Carly E. Calabrese

Micrognathia and Oropharyngeal Space in Patients With Robin Sequence: Prenatal MRI Measurements

PURPOSEnMicrognathia is the initiating feature of Robin sequence (RS) and leads to airway obstruction. Prenatal identification of micrognathia is currently qualitative and has not correlated with postnatal findings in previous studies. Oropharyngeal airway space has not been evaluated prenatally. The purposes of this study were to 1) quantitate mandibular characteristics and oropharyngeal size at prenatal magnetic resonance imaging (MRI) and 2) identify differences in fetuses with postnatal RS compared with those with micrognathia (without RS) and normal controls.nnnMATERIALS AND METHODSnThis is a retrospective case-control study of fetuses with prenatal MRIs performed from 2002 through 2017 who were live born and evaluated postnatally for craniofacial findings. Postnatal findings were used to divide patients into 3 groups: 1) RS (micrognathia, glossoptosis, and airway obstruction), 2) micrognathia without RS (micrognathia), and 3) a gestational-age matched control group with normal craniofacial morphology (control). Inferior facial angle (IFA), jaw index, and oropharyngeal space (OPS) were calculated and compared among groups.nnnRESULTSnOf 116 patients in this study, 27 had RS (23%), 35 had micrognathia (30%), and 54 were control subjects (47%). IFA, jaw index, and OPS were statistically significantly smaller in the RS group compared with the comparison groups (Pxa0<xa0.0001).nnnCONCLUSIONSnPrenatal MRI measurements of micrognathia and OPS are considerably different in patients with RS compared with other groups, including those with micrognathia alone. These measurements might serve as reliable prenatal predictors of RS.

In Utero Glossoptosis in Fetuses With Robin Sequence: Measurements From Prenatal MRI

Objective: Glossoptosis causes airway obstruction in patients with Robin sequence (RS), but little is known about the in-utero tongue. The purpose of this study was to assess shape and position of the fetal tongue on prenatal magnetic resonance imaging (MRI) to determine if this is predictive of postnatal RS. Design: Retrospective case-control study including fetuses with prenatal MRIs performed from 2002 to 2017. Inclusion criteria were (1) prenatal MRI of adequate quality, (2) live born and evaluated postnatally for craniofacial findings. Subjects were divided into groups based on postnatal findings: (1) RS, (2) micrognathia without RS, and (3) a gestational-age-matched control group with normal craniofacial morphology. Outcome variables were based on the prenatal MRI and included fetal tongue height, length, and width, tongue shape index (TSI, ratio of height to length), and observation of tongue touching the posterior pharyngeal wall. Results: A total of 116 subjects with mean gestational age at MRI of 25.6 ± 5.1 weeks were included: RS, n = 27 (23%); micrognathia, n = 35 (30%); control, n = 54 (47%). Tongue length was significantly shorter (P = .009) and TSI was significantly larger in the RS group (P < .0001). The tongue touched the posterior pharyngeal wall in 5 (19%) of the RS group and in no subjects in the other groups (P < .0001). Conclusion: In utero tongue shape and position were significantly different in fetuses with postnatal RS compared to those with isolated micrognathia and controls. Prenatal MRI tongue characteristics may be predictors for postnatal RS.

An algorithm for predicting Robin sequence from fetal MRI

Infants with Robin sequence (RS) may present with airway compromise at delivery. Prenatal diagnosis would improve preparation and postnatal care. The purpose of this study was to devise a predictive algorithm for RS based on fetal magnetic resonance imaging (MRI).

Pathogenesis of Cleft Palate in Robin Sequence: Observations From Prenatal Magnetic Resonance Imaging

PURPOSEnThe etiology of the palatal cleft in Robin sequence (RS) is unknown. The purpose of this study was to assess the position of the fetal tongue at prenatal magnetic resonance imaging (MRI) and to suggest a potential relation between tongue position and development of the cleft palate seen in most patients with RS.nnnMATERIALS AND METHODSnThis is a retrospective case-and-control study including fetuses with prenatal MRIs performed in the authors center from 2002 to 2017. Inclusion criteria were 1) prenatal MRI of adequate quality, 2) liveborn infant, and 3) postnatal diagnosis of RS (Robin group) or cleft lip and palate (CLP group). Patients with postnatal RS without a palatal cleft were excluded. A control group with normal facial morphology was matched by gestational age. The outcome variable was tongue position at fetal MRI, described as within the cleft, along the floor of the mouth (normal), other, or indeterminate.nnnRESULTSnOne hundred twenty-two patients with mean gestational age at MRI of 25.8xa0±xa04.9xa0weeks were included (Robin, nxa0=xa021 [17%]; CLP, nxa0=xa047 [39%]; control, nxa0=xa054 [44%]). The tongue was visualized within the palatal cleft in 76.2% of the Robin group and 4.3% of the CLP group. The tongue was found along the floor of the mouth (normal) in the remainder of the Robin and CLP groups and in 100% of the control group.nnnCONCLUSIONnThese findings suggest a relation between in utero tongue position and the development of cleft palate in RS.

Is Tongue-Lip Adhesion or Mandibular Distraction More Effective in Relieving Obstructive Apnea in Infants With Robin Sequence?

PURPOSEnTongue-lip adhesion (TLA) and mandibular distraction osteogenesis (MDO) are the most common operations for obstructive apnea (OA) in infants with Robin sequence (RS). The purpose of this study was to compare early outcomes of TLA and MDO on resolution of OA.nnnMATERIALS AND METHODSnThis is a retrospective cohort study of infants with RS treated with TLA or MDO from 2005 through 2018. To be included, patients had to have polysomnograms pre- and postoperatively unless intubated or tracheostomy dependent. Patients were excluded if the operation occurred after 1xa0year of age. The primary predictor variable was type of operation (TLA or MDO). The primary outcome variable was successful resolution of OA, defined as 1) postoperative apnea-hypopnea index (AHI) score lower than 5, 2) postoperative OA severity score of 0 to 1 (no to mild OA), and 3) no need for additional airway intervention during the first year of life. Descriptive, bivariate, and regression analyses were performed. Statistical significance was set at a P value less than .05.nnnRESULTSnForty-three patients were included (TLA, nxa0=xa019 [44%]; MDO, nxa0=xa024 [56%]). Eighteen patients (41%) were syndromic. Patients in the TLA group were significantly younger at operation (28.2xa0±xa023.1xa0days) compared with those in the MDO group (87.1xa0±xa081.7xa0days; Pxa0=xa0.002). The MDO group had more severe preoperative OA (AHI score, 20.5xa0±xa014.9; OA severity score, 4.7xa0±xa00.8) than the TLA group (AHI score, 17.6xa0±xa031.0; OA severity score, 3.6xa0±xa01.4; Pxa0<xa0.041). Postoperative AHI score and OA severity score for the TLA group were 11.7xa0±xa019.5 (33.5% decrease; Pxa0=xa0.496) and 2.3xa0±xa01.8 (improvement by 1.3xa0±xa02.4 levels; Pxa0=xa0.051), respectively. Postoperative AHI score and OA severity score for the MDO group were 1.1xa0±xa01.8 (94.6% decrease; Pxa0<xa0.001) and 0.2xa0±xa00.4 (improvement by 4.6xa0±xa00.8 levels; Pxa0<xa0.0001), respectively. Successful resolution of OA occurred in 9 patients (47%) in the TLA group and 22 patients (92%) in the MDO group. Postoperative complications were similar.nnnCONCLUSIONnMDO is more effective than TLA in relieving OA in infants with RS.

Does Mandibular Distraction Change the Laryngoscopy Grade in Infants With Robin Sequence

PURPOSEnTo review a series of infants with Robin sequence (RS) who underwent mandibular distraction osteogenesis (MDO) at the authors institution and document changes in pre- and postoperative laryngoscopy grades.nnnMATERIALS AND METHODSnConsecutive patients with RS who underwent MDO from March 2005 to June 2017 were identified. Patients were included if they had a preoperative polysomnogram confirming obstructive sleep apnea, had failed nonoperative airway management, had undergone MDO by the senior author (C.M.R.), and had complete documentation. Patients were excluded if they did not have a laryngoscopy performed (ie, tracheostomy dependent) or if there was insufficient information in their medical record. Variables included demographic data, operative information, and laryngoscopic details at 3 time points: before MDO (T0), at device removal (T1), and at latest follow-up laryngoscopy (T2). Descriptive statistics were computed. Significance was set at a P value less than .05.nnnRESULTSnTwenty-two infants with RS underwent MDO during the study period and 13 of these met criteria for inclusion. Mean age at MDO (T0) was 19.6xa0±xa038.8xa0weeks. Mean ages at device removal (T1) and latest follow-up laryngoscopy (T2) were 28.2xa0±xa041.5 and 62.7xa0±xa049.6xa0weeks, respectively. Three patients had Stickler syndrome; the others had nonsyndromic RS. Mean apnea-hypopnea index was 23.6xa0±xa016.6 preoperatively and 0.3xa0±xa00.7 after MDO (Pxa0=xa0.011). At T0, a grade IIb laryngoscopic view was most common (46.1%) and only 3 patients (23.1%) had a grade I view. At T1, most patients (69.2%) had a grade I view and only 2 (14.4%) had a grade of IIb or worse (Pxa0=xa0.011). At T2, all but 1 patient had a grade I view.nnnCONCLUSIONnIn addition to resolving obstructive apnea, MDO is associated with an improvement in the grade of laryngoscopic view. This could increase airway safety at home and improve ease of intubation for future operations.

Early Management of Infants With Robin Sequence: An International Survey and Algorithm

PURPOSEnThere is no widely accepted protocol for management of infants with Robin sequence (RS) who present with airway obstruction and feeding impairment. The purposes of this study were to evaluate diagnostic and treatment preferences of clinicians from the United States and non-US countries and to use these data to propose an algorithm for early management of infants with RS.nnnMATERIALS AND METHODSnA cross-sectional study was implemented using a survey, which was distributed to craniofacial surgeons and non-surgeon physicians involved in management of infants with RS. Predictor variables were nationality, specialty, and surgical volume. Outcome variables included survey responses regarding preferences for evaluation and early treatment of patients with RS. A diagnostic and early treatment algorithm was proposed based on the most common survey responses. Descriptive and analytic statistics were calculated and a P less than .05 was considered significant.nnnRESULTSnA total of 275 responses were received and 82 were excluded, leaving a final sample of 193 participants: 155 (80%) surgeons and 38 (20%) non-surgeon physicians. Thirty-seven US states were represented by 143 (74%) respondents and 50 (26%) participants were from 19 non-US countries. There were 96 (50%) low-volume and 97 (50%) high-volume respondents. There was general agreement in methods for evaluation and early treatment among respondents; the only significant differences were 1) US surgeons operate earlier to address airway obstruction than those from other countries (Pxa0=xa0.001), 2) high-volume providers tend to operate earlier (Pxa0=xa0.017) and to choose mandibular distraction more frequently (Pxa0=xa0.003) than low-volume respondents, and 3) mandibular distraction is the preferred operation in the United States, but several operations for airway improvement are used equally in non-US countries (Pxa0<xa0.001).nnnCONCLUSIONnDespite variation in the evaluation and treatment of infants with RS, this international and multispecialty survey showed trends that could represent best practices. An algorithm based on these findings is proposed.

Evaluation of Andrews' Analysis as a Predictor of Ideal Sagittal Maxillary Positioning in Orthognathic Surgery

PURPOSEnThere is no universally accepted method for determining the ideal sagittal position of the maxilla in orthognathic surgery. In Element II of The Six Elements of Orofacial Harmony, Andrews used the forehead to define the goal maxillary position. The purpose of this study was to compare how well this analysis correlated with postoperative findings in patients who underwent bimaxillary orthognathic surgery planned using other guidelines. The authors hypothesized that the Andrews analysis would more consistently reflect clinical outcomes than standard angular and linear measurements.nnnMATERIALS AND METHODSnThis is a retrospective cohort study of patients who had bimaxillary orthognathic surgery and achieved an acceptable esthetic outcome. Patients with no maxillary sagittal movement, obstructive sleep apnea, cleft or craniofacial diagnoses, or who were non-Caucasian were excluded. Treatment plans were developed using photographs, radiographs, and standard cephalometric measurements. The Andrews analysis, measuring the distance from the maxillary incisor to the goal anterior limit line, and standard measurements were applied to end-treatment records. The Andrews analysis was statistically compared with standard methods.nnnRESULTSnThere were 493 patients who had orthognathic surgery from 2007 through 2014, and 60 (62% women; mean age, 22.1xa0±xa06.8xa0yr) met the criteria for inclusion in this study. The mean Andrews distances were -4.8xa0±xa02.9xa0mm for women and -8.6xa0±xa04.6xa0mm for men preoperatively and -0.6xa0±xa02.1xa0mm for women and -1.9xa0±xa03.4xa0mm for men postoperatively. For women, the Andrews analysis was closer to the goal value (0xa0mm) postoperatively than any standard measurement (Pxa0<xa0.001). For men, the linear distance from the A point to a vertical line tangent to the nasion from the McNamara analysis performed best (Pxa0<xa0.001), followed by the Andrews analysis.nnnCONCLUSIONnThe Andrews analysis correlated well with the final esthetic sagittal maxillary position in the present sample, particularly for women, and could be a useful tool for orthognathic surgical planning.

Temporomandibular Joint Ankylosis After Ramus Construction With Free Fibula Flaps in Children With Hemifacial Microsomia

PURPOSEnPatients with hemifacial microsomia (HFM) and Kaban-Pruzansky type III mandibular deformities require ramus construction with autologous tissue. The free fibula flap, an alternative to the costochondral graft, has favorable characteristics for this construction but may be associated with temporomandibular joint ankylosis. The purposes of this study were to present a series of patients with HFM who underwent free fibula flap ramus construction, to determine the incidence of ankylosis, and to identify perioperative factors associated with ankylosis.nnnMATERIALS AND METHODSnWe performed a retrospective cohort study of patients with HFM who underwent ramus construction with a free fibula flap at Boston Childrens Hospital from 2003 to 2015. Patients who had at least 1xa0year of follow-up and complete medical records were included. The predictor variables included demographic information, HFM severity, surgical history, and operative details. The primary outcome variable was the occurrence of ankylosis. Descriptive statistics were calculated, and significance was set at Pxa0<xa0.05.nnnRESULTSnWe included 8 patients (75% of whom were female patients) in the study sample. Patients underwent construction at a mean age of 11.4xa0±xa05.9xa0years (range, 5 to 21xa0years). In 5 patients (63%), ankylosis developed during the follow-up period of 7.3xa0±xa04.8xa0years. The average time from construction to ankylosis was 4.2xa0±xa03.7xa0years. The only predictor variable statistically significantly associated with ankylosis was the use of a contralateral releasing osteotomy, which reduced the rate of ankylosis (Pxa0=xa0.035). There was a trend toward a younger age in patients in whom ankylosis developed (8.8xa0±xa02.6xa0years) compared with those without ankylosis (15.5xa0±xa08.1xa0years, Pxa0=xa0.392).nnnCONCLUSIONSnThe free fibula flap can be associated with a high rate of ankylosis when used for ramus construction in patients with HFM. Passive flap insertion and/or use of a contralateral releasing osteotomy may reduce this risk.