Predictors of Respiratory Dysfunction at Diagnosis of Robin Sequence.

OBJECTIVES/HYPOTHESIS: Robin sequence (RS) consists of associated micrognathia, glossoptosis, and respiratory dysfunction, with or without cleft palate. Studies on how different patient characteristics impact the severity of respiratory dysfunction are scarce and contradictory; this study investigates how different features affect respiratory obstruction severity at diagnosis of RS in controlled analysis. STUDY DESIGN: Retrospective cohort study that enrolled 71 RS patients under 90 days old who received care in our institution from 2009 to 2020. METHODS: The primary outcome, respiratory dysfunction, was categorized into four severity groups and analyzed using a multinomial logistic regression model that considered age, sex, mandible length, cleft palate, syndromic diagnosis, other airway anomalies, and degree of glossoptosis. RESULTS: Mandible length, syndromic diagnosis, and Yellon grade 3 glossoptosis were related to poorer respiratory outcomes (need for respiratory support). In univariate analysis, for each additional 1 mm of mandible length at diagnosis, a mean reduction of 28% in the risk of needing respiratory support was observed (OR = 0.72; 0.58-0.89); syndromic diagnosis and grade 3 glossoptosis also raised the risk (OR = 6.50; 1.59-26.51 and OR = 12.75; 1.03-157.14, respectively). In multivariate analysis, only mandible length significantly maintained its effects (OR = 0.73; 0.56-0.96), a 27% reduction. CONCLUSIONS: Mandible length was an independent predictor for more severe respiratory dysfunction in RS patients, with larger mandibles showing protective effects. Syndromic diagnosis and Yellon grade 3 glossoptosis are also likely to be associated with poorer respiratory outcomes, although this was not demonstrated in multivariate analysis. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2811-2816, 2021.

Robin Sequence: Neonatal Mandibular Distraction.

Pierre Robin sequence is defined by the clinical triad: mandibular hypoplasia, glossoptosis, and airway obstruction. Mandibular distraction osteogenesis (MDO) is a standard treatment of Robin sequence associated with severe airway obstruction and is the only intervention that directly corrects the underlying anatomic pathologic condition. Compared with tongue-lip adhesion, MDO has demonstrated more success in treating airway obstruction in infants with Pierre Robin sequence, including patients with syndromic diagnoses and concomitant anomalies. This article provides a current, comprehensive review of neonatal mandibular distraction and offers treatment guidelines based on a combined surgical experience of more than 400 patients.

Best Practices for the Diagnosis and Evaluation of Infants With Robin Sequence: A Clinical Consensus Report.

IMPORTANCE: Robin sequence (RS) is a congenital condition characterized by micrognathia, glossoptosis, and upper airway obstruction. Currently, no consensus exists regarding the diagnosis and evaluation of children with RS. An international, multidisciplinary consensus group was formed to begin to overcome this limitation. OBJECTIVE: To report a consensus-derived set of best practices for the diagnosis and evaluation of infants with RS as a starting point for defining standards and management. EVIDENCE REVIEW: Based on a literature review and expert opinion, a clinical consensus report was generated. FINDINGS: Because RS can occur as an isolated condition or as part of a syndrome or multiple-anomaly disorder, the diagnostic process for each newborn may differ. Micrognathia is hypothesized as the initiating event, but the diagnosis of micrognathia is subjective. Glossoptosis and upper airway compromise complete the primary characteristics of RS. It can be difficult to judge the severity of tongue base airway obstruction, and the possibility of multilevel obstruction exists. The initial assessment of the clinical features and severity of respiratory distress is important and has practical implications. Signs of upper airway obstruction can be intermittent and are more likely to be present when the infant is asleep. Therefore, sleep studies are recommended. Feeding problems are common and may be exacerbated by the presence of a cleft palate. The clinical features and their severity can vary widely and ultimately dictate the required investigations and treatments. CONCLUSIONS AND RELEVANCE: Agreed-on recommendations for the initial evaluation of RS and clinical descriptors are provided in this consensus report. Researchers and clinicians will ideally use uniform definitions and comparable assessments. Prospective studies and the standard application of validated assessments are needed to build an evidence base guiding standards of care for infants and children with RS.

Neonatal Mandibular Distraction Osteogenesis Reduces Cleft Palate Width and Lengthens Soft Palate, Influencing Palatoplasty in Patients With Pierre Robin Sequence.

The aim of this study was to evaluate the influence of neonatal mandibular distraction osteogenesis (MDO) on cleft dimensions and on early palatoplasty outcomes in patients with Pierre Robin Sequence (PRS). In a prospective cohort study that enrolled 24 nonsyndromic patients with PRS, 12 submitted to the MDO group and 12 patients not treated (non-MDO group), the authors compared patients for cleft palate dimensions through 7 morphometric measurements at the moment of palatoplasty and for early palatoplasty outcomes. At palatoplasty, the MDO group presented a significant shorter distance between the posterior nasal spines (PNS-PNS, P < 0.001) and between uvular bases (UB-UB, P < 0.001), representing a reduction in cleft palate width. They also had significant soft palate lengthening represented by a larger distance between UB and retromolar space (UB-RM, P < 0.001) and UB and PNS (UB-PNS, P = 0.014). Their UB moved away from the posterior wall of the nasopharynx (UB-NPH, P < 0.001). The MDO group had a length of operative time significantly shorter (P < 0.001) and no early palatoplasty complications compared with the non-MDO group. In conclusion, MDO acted as an orthopedic procedure that reduced cleft palate width and elongated the soft palate in patients with PRS. These modifications enabled a reduction of around 11% in the length of operative time of palatoplasty (P < 0.001).

Tomographic Assessment of Bone Formation After the Collares Technique of Gingivoperiosteoplasty in Patients With Cleft Lip and Palate.

OBJECTIVES: This cross-sectional study sought to assess bone formation and spontaneous tooth eruption in a cohort of 25 consecutive patients aged 6 to 11 years who underwent primary gingivoperiosteoplasty by the Collares technique. DESIGN: Cross-sectional study assessing bone formation in the cleft area using a within-group time series design. SETTING: Hospital de Clínicas de Porto Alegre (HCPA), a tertiary hospital in Brazil. PATIENTS: Twenty-five patients with nonsyndromic, complete unilateral cleft lip and palate, no comorbidities, and unerupted permanent canines. INTERVENTION: Cheiloplasty was performed by means of the Millard II technique, with the addition of a triangle at the mucocutaneous junction, vomer flap nasal floor closure, and wide subperiosteal elevation, followed by gingivoperiosteoplasty by the Collares technique. MAIN OUTCOME MEASURES: Cone-beam computed tomography was used to assess treatment effect. In a novel method, software was used to obtain two three-dimensional reconstructions, one each of the cleft and noncleft sides, enabling quantitative comparison of bone presence in the alveolar defect area. RESULTS: Of the 25 patients, 24 achieved bone bridge formation. The cleft side had 75.1% (67.9%-82.3%) of the bone volume, 70.5% (53.1%-87.9%) of the height, and 63.3% (44.1%-82.5%) of the width of the noncleft side. Bone formation was 17.28% lower in patients with lateral incisor agenesis. CONCLUSION: Collares gingivoperiosteoplasty performed well as a technique for alveolar repair in patients with cleft lip and palate, allowing spontaneous eruption of deciduous and permanent lateral incisors through the bone bridge created.

Evaluation of nasal patency by acoustic rhinometry after repair of complete unilateral cleft lip and palate.

PURPOSE: The aim of the current study was to evaluate nasal patency by acoustic rhinometry in children aged 4-8 years with repaired complete unilateral cleft lip and palate (UCLP) compared with an age-matched control group without cleft lip and palate (CLP). METHODS: This comparative cross-sectional study was conducted at a tertiary care teaching hospital and a private ENT clinic in Porto Alegre, southern Brazil. The case group consisted of 38 children who had undergone surgery for complete UCLP (mean age, 6.44 years), and the control group of 21 children without CLP (mean age, 6.21 years) recruited among patients seeking medical care for ear diseases at the private clinic. Acoustic rhinometry was performed in all children after administration of oxymetazoline hydrochloride (2 × 0.25 mg/mL) for nasal vasoconstriction. RESULTS: The minimal cross-sectional area and nasal volume of the cleft side were smaller than those of the non-cleft side in the case group (p = 0.001). When the two groups were compared, the non-cleft side in the case group did not differ from the control group (p = 0.175), but the minimal cross-sectional area and volume of the cleft nasal cavity were smaller than the mean values of the two nostrils of controls (p = 0.001). CONCLUSION: In conclusion, our findings show that nasal patency on the cleft side is impaired in children surgically treated for complete UCLP.

Comparative study between cortical bone graft versus bone dust for reconstruction of cranial burr holes.

BACKGROUND: As a consequence of the progressive evolution of neurosurgical techniques, there has been increasing concern with the esthetic aspects of burr holes. Therefore, the objective of this study was to compare the use of cortical bone graft and bone dust for correcting cranial deformities caused by neurosurgical trephines. METHODS: Twenty-three patients were enrolled for cranial burr hole reconstruction with a 1-year follow-up. A total of 108 burr holes were treated; 36 burr holes were reconstructed with autogenous cortical bone discs (33.3%), and the remaining 72 with autogenous wet bone powder (66.6%). A trephine was specifically designed to produce this coin-shaped bone plug of 14 mm in diameter, which fit perfectly over the burr holes. The reconstructions were studied 12 months after the surgical procedure, using three-dimensional quantitative computed tomography. Additionally, general and plastic surgeons blinded for the study evaluated the cosmetic results of those areas, attributing scores from 0 to 10. RESULTS: The mean bone densities were 987.95 ± 186.83 Hounsfield units (HU) for bone fragment and 473.55 ± 220.34 HU for bone dust (P < 0.001); the mean cosmetic scores were 9.5 for bone fragment and 5.7 for bone dust (P < 0.001). CONCLUSIONS: The use of autologous bone discs showed better results than bone dust for the reconstruction of cranial burr holes because of their lower degree of bone resorption and, consequently, better cosmetic results. The lack of donor site morbidity associated with procedural low cost qualifies the cortical autograft as the first choice for correcting cranial defects created by neurosurgical trephines.