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Final ID: Poster #: SCI-028

Mandibular Ramus Width Measurements in Pierre Robin Sequence Patients as a Reliable Pre-operative Planning Tool

Purpose or Case Report: Infants with Pierre Robin sequence characteristically suffer from gestational micrognathia resulting in development of congenital glossoptosis, putting them at high risk for life-threatening upper airway obstruction. Airway management needs often necessitate prolonged NICU hospitalization.

Heterogeneity of presentation leaves much to be desired in terms of data-driven risk stratification and surgical planning for this population. The decision to proceed with mandibular distraction is multifactorial, incorporating the patient's respiratory status, comorbidities, family preferences, physician preference, and availability of procedure.

Mandibular distraction osteogenesis surgery is a definitive treatment, with effective elimination of soft-tissue obstruction of the micrognathic airway regardless of the patient’s syndromic picture. Standardized, reproducible measurements ensuring fit prior to distractor placement allow selection for mandibular distraction osteogenesis surgery at a younger age.
Methods & Materials: Upon 3D reconstruction of standard pediatric low dose CT, the mandibular ramus is measured parallel to the inferior plane of the mandibular body. A secondary measurement is taken from the midpoint of the MRW line parallel to the posterior border of the ramus corresponding to the vertical component of potential osteotomy site.
Results: Inter-observer reliability of these measurements was assessed by blinded independent review of 23 anonymized cases conducted by a medical intern, a first-year radiology resident (junior), a third-year radiology resident (senior), and a pediatric radiology attending. Data was analyzed by intraclass correlation coefficient.

The senior resident and pediatric attending notably produced identical data sets. When these two were grouped with a junior resident, data demonstrates excellent reliability for all individual measured components ICC(3,1)=0.946.
Conclusions: By establishing a standardized, reproducible measurement criteria, multiple candidates have been selected for mandibular distraction osteogenesis surgery at a younger age. This reduces hospitalization length and the associated financial burden on families. There is also the potential to reduce morbidity and mortality.

We have performed 25 mandibular distraction surgeries since 2016 at a mean age of less than 27 days, which compares favorably to the published literature suggesting a mean age of approximately 53-87 days.

Our new data shows excellent inter-observer reliability among radiology residents and attendings.
  • Mayock, Patrick  ( Children's Hospital of Los Angeles , Los Angeles , California , United States )
  • Francis, Stacey  ( Kaiser Permanente Los Angeles Medical Center , Los Angeles , California , United States )
  • Dang, Jeannie  ( Kaiser Permanente Los Angeles Medical Center , Los Angeles , California , United States )
  • Peng, Lauren  ( Kaiser Permanente Los Angeles Medical Center , Los Angeles , California , United States )
Session Info:

Posters - Scientific

Neuroradiology

SPR Posters - Scientific

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