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Long-Term Orthognathic Considerations in the Pierre Robin Sequence Patient
Miles J Pfaff1, Fransia De Leon1, Laura Le1
1From the Division of Plastic and Reconstructive Surgery, University of California, Los Angeles, David Geffen School of Medicine; and the Cleft Palate Program, Orthopaedic Institute for Children.
Plastic and Reconstructive Surgery
|November 2, 2020
Summary
Many Pierre Robin sequence patients require orthognathic surgery, particularly mandibular advancement, by skeletal maturity. This suggests that micrognathia in Pierre Robin sequence may not self-correct without intervention.
Area of Science:
- Craniofacial surgery
- Pediatric dentistry
- Genetics
Background:
- Pierre Robin sequence (PRS) is characterized by micrognathia, glossoptosis, and airway obstruction.
- Conservative management of PRS often assumes spontaneous mandibular growth, but long-term outcomes are unclear.
- This study investigates the need for orthognathic surgery in PRS patients at skeletal maturity.
Purpose of the Study:
- To evaluate the long-term orthognathic surgical requirements in conservatively managed Pierre Robin sequence patients.
- To compare surgical needs between syndromic and nonsyndromic PRS patients.
- To assess the frequency of mandibular advancement in PRS patients compared to isolated cleft palate patients.
Main Methods:
- Retrospective review of conservatively managed PRS and isolated cleft palate patients (≥13 years) at two institutions.
- Analysis of orthognathic surgical requirements at skeletal maturity.
- Statistical analysis including t tests, chi-square, and Fisher's exact tests (p < 0.05 significant).
Main Results:
- 39.1% of PRS patients (n=64) required orthognathic surgery at skeletal maturity.
- The majority of PRS patients needing surgery had a Class II malocclusion, requiring mandibular advancement.
- No significant difference in surgical needs between syndromic and nonsyndromic PRS patients; PRS patients had higher mandibular advancement frequency than cleft palate patients (p=0.006).
Conclusions:
- Mandibular micrognathia in conservatively managed Pierre Robin sequence patients often necessitates orthognathic surgery, primarily mandibular advancement for Class II malocclusion.
- These findings challenge the assumption of spontaneous mandibular catch-up growth in PRS.
- Early intervention may be crucial for optimal outcomes in Pierre Robin sequence.

