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What is PIERRE ROBIN SEQUENCE?

Children with Pierre Robin sequence have a smaller-than-normal lower jaw, and their tongue falls back in the throat. This can lead to problems breathing. In many cases, children will also have cleft palate. Pierre Robin sequence can happen by itself or with other conditions (syndromes). Genetic evaluation is recommended.

Symptoms SYMPTOMS

In all patients with Robin sequence, a complete airway/breathing assessment must be done. If there is problem with the airway, the child may need a tracheostomy. This is a tube that goes into the child’s neck to help them breath. Children usually need to be in the hospital to be observed and to have a sleep study for this to be confirmed.

Treatment TREATMENT & MANAGEMENT

In more mild cases, simple positioning changes (sleeping on their stomach or side) can help your child. Care may also include a tube in the nose to help with breathing for a temporary period of time. In more severe cases or in children who have abnormal sleep study results, surgical options may be best. Surgical intervention is provided a craniofacial plastic surgeon and includes tongue-lip adhesion, mandibular distraction or tracheostomy by otolaryngology (ENT).

Tongue-lip adhesion: The undersurface of the tip of the tongue is connected with stitches to the inside of the lower lip to hold it in a more forward position. This adhesion is temporary and removed at time of palate repair. Mandibular Distraction: A device is put inside or outside of the jaw. By working with the device, the jaw is slowly brought forward carrying the tongue with it. This procedure improves the airway as new bone forms in the gap.

Tracheostomy: If neither of these procedures is successful or your child has disease below the base of the tongue, he/she may need a tracheostomy.