Is a Difficult Airway Team Needed for Intubation at Removal of Mandibular Distraction Devices for Infants With Robin Sequence?

PURPOSE: Difficult airway teams (DATs) are typically present to assist intubation at the initial mandibular distraction osteogenesis (MDO) operation for infants with Robin sequence (RS). In some institutions, the RS diagnosis triggers a "difficult airway" label for the infant, requiring DAT presence for future operations. By the time of distractor removal, however, breathing and airway anatomy are significantly improved. The objective of this study was to measure intubation difficulty and perioperative respiratory complications at MDO device removal as a proxy for the necessity for coordination with a DAT. METHODS: This is a retrospective study including infants with RS from 2013 to 2021 who had MDO during infancy. Patients were excluded if they had a tracheostomy or MDO device failure. Predictor variables included demographic data, comorbidities, and apnea-hypopnea indices (AHIs) from pre- and immediate post-MDO polysomnograms. The primary outcome measures were number of intubation attempts, laryngoscopy grade, and perioperative respiratory events at the distractor removal operation. Descriptive statistics were computed including Fisher's exact, paired sample t-tests, and Wilcoxon rank tests, and P < .05 was considered statistically significant. RESULTS: The sample included 47 (60% male) patients with a mean age at MDO of 12.0 ± 15.7 weeks. Significant improvement in AHI was seen after MDO (pre-MDO: 26.8 ± 18.4 events/hour; post-MDO 2.78 ± 2.66 events/hour; P < .001). Average number of intubation attempts decreased from 2.09 ± 1.36 to 1.30 ± 0.75 (P < .001) and the most common post-MDO laryngoscopy grade was 1 (69%). There were no intraoperative and 2 (4%) minor postoperative respiratory events, both in patients with repaired congenital cardiac disease and not related to traumatic intubation. CONCLUSION: Neither difficult intubations nor perioperative respiratory events associated with intubation trauma were seen at distractor removal, suggesting that specialty airway assistance is not routinely needed after successful MDO. DAT presence should be determined on a case-by-case basis based on specific patient risk factors.

Does Magnetic Resonance Imaging Distinguish Juvenile Idiopathic Arthritis From Other Causes of Progressive Temporomandibular Joint Destruction?

PURPOSE: Similarities in initial presentations of temporomandibular joint (TMJ) involvement from juvenile idiopathic arthritis (JIA), idiopathic condylar resorption, and other forms of progressive TMJ destruction in children create diagnostic confusion. Treatment pathways, however, depend on determination of etiology. The purpose of this study was to compare TMJ magnetic resonance images (MRIs) of patients with joint degeneration localized to the TMJs to those with JIA and TMJ involvement. STUDY DESIGN, SETTING, SAMPLE: This is a retrospective cross-sectional study including subjects younger than 18 years that presented from February 2008 to October 2019 with clinical TMJ degeneration, a gadolinium-enhanced TMJ MRI and a negative pediatric rheumatologic workup (non-JIA group), and a series of age and sex-matched subjects with TMJ degeneration on gadolinium-enhanced MRI and JIA (JIA group). MRIs were evaluated in a blinded fashion by 1 pediatric radiologist. The primary outcome variable was the radiologist's accuracy in predicting study grouping, assessed in 1 randomly-selected joint per patient. Secondary outcome variables included MRI characteristics of inflammation, osseous damage and articular disc morphology. Independent samples t-tests, sensitivity/specificity, Fisher's exact and Mann-Whitney tests were computed as applicable, and P < .05 was considered significant. RESULTS: The sample included 34 subjects: 16 non-JIA (75% female, age 13.9 ± 2.8 years) and 18 JIA (77% female, age 13.6 ± 2.8 years) (P ≥ .738). The radiologist correctly classified 64.7% of subjects as non-JIA or JIA (P = .078, sensitivity = 94.4%, specificity = 31.3%). Inflammatory and osseous findings were similar between groups (P ≥ .073). The disc was anteriorly displaced in 9 non-JIA and 0 JIA joints (P < .001, sensitivity = 100%, specificity = 100%) and flattened in 3 non-JIA and 14 JIA joints (P = .006, sensitivity = 38.9%, specificity = 90.6%). CONCLUSION AND RELEVANCE: Inflammatory and osseous findings on gadolinium-enhanced TMJ MRIs are insufficient to determine the etiology of progressive TMJ destruction. Disc characteristics, however, significantly differ between JIA and non-JIA etiologies and may be important in differentiating these conditions.

Can Dynamic Magnetic Resonance Images Improve Prenatal Diagnosis of Robin Sequence.

BACKGROUND: Robin sequence (RS) is a triad of micrognathia, glossoptosis, and airway obstruction. Prenatal diagnosis of RS improves delivery planning and postnatal care, but the process for prenatal diagnosis has not been refined. The purpose of this study was to determine if dynamic cine magnetic resonance imaging (MRI) can improve the reliability of prenatal diagnosis for RS compared to current static imaging techniques. MATERIALS AND METHODS: This is a retrospective cross-sectional study including fetuses with prenatal MRIs obtained in a single center from January 2014 to November 2019. Fetuses were included if they: 1) had a prenatal MRI with cine dynamic sequences of adequate quality, 2) were live born, and 3) had postnatal craniofacial evaluation to confirm RS. Patients without postnatal confirmation of their prenatal findings were excluded. The primary predictor variable was imaging type (cine or static MRI). Outcome variables were tongue and airway measurements: 1) tongue height, 2) length and width, 3) tongue shape index, 4) observation of tongue touching the posterior pharyngeal wall, and 5) measurement of oropharyngeal space. All measurements were made independently on the cine images and on static MRI sequences for the same cohort of subjects by a pediatric radiologist. Data were analyzed using paired samples t tests and Fisher exact tests, and significance was set as P < .05. RESULTS: A total of 11 patients with RS were included in the study. The smallest airway space consistently demonstrated complete collapse on the cine series compared to partial collapse on static images (0 mm vs 1.7 ± 1.4 mm, P = .002). No other imaging variable was statistically significantly different between techniques. CONCLUSIONS: Cine imaging sequences on prenatal MRI were superior to static images in discerning complete collapse of the smallest airway space, an important marker of RS. This suggests a possible benefit to adding dynamic MRI evaluation for prenatal diagnosis of this condition.

The Family Impact of a Robin Sequence Prenatal Diagnosis.

OBJECTIVE: Assess the impact of prenatal diagnosis of Robin sequence (RS) on parental experience during gestation and early infancy. DESIGN: Prospective case-control study. An online survey was administered via email to 44 parents representing 34 unique patients with RS. PARTICIPANTS: Parents of children diagnosed with RS and who received mandibular distraction at our tertiary care children's hospital. Participants were separated by the timing of RS diagnosis into prenatal and control postnatal groups. MAIN OUTCOME MEASURES: Effects of timing of diagnosis on parents' preparation, caregiver support, education about the condition, stress, and overall mental health. RESULTS: Complete responses were received from 44 parents representing 34 unique patients (50% response rate): prenatal, n = 17; postnatal, n = 27. Prenatal diagnosis improved parents' satisfaction regarding time to prepare for treatment (P = .001), stress of uncertainty about their child's health (P = .018), and stress about the operation(s) their child would need (P = .001). Both the prenatal (82%) and postnatal (78%) groups reported a negative impact on mental health based on diagnosis timing. All parents in the prenatal group preferred having received a prenatal diagnosis and the majority of the postnatal group (85%) would have preferred to have received the diagnosis prenatally. CONCLUSIONS: Prenatal diagnosis of RS provided tangible benefits for parents by allowing them to mentally prepare, make plans for delivery and treatment, and become educated about the condition. Parents in both groups reported a negative impact on their mental health based on diagnosis timing and the majority of parents consistently preferred prenatal diagnosis.