Congenital Anomalies of The Larynx

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Congenital anomalies of the larynx

Author: Glenn C Isaacson, MD, FAAP
Section Editor: Anna H Messner, MD
Deputy Editor: Carrie Armsby, MD, MPH
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Aug 2020. | This topic last updated: Dec 03, 2018.
INTRODUCTION
Congenital anomalies are the product of errors in embryogenesis (malformations) or the result of intrauterine events that affect
embryonic and fetal growth (deformations and disruptions) [1]. The more complex the formation of a structure, the more
opportunities for malformation.
Defects in the formation and growth of the larynx lead to a variety of malformations. The embryology, clinical features, and
management of congenital anomalies of the larynx are reviewed here. Congenital anomalies of the intrathoracic airways are
discussed separately. (See "Congenital anomalies of the intrathoracic airways and tracheoesophageal fistula".)
EMBRYOLOGY
The formation of a median pharyngeal groove presages the appearance of the respiratory tract. At approximately 25 days of
intrauterine life, the anlagen of the larynx, trachea, bronchi, and lungs arise from a ventromedial diverticulum of the foregut
called the tracheobronchial groove. The cartilage of the trachea and connective tissue and muscle of the trachea and
esophagus are derived from splanchnic mesenchyme. Lateral furrows develop on each side of the ventromedial diverticulum,
deepen, and join to form the tracheoesophageal septum [2-4].
The distal esophagus can be distinguished from the stomach and the laryngeal primordia appear at approximately 33 days of
intrauterine life. The T-shaped laryngeal slit (aditus) is formed anteriorly by the growth of the primordium of the epiglottis (from
the hypobranchial eminence, arches III and IV) and laterally by the precursors of the arytenoid cartilages (ventral ends of arch
VI) [2-4].
During the fifth and sixth weeks, the tracheoesophageal septum extends to the first tracheal ring. By the time the embryo is 13
to 17 mm in length, laryngeal cartilage and muscle development are clearly identifiable, and lateral cricoid condensation is
underway. By the seventh week of development, the cricoid ring is complete, and the cartilaginous hyoid is visible below the
epiglottis (picture 1). Definitive tracheal cartilage appears at this stage and the esophagus has four discrete layers. The larynx,
trachea, and esophagus are well formed by the end of the embryologic period (image 1 and picture 2) [2-4].
LARYNGOMALACIA
Laryngomalacia refers to collapse of the supraglottic structures during inspiration (movie 1 and picture 3) [5,6]. It is the most
common congenital anomaly of the larynx [7,8]. It is distinct from tracheomalacia (an abnormally compliant trachea), which is far
less common. Laryngomalacia causes inspiratory stridor, whereas tracheomalacia causes expiratory or biphasic stridor. In
addition, tracheomalacia is often associated with other congenital malformations such as trachea-esophageal fistula or vascular
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rings. Tracheomalacia is discussed in greater detail separately. (See "Congenital anomalies of the intrathoracic airways and
tracheoesophageal fistula", section on 'Tracheomalacia'.)
Etiology — The etiology of laryngomalacia is not clearly defined, and different mechanisms may apply in different infants.
Proposed mechanisms include [8-10]:
● Delayed maturation or "hypotonia" of the supporting cartilaginous structures of the larynx

● Redundant soft tissue in the supraglottis
● A foreshortened or tight aryepiglottic fold
● Underlying neuromuscular disorders
● Supraglottic inflammation or edema
Gastroesophageal reflux (GER) has been reported to occur more commonly in children with laryngomalacia compared with
other children [11]. Whether GER causes laryngomalacia is uncertain. It is quite possible that the two occur together because of
a common underlying mechanism (immature airway and sphincter tone), Nevertheless, in clinical practice it is widely noted that
antireflux medications seem to improve laryngomalacia symptoms. The reason for this effect is unclear since infants typically do
not produce much gastric acid.
In histologic studies, surgically excised supraglottic tissues from infants with laryngomalacia are not distinct from supraglottic
tissues from infants without laryngomalacia, weakening the "malacia" or soft-cartilage theory. The frequent concurrence of
gastroesophageal reflux, the variation in severity with state of consciousness, and the new onset of laryngomalacia after
neurologic injuries support the hypothesis that it is a neuromuscular disorder [9].
Clinical features — Laryngomalacia manifests with intermittent low-pitched "wet" inspiratory stridor, usually in the neonatal
period [7,8,12,13]. In most children with laryngomalacia, stridor is loudest at four to eight months of age and resolves by 12 to
18 months. Hoarseness is not a characteristic finding.
The degree of airway obstruction is dynamic and thus the intensity of stridor fluctuates. Stridor is more intense during upper
respiratory tract infections and is often worse in the supine position, improving when prone [7]. In infants with mild to moderate
laryngomalacia, the stridor is usually loudest when feeding or sleeping and may disappear completely when crying (although
this may be difficult to detect due to the noise of the cry). Patients with severe laryngomalacia, conversely, may have the loudest
stridor during crying [7]. In some infants, the stridor may be present only during sleep or relaxation (state-dependent
laryngomalacia) [12,13].
Other presenting features include [7,9,14-16]:
● Snoring and/or sleep-disordered breathing – 25 percent

● Swallowing dysfunction and/or feeding difficulties – 10 to 50 percent
● Gastroesophageal reflux and laryngopharyngeal reflux (reflux beyond the esophagus to the larynx, oropharynx, or
nasopharynx) – 60 to 70 percent
Children with mild forms of laryngomalacia breathe and feed well despite their noisy respiration. More severe forms result in
suprasternal or substernal retractions, poor feeding, sleep-disordered breathing, and failure to thrive [15,16].
Up to 20 percent of infants with laryngomalacia have additional airway anomalies, but life-threatening anomalies are uncommon
[5,17]. Laryngomalacia may be isolated or may occur in association with congenital syndromes (eg, Down syndrome, DiGeorge
(22q11 deletion) syndrome) or other non-airway anomalies [5]. (See "Down syndrome: Clinical features and diagnosis" and
"DiGeorge (22q11.2 deletion) syndrome: Clinical features and diagnosis".)
Diagnosis — The diagnosis of laryngomalacia is usually suspected based upon the history and physical examination [7]. It is
confirmed with flexible fiberoptic laryngoscopy (movie 1) [10]. An experienced pediatric care provider need not refer every child
with mild inspiratory stridor to a pediatric otolaryngologist. Children with significant or progressive stridor, apnea, cyanotic
episodes, or poor growth require endoscopic confirmation.
Once the diagnosis of laryngomalacia is confirmed with flexible fiberoptic laryngoscopy, the clinician should assess severity and
consider additional evaluation for associated airway abnormalities.
● Severity – The severity of laryngomalacia is determined by the degree and sequelae of airway obstruction and the response
to conservative interventions (eg, position change, feeding modifications, treatment for gastroesophageal reflux). For
example, intermittent noisy breathing in an infant who is otherwise feeding and growing well would be considered mild,
whereas an infant with considerable stridor associated with feeding difficulties, poor growth, apnea, or cyanotic episodes is
considered to have severe laryngomalacia.
● Associated anomalies – Ancillary airway evaluation (eg, direct laryngoscopy and bronchoscopy or flexible fiberoptic
bronchoscopy) for concurrent laryngotracheal malformations (eg, esophageal atresia, tracheoesophageal atresia) is
warranted in infants with cyanosis, apnea, hoarseness, feeding difficulty, poor growth, or atypical stridor [5,10,17,18].
Ancillary airway evaluation is lower yield in infants with less severe symptoms, and performing it as a routine in this setting
is controversial [5,10,17,18].
For children who have normal or equivocal findings on awake laryngoscopy yet have a clinical presentation highly suggestive of
laryngomalacia, drug-induced sleep endoscopy (DISE) may be useful to evaluate for state-dependent laryngomalacia [19].
Some children show signs of supraglottic collapse only during sleep. DISE can identify this variant and help define the areas of
supraglottic prolapse [20]. DISE is not without risk and thus should only be pursued in patients with considerable symptoms. We
do not advise DISE in young infants. DISE is most commonly used to evaluate older children with residual obstructive sleep
apnea following adenotonsillectomy. The procedure is discussed in greater detail separately. (See "Adenotonsillectomy for
obstructive sleep apnea in children", section on 'Drug-induced sleep endoscopy'.)
Numerous other potentially dangerous airway abnormalities may have similar clinical presentations. Hoarseness is not
characteristic of laryngomalacia. Other causes of stridor include subglottic stenosis, vocal cord paralysis, vascular ring, laryngeal
mass (eg, cyst or hemangioma), subglottic hemangioma, and tracheomalacia (table 1A-B). The assessment of stridor in children
is discussed separately. (See "Assessment of stridor in children".)
Management — The management of laryngomalacia depends upon the severity. In the majority of otherwise normal children,
laryngomalacia is not dangerous and resolves spontaneously.
● Mild laryngomalacia – Infants with mild laryngomalacia (intermittent mild stridor with no other symptoms) may be followed
clinically with frequent monitoring to make sure that weight gain is adequate [7,10]. Stridor usually resolves by 12 to 18
months of age.
● Moderate to severe laryngomalacia – Infants with moderate or severe laryngomalacia (stridor with feeding difficulty,
dyspnea, tachypnea, cyanosis, apnea) should be referred to an otolaryngologist for full endoscopic evaluation and possible
intervention [7,10]. Medical management may be adequate therapy for some infants with moderate laryngomalacia [21];
however, infants with severe laryngomalacia often require surgical intervention.
Medical management consists of acid suppression, speech and swallow therapy, and/or high-calorie formula. Infants known
to have associated GER should be treated for reflux [10]. Therapy for GER is often empiric in patients with laryngomalacia,
though a diagnostic evaluation may be appropriate for some children. Data supporting one approach versus the other are
extremely limited [22-25]. Feeding evaluation and speech therapy services can help guide interventions to improve feeding
(eg, texture modification, bottle pacing, augmenting feeding schedule). Management of GER and swallowing dysfunction
are discussed in greater detail separately. (See "Gastroesophageal reflux in infants", section on 'Treatment options' and
"Aspiration due to swallowing dysfunction in children", section on 'Management'.)
Children with severe laryngomalacia often benefit from surgery to remove redundant supraglottic tissue (picture 4). In
experienced hands, this surgery can produce dramatic improvements in breathing, feeding, and growth with little morbidity
[5,7,26-28]. Parents report improved quality of life for children undergoing supraglottoplasty [29]. Two meta-analyses found
that for children with obstructive sleep apnea due to laryngomalacia, supraglottoplasty is associated with improvement in
the apnea-hypopnea index and oxygen saturations; however, most children have residual symptoms after the procedure
[30,31]. (See "Adenotonsillectomy for obstructive sleep apnea in children", section on 'Supraglottoplasty'.)
Possible complications of supraglottoplasty include supraglottic or glottic scarring and subsequent chronic aspiration or
dysphonia, so surgery should not be undertaken for minimal indications (ie, to ease parental anxiety about noisy breathing).
The risks and benefits of supraglottoplasty should be carefully considered in children with underlying neuromuscular
disease. Neuromuscular disease is not a contraindication to supraglottoplasty, but the benefit of improving airway
obstruction must be weighed with the risk of worsening aspiration in this setting [10].
MALFORMATIONS
The complex structure of the larynx and its numerous folds and outpouchings predispose this region to congenital
malformations.
Cysts
Vallecular cysts — Congenital vallecular cysts contain respiratory epithelium and mucous glands. They arise in the
hypopharynx and are an uncommon cause of respiratory distress in infancy (picture 5 and picture 6) [32,33]. Infants with
vallecular cysts typically present with feeding difficulty, failure to thrive, stridor, noisy breathing, respiratory distress, or a hoarse
cry. Laryngomalacia is an associated finding [32-35]. Diagnosis is confirmed with endoscopy of the hypopharynx.
Definitive treatment requires marsupialization, which can be performed transorally with microscissors or laser, or through a
transhyoid resection [32,36-38]. Aspiration or rupture may provide temporary improvement in symptoms, but seldom provides
permanent treatment.
Laryngoceles and saccular cysts — Congenital laryngoceles and saccular cysts are abnormal dilations of the laryngeal
saccule and arise in the region of the laryngeal ventricle. Laryngoceles are outpouchings of the saccular mucosa. They may
remain within the confines of the cartilaginous larynx (internal laryngoceles) or extend through the thyrohyoid membrane to
present as neck masses (combined laryngoceles). Saccular cysts are fluid-filled submucosal cysts that are covered by a normal
mucous membrane and isolated from the interior of the larynx [39].
These anomalies typically are asymptomatic; however, they may cause upper airway obstruction (particularly in the neonate),
hoarseness, dysphagia, dyspnea, and laryngeal discomfort [40-43]. The symptoms caused by laryngoceles are episodic
because laryngoceles intermittently fill with air. In contrast, the symptoms produced by saccular cysts are constant [43].
Laryngoceles and saccular cysts usually are discovered incidentally during radiologic evaluation for unrelated symptoms.
Laryngoceles are visualized as fluid- and air-containing cystic masses on plain radiography, ultrasound, or computed
tomography (CT) [42]. CT is preferred for determination of the full extent of the lesion and its relation to the larynx.
Laryngeal atresia — Laryngeal atresia is thought to be caused by the failure of epithelial growth and recanalization in the
vestibule and subglottic regions [44].
Most infants with laryngeal atresia present with asphyxia at the time of birth. Performing an emergency tracheotomy soon after
delivery is necessary for survival because the imperforate larynx cannot be intubated [45]. If detected in utero, an ex utero
intrapartum treatment (EXIT) procedure with tracheotomy can be lifesaving [46].
Laryngeal webs — Laryngeal webs are rare congenital anomalies caused by failure of resorption of the epithelial layer that
obliterates the laryngeal opening in normal development. Failure of resorption results in incomplete separation of the vocal folds
(picture 7). Webs can occur anywhere along the anterior or posterior larynx. Laryngeal webs also can result from trauma to the
airway (eg, because of intubation, traumatic injury, or prior surgical manipulation). (See "Common causes of hoarseness in
children", section on 'Trauma'.)
Approximately 10 percent of patients with laryngeal webs have other associated anomalies, principally of the upper respiratory
tract (eg, subglottic stenosis, submucous cleft palate) [47,48]. In addition, laryngeal webs are associated with cardiac defects
(particularly ventricular septal defects [49,50]) and may occur in conjunction with 22q11 deletion as part of the
DiGeorge/velocardiofacial syndrome. (See "DiGeorge (22q11.2 deletion) syndrome: Epidemiology and pathogenesis" and
"Syndromes with craniofacial abnormalities", section on 'Velocardiofacial (Shprintzen) syndrome'.)
Patients with laryngeal webs usually present in infancy with respiratory distress and a weak or high-pitched cry [47,51].
Symptoms in those who present later in life include hoarse or weak voice, breathiness, and varying degrees of dyspnea and
stridor depending upon the degree of airway compromise. (See "Assessment of stridor in children".)
Treatment of laryngeal webs depends upon the degree of airway obstruction. Simple anterior commissure webs are treated
surgically, either by a simple lysis of the web with a CO2 laser or sharp dissection with a knife. Laryngeal reconstruction,
laryngeal stent, and tracheotomy may be indicated in patients with webs that include larger portions of the anterior and posterior
glottis.
Congenital subglottic stenosis — Congenital subglottic stenosis is a narrowing of the lumen of the cricoid region possibly
caused by incomplete canalization of the cricoid ring (diameter less than 4 mm in the term infant and 3 mm in the preterm infant)
[44,52]. It presents most commonly as recurrent croup but may cause biphasic stridor in a newborn if severe. It is differentiated
from acquired subglottic stenosis by the absence of history of trauma or instrumentation and by less severe symptoms. (See
"Complications and long-term pulmonary outcomes of bronchopulmonary dysplasia", section on 'Glottic and subglottic damage'.)
Congenital subglottic stenosis typically improves as the larynx grows. Fewer than one-half of the children with this disorder
require tracheostomy. Depending upon the severity of the obstruction and the need for supplemental oxygen therapy, congenital
subglottic stenosis also may be treated by anterior-cricoid split, endoscopic laser therapy, or endoscopic division of cartilage with
balloon dilation [44,53-55].
Laryngeal clefts — Posterior laryngeal clefts are thought to result from failed fusion of the two lateral growth centers of the
posterior-cricoid cartilage at six to seven weeks of intrauterine life. The smallest clefts may involve only the interarytenoid region,
whereas more extensive aborted development of the tracheoesophageal septum can result in a large laryngotracheoesophageal
cleft that extends to the carina [56,57].
Laryngeal clefts occur in approximately 1 in 10,000 to 1 in 20,000 live births [58]. They are more common in boys than girls, with
a male:female ratio of 5:3 [59,60]. Clefts of the larynx and trachea/esophagus can occur in isolation, as part of a syndrome (eg,
Opitz-Frias, VATER/VACTERL, Pallister-Hall, CHARGE), or with other associated malformations (gastrointestinal, genitourinary,
cardiac, craniofacial) [61].
Children with posterior laryngeal clefts may present with increased secretions, feeding difficulty, failure to thrive, wheezing,
stridor, noisy breathing, aspiration, respiratory distress, recurrent pulmonary infections, and/or hoarseness [44,62,63]. In one
series of 41 children with type 1 posterior laryngeal clefts, the average age at onset of symptoms was five months, and the
average age at diagnosis was three years [62]. Children with posterior laryngeal clefts often have associated gastroesophageal
reflux, tracheomalacia, developmental delay, and/or additional congenital anomalies [44,62-64].
The diagnosis is based upon a high index of suspicion and is supported by modified barium swallow that shows leakage of
barium into the airway [44,63]. Leakage occurs at the laryngeal introitus rather than the distal trachea as is seen in H-type
fistula. Rigid endoscopy is the standard for diagnosis and should include endoscopic palpation of the interarytenoid area (picture
8) [62].
Posterior laryngeal clefts and laryngotracheoesophageal clefts may be classified according to anatomic or clinical criteria. The
Benjamin classification describes five types (figure 1) [65]:
● Occult clefts can be appreciated only by palpation and measurement of the interarytenoid height
● Type 1 clefts are limited to the supraglottic, interarytenoid area (picture 8)
● Type 2 clefts show partial clefting of the posterior cricoid cartilage, sometimes with a mucosal bridge across the
cartilaginous gap
● Type 3 clefts involve the entire cricoid and the cervical portion of the tracheoesophageal membrane, stopping above the
thoracic inlet
● Type 4 clefts involve a major portion of the intrathoracic tracheoesophageal wall (picture 9)
The treatment for laryngotracheoesophageal clefts depends upon the type of cleft and severity of associated symptoms. Minor
clefts (type 1 and occult clefts) sometimes can be managed without surgery if medical therapy can control gastroesophageal
reflux and aspiration [44,62]. Injection of the interarytenoid region with fillers, such as hyaluronic acid, can help decrease
aspiration temporarily as the child grows [66-68]. Local endoscopic procedures can also be attempted on smaller-sized clefts.
Operative repair typically requires collaboration of the otolaryngologist and thoracic surgeon, depending on the length of the
cleft. (See "Management of gastroesophageal reflux disease in children and adolescents".)
Subglottic hemangiomas — Hemangiomas are congenital vascular lesions that undergo a phase of rapid growth that is
initiated during the first few weeks to months of life [69]. They may present as solitary lesions or as part of a segmental
hemangioma syndrome [70,71]. The growth phase lasts for 12 to 18 months, after which the lesion stabilizes in size for a period
of time. Finally, the lesion undergoes involution, typically with complete resolution of the lesion by age 5 in 50 percent of cases
and age 12 in the remainder of cases. (See "Infantile hemangiomas: Epidemiology, pathogenesis, clinical features, and
complications".)
Congenital subglottic hemangiomas are rare, but potentially fatal lesions. They account for 1.5 percent of congenital laryngeal
abnormalities. There is a higher prevalence in females with a female:male ratio of 2:1. Presentation may be similar to that of
subglottic stenosis, with recurrent croup and biphasic stridor.
Clinical diagnosis can be made based upon history and complete physical examination, including rigid endoscopy. Plain
radiographs of the neck typically demonstrate asymmetric narrowing of the subglottis. CT with contrast may be useful in
delineating large hemangiomas or those extending beyond the confines of the larynx. Rigid endoscopy reveals a red to blue,
compressible, sessile lesion that is most commonly located in the posterolateral subglottis (picture 10 and picture 11).
Systemic therapy with propranolol is the first-line therapy for subglottic hemangiomas and has produced dramatic results in case
series with a low incidence of complications [72,73]. Historically, a watchful waiting approach with or without a tracheotomy was
taken. Locally injected or systemic glucocorticoids may hasten resolution. Laser or open surgical resections have been used
with mixed results. (See "Infantile hemangiomas: Management", section on 'Propranolol'.)
Subglottic hemangioma associated with cutaneous hemangiomas in a "beard" distribution may be seen in PHACE syndrome
(posterior fossa brain malformations, hemangiomas of the face (large or complex), arterial anomalies, cardiac anomalies, and
eye abnormalities) (table 2) [74,75]. (See "Infantile hemangiomas: Epidemiology, pathogenesis, clinical features, and
complications", section on 'PHACE syndrome'.)
SUMMARY
● Laryngomalacia refers to collapse of the supraglottic structures during inspiration (movie 1 and picture 3). Clinical
manifestations include stridor that worsens in the supine position, during feeding, and with upper respiratory infections.
Laryngomalacia is suspected based upon the history and physical examination and confirmed with flexible fiberoptic
laryngoscopy. In most otherwise normal children, laryngomalacia resolves spontaneously by 12 to 18 month of age. Infants
who have hoarseness, atypical stridor, or stridor with feeding difficulty, dyspnea, tachypnea, cyanosis, or apnea should be
referred to an otolaryngologist for full endoscopic evaluation and possible intervention. (See 'Laryngomalacia' above.)
● Infants with vallecular cysts (picture 5 and picture 6) typically present with feeding difficulty, failure to thrive, stridor, noisy
breathing, respiratory distress, or a hoarse cry. Laryngomalacia is an associated finding. Diagnosis is confirmed with
endoscopy of the hypopharynx. (See 'Vallecular cysts' above.)
● Congenital laryngoceles and saccular cysts typically are asymptomatic; however, they may cause upper airway obstruction
(particularly in the neonate), hoarseness, dysphagia, dyspnea, and laryngeal discomfort. The symptoms caused by
laryngoceles are episodic because laryngoceles intermittently fill with air. The symptoms produced by saccular cysts are
constant. (See 'Laryngoceles and saccular cysts' above.)
● Most infants with laryngeal atresia present with asphyxia at the time of birth. Emergency tracheotomy is necessary for
survival. (See 'Laryngeal atresia' above.)
● Laryngeal webs are rare congenital anomalies caused by failure of resorption of the epithelial layer at the laryngeal
opening, resulting in incomplete separation of the vocal folds (picture 7). Patients with laryngeal webs usually present in
infancy with respiratory distress and an unusual cry. Approximately 10 percent of patients with laryngeal webs have other
associated anomalies. Treatment is surgical and depends on the degree of airway obstruction. (See 'Laryngeal webs'
above.)
● Congenital subglottic stenosis is a narrowing of the lumen of the cricoid region. It presents most commonly as recurrent
croup but may cause biphasic stridor in a newborn if severe. It is differentiated from acquired subglottic stenosis by the
absence of history of trauma or instrumentation and by less severe symptoms. Congenital subglottic stenosis typically
improves as the larynx grows; however, surgical intervention may be necessary in some children. (See 'Congenital
subglottic stenosis' above.)
● Children with posterior laryngeal clefts may present with increased secretions, feeding difficulty, failure to thrive, wheezing,
stridor, noisy breathing, aspiration, respiratory distress, recurrent pulmonary infections, and/or hoarseness. Contrast studies
of the esophagus may show spillage of barium into the airway. Rigid endoscopy is the standard for diagnosis and should
include palpation of the interarytenoid area (picture 8 and picture 9). (See 'Laryngeal clefts' above.)
● Congenital subglottic hemangiomas are rare, but potentially fatal lesions. The presentation may be similar to that of
subglottic stenosis with recurrent croup and biphasic stridor. Plain radiographs typically demonstrate asymmetric narrowing
of the subglottis. The diagnosis is confirmed endoscopically (picture 10 and picture 11). Propranolol is generally the first-line
therapy for subglottic hemangiomas. (See 'Subglottic hemangiomas' above and "Infantile hemangiomas: Management".)
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33. Breysem L, Goosens V, Vander Poorten V, et al. Vallecular cyst as a cause of congenital stridor: report of five patients.
Pediatr Radiol 2009; 39:828.
34. Ku AS. Vallecular cyst: report of four cases--one with co-existing laryngomalacia. J Laryngol Otol 2000; 114:224.
35. Hsieh WS, Yang PH, Wong KS, et al. Vallecular cyst: an uncommon cause of stridor in newborn infants. Eur J Pediatr
2000; 159:79.
36. Myer CM. Vallecular cyst in the newborn. Ear Nose Throat J 1988; 67:122.
37. Oluwole M. Congenital vallecular cyst: a cause of failure to thrive. Br J Clin Pract 1996; 50:170.
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40. Pennings RJ, van den Hoogen FJ, Marres HA. Giant laryngoceles: a cause of upper airway obstruction. Eur Arch
41. Sniezek JC, Johnson RE, Ramirez SG, Hayes DK. Laryngoceles and saccular cysts. South Med J 1996; 89:427.
42. Chu L, Gussack GS, Orr JB, Hood D. Neonatal laryngoceles. A cause for airway obstruction. Arch Otolaryngol Head Neck
Surg 1994; 120:454.
43. Civantos FJ, Holinger LD. Laryngoceles and saccular cysts in infants and children. Arch Otolaryngol Head Neck Surg
1992; 118:296.
44. Hartnick CJ, Cotton RT. Congenital laryngeal anomalies. Laryngeal atresia, stenosis, webs, and clefts. Otolaryngol Clin
North Am 2000; 33:1293.
45. Okada T, Ohnuma N, Tanabe M, et al. Long-term survival in a patient with congenital laryngeal atresia and multiple
malformations. Pediatr Surg Int 1998; 13:521.
46. Elliott R, Vallera C, Heitmiller ES, et al. Ex utero intrapartum treatment procedure for management of congenital high
airway obstruction syndrome in a vertex/breech twin gestation. Int J Pediatr Otorhinolaryngol 2013; 77:439.
47. Benjamin B. Chevalier Jackson Lecture. Congenital laryngeal webs. Ann Otol Rhinol Laryngol 1983; 92:317.
48. McHugh HE, Loch WE. Congenital webs of the larynx. Laryngoscope 1942; 52:43.
49. Levitsky SE. Hoarseness. In: Primary pediatric care, 4th ed, Hoekelman RA (Ed), Mosby, St. Louis 2001. p.1156.
50. Shearer WT, Biller HF, Ogura JH, Goldring D. Congenital laryngeal web and interventricular septal defect. First reported
cases. Am J Dis Child 1972; 123:605.
51. Strakowski SM, Butler MG, Cheek JW, et al. Familial laryngeal web in three generations with probable autosomal
dominant transmission. Dysmorph Clin Genet 1988; 2:9.
52. Weintraub AS, Holzman IR. Neonatal care of infants with head and neck anomalies. Otolaryngol Clin North Am 2000;
33:1171.
53. Santos D, Mitchell R. The history of pediatric airway reconstruction. Laryngoscope 2010; 120:815.
54. Blanchard M, Leboulanger N, Thierry B, et al. Management specificities of congenital laryngeal stenosis: external and
endoscopic approaches. Laryngoscope 2014; 124:1013.
55. Dahl JP, Purcell PL, Parikh SR, Inglis AF Jr. Endoscopic posterior cricoid split with costal cartilage graft: A fifteen-year
experience. Laryngoscope 2017; 127:252.
56. Lim TA, Spanier SS, Kohut RI. Laryngeal clefts: a histopathologic study and review. Ann Otol Rhinol Laryngol 1979;
88:837.
57. Moungthong G, Holinger LD. Laryngotracheoesophageal clefts. Ann Otol Rhinol Laryngol 1997; 106:1002.
58. Roth B, Rose KG, Benz-Bohm G, Günther H. Laryngo-tracheo-oesophageal cleft. Clinical features, diagnosis and therapy.
Eur J Pediatr 1983; 140:41.
59. Phelan PD, Stocks JG, Williams HE, Danks DM. Familial occurrence of congenital laryngeal clefts. Arch Dis Child 1973;
48:275.
60. DuBois JJ, Pokorny WJ, Harberg FJ, Smith RJ. Current management of laryngeal and laryngotracheoesophageal clefts. J
Pediatr Surg 1990; 25:855.
61. Johnston DR, Watters K, Ferrari LR, Rahbar R. Laryngeal cleft: evaluation and management. Int J Pediatr
62. Parsons DS, Stivers FE, Giovanetto DR, Phillips SE. Type I posterior laryngeal clefts. Laryngoscope 1998; 108:403.
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63. Rahbar R, Rouillon I, Roger G, et al. The presentation and management of laryngeal cleft: a 10-year experience. Arch
Otolaryngol Head Neck Surg 2006; 132:1335.
64. Kennedy CA, Heimbach M, Rimell FL. Diagnosis and determination of the clinical significance of type 1A laryngeal clefts
by gelfoam injection. Ann Otol Rhinol Laryngol 2000; 109:991.
65. Benjamin B, Inglis A. Minor congenital laryngeal clefts: diagnosis and classification. Ann Otol Rhinol Laryngol 1989;
98:417.
66. Nakahara S, Tayama N, Tsuchida Y. A minor laryngeal cleft (type 1-a) diagnosed in infancy. Int J Pediatr Otorhinolaryngol
1995; 32:187.
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69. Ahmad SM, Soliman AM. Congenital anomalies of the larynx. Otolaryngol Clin North Am 2007; 40:177.
70. O TM, Alexander RE, Lando T, et al. Segmental hemangiomas of the upper airway. Laryngoscope 2009; 119:2242.
71. Vermeer S, van Oostrom CG, Boetes C, et al. A unique case of PHACES syndrome confirming the assumption that
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Otolaryngol Head Neck Surg 2010; 142:463.
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Treatment of Airway Hemangiomas. Otolaryngol Head Neck Surg 2015; 153:452.
74. O-Lee TJ, Messner A. Subglottic hemangioma. Otolaryngol Clin North Am 2008; 41:903.
75. Garzon MC, Epstein LG, Heyer GL, et al. PHACE Syndrome: Consensus-Derived Diagnosis and Care Recommendations.
J Pediatr 2016; 178:24.
Topic 6300 Version 20.0
GRAPHICS
Coronal section through eight-week embryo
S: nasal septum; T: tongue; E: eyeball; h: hyoid; t: thyroid cartillage; i: laryngeal

introitus.
Graphic 74038 Version 2.0
Sagittal airway 20-week fetus
e: epiglotis; l: larynx; t: trachea; l: lung; h: heart.
Normal newborn larynx
Note the healthy appearance of the normal structures: epiglottis (E); glottis (G);
aryepiglottic folds (AF); vocal folds (vf); and vestibular folds (VF).
Courtesy of Glenn C Isaacson, MD, FAAP, FACS.
Laryngomalacia
Panel A: Flexible laryngoscopy of laryngomalacia during inspiration. Note collapse of the

epiglottis. Panel B: Flexible laryngoscopy of laryngomalacia during expiration. Note the
omega-shaped epiglottis.
Congenital anomalies associated with stridor
Malformation Characteristics
Nose* Nasal deformities Choanal atresia or agenesis, septum deformities, turbinate hypertrophy, vestibular atresia,
or stenosis.
Pharynx* Craniofacial anomalies Anomalies causing facial retrusion are associated with upper airway obstruction, including
Crouzon, Pierre Robin, and Apert syndromes.
Tongue Macroglossia and glossoptosis.
Larynx Laryngomalacia Most common cause of chronic stridor in infants. Almost all patients present by 6 weeks of
age. Symptoms are more pronounced after upper respiratory infections.
Laryngeal webs 75% located in the glottic area. Complete webs cause respiratory distress at birth
and partial webs produce stridor, weak cry, and different degrees of respiratory distress.
Associated anomalies are common.
Laryngeal cysts If located in supraglottic area, may cause respiratory distress and stridor.
Laryngeal clefts Characterized by abnormal communication between the larynx and pharynx, sometimes
extending downward between the trachea and esophagus. Patients may present with
aspiration, cough, swallowing difficulties, respiratory distress, hoarse cry, or occasionally
with stridor; often associated with other congenital anomalies.
Subglottic hemangioma Presents as with stridor and respiratory distress, usually worsening during the first few
months of life. Often associated with cutaneous hemangiomas.
Subglottic stenosis May be congenital but more often acquired secondary to intubation. Usually located 2 to 3
mm below the glottis.
Vocal cord paralysis Idiopathic or secondary to a neurologic disorder (including Chiari II malformation,
hydrocephalus, meningomyelocele, hypoxic cerebral palsy, and cerebral hemorrhage) [1,2].
Trachea ¶ Tracheal stenosis Usually presents with stridor or both stridor and wheezing. If stenosis is significant,
respiratory distress occurs.
Vascular rings or slings 74% of vascular rings are symptomatic. The airway compression usually is intrathoracic,
causing expiratory stridor. Associated anomalies are common.
Tracheomalacia Often associated with other congenital anomalies. May be secondary to a vascular ring or
cysts. Worsens with upper respiratory infections, crying, coughing, or feeding. May cause
severe spells with cyanosis.
Bronchi and distal Bronchogenic cyst May occur at any point throughout the tracheobronchial tree. Typically present during
airways ¶ childhood with recurrent coughing, wheezing, or pneumonia, but may become symptomatic
during infancy or adulthood or present as an incidental finding on chest radiographs.
* Noise generated from the nose or pharynx is typically low in pitch and is referred to as snoring or stertor.
¶ Noise generated from the trachea, bronchi, or distal airways is mostly wheezing.
References:
1. Nisa L, Holtz F, Sandu K. Paralyzed neonatal larynx in adduction. Case series, systematic review and analysis. Int J Pediatr Otorhinolaryngol 2013; 77:13.
2. Holinger LD, Holinger PC, Holinger PH. Etiology of bilateral abductor vocal cord paralysis: a review of 389 cases. Ann Otol Rhinol Laryngol 1976; 85:428.
Noncongenital causes of stridor in children
Typical age of presentation
Infants
Inspiratory Expiratory Other
Cause and Preschool
School-
Fever/toxic
Neonate toddlers (3 to 5 Adolescents stridor stridor* characteristics
aged
(6 to 24 years)
months)
Acute onset
Foreign body X X + +/– A history of a

aspiration ¶ witnessed choking
episode is common,
but the acute
symptoms often
resolve and may
not be immediately
recalled by the
caregiver.
Airway burn X X X X + +/– Due to thermal

injury or caustic
ingestion.
Bacterial X X X + +/– + Among the most

tracheitis Δ common airway
emergencies
requiring PICU
admission. Most
patients have
prodromal
symptoms of viral
URI.
Anaphylaxis X X X X + +/– Often associated

with hives, choking,
vomiting, and/or
wheezing.
Epiglottitis X X X + +
Subacute or intermittent onset
Laryngotracheitis X X + +/– URI symptoms,

(croup) Δ fever, cough,
hoarseness.
Spasmodic croup X X + Mild URI symptoms

may be present.
Retropharyngeal X X + + Sore throat

abscess progressing to
fever, dysphagia,
and muffled
"hot potato" voice.
Peritonsillar X X X + + Usually preceded by

abscess tonsillitis or
pharyngitis (eg,
streptococcal), with
muffled voice.
Chronic or recurrent
Congenital X X +/– +/–

anomalies ◊
Vocal cord X X X X X + +/– May be congenital,

dysfunction Δ iatrogenic, or
acquired (idiopathic
or neurologic).
Children with
bilateral vocal cord
paralysis typically
present with stridor
and respiratory
insufficiency and
may have normal
voice/cry.
Recurrent X X + +/– Typically presents
respiratory with chronic or
papillomatosis progressive
stridor,
hoarseness of
voice or weak cry,
choking episodes,
and cough, with
wheezing if
trachea is also
involved. In some
cases, upper
airway
obstruc on may
be life-
threatening and
may be the
presen ng
symptom.
Hypocalcemic X X +/– May be caused by
laryngeal severe
spasm hypocalcemia
from any cause
(eg, calcipenic
rickets). The
associated stridor
can be chronic or
intermi ent but
also may present
with acute severe
onset.
Tumor X X X X X +/– +/– Airway compression
usually is extrinsic.
Stridor symptoms
depend on location
of compression.
+: usually present; +/-: may or may not be present; PICU: pediatric intensive care unit; URI: upper respiratory tract infection.
* Any obstructive process that leads to a fixed airway narrowing will produce both inspiratory and expiratory noise.
¶ Foreign body aspiration can occur in any age group but is most common in toddlers and preschool-aged children.
Δ Onset either acute or subacute/gradual.
◊ Congenital anomalies that cause stridor include laryngomalacia, tracheomalacia, subglottic stenosis, bronchogenic cysts, laryngeal malformations,
hemangiomas, and vascular rings. These are outlined in a separate table.
Laryngomalacia surgery
Panel A: Rigid laryngoscopy of laryngomalacia with redundant supraglottic tissue

hiding the vocal cords. Panel B: Rigid laryngoscopy of laryngomalacia following
resection of redundant tissue from epiglottis and arytenoids.
Vallecular cyst
Congenital vallecular cysts arise in the hypopharynx. They contain respiratory

epithelium and mucous glands.
u: uvula; vc: vallecular cyst.
Cyst of aryepiglottic fold
Note obstruction of the glottic opening.
C: cyst.
Courtesy of Ellen S Deutsch, MD.
Anterior laryngeal web (type 3 laryngeal atresia)
A perforated membrane (*) partially obstructs the glottis (G).
Type 1 laryngeal cleft
Images obtained on airway endoscopy in a child with a type 1 laryngeal cleft. Type 1 laryngeal clefts are limited to the supraglottic,
interarytenoid area. Panel A shows the appearance of the cleft without any instrumentation. Panel B shows the cleft as the endoscopist
palpates the interarytenoid area with a probe.
Courtesy of Glenn C Isaacson, MD, FAAP.
Classification of laryngotracheal clefts
Based on the Benjamin classification. Benjamin B, Inglis A. Minor congenital laryngeal

clefts: Diagnosis and classification. Ann Otol Rhinol Laryngol 1989; 98:417.
Endoscopic view of type 4 laryngeal cleft
Type 4 laryngeal clefts involve a major portion of the intrathoracic

tracheoesophageal wall. In the image above, the endotracheal tube is displaced
into the laryngeal cleft.
Endoscopic view of subglottic hemangioma
Note the red-blue sessile lesion in the posterolateral subglottis.
Subglottic hemangioma in a young child
(Panel A) Subglottic hemangioma (arrow) seen from above the vocal cords. There is near complete obstruction of the subglottic
airway.
(Panel B) Normal pediatric larynx.
AC: arytenoid cartilage; AF: aryepiglottic fold; E: epiglottis; VC: vocal cord.
(Panel A) Courtesy of Anna Messner, MD.

(Panel B) Reproduced with permission from: Nagdev A. Airway, breathing, circulation: Normal airway. In: Greenberg's Text-Atlas of
Emergency Medicine, Greenberg MI, Hendrickson RG, Silverberg M, et al (Eds), Lippincott Williams & Wilkins, Philadelphia 2005.
Copyright © 2005 Lippincott Williams & Wilkins. www.lww.com.
Characteristics of PHACE syndrome
P Posterior fossa brain malformations, most commonly the Dandy-Walker variant
H Hemangiomas, particularly large, segmental facial lesions
A Arterial anomalies
C Cardiac anomalies and coarctation of the aorta
E Eye abnormalities and endocrine abnormalities
S* Sternal cleft, supraumbilical raphe, or both
* The term "PHACE(S)" is sometimes used in the presence of ventral developmental defects.
Contributor Disclosures
Glenn C Isaacson, MD, FAAP Nothing to disclose Anna H Messner, MD Nothing to disclose Carrie Armsby, MD, MPH Nothing to disclose
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a
multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced content is
required of all authors and must conform to UpToDate standards of evidence.
Conflict of interest policy

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● Delayed maturation or "hypotonia" of the supporting cartilaginous structures of the larynx

Other presenting features include [7,9,14-16]:

● Snoring and/or sleep-disordered breathing – 25 percent

Use of UpToDate is subject to the Subscription and License Agreement.

Topic 6300 Version 20.0

Coronal section through eight-week embryo

S: nasal septum; T: tongue; E: eyeball; h: hyoid; t: thyroid cartillage; i: laryngeal

Graphic 74038 Version 2.0

Sagittal airway 20-week fetus

e: epiglotis; l: larynx; t: trachea; l: lung; h: heart.

Graphic 50788 Version 3.0

Normal newborn larynx

Courtesy of Glenn C Isaacson, MD, FAAP, FACS.

Graphic 73785 Version 1.0

Panel A: Flexible laryngoscopy of laryngomalacia during inspiration. Note collapse of the

Courtesy of Glenn C Isaacson, MD, FAAP, FACS.

Graphic 79457 Version 1.0

Congenital anomalies associated with stridor

Tongue Macroglossia and glossoptosis.

Graphic 62718 Version 8.0

Noncongenital causes of stridor in children

Typical age of presentation

Foreign body X X + +/– A history of a

Airway burn X X X X + +/– Due to thermal

Bacterial X X X + +/– + Among the most

Anaphylaxis X X X X + +/– Often associated

Subacute or intermittent onset

Laryngotracheitis X X + +/– URI symptoms,

Spasmodic croup X X + Mild URI symptoms

Retropharyngeal X X + + Sore throat

Peritonsillar X X X + + Usually preceded by

Congenital X X +/– +/–

Vocal cord X X X X X + +/– May be congenital,

Recurrent X X + +/– Typically presents

Graphic 101526 Version 6.0

Panel A: Rigid laryngoscopy of laryngomalacia with redundant supraglottic tissue

Courtesy of Glenn C Isaacson, MD, FAAP, FACS.

Graphic 56479 Version 1.0

Congenital vallecular cysts arise in the hypopharynx. They contain respiratory

u: uvula; vc: vallecular cyst.

Courtesy of Glenn C Isaacson, MD, FAAP, FACS.

Graphic 79138 Version 2.0

Cyst of aryepiglottic fold

Note obstruction of the glottic opening.

Courtesy of Ellen S Deutsch, MD.

Graphic 69715 Version 3.0

Anterior laryngeal web (type 3 laryngeal atresia)

A perforated membrane (*) partially obstructs the glottis (G).

Courtesy of Glenn C Isaacson, MD, FAAP, FACS.

Graphic 57415 Version 3.0

Type 1 laryngeal cleft

Courtesy of Glenn C Isaacson, MD, FAAP.

Graphic 74786 Version 2.0

Classification of laryngotracheal clefts

Based on the Benjamin classification. Benjamin B, Inglis A. Minor congenital laryngeal

Graphic 66067 Version 3.0

Endoscopic view of type 4 laryngeal cleft

Type 4 laryngeal clefts involve a major portion of the intrathoracic

Courtesy of Glenn C Isaacson, MD, FAAP, FACS.

Graphic 67712 Version 1.0