide usefulinformation about sphincter function and peristalsis.

Other tests depend on suspected diagnosisbut may include chest and neck radiography, endoscopy, and chestor brain imaging with CT or MRI.

References

1. Altshuler SM, Liacouras CA, eds. Clinicalpediatric gastroenterology. Philadelphia: Churchill Livingstone,1998.
2. Behrman RE, et al., eds. Nelson textbook of pediatrics,16th ed. Philadelphia: WB Saunders, 2000.
3. Illingworth RS. Sucking and swallowing difficultiesin infancy: diagnostic problem of dysphagia. Arch Dis Child 1969;44:655–665.
4. Long SS, et al., eds. Principles and practice of pediatricinfectious diseases. New York: Churchill Livingstone, 1997.
5. Online Mendelian Inheritance in Man (OMIM). McKusick-NathansInstitute for Genetic Medicine, Johns Hopkins University (Baltimore,MD) and National Center for Biotechnology Information, NationalLibrary of Medicine (Bethesda, MD), 2000. World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim.
6. Pickering LK, ed. 2000 Red book: report of the Committeeon Infectious Diseases, 25th ed. Elk Grove Village, IL: AmericanAcademy of Pediatrics, 2000.
7. Rudolph AM, ed. Rudolph's pediatrics, 20thed. Stamford, CT: Appleton & Lange, 1996.
8. Rudolph CD. Feeding disorders in infants and children.J Pediatr 1994;125(suppl):116–124.
9. Volpe JJ. Neurology of the newborn, 4th ed. Philadelphia:WB Saunders, 2001.
10. Walker WA, et al., eds. Pediatric gastrointestinaldisease, 3rd ed. Hamilton, Ontario, Canada: BC Decker, 2000.
11. Willging JP, et al. Feeding disorders in children.In: Cotton RT, Myer CM III, eds. Practical pediatric otolaryngology.Philadelphia: Lippincott-Raven, 1999:603–612.

Book Source Details

• Book Title: The Diagnostic Approach to Symptoms and Signs in Pediatrics
• Author(s): Paul S. Bellet
• Year of Publication: 2006
• Copyright Details: The Diagnostic Approach to Symptoms and Signs in Pediatrics, Copyright © 2006 Lippincott Williams & Wilkins.

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