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Stertor, Stridor, and Airway Obstruction

Stertor, Stridor, and Airway Obstruction: Excerpt from The Diagnostic Approach to Symptoms and Signs in Pediatrics

Stertoris noisy, snoring-type breathing that results from airway obstructionin the nose, nasopharynx, or oropharynx. The sounds are heard onlyduring inspiration.

Stridor is a low- to medium-pitchedsound that signifies airway obstruction in the supraglottic, glottic,subglottic, or tracheal regions. Any obstruction in the extrathoracicairway including the upper trachea is likely to cause inspiratorystridor, whereas expiratory stridor is usually generated withinthe intrathoracic trachea.

In many cases, careful history andphysical exam can identify level of lesion. Precise diagnosis mustbe sought in every child because of danger of further compromiseof the airway. With rare exceptions, only 1 diagnosis exists.

Principal Causes of Airway Obstruction

1. Noseand nasopharynx
   1. Congenital
      1. Choanal atresia
      2. Craniofacial anomalies
      3. Midline masses
   2. Infection/inflammation
      1. Rhinitis
      2. Adenoid hypertrophy
      3. Polyps
   3. Trauma
   4. Neoplasm
2. Oropharynx and hypopharynx
   1. Congenital
      1. Micrognathiaand other skull base abnormalities
      2. Macroglossia
      3. Decreased pharyngeal muscle tone
   2. Infection/inflammation
      1. Tonsillarhypertrophy
      2. Abscess
   3. Foreign body
   4. Trauma
   5. Neoplasm
3. Supraglottic
   1. Congenital
      1. Laryngomalacia
      2. Laryngeal cyst and laryngocele
   2. Infection/inflammation
      1. Supraglottitis
      2. Gastroesophageal reflux
      3. Hereditary angioedema
   3. Trauma
   4. Neoplasm
4. Glottic
   1. Congenital
      1. Laryngeal web
      2. Laryngeal cleft
      3. Vocal cord paralysis
   2. Infection/inflammation
      1. Laryngitis
      2. Laryngeal spasm
   3. Foreign body
   4. Trauma
   5. Neoplasm
5. Subglottic
   1. Congenital
      1. Subglottic stenosis
      2. Cysts
   2. Infection/inflammation
      1. Croup
      2. Bacterial tracheitis
   3. Trauma
   4. Neoplasm
6. Tracheobronchial
   1. Congenital
      1. Tracheomalacia
      2. Tracheal web
      3. Tracheal cysts
      4. Tracheal stenosis
      5. Vascular anomalies
   2. Infection/inflammation
   3. Foreign body
   4. Trauma
   5. Neoplasm
      1. Tracheal
      2. Thyroid
      3. Mediastinal masses
7. Psychogenic

Clinical Features and Diagnosis

Nose and Nasopharynx

Congenital

Choanal Atresia

Causes obstructionof nasopharynx that may be unilateral or bilateral.

Respiratory distress occurs with bilateraldisease unless the mouth is open because neonates are obligate nosebreathers.

Best diagnostic test is flexible endoscopy.CT is used to delineate anatomy prior to surgery.

Craniofacial Anomalies

2 autosomal-dominantdisorders, Apert syndrome and Crouzon syndrome, are caused by mutationsin gene encoding fibroblast growth factor receptor-2 on chromosome10q26. In both disorders, midface hypoplasia and shape of palatecontribute to airway obstruction.

Apert syndrome is characterized byacrobrachycephaly, coronal craniosynostosis, shallow orbits, proptosis,hypertelorism, cutaneous and osseous syndactyly, broad distal phalanxof thumb and hallux of big toe, and variable mental retardation.

Characteristic features of Crouzonsyndrome include craniosynostosis (especially of coronal, sagittal,and lambdoid sutures), shallow orbits, proptosis, hypertelorism,parrot-beaked nose, and variable mental retardation.

Midline Masses

Nasal dermoidcyst may be suspected when pit or swelling is seen on dorsum ofnose in midline.

Encephalocele is herniation of meningesand brain tissue into nasal area producing mass or swelling.

CT demonstrates location and extentof these lesions. Biopsy is never performed if there is suspicionof intracranial communication.

Infection/Inflammation

Rhinitis

Infectious or allergic rhinitis may causeinflammation and edema of nasal mucosa, causing upper airway obstruction(see Chap. 41, Nasal Discharge).

Adenoid Hypertrophy

Recurrentviral URIs and allergic rhinitis contribute to adenoid hypertrophy.

Persistent snoring is common finding,and sleep apnea also may occur.

History, physical exam, and lateralneck radiograph confirm diagnosis.

Polyps

Nasal polyps can be visualized on nasal examas gray, smooth, soft, mobile masses. Predisposing causes are allergicrhinitis and cystic fibrosis.

Trauma

Trauma tonose and nasopharynx may produce significant swelling and bleeding, whichcause airway obstruction.

Plain radiographs and CT provide valuablediagnostic information.

Neoplasm

In childhood,most common benign tumors of nasal cavity are hemangioma and lymphangioma.In adolescence, most frequent benign tumor is juvenile angiofibroma.Malignant tumors include rhabdomyosarcoma, carcinoma, and lymphoma.

Common presenting feature of thesetumors is nasal obstruction.

Nasal speculum can be used to examinenasal cavity, while flexible nasopharyngoscopy permits visualizationof nasopharynx.

CT of nasal cavity, nasopharynx, andsinuses locates mass and defines its extent. Histologic diagnosisis definitive.

Oropharynx and Hypopharynx

Congenital

Micrognathia and Other Skull Base Abnormalities

Althoughmicrognathia can occur as isolated lesion, also can occur as partof Pierre Robin syndrome, which includes glossoptosis and cleftpalate.

Another disorder with significant micrognathiais autosomal-dominant Treacher-Collins-Franceschetti syndrome, whosegene locus has been mapped to chromosome 5q32-33.1. Other characteristicfindings include downward slanting palpebral fissures, colobomaof lower lid, malar hypoplasia, and malformations of external ear(commonly microtia).

Macroglossia

Can be associated with congenital hypothyroidism,Beckwith-Wiedemann syndrome, Hurler syndrome, and glycogen storagedisease type II. Diagnosis may be suspected from history and physicalexam. These disorders are discussed in other chapters.

Decreased Pharyngeal Muscle Tone

Decreased tone of pharyngeal musculaturemay contribute to noisy breathing, especially with cerebral palsyand trisomy 21.

Infection/Inflammation

Tonsillar Hypertrophy

Hypertrophy of tonsils may be seen with recurrentviral URI or allergic rhinitis. Tonsils are enlarged and may causeupper airway obstruction.

Abscess

Peritonsillar, retropharyngeal, or lateralpharyngeal abscess may cause fever, difficulty swallowing, drooling,neck pain, and respiratory distress, including stridor (see Chap. 61, Sore Throat).

Foreign Body

Foreignbody in pharynx may cause acute onset of choking and airway obstruction.

History and physical exam can be diagnostic;however, in some cases neck radiographs and nasopharyngoscopy maybe needed to locate the foreign body.

Trauma

Any facialor neck injury can cause edema or hematoma formation that compromises airway.

History, physical exam, radiographsof face and neck, and nasopharyngoscopy are usually diagnostic.

Laryngoscopy and bronchoscopy alsomay be necessary depending on extent of injury. Early tracheostomyshould be considered.

Inhalation injury from fire may causesevere edema of mouth, pharynx, and larynx, producing combinationof cough, hoarseness, and stridor. Visualization of larynx may beaccomplished by endoscopy.

Neoplasm

Benign tumorsof tongue include hemangioma and lymphangioma. Most common malignanttumor of tongue is rhabdomyosarcoma.

Hemangioma can usually be diagnosedclinically.

CT or MRI defines extent of mass. Histologicdiagnosis is definitive.

Supraglottic

Congenital

Laryngomalacia

Most commoncongenital anomaly affecting larynx.

Inward collapse of aryepiglottic foldsis usual mechanism of stridor, which may occur at birth or may bedelayed for a few weeks.

Respiratory distress and feeding difficultiesoccasionally occur. Stridor often improves with placement in proneposition, whereas agitation makes it worse.

Flexible endoscopy is diagnostic procedureof choice.

Resolution usually occurs in firstyear of life when these tissues become firmer. Only in extreme casesis tracheostomy required.

Laryngeal Cyst and Laryngocele

Unusuallesions that are thought to arise from saccule of laryngeal ventricle.

Fluid-filled mass is called cyst andair-filled mass is called laryngocele.

Cyst usually causes airway obstructionat birth, whereas laryngocele usually causes airway problems laterin infancy.

Lateral neck radiography shows massthat may be seen by flexible laryngoscopy. CT and MRI are usefulin determination of location and extent of mass.

Infection/Inflammation

Supraglottitis

Infectionof supraglottic tissues that includes epiglottis.

Generally caused by infection of encapsulatedorganisms.

Usual age range is 1–5 yrs.

Since introduction of H. influenzaetype b vaccine, occurrence of epiglottitis has markedly decreased.Now organisms usually cultured are S. pneumoniae, S. aureus, nontypeableH. influenzae, and group A Streptococcus.

Onset is acute, with fever, inspiratorystridor, pain with swallowing, and drooling.

Laryngoscopy should be performed toconfirm diagnosis but should be done with utmost caution and withanesthesiologist or otolaryngologist present.

Gastroesophageal Reflux

Can be associatedwith coughing, gagging, and choking.

Persistent stridor may occur as consequenceof severe gastroesophageal reflux, and laryngoscopic visualizationof upper airway may reveal inflammatory changes (see Chap. 55, Regurgitation and Vomiting).

Hereditary Angioedema

Laryngeal edema can occur in this autosomal-dominantdisorder in which serum levels of C1 inhibitor are low or its functionis defective (see Chap. 17, Edema).

Trauma

Supraglottic stenosis may be due to traumaor may result from surgical procedures involving the airway.

Neoplasm

Laryngealpapillomas are caused by human papilloma viruses, which can be transmittedduring birth from infected mother to newborn.

They may occur anywhere in airway,but most common location is larynx.

Flexible laryngoscopy reveals mass,and histologic diagnosis is confirmatory.

Glottic

Congenital

Laryngeal Web

Incomplete canalization of laryngeal airwayresults in formation of laryngeal web. This lesion usually occursat glottic level and produces aphonia or weak or husky voice. Stridoralso may occur, and laryngoscopy is diagnostic.

Laryngeal Cleft

Cleft inlarynx is produced by failure of rostral development of tracheoesophageal septum.

Stridor is often heard, and attemptsat feeding produce choking and coughing. Recurrent aspiration mayresult in pneumonia.

Esophagraphy shows spillage of contrastinto trachea with swallowing. Endoscopy is confirmatory.

Vocal Cord Paralysis

Vagus nerveby means of recurrent laryngeal nerve helps control vocal cord movement.Any lesion that affects these nerves may cause unilateral or bilateralvocal cord paralysis, which may be congenital or acquired.

When 1 vocal cord is involved, airwaycompromise is usually minimal and weak or hoarse cry occurs. Onlywith periods of agitation or stress does stridor appear. Causesof unilateral vocal cord paralysis include recurrent laryngeal nervecompression or injury secondary to neck or mediastinal mass, enlargedleft atrium or pulmonary artery, and surgery, particularly cardiovascularor neck surgery.

Stridor is characteristic of bilateralvocal cord paralysis. Cause is usually CNS lesion (e.g., hydrocephalus,intracranial hemorrhage, or Chiari malformation).

Diagnosis of vocal cord paralysis canbe made by flexible laryngoscopy. Vocal cord(s) fails to abducton inspiration. Further investigation depends on suspected cause.

Infection/Inflammation

Laryngitis

Usually associated with hoarseness and notwith stridor, unless inflammation is so severe that airway obstructionoccurs (see Chap. 31, Hoarseness).

Laryngeal Spasm

May be caused by repeated attempts at intubation,allergic reactions, and hypocalcemia. Manifestations of allergicreaction include urticaria, wheezing, and hypotension. Carpopedalspasm suggests tetany, in which case serum calcium should be measuredand intravenous calcium given immediately.

Foreign Body

Foreign body in glottic area may cause acuteonset of choking and coughing. Persistent stridor may be only manifestationof retained foreign body. Diagnosis may be suspected from historyand confirmed by neck radiography or laryngoscopy.

Trauma

Granuloma formation may occur secondary toendotracheal intubation. Obstruction can be minimal or severe.

Neoplasm

Laryngeal papillomas are discussed in thesection Supraglottic: Neoplasm.

Subglottic

Congenital

Subglottic Stenosis

Biphasicstridor usually occurs with congenital subglottic stenosis, yetvoice remains normal.

Neck radiography may demonstrate subglotticnarrowing.

Diagnosis is confirmed by rigid endoscopyunder general anesthesia.

Cysts

Subglottic cysts can be congenital or occursecondary to airway intubation. Rigid endoscopy under general anesthesiais diagnostic.

Infection/Inflammation

Croup

In viralcroup subglottic edema produces airway obstruction and stridor.Most common cause is parainfluenza viral infection. Influenza viruses,rhinoviruses, respiratory syncytial virus, adenoviruses, and enterovirusesalso can cause croup. Age range is usually 6–36 mos.

Symptoms of upper respiratory infectionare followed in 12–24 hrs by barky cough and stridor. Feveris variable finding. This is usually self-limited illness lasting3–7 days.

Neck radiography may be performed ifpresentation is unusual or puzzling and diagnosis is uncertain. "Steeplesign" indicates airway obstruction and is consistent withdiagnosis of croup.

Except for signs of infection, spasmodiccroup cannot be distinguished from viral croup. Onset is sudden,and episodes usually occur during night. Although pathogenesis remainsuncertain, episodes usually resolve during day and subside in afew days. Diagnosis is clinical.

Bacterial Tracheitis

Usuallyfollows preexisting viral infection.

Usual pathogens are S. aureus, S. pneumoniae,M. catarrhalis, and nontypeable H. influenzae.

Average age of onset is 5 yrs.

Clinical presentation is similar tothat of viral croup except these children appear more toxic. Theyhave worsening stridor with purulent tracheal secretions and areless responsive to racemic epinephrine. In most cases, trachealintubation is required because of severe airway obstruction.

Radiography shows subglottic edemaand irregular tracheal densities. Flexible nasopharyngoscopy canidentify purulent secretions in airway, and rigid endoscopy shouldbe performed for debridement. This diagnosis should be consideredin older child with unusually severe croup.

Trauma

Acquiredsubglottic stenosis is usually complication of prolonged endotrachealintubation and is generally more severe than congenital subglotticstenosis.

Although airway films may be diagnostic,flexible endoscopy should be performed to visualize larynx and otherstructures above vocal cords. Occasionally, view of subglottis ispossible. Rigid endoscopy confirms diagnosis.

Neoplasm

Subglottichemangioma is benign tumor that usually becomes symptomatic by 3–6 mosof age.

Stridor may be inspiratory or biphasic.Barky cough and hoarseness also may occur.

Neck radiography may show asymmetricsubglottic narrowing. CT or MRI is used to determine extent of thelesion.

Diagnosis is confirmed by endoscopicexam under general anesthesia.

Tracheobronchial

Congenital

Tracheomalacia

Presenceof misshapen or deficient tracheal cartilage rings contributes toabnormal collapse of trachea, called tracheomalacia.

Onset is insidious and often episodic.Crying, coughing or feeding may lead to acute exacerbation. Stridoris typically expiratory. Cartilage becomes firmer with growth, andstridor usually resolves by 12–18 mos of age.

Diagnosis can be confirmed by fluoroscopyor bronchoscopy, which demonstrates inspiratory collapse of extrathoracictrachea and expiratory collapse of intrathoracic trachea.

Tracheal Web

Local narrowing of trachea produced by fibroustissue, and bronchoscopy is diagnostic.

Tracheal Cysts

Usuallyasymptomatic, but large or infected ones may produce stridor and cough.

Chest radiography usually shows cysticmediastinal structure adjacent to trachea.

Other useful diagnostic tests includeesophagraphy, CT, and bronchoscopy.

Tracheal Stenosis

Fibrousstrictures that narrow airway may occur anywhere in trachea, andbronchoscopy is diagnostic.

Tracheal ring involves individual trachealrings or segmental areas of trachea that may not increase in sizewith growth. Rings resemble cartilage and lack membranous posteriorsegment.

Chest radiography may show narrow orfunnel-shaped trachea. Bronchoscopy confirms diagnosis.

Vascular Anomalies

Double aorticarch can compress trachea and esophagus and cause stridor, dysphagia, andapnea. Esophagraphy shows characteristic indentations of esophagus,with higher and larger indentation caused by right aortic arch andlower indentation caused by left aortic arch. MRI is confirmatory.

Anomalous innominate artery originatesmore distally from aortic arch than normal and compresses rightanterior aspect of trachea just above carina. Stridor, persistentcough, and apnea may occur. Chest radiography shows anterior indentationof trachea. Demonstration of anterior compression of distal tracheaby pulsatile vessel at bronchoscopy is diagnostic.

In pulmonary artery sling, left pulmonaryartery arises as branch of right pulmonary artery, courses betweentrachea and esophagus to reach left lung, and compresses right mainbronchus and esophagus. Chest radiograph shows anterior bowing ofright main bronchus, deviation of lower trachea to the left, unequalaeration of lungs, and mediastinal density between trachea and esophagus.Esophagraphy may show anterior indentation of esophagus just abovelevel of carina. MRI or cardiac catheterization with pulmonary angiographyis diagnostic.

Infection/Inflammation

Retroesophagealabscess may cause fever and stridor due to compression of trachea.

Chest radiography, esophagraphy, andCT are useful in diagnosis. Confirmation of diagnosis may only bepossible at surgery.

Foreign Body

Trachealforeign bodies may produce choking and gagging followed by persistent coughingand stridor. Radiopaque foreign body may be recognized on chestradiograph; otherwise, bronchoscopy is confirmatory.

Esophageal foreign body may compresstrachea and cause stridor, cough, and respiratory distress, sometimeswithout any esophageal symptoms (e.g., drooling, dysphagia, or pain).

Chest radiography may show its location,whereas esophagraphy usually shows filling defect.

Esophagoscopy is confirmatory.

Trauma

Tracheal stenosis may occur from complicationsof intubation or, less commonly, tracheostomy. Bronchoscopy is diagnostic.

Neoplasm

Tracheal

Rare inpediatric population.

Benign tumors include squamous papilloma,chondroma, and hemangioma, whereas malignant tumors include varioussarcomas and carcinomas.

Presenting symptoms include persistentcough, expiratory stridor, and hemoptysis.

Chest and airway radiography may identifymass lesion. Combination of tests that includes bronchoscopy, CT,and MRI can locate site and extent of lesion.

Histologic diagnosis is definitive.

Thyroid

Thyroid neoplasm may compress trachea andcause stridor. CT can demonstrate compression. See further discussionof thyroid neoplasms in Chap.42, Neck Masses.

Mediastinal Masses

Some mediastinalmasses including teratoma, lymphangioma, and lymphoma may compresstrachea and cause stridor.

Combination of tests, including chestCT and MRI as well as bronchoscopy, can locate and determine extentof lesion.

Histologic diagnosis is definitive.

Psychogenic

In pediatricpopulation, this type of stridor is most common in adolescent girls.Often history of psychologic stress exists.

Stridor may be heard in inspiration,expiration or both. Associated findings include cough, hoarseness,and dyspnea.

In some cases, flexible laryngoscopymay reveal paradoxic vocal cord motion, in which vocal cords adductrather than abduct in inspiration.

Diagnostic Approach

Stertorand stridor are signs of upper airway obstruction. With seriousairway obstruction, establishing control of the airway and maintainingventilation are the most important immediate priorities. In absenceof life-threatening airway obstruction, prompt but thorough investigationis essential.

Age of onset is useful in diagnosis.Onset of stridor at birth or during neonatal period suggests laryngomalacia,laryngeal web, vocal cord paralysis, or vascular ring. In infancyand childhood, onset of acute stridor suggests croup, supraglottitis,or foreign body. Most common cause of persistent stertor in children2–8 yrs of age is adenoid or tonsillar hypertrophy.

Phase of respiration in which stridoroccurs, pitch of the sound, character of voice or cry, and changein position help in assessing degree of obstruction and its localization.

The phaseof respiration associated with different levels of airway obstructionhas already been described.

Variation in pitch depends on leveland degree of obstruction. Coarse low-pitched snoring sounds (stertor)localize lesion to nose, nasopharynx, or oropharynx. Harsh inspiratorystridor may occur with supraglottic, glottic, or subglottic lesions.Stridor associated with deep barking cough signifies subglotticor tracheal obstruction, whereas stridor associated with hoarsenessor change in character of voice or cry signifies glottic lesion.

When infants with laryngomalacia orinnominate artery compression are placed in prone position withneck extended, stridor decreases.

After history and physical exam, othertests may be useful depending on suspected diagnosis. These includeneck and chest radiography and flexible laryngoscopy. If resultsof these tests are normal, upper GI radiographic series with attentionto the pharynx and esophagus should be considered. With suspectedobstruction below glottis, bronchoscopy is necessary. Usefulnessof esophagoscopy, CT, and MRI depends on suspected diagnosis.

Histologic diagnosis is necessary forany suspected neoplasm except perhaps hemangioma, which can usuallybe recognized clinically.

References

1. Cotton RT, Myer CM III, eds. Practicalpediatric otolaryngology. Philadelphia: Lippincott-Raven, 1999.
2. Fleisher GR, Ludwig S, eds. Textbook of pediatric emergencymedicine, 4th ed. Philadelphia: Lippincott Williams & Wilkins,2000.
3. Jones KL. Smith's recognizable patterns ofhuman malformation, 5th ed. Philadelphia: WB Saunders, 1997.
4. Kirks DR, ed. Practical pediatric imaging: diagnosticradiology of infants and children, 3rd ed. Philadelphia: Lippincott-Raven,1998.
5. Kissoon N, et al. Psychogenic upper airway obstruction.Pediatrics 1988;81:714–717.
6. Kuppersmith R, et al. Functional stridor in adolescents.J Adolesc Health Care 1993;14:166–171.
7. Long SS, et al., eds. Principles and practice of pediatricinfectious diseases. New York; Churchill Livingstone, 1997.
8. Myer CM III, Cotton RT. Pediatric airway and laryngealproblems. In: Lee KJ, ed. Textbook of otolaryngology and head andneck surgery. New York: Elsevier, 1989:658–673.
9. Nielson D, et al. Stridor and gastroesophageal refluxin infants. Pediatrics 1990;85:1034–1039.
10. 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.
11. Rudolph AM, ed. Rudolph's pediatrics, 20thed. Stamford, CT: Appleton & Lange, 1996.

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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