pierre robin syndrome intubation

OBJECTIVE: To find predictors of a difficult intubation in infants with an isolated or a syndromic cleft lip/palate. STUDY DESIGN: Retrospective review: single-blind trial. SETTINGS: Tertiary care centre. METHODS: A total of 145 infants born with cleft lip/palate were enrolled. Three clinical and seven lip/palate anatomic parameters were evaluated. The grade of intubation was determined by the anesthesiologist at the time of the labioplasty/staphylorrhaphy surgery at 3 and 10 months, respectively...

Symptomatic micrognathia, as seen in syndromic and isolated presentations of the Robin sequence (RS), can pose immediate an ongoing threats to the well-being of neonates. Upper airway obstruction can manifest as acute respiratory insufficiency requiring postpartum intubation and mechanical ventilation or as a mild irregularity in the oropharyngeal airflow that can be managed by positioning the newborn in a prone or decubitus position. Clinically significant micrognathia is often accompanied by some degree of feeding difficulty, obstructive sleep apnea, and gastroesophageal reflux disease, all of which should be evaluated by a multidisciplinary team of specialists before a definitive treatment plan is formulated...

INTRODUCTION: Pierre Robin sequence (PRS) is a congenital anomaly presenting with micrognathia, glossoptosis and a cleft palate. This study describes a decade's experience of the management of upper airway obstruction (UAO) in PRS patients with a nasopharyngeal airway (NPA). METHODS: This study was conducted by paediatric respiratory and otolaryngology departments. Children with PRS referred with UAO were evaluated according to a standard protocol. Data collected included the degree of airway obstruction, method of airway management, polysomnography data before and after intervention, and longer term follow-up...

BACKGROUND: Children with Pierre Robin sequence (PRS) have significant oropharyngeal abnormalities, with respiratory and feeding difficulties. Gastrostomy tubes (G-tube) provide a means for nutrition. OBJECTIVE: To evaluate the safety and efficacy of percutaneous G-tube insertion in children with PRS. MATERIALS AND METHODS: Of 120 children with PRS (1996-2009), 40 were referred for G-tube insertion; clinical details were reviewed in 37/40 children (18M, 19F) at three time periods: (1) pre-G-tube insertion, (2) at G-tube insertion, (3) at G-tube removal...

Pierre-Robin syndrome (PRS) is often associated with difficulty in endotracheal intubation. We present the use of percutaneous dilational tracheotomy (PDT) for airway management of a newborn with PRS and a glossopharyngeal web. A 2-day-old term newborn with PRS and severe obstructive dyspnea was evaluated by the anesthesiology team for airway management. A direct laryngoscopy revealed a glossopharyngeal web extending from the base of the tongue to the posterior pharyngeal wall. The infant was spontaneously breathing through a 2 mm diameter fistula in the center of this web...

OBJECTIVES: To evaluate the course and prognosis of airway obstruction, feeding difficulties and hearing abnormalities in patients with Pierre Robin sequence (PRS). METHODS: A retrospective review was conducted, of 69 patients with PRS, attending between 1991 and 2010 at the Children's University Hospital in Dublin. Data regarding airway management, nutritional status and hearing difficulties was collected prospectively. RESULTS: Airway obstruction requiring intervention other than positional therapy was seen in 39% (27) patients...

BACKGROUND: Pierre Robin Sequence (PRS) is characterised by mandibular micrognathia and/or retrognathia, glossoptosis and upper airway obstruction (UAO). In severe cases, UAO and cyanosis occur immediately after birth and endoscopic intubation may become necessary. Therefore, prenatal diagnosis with referral to a specialized department is important. METHOD: A non-invasive interdisciplinary treatment protocol is presented. The postnatal adjustment of the preepiglottic baton plate (PEBP) as early as possible is essential in this concept...

OBJECTIVE: To determine the endotracheal tube (ETT) size and presence of subglottic narrowing in children less than 1 year old with Pierre Robin Sequence (PRS). MATERIALS AND METHODS: We performed a retrospective review from 2005 to 2009 of infants with PRS who underwent diagnostic laryngoscopy (DL) and intubation. RESULTS: Fifteen children with a median age of 25 days were reviewed. All patients, except one, were born full term. Subglottic narrowing was visualized in 5 patients (33%)...

OBJECTIVE: The purpose of this study was to evaluate subperiosteal release of the floor of the mouth (SRFM) musculature to correct neonatal airway obstruction in children with Pierre Robin sequence and to compare this procedure with other surgical options available. METHODS: A retrospective chart review of patients who underwent a SRFM for neonatal severe airway obstruction unresponsive to conservative management was performed. Preoperative and postoperative oxygenation parameters, sleep studies, feeding difficulties, and long-term evolution were documented...

BACKGROUND: The compromised airway in Robin sequence demands prompt operative intervention. Tongue-lip adhesion is one alternative; however, the outcome of this technique is variable. The purpose of this study was to identify variables that preoperatively predict the success of adhesion in Robin sequence patients with life-threatening respiratory distress. METHODS: This is a retrospective review of infants with severe (Laberge grade II or III) Robin sequence managed by tongue-lip adhesion...

We report a successful use of pediatric Airtraq optical laryngoscope in a pediatric patient with Robin sequence. Robin sequence accompanies a characteristic facies and its clinical presentation is marked by micrognathia, retrognathia, glossoptosis, and respiratory obstruction. A 1-year-5 month-old boy with Robin sequence was scheduled for palate repair under general anesthesia. After anesthetic induction with thiopental and neuromuscular paralysis with rocuronium, initial laryngeal view with the conventional Macintosh laryngoscope was Cormack-Lehane grade II due to restricted mouth opening and micrognathia...

Fiberoptic intubation in pediatric patients is often required especially in difficult airways of syndromic patients i.e. Pierre Robin Syndrome. Small babies will desaturate very quickly if ventilation is interrupted mainly to high metabolic rate. We describe guidelines to perform a safe fiberoptic intubation while maintaining spontaneous breathing throughout the procedure. Steps requiring the use of propofol pump, fentanyl, glycopyrrolate, red rubber catheter, metal insuflation hook, afrin, lubricant and lidocaine spray are shown...

Pierre Robin Sequence (PRS) is a craniofacial anomaly characterized by a triad of micrognathia, glossoptosis, and cleft palate. Infants with PRS frequently have feeding problems that may require supplemental nutrition through a nasogastric or gastrostomy tube. Very few published studies have illustrated the most appropriate method for securing an enteral feeding route in this patient population. One case report described a major complication leading to death from airway compromise following percutaneous endoscopic gastrostomy (PEG) tube placement...

Congenital retrognathia and glossoptosis characterize isolated Pierre Robin sequence (iPRS); the small mandible and its retracted position cause retrodisplacement of the tongue and reduction of the oropharyngeal airway. These neonates may be affected by airway obstruction, feeding difficulties, failure to thrive, and chronic hypoxaemia. To solve the respiratory problems secondary to glossoptosis, various treatments have been described including prone positioning, a nasopharyngeal tube, glossopexy, and mandibular distraction...

The Pierre Robin sequence (PRS) characterized by mandibular micro- or retrognathia and glossoptosis with or without cleft palate, presents clinically with intermittent upper airway obstruction (UAO). It is associated with other malformations in about half the cases. The incidence is about 1:8,500. Isolated PRS without other malformations does not, by itself, appear to affect neurodevelopment. Active intervention may therefore be required to reduce the risk for neurocognitive impairment resulting from UAO. Current treatment options for UAO range from prone positioning, use of a nasopharyngeal tube, glossopexy via tongue lip adhesion, mandibular distraction to tracheostomy...

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OBJECTIVE: Describe airway management using nasopharyngeal airway in infants. DESIGN: Retrospective case series (1996 to 2006). SETTING: Tertiary pediatric hospital. PATIENTS, PARTICIPANTS: The craniofacial database of Seattle Children's Hospital was searched to identify patients with one of the following diagnoses: micrognathia, secondary cleft palate, branchial arch anomalies, Pierre Robin sequence (PRS), or velocardiofacial syndrome...

This four-month-old Pierre Robin child was admitted for cleft lip repair with history of two failed attempts at intubation and subsequent cancellation of surgery. The capnography guided awake nasal intubation was considered as the child's parents were desperate to get the surgery done. A modified cuffless endotracheal tube was used with a capnography sampling tube placed within it. With the capnograph guidance the expiratory gas flow was followed to successfully intubate the child.This technique was found to be very convenient and helpful...

INTRODUCTION: Mandibular distraction is recognized as a treatment of respiratory distress in neonates with microretrognathia as seen in the Pierre Robin syndrome. However, mandibular distraction is a complex and lengthy treatment involving 2 to 4 weeks of distraction and another 4 to 12 weeks for bone consolidation. This study was performed to establish the safety and effectiveness of rapid protocol distraction osteogenesis with recombinant human bone morphogenetic protein 2 (rhBMP-2) in neonates with the Pierre Robin syndrome...

OBJECTIVE: To present a new orthopedic method for treatment of infants with Pierre Robin sequence (PRS) and upper airway obstruction (UAO) as an alternative to other established nonsurgical and surgical techniques such as positioning, nasopharyngeal or endotracheal intubation, tongue-lip adhesion, extension, distraction, or tracheostomy. DESIGN: Review of the literature and presentation of novel orthopedic appliances. SETTING: Department of Orthodontics, Dental Clinic, Medical Faculty of the University of Wuerzburg, Germany, Department and Clinic of Pediatrics, Medical Faculty of the University of Wuerzburg, Germany, 2005 to 2008...