Long-Term Orthognathic Considerations in the Pierre Robin Sequence Patient
Miles J. Pfaff, M.D., M.H.S.1, Christos Haveles1, Claire Liu1, Emily Berthiaume1, Julia R. Ayeroff1, Omotayo Arowojolu, M.D.2, James P. Bradley, M.D.3, Libby F. Wilson, M.D.4, Justine C. Lee, M.D., Ph.D.1.
1UCLA Medical Center, Los Angeles, CA, USA, 2New York University, New York, NY, USA, 3Division of Plastic and Reconstructive Surgery, Northwell Health Hofstra School of Medicine, NY, USA, 4Orthopaedic Institute for Children, Cleft Palate Program, Los Angeles, CA, USA.
PURPOSE: Pierre Robin Sequence (PRS) is manifested by the constellation of micrognathia, glossoptosis, and airway obstruction. While syndromic children may require multiple surgical treatments aimed towards correction of airway obstruction as well as malocclusion, one of the controversies in nonsyndromic PRS is the potential for "catch-up" mandibular growth equivalent to children without craniofacial diagnoses. In this work, we evaluated the long-term requirements of orthognathic surgery at skeletal maturity in children diagnosed with PRS.
METHODS: 115 children diagnosed with PRS were retrospectively evaluated from two craniofacial centers. Children ≥ 13 years of age were included and patients who underwent any type of mandibular distraction or surgery prior to skeletal maturity were excluded. Demographics, surgical, and orthodontic histories were reviewed. Descriptive statistics were reported. Chi-square was used to compare groups.
RESULTS: 43 patients with PRS without mandibular surgery prior to skeletal maturity were identified (57% female; mean age: 20 ± 3.3 years). Mean length of follow up was 14.2 ± 5.2 years. 18 patients (41%) were diagnosed as syndromic, the majority of which was Stickler Syndrome. 41 patients (95%) had a history of a cleft palate and of those patients, 18 (44%) had velopharyngeal insufficiency necessitating surgery. Data was available for 42 patients in regards to orthodontic and orthognathic care. Orthodontic evaluation revealed that 41 patients (98%) had a history of orthodontic treatment and 20 (48%), 17 (40%), and 5 (12%) were classified as Angle Class I, II, and III, respectively. Subset analysis revealed that 13 and 4 patients with a syndromic diagnosis were Class I and class II, respectively, while 1 was class III (p> 0.05). A total of 17 (39.5%) patients underwent or were recommended to undergo orthognathic surgery due to Class II (n=11/17, 65%) or Class III (n=3/17, 18%) malocclusion. When PRS children with surgical Class II malocclusion were separated into nonsyndromic versus syndromic subgroups, 9/11 (80%) were nonsyndromic and 2/11 (18%) were syndromic. Overall, 9/25 (36%) of nonsyndromic and 2/18 (11%) of syndromic PRS children required orthognathic surgery to specifically address mandibular and occlusal abnormalities (p=0.09).
CONCLUSION:The current study suggests deficient long-term mandibular growth of nonsyndromic PRS children is relatively similar to that of children with syndromic PRS. These findings lie in contrast to the concept of mandibular "catch-up" growth and suggest that the maxillomandibular relationship in PRS may not resolve without intervention.
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