[Pierre Robin Sequence: interdisciplinary treatment after prenatal diagnosis]

Area of Science:

• Craniofacial anomalies
• Pediatric airway management
• Neonatal care

Context:

• Pierre Robin Sequence (PRS) presents with micrognathia, glossoptosis, and upper airway obstruction (UAO), often requiring immediate intervention.
• Severe UAO and cyanosis at birth necessitate prompt diagnosis and specialized care.
• Prenatal diagnosis is crucial for timely referral and management planning.

Purpose:

• To present a non-invasive interdisciplinary treatment protocol for Pierre Robin Sequence.
• To evaluate the efficacy of the preepiglottic baton plate (PEBP) in managing UAO in infants with PRS.
• To compare PEBP treatment with conventional methods and assess long-term outcomes.

Summary:

• A randomized crossover trial demonstrated that the PEBP significantly reduced the apnea index in 11 infants with isolated PRS, outperforming conventional palatal plates.
• An uncontrolled longitudinal study showed normalization of UAO within 3 months post-discharge, with adequate weight gain and successful bottle feeding.
• A comparative study found no significant cognitive differences between children with PRS treated with PEBP and healthy children.

Impact:

• Early and adequate PEBP treatment effectively avoids complications of UAO, leading to improved feeding, speech, and facial profile development.
• This non-invasive approach renders traditional surgical interventions like tongue-lip adhesion or mandibular distraction obsolete.
• Optimal postnatal therapy requires interdisciplinary collaboration among prenatal care, neonatology, and orthodontics.