PIERRE ROBIN SYNDROME TRACHEAL WEBBING

Pierre Robin syndrome :
Pierre Robin syndrome presents with micrognathia and with a resultant displacement of the tongue into the posterior pharynx, which may occlude the upper airway. A central cleft of the soft palate usually is present in Pierre Robin syndrome.

Respiratory distress and cyanosis are caused by the obstruction of the upper airway. In the delivery room, the infant should be given supplemental oxygen and placed in a prone position in an attempt to have the tongue move forward in a dependent fashion from the posterior pharynx, relieving the airway obstruction.

If the infant continues to have persistent respiratory distress, an oral airway may be placed. Alternatively, an appropriately sized endotracheal tube may be passed through the nose into the hypopharynx.

Tracheotomies are generally not necessary and should be avoided in Pierre Robin syndrome. Intubation of these infants often is not easy because visualization of the larynx is difficult.

Tracheal webbing
The pathogenesis of tracheal webbing originates in the tenth week of gestation when an arrest in the development of the larynx near the vocal cords results in a residual web of tissue persisting in the airway. Approximately 75% of tracheal webs occur at the level of the vocal cords.

These lesions are critical if more than 50% of the airway diameter is occluded, but this is rare. These disorders may be relatively asymptomatic at birth, with the development of distress later when activity increases and the need for airway flow increases.

When attempting to intubate these infants, an obstructive covering may be observed over the larynx and may occlude the airway completely. If the web consists of a thin membrane, the ETT may be pushed beyond the obstruction. However, if the membrane is thick, the infant requires an emergency tracheotomy. If the infant is manifesting severe distress, a large bore needle or catheter may be placed into the trachea to allow for gas exchange while arranging for emergency treatment.

Caution must be used because inexperienced personnel may confuse this rare disorder with simple inability to visualize the vocal cords.