Endoscopic release of intraoral synechiae in popliteal pterygium syndrome

Area of Science:

• Pediatric Surgery
• Genetics
• Oral and Maxillofacial Surgery

Background:

• Popliteal pterygium syndrome is a rare genetic disorder characterized by webbing of the skin behind the knees and other congenital anomalies.
• Intraoral manifestations, such as fibrous bands restricting mouth opening, can occur but are less common.

Observation:

• An infant diagnosed with typical popliteal pterygium syndrome presented with significant microstomia.
• Intraoral examination revealed fibrous bands tethering the maxillary and mandibular alveolar ridges, severely limiting mandibular excursion.

Findings:

• Surgical intervention was performed to release the restrictive intraoral fibrous bands.
• Endoscopic assistance was utilized during the surgical division of the bands to ensure precise and complete release.

Implications:

• This case highlights the importance of recognizing and managing intraoral complications associated with popliteal pterygium syndrome.
• Endoscopic techniques offer a minimally invasive approach for treating intraoral fibrous bands, potentially reducing morbidity and improving functional outcomes in infants.