CONGENITAL ANOMALIES OF THE LARYNX

WHEN TO SUSPECT?

The onset of congenital anomalies is usually in the 1st 6- 12 months of age. The primary manifestation will be prominent stridor which can be inspiratory or biphasic and will not be associated with fever. Hoarseness of voice will be present. There will be feeding difficulties. Direct laryngoscopy is the line of treatment useful in >85% of cases for diagnosis.

LARYNGOMALACIA 

• It is the softening of the larynx or having less tone. It is the most common congenital anomaly of the larynx. The most common cause of stridor <2 years of age. The most commonly affected part is the supraglottic larynx.

PATHOLOGY OF LARYNGOMALACIA 

• The abnormally less tone in the supraglottic part leads to the partial collapse of the larynx in forceful inspiration. This leads to intermittent stridor. In 90% of cases, it is a mild, self-limiting illness. In 10% of severe and problematic cases, it co-exists with: Neurological disease and reflux which can be GER (Gastro-oesophageal reflux) or Laryngeal reflux.

CLINICAL FEATURES OF LARYNGOMALACIA

• The age of onset is between 2 weeks - 6 months. The clinical hallmark is Episodic/intermittent stridor, which increases during crying, URI (upper respiratory infection) or when the child is put in the supine position. It decreases when the child is calm or in a prone position. Many patients have coexisting dysphagia or feeding difficulties.

INVESTIGATION OF CHOICE 

• The investigation of choice is Direct laryngoscopy. It shows omega epiglottis, which indicates underlying laryngomalacia.

TREATMENT OF LARYNGOMALACIA

As the disease improved in >90% of cases by the age of 2 years, it is advised for observation of the child and reassurance of the parents. If the patient has reflux, add PPI (proton pump inhibitor) for management. A minor surgical procedure is done in case of no response - supraglottoplasty.

SUBGLOTTIC STENOSIS

It is the second most common congenital anomaly of the larynx. It is usually congenital but can also be acquired. It occurs due to prolonged intubation. It is the narrowing in the subglottic region or an opening present due to stenosis. The manifestation is Stridor, usually inspirational or rarely biphasic without fever. 

CHOICE OF INVESTIGATIONS 

• The investigation of choice is Direct laryngoscopy.

MEYERS - CLASSIFICATION AND TREATMENT

TREATMENT OF SUBGLOTTIC STENOSIS

• For grade I and grade II - Initially, dilatation is done, followed by Endoscopic LASER surgery. For grade III and grade IV - 

Anterior cricoid split is done, followed by laryngotracheal reconstruction with cartilage graft augmentation.

Also Read: Crohn's disease: Causes, Types, Symptoms, Diagnosis, Treatment, Complications And Prevention

OTHER CONGENITAL LARYNGEAL ANOMALIES 

VOCAL CORD PALSY

• It is the 3rd most common congenital anomaly of the larynx. It can be either unilateral or bilateral and associated with CNS disease. If idiopathic and unilateral, it improves spontaneously in infancy until 6-12 months. In bilateral vocal cord palsy, required emergency tracheostomy followed by surgery.

CONGENITAL LARYNGEAL WEBS 

• It can manifest with congenital heart disease or 22q del syndrome.

 TREATMENT 

• The treatment is symptomatic.  If symptoms are severe and causing problems, it is surgically removed.

LARYNGOCELE 

• It is an abnormal, vertical, air-filled laryngeal saccule. It is between the false vocal cord, the epiglottis base, and the thyroid cartilage's inner surface. It communicates with the laryngeal lumen and, when intermittently filled with air, causes hoarseness and dyspnea. The hoarseness increases with the crying. A saccular/ laryngeal cyst filled with mucus, not air. Surgical excision is the therapy of choice.

LARYNGEAL CLEFTS

• It is associated with increased aspirations.  It should be repaired as soon as it is discovered.

VASCULAR RINGS

It is an apparent blood vessel that can cause circling and compression of the airways of the larynx, trachea, and sometimes the oesophagus. There are two types of vascular rings:

Also Read: KEY POINTS AND RECOMMENDATIONS IN PEDIATRIC ADVANCED LIFE SUPPORT

Hope you found this blog helpful for your NEET SS ENT preparation. For more informative and interesting posts like these, keep reading PrepLadder’s blogs.