Evaluation of acute dysphagia in children

INTRODUCTION — The primary function of swallowing is the ingestion, preparation, and transportation of nutrients to the digestive tract. Secondary functions of swallowing are the control of nasal and oropharyngeal secretions, clearance of mucous and contaminants from the lower respiratory tract, protection of the upper airway, and equalization of pressure across the tympanic membrane through the Eustachian tube.

This topic will review the differential diagnosis and evaluation of dysphagia in children with a focus on conditions that present with acute-onset dysphagia. The evaluation and management of chronic swallowing dysfunction resulting in chronic aspiration in children are discussed separately. (See "Aspiration due to swallowing dysfunction in children".)

DEFINITIONS

●Dysphagia – Dysphagia is defined as any difficulty or abnormality of swallowing. Dysphagia is not a specific disease entity but is a symptom of other conditions, some of which may be life-threatening.

●Odynophagia – Odynophagia refers to pain on swallowing and may also be present in the dysphagic child.

●Sialorrhea – Sialorrhea (drooling) may accompany dysphagia and often indicates oropharyngeal, upper airway, and/or esophageal infection or obstruction.

PATHOPHYSIOLOGY — Dysphagia can be further characterized by the phase of swallowing that is affected; this identification may be informative in guiding evaluation (see "Aspiration due to swallowing dysfunction in children", section on 'Physiology of feeding and swallowing'):

●Oral dysphagia – Poor handling of the food bolus or difficulty in propulsion of contents into the oropharynx, which is initiated by the swallow reflex and tongue (eg, caused by an absent or impaired swallowing reflex, poor coordination of bite/chew, or infectious mouth lesions).

●Pharyngeal dysphagia – Uncoordinated muscle contraction in the effort to safely propel food from the oropharynx to the esophagus (eg, resulting in aspiration, choking, or parapharyngeal reflux).

●Esophageal dysphagia – Difficulty with cricopharyngeal relaxation or smooth muscle contraction to send contents into the stomach (eg, suspected obstruction [esophageal foreign body] or inability to achieve effective muscle contraction).

DIFFERENTIAL DIAGNOSIS — The table provides a listing of life-threatening, common, and other conditions associated with dysphagia in children (table 1).

Life-threatening conditions — Life-threatening etiologies of dysphagia may cause airway compromise, pulmonary aspiration, serious local or systemic infection, or inflammatory disease.

Oropharyngeal infection or inflammation

Retropharyngeal cellulitis and abscess — Retropharyngeal cellulitis and abscess occur most commonly in children between the ages of two and four years. Retropharyngeal abscess often is a polymicrobial infection. The predominant bacterial species are Streptococcus pyogenes (group A streptococcus), Staphylococcus aureus (including methicillin-resistant S. aureus), and respiratory anaerobes (including Fusobacteria, Prevotella, and Veillonella species).

Early in the disease process, the findings may be indistinguishable from those of uncomplicated pharyngitis. With disease progression, symptoms related to inflammation and obstruction of the upper aerodigestive tract develop. Children with retropharyngeal cellulitis with or without abscess eventually appear ill with fever. Symptoms may include dysphagia, odynophagia, drooling, pain on neck extension, and torticollis. In addition, respiratory distress, stridor, and/or neck swelling, mass, or lymphadenopathy may be present. In stable patients, the diagnosis is best confirmed by contrast computed tomography (CT) of the neck. (See "Retropharyngeal infections in children".)

Acute epiglottitis — Acute epiglottitis, which occurs rarely among children immunized against Haemophilus influenzae type b, is a medical emergency. To minimize morbidity and mortality, prompt recognition (algorithm 1) and treatment is critical (table 2). Clinical features, diagnosis, and management are discussed separately. (See "Epiglottitis (supraglottitis): Clinical features and diagnosis" and "Epiglottitis (supraglottitis): Management".)

Diphtheria — Diphtheria occurs rarely in regions with high vaccination rates but remains a serious disease throughout much of the world. It is an acute, communicable disease caused by the Gram-positive bacillus Corynebacterium diphtheriae. Respiratory diphtheria has a gradual onset of symptoms, with sore throat, odynophagia, malaise, and low-grade fever being the most common. In a significant percentage of patients, diphtheria toxin locally induces the formation of a coalescing pseudomembrane, which can extend to any portion of the respiratory tract from the nasal passages to the tracheobronchial tree and cause odynophagia. Neurologic toxicity with uncoordinated swallowing, dysphagia, upper airway obstruction, and/or pulmonary aspiration is unusual in mild disease but develops in up to three-fourths of patients with severe diphtheria. The diagnosis is confirmed by positive culture and positive toxin assay. (See "Clinical manifestations, diagnosis, and treatment of diphtheria".)

Stevens-Johnson syndrome — Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome represent disorders of uncertain etiology that are characterized by desquamative lesions of the skin and mucous membranes (picture 1 and picture 2 and picture 3 and picture 4). Drooling and dysphagia commonly accompany severe oropharyngeal mucosal sloughing. Clinical findings establish the diagnosis in most cases, although, rarely, a skin biopsy may be performed.

TEN is almost invariably drug-induced (eg, sulfonamide antimicrobials, phenobarbital, carbamazepine, or lamotrigine), while SJS is associated with infections, as well as drug administration. SJS and TEN begin with a prodrome of fever and influenza-like symptoms one to three days before the development of mucocutaneous and skin lesions. Characteristic vesicular and bullous skin lesions then appear and progress over several days, followed by sloughing (picture 5 and picture 6 and picture 7 and picture 1 and picture 3). Multiorgan involvement, including tracheobronchitis, may occur. (See "Stevens-Johnson syndrome and toxic epidermal necrolysis: Pathogenesis, clinical manifestations, and diagnosis".)

Disordered control of swallowing

Central nervous system infection — Children with meningitis, encephalitis, or cerebral abscess may have impaired consciousness (lethargy, obtundation, or coma) with loss of the gag reflex and dysphagia. They may also rarely exhibit bulbar signs with difficulty swallowing due to infection of the components of the swallowing pathway (eg, cortical motor stripe, vagus nerve nucleus). Fever is usually present.

Most patients with bacterial meningitis present with fever and with clinical findings of meningeal inflammation (nausea, vomiting, irritability, anorexia, headache, confusion, back pain, and nuchal rigidity). However, the clinical manifestations of bacterial meningitis are variable and nonspecific; no single sign is pathognomonic. (See "Bacterial meningitis in children older than one month: Clinical features and diagnosis", section on 'Clinical features'.)

Children with encephalitis often have fever, altered mental status, seizures, and/or focal neurologic signs. (See "Acute viral encephalitis in children: Clinical manifestations and diagnosis", section on 'Clinical features'.)

For patients without contraindications to lumbar puncture, the diagnosis of meningitis or encephalitis is confirmed by the presence of cerebrospinal fluid (CSF) pleocytosis. Presumptive antimicrobial therapy should be given to patients in whom lumbar puncture cannot be safely performed. Specific pathogens are identified by isolation from CSF by culture or other molecular methods such as polymerase chain reaction (PCR) as well as similar detection methods in other samples such as blood. The approach to diagnosis of bacterial meningitis and viral meningitis or encephalitis is discussed in greater detail separately. (See "Bacterial meningitis in children older than one month: Clinical features and diagnosis" and "Viral meningitis in children: Clinical features and diagnosis" and "Acute viral encephalitis in children: Clinical manifestations and diagnosis".)

Central nervous system tumor — Cranial neuropathies with drooling, dysphagia, diplopia, impairment of ocular movements, and/or facial palsy suggest underlying brainstem pathology. These findings represent common localizing signs of central nervous system tumors and may be associated with new-onset seizures or signs of increased intracranial pressure such as progressive headache or morning vomiting. Neuroimaging establishes the diagnosis. (See "Clinical manifestations and diagnosis of central nervous system tumors in children", section on 'Cranial nerve palsies' and "Elevated intracranial pressure (ICP) in children: Clinical manifestations and diagnosis".)

Other neurologic conditions — A diverse group of central and peripheral neurologic conditions, neuromuscular diseases, and myopathies are associated with poor coordination of swallowing and/or pharyngeal muscle weakness that results in dysphagia. Etiologies include (see "Aspiration due to swallowing dysfunction in children"):

●Cerebral palsy (see "Cerebral palsy: Classification and clinical features")

●Traumatic brain injury

●Demyelinating diseases (eg, Guillain-Barré syndrome with bulbar weakness [Miller Fisher syndrome]) (see "Guillain-Barré syndrome in children: Epidemiology, clinical features, and diagnosis")

●Botulism (see "Botulism")

●Congenital myopathies (see "Congenital myopathies")

●Mitochondrial myopathies (see "Mitochondrial myopathies: Clinical features and diagnosis")

●Myasthenia gravis (see "Clinical manifestations of myasthenia gravis")

●Spinal muscular atrophy and neurodegenerative diseases (eg, metachromatic leukodystrophy, Alexander disease, Krabbe disease, juvenile amyotrophic lateral sclerosis) (see "Spinal muscular atrophy" and "Metachromatic leukodystrophy" and "Neuropathies associated with hereditary disorders")

Patients with poor swallow coordination may present with brief resolved unexplained events (BRUE) or aspiration pneumonia. Diagnosis of aspiration requires specialized testing, as discussed separately. (See "Aspiration due to swallowing dysfunction in children".)

Tetanus — Tetanus is a nervous system disorder characterized by muscle spasms that is caused by the toxin-producing anaerobe Clostridium tetani. Dysphagia and trismus are common presenting symptoms in patients with generalized tetanus. Other clinical findings may include stiff neck, severe neck arching (opisthotonos), or risus sardonicus (sardonic smile). Patients with generalized tetanus characteristically have tonic contraction of their skeletal muscles and intermittent intense muscular spasms. Since patients with tetanus have no impairment of consciousness or awareness, both the tonic contractions and spasms are intensely painful. Tetanic spasms may be triggered by loud noises or other sensory stimuli, such as physical contact or light. The diagnosis of tetanus is made based upon typical clinical findings. (See "Tetanus".)

Poliomyelitis — Although polio no longer poses the public health threat in the United States that it once did, areas of endemic wild-type poliovirus still exist in other areas of the world and decreasing rates of vaccination of children in some regions increases the risk of resurgence. The hallmark of poliomyelitis is acute flaccid weakness due to anterior horn cell injury. The onset of weakness typically coincides with signs and symptoms of viral meningitis (eg, neck stiffness, headache, fever, and vomiting) as well as muscle pain. The distribution and extent of weakness may vary, ranging from one muscle or group of muscles to quadriplegia and respiratory failure. Tone is reduced, nearly always in an asymmetric manner. Proximal muscles usually are affected more than distal muscles, and legs more commonly than arms. Reflexes are decreased or absent. The sensory examination is almost always normal. Up to one-third of patients may develop bulbar involvement with dysphagia, dysarthria, and difficulty handling secretions. The diagnosis of poliomyelitis is suspected based on the clinical presentation and high index of suspicion from epidemiology or exposure history. Isolation of the poliovirus from stool is the gold standard for confirming the diagnosis. (See "Poliomyelitis and post-polio syndrome", section on 'Poliomyelitis'.)

Esophageal injury

Esophageal foreign body — Patients of any age with an esophageal foreign body may present with inability or refusal of oral fluids or solids (dysphagia), drooling, or respiratory symptoms, including wheezing, stridor, or choking. The majority of foreign body ingestions occur in children between the ages of six months and three years. Most children with esophageal foreign bodies are brought to medical attention by their parents because the ingestion was witnessed or reported to them.

When symptoms do occur, they are often related to the location of the foreign body. Older children may localize the sensation of something "stuck" in the neck or lower chest, suggesting irritation in the upper or lower esophagus, respectively. Foreign bodies lodged in the esophagus at the level of the cricoid cartilage or the tracheal bifurcation can compress the airway causing partial airway obstruction (image 1). It is also possible that an esophageal foreign body will become dislodged into the upper airway. (See "Emergency evaluation of acute upper airway obstruction in children".)

Esophageal perforation from ingested sharp objects or erosion from retained smooth foreign bodies, particularly "button" batteries, may cause serious sequelae, including mediastinitis, sepsis, and death (picture 8 and picture 9). Children with suspected foreign body or "button" battery ingestion should have prompt anterior-posterior (AP) and lateral radiographs performed to identify possible esophageal or gastrointestinal retention. Evidence of a double-ring or halo on the AP view (image 2), or step-off of the foreign body on the lateral view (image 3), indicates the presence of a "button" battery rather than a coin and requires immediate removal. "Button" batteries can cause severe esophageal injury within two hours of ingestion. (See "Foreign bodies of the esophagus and gastrointestinal tract in children" and "Button and cylindrical battery ingestion: Clinical features, diagnosis, and initial management", section on 'Esophageal impaction'.)

Impacted meat or other food bolus is the most common esophageal foreign body in adults but is relatively rare in children. It usually presents as dysphagia beginning acutely while eating. In children presenting with a food impaction, there is a higher incidence of underlying esophageal pathology (strictures, achalasia, eosinophilic esophagitis [EoE], or esophageal motility disorders) as compared with children with other esophageal foreign bodies [1]. Children who are in acute distress or unable to swallow oral secretions following food impaction require immediate attention and removal of the impaction. If the patient is comfortable and able to handle oral secretions, endoscopic intervention can be delayed because many food impactions will pass spontaneously. (See "Foreign bodies of the esophagus and gastrointestinal tract in children", section on 'Esophageal food impaction'.)

Caustic ingestion — Dysphagia is the most common symptom of caustic oropharyngeal and esophageal injury. Patients may also present with drooling, oral burns, retrosternal or abdominal pain, hematemesis, and features suggesting upper airway injury, such as stridor, hoarseness, nasal flaring, and retractions. Caustic ingestion may also cause a chemical epiglottitis or an esophageal perforation with mediastinitis. The diagnosis is typically established based upon history with endoscopy confirming esophageal injury in patients with symptoms (picture 10) and asymptomatic patients who have ingested more toxic substances (eg, concentrated acids or bases found in drain cleaners) or basic substances (eg, lye [NaOH or KOH]). (See "Caustic esophageal injury in children".)

Esophageal perforation — Esophageal perforation and mediastinitis occur rarely in children. Caustic ingestions, erosive esophageal foreign bodies (ie, "button" batteries), and esophageal procedures (eg, endoscopy, dilation of esophageal strictures) are the most frequent causes [2,3].

●Etiology and clinical manifestations – Clinical findings of esophageal perforation include:

•Dysphagia with odynophagia

•Neck pain (cervical esophageal perforation)

•Chest pain (thoracic esophageal perforation)

•Dyspnea

In addition, hematemesis may occur if the foreign body erodes into adjacent vascular structures. (See "Button and cylindrical battery ingestion: Clinical features, diagnosis, and initial management", section on 'Esophageal impaction'.)

Esophageal perforation progresses to mediastinitis when oral and gastric fluids contaminate the mediastinal space. Mediastinitis typically presents with fever and subcutaneous emphysema and rapidly progresses to septic shock within hours if not rapidly detected and treated. This infection is usually polymicrobial and is caused by mouth flora, including Staphylococcus species, Streptococcus species, anaerobic bacteria, and Gram-negative bacteria [3]. Laboratory evaluation of these patients should include a complete blood count, blood culture, electrolytes, and type and screen.

●Diagnosis – Plain radiographs of the chest (AP and lateral) and neck (soft tissue lateral and AP) will detect esophageal perforation in 90 to 95 percent of patients [2]. Findings depend on the location of the perforation. Neck films demonstrate air in the prevertebral fascial planes of the retropharynx and subcutaneous emphysema in patients with cervical esophageal perforations. Findings associated with more distal esophageal perforations on chest radiograph include pneumomediastinum, subcutaneous emphysema, pleural effusion, hydrothorax, hydropneumothorax, and subdiaphragmatic air (upright film).

Contrast esophagography or CT should be obtained in children with a high level of suspicion for a perforation despite a negative chest radiograph. In addition, these studies may identify the specific site of esophageal perforation and are confirmatory studies [2]. The choice of confirmatory test is best guided by a surgical consultant.

●Management – Management priorities are as follows:

•Aggressive treatment of hypovolemia and shock as determined by the patient's circulatory status (algorithm 2) (see "Shock in children in resource-abundant settings: Initial management")

•Administration of a broad-spectrum antibiotic regimen (eg, ceftazidime and clindamycin) that provides coverage for Gram-positive cocci (including methicillin-resistant S. aureus), anaerobic bacteria, and Gram-negative bacteria

•Emergency consultation with a surgeon with pediatric thoracic surgery expertise who can perform surgical drainage, ideally within 24 hours of esophageal perforation

•In patients with active bleeding, endotracheal intubation, hemodynamic stabilization, and emergent thoracotomy in the operating room

Mortality from esophageal perforation varies from approximately 0 to 10 percent in patients who undergo drainage procedures within 24 hours to as high as 31 percent in patients where definitive drainage is delayed beyond 24 hours [4,5].

Extrinsic compression (intrathoracic tumor) — Extrinsic compression from mediastinal or chest tumors (eg, lymphoma) may rarely compromise esophageal motility. However, these tumors are most notable for causing respiratory distress, stridor, wheezing, and/or anxiety, especially when the patient is in a supine position, and for complicating airway management in patients with respiratory failure. Patients with stridor, obstructive upper airway swelling, or cerebral edema caused by superior vena cava syndrome warrant emergency radiotherapy and/or vascular stenting. The diagnosis is typically established through chest radiograph or CT. (See "Malignancy-related superior vena cava syndrome", section on 'Treatment'.)

Common conditions

Oropharyngeal trauma — Penetrating oropharyngeal wounds in children typically result from low-force mechanisms and usually heal spontaneously with no sequelae. Rarely, internal carotid artery injury with neurologic deficit or deep space neck infection with drooling and dysphagia may complicate oropharyngeal trauma involving the lateral aspect of the palate. (See "Oropharyngeal trauma in children".)

Infants and toddlers with unexplained oral injuries, including frenulum tears, intraoral or lip bruising, or wounds to the palate or oral mucosa warrant further evaluation for physical or sexual abuse (table 3). Intentional trauma to the posterior oropharynx of a nonverbal child by the caregiver to induce hematemesis has also been described as a form of Munchausen syndrome by proxy. (See "Physical child abuse: Recognition" and "Medical child abuse (Munchausen syndrome by proxy)".)

Stomatitis — Stomatitis, the inflammation of the mucous lining of the mouth and throat, is one of the most common causes of dysphagia in children and is typically associated with viral infection (eg, enterovirus or herpesvirus infection).

●Enteroviruses cause hand, foot, and mouth syndrome which is characterized by fever, oral vesicles on the buccal mucosa (picture 11) and tongue (picture 12), and peripherally distributed small, tender cutaneous lesions on the hands (picture 13), feet (picture 14), buttocks, and (less commonly) genitalia. The Coxsackie A enterovirus is also the etiologic agent of herpangina, a vesicular enanthem of the tonsillar fauces and soft palate that principally affects children 3 to 10 years of age (picture 15). Oropharyngeal symptoms of sore throat are accompanied by fever and odynophagia. Most disease occurs during summer outbreaks. (See "Hand, foot, and mouth disease and herpangina".)

●Herpetic gingivostomatitis is most often caused by herpes simplex virus type 1 (HSV-1) and typically occurs in children between six months and five years of age. Clinical features of HSV-1 stomatitis include a prodrome of fever and constitutional symptoms, followed by anterior oral and extraoral lesions. The lesions begin as vesicles, which coalesce to form painful ulcers (picture 16). Associated findings include fever, bad breath, odynophagia, anorexia, and submandibular or cervical lymphadenitis. (See "Herpetic gingivostomatitis in young children".)

Treatment of viral stomatitis caused by enterovirus is primarily supportive and consists of administration of appropriate oral pain medications (eg, ibuprofen or acetaminophen, as needed) and encouragement of adequate fluid intake. On occasion, children will refuse to drink and require intravenous treatment for dehydration. (See "Treatment of hypovolemia (dehydration) in children in resource-abundant settings".)

We do not routinely suggest "magic mouthwash" (equal parts of diphenhydramine, aluminum and magnesium hydroxide antacid, and 2% viscous lidocaine or similar solutions) to coat lesions and/or soothe pain given the lack of evidence of benefit from clinical trials, the potential for harm (eg, toxicity from systemic absorption, allergic reaction), and difficulty of application in young children (see "Herpetic gingivostomatitis in young children", section on '"Magic mouthwash" and other topical therapies'). The management of herpetic gingivostomatitis is discussed in greater detail separately. (See "Herpetic gingivostomatitis in young children", section on 'Management of gingivostomatitis'.)

Infectious pharyngitis — Patients with dysphagia and clinical evidence of infectious pharyngitis are managed according to the infectious etiology (algorithm 3). Common pathogens include enterovirus, adenovirus, Epstein-Barr virus, Group A Streptococcus, and Neisseria gonorrhoeae. On occasion, children with pharyngitis have such severe pain with swallowing that they refuse fluid intake and become dehydrated. The approach to the diagnosis of infectious pharyngitis and the treatment of dehydration in children is discussed in greater detail separately. (See "Group A streptococcal tonsillopharyngitis in children and adolescents: Clinical features and diagnosis" and "Treatment of hypovolemia (dehydration) in children in resource-abundant settings".)

Peritonsillar abscess — Children with peritonsillar cellulitis and abscess (PTA) have a severe sore throat (usually unilateral), fever, and a "hot potato" or muffled voice. Pooling of saliva or drooling may be present. Trismus, related to irritation and reflex spasm of the internal pterygoid muscle, occurs in nearly two-thirds of patients and helps to distinguish PTA from severe pharyngitis or tonsillitis. Although most children with PTA are adolescents, rarely, it may present in younger children and be difficult to differentiate from retropharyngeal abscess or epiglottitis, especially if drooling and trismus are present. (See "Peritonsillar cellulitis and abscess", section on 'Evaluation'.)

Examination findings consistent with PTA include an extremely swollen and fluctuant tonsil often with deviation of the uvula to the opposite side (picture 17). Alternatively, there may be visible swelling or bulging of the posterior soft palate near the tonsil, with palpable fullness and/or fluctuance.

Drainage, antimicrobial therapy, and supportive care are the cornerstones of management for PTA. (See "Peritonsillar cellulitis and abscess", section on 'Management'.)

Esophagitis — Esophageal injury with inflammation and dysphagia most commonly occurs as a complication of gastroesophageal reflux (picture 18). Medication-induced esophagitis (or "pill esophagitis"), EoE (picture 19), and esophageal infection are additional etiologies to consider and are more likely in children with dysphagia and odynophagia (see "Clinical manifestations and diagnosis of gastroesophageal reflux disease in children and adolescents", section on 'Clinical manifestations'):

●Gastroesophageal reflux disease – Gastroesophageal reflux disease (GERD) with esophagitis is suggested by recurrent regurgitation after two years of age with dysphagia, frequent complaints of heartburn, and refusal of food (especially solids). The presence of dysphagia is an indication for diagnostic endoscopy. (See "Clinical manifestations and diagnosis of gastroesophageal reflux disease in children and adolescents".)

●Medication-induced ("pill") esophagitis – The typical patient with medication-induced esophagitis does not have a history of prior esophageal disease. Patients will often present with the sudden onset of odynophagia and retrosternal pain; the pain may be so severe that swallowing saliva is difficult. An adolescent with acute onset of heartburn or odynophagia in association with ingestion of tetracyclines (doxycycline or minocycline) that are frequently used for treatment of acne is a common scenario. Other medications that injure the esophageal mucosa include clindamycin, aspirin and other antiinflammatory agents, and bisphosphonates. In such cases, a clinical diagnosis may be made without the requirement for confirmatory endoscopy. (See "Pill esophagitis".)

●Eosinophilic esophagitis – EoE is one of the leading causes of dysphagia in the pediatric population. Males are more frequently affected (3:1 ratio), and patient history of atopy, asthma, and allergy is common. In younger children, vague feeding difficulties may be the only presenting symptom, while older children and adolescents present more commonly with dysphagia or food impaction. The diagnosis of EoE is based on clinical manifestations, endoscopic findings with biopsy demonstrating ≥15 eosinophils/high-powered field, and exclusion of other causes that may be responsible for, or contributing to, symptoms and esophageal eosinophilia (table 4). (See "Clinical manifestations and diagnosis of eosinophilic esophagitis (EoE)".)

●Infectious esophagitis – Infectious esophagitis most commonly occurs in immunocompromised patients with human immunodeficiency virus or hematologic malignancies, although it may occasionally occur in immunocompetent hosts. Candidal esophagitis is most frequently identified. Other etiologies include HSV-1, Mycobacterium tuberculosis, Cryptosporidium, and Pneumocystis jirovecii [6]. (See "Candida infections in children", section on 'Esophagitis' and "Herpes simplex virus infection of the esophagus".)

Referral to a pediatric gastroenterologist for upper endoscopy provides the best means to definitively diagnose children with suspected esophagitis other than those with uncomplicated medication-induced esophagitis. Unlike esophageal pH monitoring studies, endoscopy permits visualization of the esophageal epithelium, histologic evaluation to establish the presence of other disorders (eg, eosinophilic or infectious esophagitis), and identification of the presence and severity of esophagitis as well as complications, such as strictures (picture 20) or Barrett's esophagus. (See "Clinical manifestations and diagnosis of eosinophilic esophagitis (EoE)" and "Clinical manifestations and diagnosis of gastroesophageal reflux disease in children and adolescents", section on 'Endoscopy and histology'.)

Dystonic reaction — Dysphagia may occur as part of a dystonic reaction to certain medications, including typical antipsychotics (eg, chlorpromazine, haloperidol), anticonvulsants (eg, carbamazepine, phenytoin), and antiemetics (eg, metoclopramide, promethazine). The reaction causes acute spasm of multiple muscle groups, including oropharyngeal muscle spasm. Patients may also manifest a fixed upper gaze (oculogyric crisis), neck twisting, grimace, clenched jaw, and dysarthria. Dystonic reactions that are drug-induced can be treated with diphenhydramine or benztropine. (See "First-generation (typical) antipsychotic medication poisoning", section on 'Acute extrapyramidal syndromes'.)

Other conditions — Other conditions that may cause dysphagia include:

●Achalasia – Dysphagia for solids and liquids during swallowing, with a gradual but progressive onset and sometimes associated with regurgitation, is the primary clinical feature of achalasia (image 4). An association with adrenal glucocorticoid deficiency and alacrima (triple A syndrome or Allgrove syndrome) has been described in children with achalasia. (See "Causes of primary adrenal insufficiency in children", section on 'Triple A syndrome'.)

A contrast swallow is the primary screening test when achalasia is suspected on clinical grounds. The diagnostic accuracy of contrast swallow for achalasia is approximately 95 percent. Patients who have clinical and radiographic findings suggestive of achalasia require manometric and endoscopic evaluation to confirm the diagnosis. (See "Achalasia: Pathogenesis, clinical manifestations, and diagnosis".)

●Rheumatic disease – Gastrointestinal involvement is found in 30 to 74 percent of children with juvenile systemic sclerosis (scleroderma [JSSc]). Radiological and functional studies of the gastrointestinal tract often demonstrate abnormalities, such as gastroesophageal reflux and esophageal dysmotility, even in the absence of symptoms. Manometry and intraesophageal 24-hour pH monitoring are sensitive indicators of diminished lower esophageal sphincter tone and gastroesophageal reflux. (See "Juvenile systemic sclerosis (scleroderma): Classification, clinical manifestations, and diagnosis", section on 'Organ involvement' and "Gastrointestinal manifestations of systemic sclerosis (scleroderma)".)

In children with juvenile dermatomyositis, weakness of the palate and cricopharyngeal muscle may result in dysphagia, a nasal voice, tracheal aspiration, and reflux of food into the nasopharynx. Involvement of the upper esophagus can lead to dysphagia for solids and liquids. Dysphagia may also result from weakness of striated muscle in the lower third of the esophagus with esophageal reflux and esophageal dysmotility [7]. (See "Juvenile dermatomyositis and other idiopathic inflammatory myopathies: Epidemiology, pathogenesis, and clinical manifestations", section on 'Muscle weakness'.)

Children with juvenile idiopathic arthritis may rarely develop dysphagia caused by cricoarytenoid arthritis [8].

●Crohn disease – In children with Crohn disease, esophageal involvement is present histologically in 18 to 43 percent of patients and may cause dysphagia and odynophagia. This condition is primarily seen in children with advanced ileocolonic disease. The symptoms usually improve with effective management of the Crohn disease. (See "Clinical manifestations and complications of inflammatory bowel disease in children and adolescents" and "Overview of the management of Crohn disease in children and adolescents".)

●Thyroid enlargement – Enlarging thyroid lobes usually grow outward because of their location in the anterior neck in front of the trachea and are covered only by thin muscles, subcutaneous tissue, and skin. As a result of this outward growth, even very large goiters may not compress the trachea or impinge on the great vessels lateral to the lobes. However, in patients with substantial enlargement of one lobe or asymmetric enlargement of both lobes, the trachea, esophagus, or blood vessels may be displaced or, less often, compressed. Bilateral lobar enlargement, especially if the goiter extends posterior to the trachea, may cause either compression or concentric narrowing of the trachea or compression of the esophagus or jugular veins. Dysphagia is less common than respiratory distress because of the posterior position of the esophagus relative to the trachea. (See "Approach to acquired goiter in children and adolescents".)

Among the various causes of thyroid enlargement in children, acute suppurative thyroiditis is most commonly associated with dysphagia. (See "Approach to acquired goiter in children and adolescents", section on 'Acute suppurative thyroiditis'.)

●Mucositis – Oral mucositis commonly occurs in children receiving cytotoxic chemotherapy and other anticancer treatments (table 5) and can cause significant dysphagia. (See "Oral toxicity associated with systemic anticancer therapy".)

●Esophageal tumor – Adenocarcinoma or leiomyoma of the esophagus are extremely rare causes of dysphagia for solids more than liquids in children [9,10]. Upper endoscopy and biopsy confirm the diagnosis.

●Vascular ring – Although vascular anomalies of the aortic arch with an encircling ring around the esophagus and trachea usually present as respiratory distress and/or stridor, they may rarely present as dysphagia in children. These anatomic anomalies typically present in an infant less than one year of age. Dysphagia, vomiting, and feeding difficulty are also common. Vascular rings may be suggested by findings on plain radiographs of the chest and confirmed by CT angiography, magnetic resonance angiography, or echocardiography depending upon available technology and expertise. (See "Vascular rings and slings".)

●Globus sensation – Globus sensation is defined as an intermittent feeling of a lump or foreign body in the throat that occurs between meals, has lasted for at least six months, and is not associated with gastroesophageal reflux or esophageal motility disorders. In addition, patients with globus sensation do not have true dysphagia. Although common in adults, globus sensation is rare in children and warrants appropriate diagnostic evaluation to exclude the presence of a true foreign body or other esophageal pathology. (See "Globus sensation", section on 'Diagnosis'.)

STABILIZATION — The initial evaluation of children with signs and symptoms of dysphagia must begin with a rapid assessment of respiratory status to identify those who need resuscitation.

Signs suggesting upper airway obstruction and/or serious respiratory distress include:

●New onset of drooling

●Stridor

●Suprasternal retractions

●Difficulty speaking (dysphonia)

●Inability to speak (aphonia)

●"Hot potato" voice

●Tachypnea

●Cyanosis or low oxygen saturation by pulse oximetry

●Poor respiratory effort with hypercapnia in patients with muscle weakness

●Worsening respiratory status in the supine position indicating a mediastinal mass

Priorities for these patients include providing supplemental oxygen, support of breathing, and rapid sequence intubation (table 6). The management of complete or rapidly progressing partial upper airway obstruction requires an emergency call for assistance from an airway specialist (eg, anesthesiologist or pediatric critical care specialist). Management of children with upper airway obstruction is summarized in the algorithms (algorithm 4A-B) and described in detail separately. (See "Emergency evaluation of acute upper airway obstruction in children".)

Patients with a mediastinal mass, dysphagia, and tracheal impingement should be maintained in an upright position and, whenever possible, undergo endotracheal intubation in a controlled environment by a pediatric anesthesiologist or other airway expert with similar expertise.

EVALUATION

History — An accurate and complete history should suggest the diagnosis in the majority of patients. Key historical information includes:

●Likely triggers:

•Witnessed foreign body ingestion (or missing small foreign bodies or "button" batteries)

•Known or suspected caustic ingestion

•Pain exacerbated by eating, suggesting gastroesophageal reflux and/or esophagitis

•Substernal pain after taking an oral medication, especially tetracycline antibiotics (eg, doxycycline)

•Prior history of rheumatic disease, Crohn disease, or chronic neurologic impairment (eg, cerebral palsy, traumatic brain injury)

●Related symptoms:

•Dysphonia or aphonia

•Presence of fever, especially with oral lesions, drooling, sore throat, trismus, or stiff neck

•Erythema multiforme rash or desquamation with mucous membrane changes in patients with risk factors for Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) (picture 1 and picture 2 and picture 3 and picture 4) (see 'Stevens-Johnson syndrome' above)

•Symptoms of cranial nerve weakness, including vision changes, ptosis, nasal regurgitation of food or liquid with swallowing

●Type of dysphagia:

•Dysphagia for solids more than liquids suggests some degree of esophageal obstruction (eg, foreign body, food bolus, esophageal tumor [rare], vascular ring [rare])

•Dysphagia that is equal for solids and liquids suggests esophageal dysmotility (eg, achalasia)

Physical examination — The child with dysphagia should undergo a thorough general physical examination, initially focusing on the patient's cardiopulmonary status. Patients with upper airway obstruction and/or serious respiratory distress require emergency stabilization (see 'Stabilization' above). Additional findings of particular importance include:

●Oropharynx and neck – In the stable dysphagic patient, inspection of the oral cavity, pharynx, and neck may reveal a cyst, mass, localized infection, trismus, crepitus, or inflammatory cause for dysphagia. Specific findings are discussed in the conditions described above.

●Chest and lungs – The pulmonary examination may detect signs of aspiration, including rales, rhonchi, decreased breath sounds, expiratory wheezing, or hoarse voice.

●Neurologic status – Neurologic examination may reveal an altered level of arousal from an underlying brain injury or depressed sensorium from drugs or infection that may limit effective swallowing. Examination of the cranial nerves, particularly V, VII, IX, X, and XII, may reveal deficits that negatively impact swallowing.

Evaluation of muscle tone, strength, and reflexes is important to identify other neuromuscular causes of dysphagia.

Ancillary studies — Children with an acute onset of dysphagia require an urgent approach to diagnosis. In most patients, a history and physical examination will identify the most likely etiology. Additional studies provide confirmation of the clinical diagnosis for each condition. (See 'Differential diagnosis' above.)

Laboratory studies — After obtaining a history and physical examination, the laboratory evaluation is determined by the most likely cause of dysphagia as described above for each specific condition (see 'Differential diagnosis' above). Laboratory studies may be limited or unnecessary for many common conditions (eg, stomatitis, mild oropharyngeal trauma, infectious pharyngitis, or dystonic reaction).

Imaging — Initial radiographic evaluation of the dysphagic child typically includes:

●Neck radiograph – Anterior-posterior (AP) and lateral plain films of the airway and soft tissues of the neck, looking for evidence of a foreign body, mass, airway impingement, air in the retropharyngeal region or subcutaneous tissues, epiglottitis, tracheitis, thickening of the prevertebral soft tissues, or other abnormality.

●Chest radiograph – AP and lateral chest radiograph to identify signs of an esophageal foreign body, aspiration pneumonia, congenital heart disease, subcutaneous air from mediastinitis, mediastinal mass, or, as in the patient with achalasia, dilated esophagus with air-fluid levels. For patients with a mediastinal mass on chest radiograph, ultrasonography of the chest may be useful to identify whether the mass is solid or liquid. Ultrasonography provides less information than chest CT, but can be done while the patient is upright, whereas chest CT requires supine positioning, which risks acute respiratory decompensation. In most cases, CT will ultimately be required but can be accomplished in a controlled, well-monitored setting, as determined by the appropriate specialist who will provide definitive care.

Other radiographic studies may be indicated based upon clinical presentation or findings on plain radiograph:

●Esophageal foreign body – For patients with a radiopaque esophageal foreign body on plain radiograph, consultation with a pediatric gastroenterologist or, if the foreign body is impinging on the airway, a pediatric otolaryngologist is usually the next step to facilitate endoscopic removal with urgency of removal determined by the type and location of the foreign body. (See "Foreign bodies of the esophagus and gastrointestinal tract in children", section on 'Indications for urgent removal'.)

Occasionally water-soluble contrast fluoroscopic studies or CT scan are necessary to confirm suspected nonradiopaque foreign bodies. (See "Foreign bodies of the esophagus and gastrointestinal tract in children", section on 'Approaches for specific types of foreign bodies' and "Emergency evaluation of acute upper airway obstruction in children", section on 'Determining the cause of upper airway obstruction'.)

●Retropharyngeal abscess or mediastinitis – For patients with suspected retropharyngeal abscess or mediastinitis, CT scan of the neck or chest provides diagnostic confirmation. Procedural sedation may be required to obtain imaging in young children, and thus, control of the airway may be necessary for safe performance of these studies.

●Increased intracranial pressure or focal neurologic findings – Patients with evidence of increased intracranial pressure or focal neurologic signs suggesting a brain abscess or tumor should undergo neuroimaging.

Although cervical ultrasonography has been used to identify abnormalities with the tissues and function of the palate, tongue, and floor of the mouth, it is less useful than contrast studies for assessing airway problems and aspiration.

Other diagnostic studies — Upper endoscopy is an essential procedure in patients who have ingested a caustic agent or have evidence of an obstructing foreign body. Optimal timing of endoscopy depends on the clinical setting. (See "Caustic esophageal injury in children", section on 'Initial evaluation' and "Foreign bodies of the esophagus and gastrointestinal tract in children", section on 'Approach to management'.)

Patients with suspected mediastinitis require emergency surgical consultation for upper endoscopy and bronchoscopy. (See 'Esophageal perforation' above.)

Esophagitis is confirmed by elective upper gastrointestinal endoscopy. In addition to upper endoscopy, achalasia is confirmed by esophageal manometry.

DIAGNOSTIC APPROACH — The approach to children with dysphagia is provided in the algorithms (algorithm 5A and algorithm 5B). Evaluation and management are strongly determined by the history, presence of respiratory compromise, fever, and clinical findings.

Respiratory compromise — Children with cyanosis or respiratory distress should receive 100% oxygen and receive no oral intake until fully evaluated and clinically stable.

Children with life-threatening upper airway obstruction require assessment and treatment as presented in the algorithms (algorithm 4A and algorithm 4B and algorithm 6).

Children with evidence of respiratory failure should receive support of breathing with bag-valve-mask ventilation and undergo endotracheal intubation (table 6). (See "Technique of emergency endotracheal intubation in children" and "Rapid sequence intubation (RSI) in children for emergency medicine: Approach".)

Dysphagia with fever — The clinician should evaluate these patients for oropharyngeal, parapharyngeal, and upper airway infection in particular (algorithm 5A). Other diagnoses, such as mediastinitis, are also possible.

●Epiglottitis manifests as aphonia, drooling, and severe respiratory distress with signs of upper airway obstruction. (See 'Acute epiglottitis' above.)

●Trismus and unilateral peritonsillar swelling with a "hot potato" (muffled, hypernasal) voice identifies peritonsillar abscess (PTA). (See 'Peritonsillar abscess' above.)

●Drooling, stiff neck, lateral neck pain, and/or pain with neck extension suggests retropharyngeal or parapharyngeal infection. (See 'Retropharyngeal cellulitis and abscess' above.)

●Fever, severe substernal pain, crepitus, and/or shock after a caustic ingestion, chronic esophageal foreign body, or esophageal procedure are manifestations of esophageal perforation with mediastinitis. (See 'Esophageal perforation' above.)

●Severe trismus, opisthotonos, and myotonia worsened by loud noise in an unimmunized or underimmunized child is diagnostic for tetanus. The presence of fever may be variable. (See "Tetanus".)

●The presence of a pseudomembrane on oropharyngeal examination suggests diphtheria. (See 'Diphtheria' above.)

●Exudative tonsillitis with pain on swallowing indicates acute pharyngitis.

●Painful mucosal ulcers are characteristic for stomatitis or Stevens-Johnson syndrome (SJS). The presence of fever may be variable.

●Acute thyroid enlargement with respiratory compromise is seen in patients with acute suppurative thyroiditis. The presence of fever may be variable.

●Infections, such as poliomyelitis, meningitis, or encephalitis may cause neurologic dysfunction (cranial nerve palsy or altered mental status) with impairment of swallowing.

●Patients with impaired swallowing from a chronic neurologic disorder (eg, cerebral palsy, traumatic brain injury) require assessment for aspiration pneumonia. (See 'Other neurologic conditions' above.)

Dysphagia without fever — Although typically evident from the history, an acute onset of dysphagia in an otherwise healthy child should raise suspicion for an esophageal foreign body, caustic ingestion, or thermal esophageal burn (algorithm 5B). Clinical features that identify other etiologies in the afebrile child with dysphagia include:

●Oral bleeding, puncture wound, or laceration (oropharyngeal trauma)

●Intermittent dysphagia in association with oculogyric crisis, dysarthria, trismus, or torticollis (dystonic reaction)

●Odynophagia exacerbated by eating or certain oral medications (eg, doxycycline) (esophagitis)

●Skin changes, arthritis, and rash characteristic of rheumatic disease (juvenile systemic sclerosis [JSSc], dermatomyositis)

●Ptosis, bulbar weakness exacerbated by eating (eg, botulism, myasthenia gravis)

●Cranial nerve dysfunction in association with visual disturbance, ataxia, and/or headache (central nervous system tumor)

●Dysphagia for solids more than liquids in association with an esophageal obstruction (eg, foreign body, food bolus, esophageal tumor [rare], vascular ring [rare])

●Dysphagia for solids and liquids in association with abnormal esophageal motility on barium swallow and/or manometry (achalasia)

●Dysphagia with no apparent anatomic or physiologic cause (globus sensation)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topic (see "Patient education: Esophagitis (The Basics)")

SUMMARY AND RECOMMENDATIONS

●Definition – Dysphagia is defined as any difficulty or abnormality of swallowing. Dysphagia is a symptom of other conditions some of which may be life-threatening (table 1). Pain on swallowing (odynophagia) commonly accompanies dysphagia. Drooling (sialorrhea) is an indicator of potentially life-threatening upper airway or esophageal obstruction.

●Stabilization – The initial evaluation of children with signs and symptoms of dysphagia must begin with a rapid assessment of respiratory status to identify those who need resuscitation. Conditions that require immediate intervention include the following (see 'Stabilization' above):

•Complete upper airway obstruction

•Rapidly progressing partial airway obstruction

•Respiratory failure

•Mediastinal mass with tracheal impingement

Priorities for these patients include providing supplemental oxygen, support of breathing, and definitive airway management (algorithm 4A and algorithm 4B and table 6).

●Evaluation

•Evaluation is strongly determined by the presence of respiratory compromise, fever, and clinical findings as shown in the algorithms (algorithm 5A-B). (See 'Evaluation' above and 'Diagnostic approach' above.)

•Key historical features include (see 'History' above):

-Presence of fever, especially with oral lesions, drooling, sore throat, trismus, or stiff neck

-Witnessed foreign body ingestion (or missing small foreign bodies or "button" batteries)

-Known or suspected caustic ingestion

-Pain exacerbated by eating suggesting gastroesophageal reflux and/or esophagitis

-Substernal pain after taking an oral medication, especially tetracycline antibiotics (eg, doxycycline)

-Dysphagia for solids more than liquids (suggests esophageal obstruction)

-Dysphagia that is equal for solids and liquids (suggests achalasia)

•Once the airway is stabilized, as needed, the dysphagic patient should undergo a complete physical examination with special emphasis on the oropharynx, neck, lungs, and neurologic examination. (See 'Physical examination' above.)

•A complete history and physical examination should suggest the diagnosis in most patients. Additional studies, including imaging in selected patients, provide confirmation of the clinical diagnosis for each condition and guide definitive management. (See 'Ancillary studies' above and 'Differential diagnosis' above.)

ACKNOWLEDGMENT — The editorial staff at UpToDate would like to acknowledge Ronald A Furnival, MD, who contributed to earlier versions of this topic review.