pierre robin sequence vs treacher collins

In this study, we report 10 selected paediatric patients with severe micrognathia and airway obstruction.

Recently, in neonates, mandibular distraction osteogenesis has been popularized in the literature and it is now widely accepted as the procedure of choice in the early management of airway obstruction due to craniofacial disproportion. Thus, management regimes differ depending on the degree of upper airway obstruction (such as feeding difficulties, pulmonary infections).Ĭurrent treatments include non-surgical (prone positioning, nasopharyngeal tube/stenting, prolonged intubation), or surgical options (tongue-lip adhesion, tracheotomy, mandibular distraction osteogenesis). However, some patients may present an adequate airway when awake, but the obstruction may arise during feeding or sleeping when the pharyngeal muscle tone decreases. It is clear that not every child with mandibular hypoplasia displays airway obstruction. A wide range of clinical manifestations exists, but the main clinical problems faced by clinicians include upper airway obstruction and feeding difficulties. Robin later revised the characteristics of the syndrome and included cleft palate as an additional factor that could be present. Its incidence has been estimated as 1:8,500 to 1:20,000 newborns in the general population, with no gender predilection. The features of PRS were first described by Robin and include micrognathia, glossoptosis and respiratory distress. Retro/micrognathia may be a feature of isolated Pierre Robin sequence (PRS), when PRS is not associated with other malformations (referred as non-syndromic PRS), or is part of several congenital craniofacial syndromes, such as Treacher Collins syndrome, Goldenhar syndrome, Nager syndrome, temporomandibular joint ankylosis and velocardiofacial syndrome. Infants with congenital craniofacial anomalies (CFAs) often display associated severe mandibular hypoplasia causing obstruction of the airway through retro-positioning of the tongue-base into the posterior pharyngeal airway. In one case, the patient with Goldenhar's Syndrome, the procedure was repeated. All patients were treated with bilateral mandibular distraction: two cases with an external multivector distraction device, six cases with an internal non-resorbable device and two cases with an internal resorbable device. The two tracheotomy patients were 5 months and 2 years old respectively, and the distraction procedure was performed to remove the tracheotomy tube. Gavage therapy was employed in all patients since oral feeding was not possible. Indications for surgery included frequent apneic episodes with severe desaturation (70%). All patients were evaluated by a multidisciplinary team. Pierre Robin sequence cephalometry craniofacial morphology mandibulofacial dysostosis.Ten patients between the ages of 1 month and 2 years with severe micrognathia and airway obstruction were treated with Mandibular Distraction Osteogenesis (MDO).Īll patients underwent fibroscopic examination of the upper airway and a radiographic imaging and/or computed tomography scans to detect malformations and to confirm that the obstruction was caused by posterior tongue displacement. Treacher Collins syndrome presented a decreased mandible and a more severe vertical growth pattern compared to PRS. No differences between TCS and PRS were observed for the sagittal position of the maxilla, maxillomandibular relationship, and dental components. Group TCS showed a smaller mandibular length (Co-Go, Co-Gn) and a higher palatal plane (SN-Palatal Plane) and mandibular plane angles (SN-Go.Gn) compared to group PRS. Intergroup differences in the mandible size and growth pattern were observed. The significance level considered was 5%. Intergroup comparisons were performed using t tests. Variables evaluating the cranial base, the maxillary and mandibular skeletal components, maxillomandibular relationship, the vertical components and the dentoalveolar region were measured. Group PRS was composed of 9 patients paired by age and sex with group TCS.Ĭone beam computed tomography-derived cephalometric images taken before the orthodontic or the orthodontic-surgical treatment were analyzed using Dolphin Imaging (Dolphin Imaging 11.0 & Management Solutions). Group TCS was composed of 9 patients (4 male, 5 female) with a mean age of 12.9 years (standard deviation = 4.8). The aim of this study was to compare the dentoskeletal pattern of Treacher Collins syndrome (TCS) and nonsyndromic Pierre Robin sequence (PRS).Įighteen patients diagnosed with TCS (Group TCS) or PRS (Group PRS) in rehabilitation treatment at a single center.

Pierre robin sequence vs treacher collins