Robin sequence: a single treatment protocol

Abstract

Robin sequence is not only an anatomic obstructive disorder to be treated with surgical procedures, but knowledge about children s growth and development must be applied by a multidisciplinary team, since this permits the maintenance of airway permeability and of the ability to feed orally, often without the need of surgical procedures and their risks, especially when applied to neonates and small infants.

“Mandibular distraction osteogenesis for severe airway obstruction in Robin Sequence. Case report”

“…Treatment protocols must be individualized for every RS patient, depending on the underlying cause of obstruction (Schaefer et al, 2004). According to Marques et al (2005) a preoperative comprehensive airway endoscopy is essential to define the type of pathology and to determine which treatment modalities are indicated. Moreover, Tibesar et al (2006) point out that the patient must fulfil certain criteria, before MDO is performed.…”

“…Its contribution to early decannulation of tracheostomy-dependent RS patients has been well documented (Denny et al, 2001;Figueroa and Polley, 2002;Monasterio et al, 2002;Steinbacher et al, 2005). The procedure in the neonatal period is not without risks and complications requiring careful patient selection (Schaefer et al, 2004;Marques et al, 2005).…”

A Pierre Robin Syndrome with Absent Anterior 2/3 Tongue—A Case Report

“…While there is a great variation in severity, PRS is characterized by the following phenotypic features: micrognathia (small and symmetrical receded mandible), glossoptosis (tongue of variable size falls backwards into the post pharyngeal wall), cleft palate (U or V shaped) [10] [11]. Infants with PRS sequence often have airway obstruction, feeding difficulties, and challenges in gaining weight, and they may have associated anomalies, including hypotonia and limb reduction defects [12].…”

Is Amniotic Fluid Level a Predictor for Syndromic Diagnosis in Robin Sequence?

“…The craniofacial findings of RS, which also frequently include a cleft of the secondary palate (Marques et al, 2005), may occur in isolation or as a component of an underlying syndrome (Shprintzen, 1992). Hundreds of syndromes have been associated with the RS phenotype (Cohen, 1999;Evans et al, 2006); Stickler syndrome and 22q11.2 deletion syndrome are the most common (Shprintzen, 1992;Evans et al, 2006).…”