Congenital disorders of the Larynx“Laryngomalacia”

By:Zainab ahmed

Audiologist and speech languuage

pathologist

PART 1 “LARYNGOMALACIA”

WHAT IS LARYNGOMALACIA?

Most common congenital anomaly of the larynx

-characterized by partial or complete collapse of the supraglottic structures on inspiration.

Laryngo malacia is a congenital softening of the tissues of the larynx (voice box) above the vocal cords. This is the most common cause of noisy breathing in infancy. The laryngeal structure is malformed and floppy, causing the tissues to fall over the airway opening and partially block it.

LARYNGOMALACIA..

Etiology: Unkown

Theories: GERD,(gastroesophageal reflux disease) Immature neuromuscular control(the mind's attempt to teach the body conscious control of a specific movement. )

Epiglottis is derived from the 3rd and 4th branchial arches. Overgrowth of the 3rd results in elongation of the structure and the observed laryngomalacia

However histological studies do NOT demonstrate a difference between the quality of the cartilage between normal/abnormal

Congenital disorders of the Larynx

OrganogenesisAt birth the larynx is located high in the larynx between levels C1- C4

By age 2 the larynx begins to descend inferiorly

By age 6 the larynx reaches the adult position between levels C4 through C7

CONGENITAL DISORDERS OF THE LARYNX

The larynx develops from the endodermal lining and the adjacent mesenchyme of the foregut between the fourth and sixth branchial arches.

At 20 days' gestation, the foregut is first identifiable with a ventral laryngotracheal groove

The laryngotracheal groove continues to deepen until its lateral edges fuse. By day 26, this tube descends caudally, where the trachea becomes separated from the esophagus by the tracheoesophageal septum with a persistent slitlike opening into the pharynx.

DETECTION OF LARYNGOMALACIA

Laryngomalacia is seen in a child’s airwayduring micro laryngoscopy which is a precise type of surgery used in the diagnosis and removal of various lesions of the vocal folds to restore a normal voice.

PART 2: LARYNGOMALACIA

CLINICAL DIAGNOSIS

It is considered a benign, transient cause of inspiratory stridor and is characterized by collapse of arytenoids, epiglottis and aryepiglottic folds during the inspiratory phase.

. The clinical diagnosis comprises of identifying three anatomic abnormalities

(1) flaccid epiglottis prolapsing backwards during inspiration; (2) poorly supported arytenoids that prolapse

forward during inspiration; (3) short aryepiglottic folds.

NORMAL VS LARYNGO MALACIAC VIEW

CAUSES: It can be due to the relaxation or a lack of muscle

tone in the upper airway.

Insufficient or delayed calcium deposition

The part of the nervous system that gives tone to the airway is most likely underdeveloped.

Gastroesophageal reflux (GER) may play an etiological role in laryngomalacia

Immature neuromuscular control may be responsible for the arytenoid prolapse observed in laryngomalacia

TYPES:

Type I: Inward collapse of aryepiglottic folds

Type II: Long tubular epiglottis infolding on itself (Omega shaped)

Type III: Anterior and medial collapse of corniculate and cuneiform cartilages

Type IV: Posterior displacement of epiglottis against posterior pharyngeal wall or inferior collapse towards vocal folds

Type V: Short aryepiglottic folds

DEGREE OF LARYNGOMALACIA

Laryngomalacia can be mild, moderate or severe. 99% of infants with laryngomalacia have mild or moderate laryngomalacia.

Mild laryngomalacia: noisy breathing or inspiratory stridor. no significant airway obstruction, feeding difficulties

or other symptoms associated with laryngomalacia. The noisy breathing is annoying to caregivers, but

does not cause other health care problems. Infants with mild laryngomalacia usually outgrow

the stridor by 12 – 18 months of age

MODERATE LARYNGOMALACIA

 noisy breathing or inspiratory stridor Feeding difficulties without poor weight gain (vomiting or spitting up) Choking on food Gastroesophageal reflux) Mild to moderate chest and/or neck

retractions (chest and/or neck sinking in with breathing), but no severe distres

. Stomach acid, if it reaches the upper part of the esophagus and voice box, can cause swelling of the floppy tissue above the vocal cords.

SEVERE LARYNGOMALACIA

Feeding difficulties with poor weight gain (chest and/or neck sinking in with breathing) Significant cyanosis (turning blue) Life threatening apnea (pauses in breathing) Heart or lung problems from chronic oxygen

deprivation (low oxygen) Airway symptoms severe enough to cause

multiple visits to an emergency department or hospital

Only 1% of infants with laryngomalacia have severe laryngomalacia. Infants with severe laryngomalacia usually need surgery.

SYMPTOMS

Noisy breathing (stridor) — An audible wheeze when the baby inhales (breathes in). It is often worse when the baby is agitated, feeding, crying or sleeping on his back.

High pitched sound

Difficulty feeding

Poor weight gain

Choking while feeding

Apnea (breathing stoppage)

Pulling in neck and chest with each breath

Cyanosis (turning blue)

Gastro esophageal reflux (spitting, vomiting and regurgitation)

Aspiration (inhalation of food into the lungs)

PART : 3 “LARYNGOMALAYCIA”

CHECKLIST :

Look out and check if the child

has difficulty keeping food down and constantly spits it up

is losing weight or is not gaining weight

begins to feed less and tires easily in the middle of feeding

begins to choke on food

struggles between eating and breathing

PHYSICAL EXAM: Elongation and lateral extension of the eiglottis (omega

shaped) that falls posteroinferiorly on inspiration

Redundant bulky arytenoids that prolapse anteromedially on inspiration

Shortening of the aryepiglottic folds, which results in tethering of the arytenoids to the epiglottis

Inward collapse of the aryepiglottic folds (cuneiform cartilages) on inspration

Visualization of the vocal cords, which are normal in structure and function

FLEXIBLE ENDOSCOPY

Elongation and lateral extension of the epiglottis (omega shaped) that falls posteroinferiorly on inspriation

MICROLARYNGOSCOPY AND BRONCHOSCOPY

This test is done in the operating room under general anesthesia by the ENT surgeon. The doctor looks at the voice box and windpipe with telescopes. Your doctor may recommend this test if the X-ray test shows something abnormal or if your doctor has a suspicion of additional airway problems

ESOPHAGOGASTRODUODENOSCOPY (EGD)

An EGD is a diagnostic test done in the operating room under general anesthesia by the gastroenterologist. The doctor looks at your child's esophagus and stomach with a lighted tube.

During an EGD, the doctor looks for signs of chronic inflammation from acid irritation that can occur in the stomach or the esophagus. Your doctor may recommend this if the pH probe is significantly abnormal or there is strong suspicion of significant GERD based on history and clinical examination

TREATMENT

In approximately 90% of reported cases, the condition is mild, no intervention is needed and the parents can be reassured accordingly (Lane et al. 1984)

Medical management should include treatment of documented gastroesophageal reflux disease (GERD) because this condition is known to contribute to laryngomalacia

SURGICAL MANAGEMENT

Surgical management is indicated in rare instances if respiratory complications develop if there is serious respiratory obstruction with substantial sternal and intercostal recession,

feeding difficulties that may be compounded by reflux enhanced by the high negative intrathoracic pressures generated, and consequent failure to thrive.

Tracheotomy can be performed for emergent episodes of distress and should be left in place until the supraglottic pathology resolves with age.

TREATMENT

Surgical management -Restoration of an adequate airway can be achieved by performing an endoscopic aryepiglottoplasty

(sometimes termed a supraglottoplasty; Jani et al. 1991).

Supraglottoplasty 1) Using cup forceps and microscissors (or alternatively

the carbon dioxide laser), each aryepiglottic fold is first divided to release it from the edge of the epiglottis,

2) the redundant mucosa and submucosal tissue are then excised from over the arytenoids, together if necessary with part or all of the cuneiform cartilages. The stridor is usually improved immediately following

PART 4 : LARYNGOMALACIA

POST OPERATIVE CARE

If a child has had an operation, there may still be a requirement for treatment for gastro esophageal reflux during and after the operation. It is also important to monitor the child for signs and symptoms of worsening LM.

POST OPERATIVE CARE Take the child to the hospital if breathing stops for longer than 10 seconds dusky or blue color around lips associated with noisy

breathing chest or neck retractions that do not stop with

repositioning your child or waking your child up Inform the child’s doctor if the child has difficulty keeping food down and constantly

spits it up. child is losing weight or is not gaining weight. child begins to feed less and tires easily in the middle

of feeding. child begins to choke on food. child struggles between eating and breathing

QUOTATION: “WHEN PEOPLE DON’T EXPRESS THEMSELVES,THEY DIE ONE PIECE AT A TIME”. LAURIE HALSE