11/22/2023 0 Comments Pierre robin sequence jacksonville flPreviously, laryngomalacia was deemed a contraindication to successful distraction in Robin sequence patients. Neonates with Robin sequence may also have secondary airway abnormalities that compound respiratory distress, such as laryngomalacia, bronchial stenosis, bronchomalacia, or tracheomalacia,, which may influence the decision to proceed with MDO. Concomitantly, under the same anesthetic before MDO, a pediatric otolaryngologist performs a DISE. A maxillofacial computed tomography (CT) scan is obtained to evaluate the quality of the bone stock as well as mandibular and temporomandibular joint (TMJ) morphology. The senior authors’ protocol of care includes PSG on all neonates under consideration for MDO unless the patient is intubated or PSG is not tolerated/possible. Establishment of reference values for neonates will provide a helpful construct to further standardize polysomnographic findings in the complex Robin sequence population. Thus, PSG indications for neonatal mandibular distraction osteogenesis (MDO) for Robin sequence are currently based largely on clinical judgment. Most craniofacial surgeons select an apnea-hypopnea index (AHI) of 11 to 29 as their minimum for surgical intervention. , A severe obstructive index in neonates has been reported from greater than 6 to greater than 24, thereby representing a wide range of interpretation in the literature. However, PSG interpretation is not standardized for neonates. PSG remains the gold standard to quantify the severity of airway obstruction and to identify the presence of central sleep apnea. Direct laryngoscopy/bronchoscopy, and polysomnography (PSG) should be performed, whenever possible, for patients under consideration for surgical intervention.Īlthough drug-induced sleep endoscopy (DISE) from nasal aperture to bronchus is the yardstick to assess glossoptosis and other associated airway anomalies, the degree of glossoptosis may not correlate with the severity of respiratory dysfunction. Although there are multiple conservative interventions available for infants expressing mild to moderate airway obstruction, this article addresses the implementation of mandibular distraction in severely affected infants and neonates. Inaccurate use of the correct Robin sequence definition hinders communication between specialists and thwarts the development of standardized care protocols.Ī multidisciplinary approach to Robin sequence evaluation and treatment is critical and includes neonatologists, geneticists, pulmonologists, developmental pediatricians, otolaryngologists, plastic surgeons, pediatric gastroenterologists, nurse feeding specialists, and speech-language pathologists. With Robin sequence, nosologic confusion is evidenced by the wide variability in defining the clinical entity, which is highlighted in a survey of American Cleft Palate-Craniofacial Association providers. , Cleft palate is not a criterion for the diagnosis of Robin sequence despite the fact that up to 90% of affected patients have a concomitant U-shaped or V-shaped cleft of the posterior palate. In his 1923 publication, Robin first described the eponymous constellation of respiratory distress associated with glossoptosis in the presence of mandibular micrognathia. Pierre Robin sequence is defined by the clinical triad: mandibular hypoplasia, glossoptosis, and airway obstruction. Specific indications for neonatal mandibular distraction have not been codified, but it is a critical next step toward the standardization of care. The complication rate associated with neonatal mandibular distraction is low in both occurrence and level of acuity. Understanding mandibular morphology in Robin sequence is crucial to plan and to properly place osteotomies and vectors. In properly selected patients, neonatal mandibular distraction osteogenesis can resolve tongue-based airway obstruction and obviate tracheostomy in Pierre Robin sequence patients with severe airway obstruction.
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