A Rare Case of Bilateral and Midline Cleft Palate With Bilateral Macrostomia

Area of Science:

• Craniofacial Surgery
• Pediatric Plastic Surgery
• Congenital Anomalies

Background:

• Pierre Robin Sequence presents challenges in airway management, often delaying surgical interventions for associated craniofacial anomalies.
• Complex cleft palate presentations, including midline, lateral, and soft palate agenesis, require tailored surgical approaches.
• Existing terminology for rare cleft palate variations lacks uniformity, potentially causing confusion in diagnosis and treatment.

Purpose of the Study:

• To describe the surgical management of a rare complex cleft palate in an infant with Pierre Robin Sequence.
• To propose standardized terminology for rare cleft palate presentations.
• To highlight the successful one-stage correction of multiple palatal clefts.

Main Methods:

• A 6-day-old infant with bilateral macrostomia, micrognathia, and a complex cleft palate was managed.
• Surgical interventions, including bilateral macrostomia repair and one-stage palatal cleft correction, were performed after achieving airway stability.
• A right buccal myomucosal flap was utilized for the oral lining during palatal repair.

Main Results:

• Successful surgical correction of bilateral macrostomia and a complex cleft palate involving midline submucous cleft, left lateral submucous cleft, and right lateral cleft with partial soft-palate agenesis.
• The patient underwent staged surgical procedures, with palatal repair completed at 17 months.
• The proposed term "lateral cleft palate" aims to unify the classification of rare cleft types.

Conclusions:

• Complex cleft palate anomalies associated with Pierre Robin Sequence necessitate careful surgical planning and execution.
• A unified terminology, such as "lateral cleft palate," is recommended for rare cleft palate presentations.
• One-stage correction of multiple palatal clefts using specialized flaps can achieve favorable outcomes.