Upper Airway Computed Tomography Measures and Receipt of Tracheotomy in Infants With Robin Sequence

Abstract

Importance: Airway management in infants with Robin sequence is challenging. Objective upper airway measures associated with severe airway compromise requiring tracheotomy are needed to guide decision making.

Objectives: To define objective upper airway measures in infants with Robin sequence from craniofacial computed tomography (CT) and to identify those measures in Robin sequence associated with tracheotomy.

Design, setting, and participants: A cohort study (2003 to 2014, over 1-year follow-up) of 37 infants with Robin sequence evaluated for surgical management and 37 selected age- and sex-matched controls without a craniofacial condition conducted in a pediatric institution's craniofacial center.

Main outcomes and measures: Define and compare CT-generated upper airway measures in these groups: infants with Robin sequence vs controls, and infants with Robin sequence with vs without tracheotomy. A negative difference signifies lower values for the Robin sequence and tracheotomy groups. Clinical data collected included age and height at time of CT scan, sex, tracheotomy presence, associated syndrome, and laboratory indicators of hypoventilation and hypoxemia. To evaluate interrater reliability, 2 raters performed each measurement in the Robin sequence group.

Results: In 74 infants, 17 of 28 measures were different between infants with Robin sequence and those in the control group. Tracheotomy was performed in 14 of 37 (38%) infants with Robin sequence. Infants with tracheotomy more commonly had associated syndromes (12 of 14 [86%] vs 11 of 23 [48%]) and a history of hypoventilation and hypoxemia (13 of 14 [93%] vs 15 of 23 [65%]). Five of the 11 measures associated with tracheotomy were reliable and simpler to measure with the following mean differences (95% CIs) between groups: tongue length, 0.87 (0.26 to 1.48); tongue position relative to palate, 0.83 (0.22 to 1.45); mandibular total length, -0.8 (-1.42 to -0.19); gonial angle, 0.71 (0.08 to 1.34); and inferior pogonial angle, 0.66 (0.02 to 1.29). Using a receiver operating characteristic analysis, a composite score of these 5 measures for predicting tracheotomy risk yielded an area under the curve of 0.83 and achieved 86% sensitivity and 74% specificity.

Conclusions and relevance: Computed tomography measures quantifying tongue position and mandibular configuration can identify infants with Robin sequence, and importantly, differentiate those who have severe upper airway compromise requiring tracheotomy. Following validation, these measures can be used for objective upper airway assessment and for expediting clinical decision-making in these challenging cases for which no such tools currently exist.

Figure 1.. Two- and Three-Dimensional Computed Tomographic Measures

Control patient airway measures (5-month-old): A, airway divisions; B, tongue size and position; C, hyoid position; D, lateral mandible measures; E, inferior pogonial angle; F, bigonial distance.

Figure 2.. Representative Significant Computed Tomographic Measures in an Infant With RS (Left) and Matched Control Patient (Right)

A, Larger tongue area, taller tongue height relative to the vallecula, and more posterior tongue position are shown in RS compared with control consistent with glossoptosis. B, Compressed hyoid position is illustrated in patient with RS compared with control. C and D, A shorter and flatter mandible is demonstrated in a patient with RS compared with control.

Figure 3.. Analysis of Computed Tomographic Composite Scores for Infants With RS With vs Without Tracheotomy

A, Boxplot of 10-measure (FDR <0.2, RPD <10%) and 5-measure simplified CT composite scores for tracheotomy vs no tracheotomy . B, ROC curves for all-measure, 10-measure (FDR <0.2, RPD <10%), and 5-measure simplified CT composite scores, with sensitivity and specificity indicated in parentheses. FDR indicates false discovery rate; ROC, receiver operating characteristic; RPD, relative percent difference.