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Objectives/Hypothesis: To review airway management in Pierre Robin sequence (PRS) newborns undergoing general anesthesia and to determine if endotracheal intubation is safe in this population. Study Design: Case series and retrospective chart review at a tertiary children's hospital. Methods: PRS newborns who underwent endotracheal intubation or other airway intervention before 3 months of age between January 2000 and July 2011 were identified from a pediatric otolaryngology practice database. Indications for airway intervention, anesthetic management, method of intubation, and comorbid conditions were collected. Results: Thirty-three PRS newborns were identified. Twenty had isolated PRS, and 13 had PRS related to a coexisting syndrome. Thirteen of 35 (37%) endotracheal intubations performed in PRS newborns prior to mandibular distraction osteogenesis were accomplished with direct laryngoscopy. The remaining 22 of 35 (63%) who failed intubation with direct laryngoscopy were intubated over a flexible fiberoptic bronchoscope. No significant difference was observed between the isolated and syndromic PRS newborns with regard to technique utilized for intubation. No patient required rescue laryngeal mask airway or emergent tracheotomy, and no case resulted in death. Conclusions: This series demonstrates that endotracheal intubation is safe and effective in PRS newborns. In patients who failed intubation with direct laryngoscopy, intubation over a flexible fiberoptic bronchoscope provided a reliable alternative method. Although airway management in PRS newborns poses a significant challenge, experienced otolaryngologists and anesthesiologists can successfully manage these difficult airway cases. [ABSTRACT FROM AUTHOR]

Abstract: Introduction: In addition to upper airway obstruction, many patients with micrognathia and Pierre Robin sequence also have swallowing abnormalities and reflux. Many studies have demonstrated the effectiveness in alleviating the airway symptoms with mandibular distraction osteogenesis, but very few studies have focused on feeding and reflux outcomes. Methods: A retrospective chart review was performed to identify patients with Pierre Robin sequence who underwent mandibular distraction osteogenesis with completed pre- and post-operative upper gastroesophageal series and videofluoroscopic swallow assessments. Results: All six children in our series demonstrated significant improvements in both airway obstructive symptoms and feeding abnormalities. More specifically, all patients showed clinical and objective improvements in reflux and swallowing function after distraction surgery. Conclusion: Objective and symptomatic improvements in swallowing function and reflux disease can be seen after mandibular distraction osteogenesis in children with Pierre Robin sequence. [Copyright &y& Elsevier]

Objective. To determine benefits of early intervention in neonates with symptomatic micrognathia who underwent bilateral mandibular distraction osteogenesis within the first 90 days of life as relates to growth, need for supportive care, and further invasive procedures.Study Design. Case series with chart review.Setting. Tertiary care, academic children’s hospital.Subjects and Methods. Review of neonates with symptomatic micrognathia who underwent bilateral mandibular distraction osteogenesis in the past 5 years. Inclusion criteria included mandibular distraction osteogenesis performed within the first 90 days of life. Outcome measures included hospital course, growth curves, supportive home care needs, and airway at cleft repair.Results. Twenty-four patients met inclusion criteria. The mean age at distraction was 30 days, and the average discharge was postoperative day 14. One patient required home oxygen, 50% were able to feed exclusively by oral diet, and no patients required tracheotomy. In addition, airway results were substantial, with 90% of patients showing objective improvement in airway grade from time of mandibular distraction to time of cleft repair.Conclusion. We present our initial outcomes on mandibular distraction osteogenesis in neonates with symptomatic micrognathia. Early intervention allows discharge to home with minimal supportive care needs by avoiding tracheostomy and facilitating transition to oral feeds. The airway improvement is significant and is sustained and allows for easier intubation at time of cleft repair. [ABSTRACT FROM PUBLISHER]

Objective. In this systematic review, the authors summarize the current evidence in the literature regarding diagnosis, treatment, and long-term outcomes in neonates with tongue-based airway obstruction (TBAO) and assess the level of evidence of included studies.Data Sources. The terms Pierre Robin syndrome/sequence, micrognathia, retrognathia, and cleft palate were combined with airway obstruction, treatment, tongue-lip plication, and osteogenesis distraction to perform an Ovid literature search, yielding 341 references. The authors excluded references containing patients with isolated choanal/nasal obstruction, patients older than 12 months, and expert opinion papers, yielding 126 articles.Review Methods. The authors searched 3 electronic databases and reference lists of existing reviews from 1980 to October 2010 for articles pertaining to the diagnosis, treatment, and outcomes of TBAO. Reviewers assigned a level of evidence score based on Oxford’s Centre for Evidence Based Medicine scoring system and recorded relevant information.Results. Most studies were case studies and single-center findings. The lack of standardization of diagnostic and treatment protocols and the heterogeneity of cohorts both within and between studies precluded a meta-analysis. There was little evidence beyond expert opinion and single-center evaluation regarding diagnosis, treatment, and long-term outcomes of neonates with TBAO.Conclusions. The variability in the phenotype of the cohorts studied and the absence of standardized indications for intervention preclude deriving any definitive conclusions regarding diagnostic tools to evaluate this patient population, treatment choices, or long-term outcomes. A coordinated multicenter study with a standardized diagnostic and treatment algorithm is recommended to develop evidence for the diagnosis and treatment of neonates with TBAO. [ABSTRACT FROM PUBLISHER]

The article presents a study on the tongue suspension technique for performing tongue-lip adhesion. It notes that tongue-lip adhesion with suspension entails several advantages including the prevention of button aspiration and potential interference in early bottle-feeding and developmental oral motor function. It mentions that the technique expands the armamentarium of craniofacial surgeons in managing difficult airways in Pierre Robin sequence.

The article presents a study which investigates the efficacy of mandibular lengthening by distraction osteogenesis (MDO) in managing severe obstructive sleep apnea among infants and neonates. It notes that the findings reveal that this procedure is essential in preventing inherent risks and complications of micrognathia and obstructive sleep apnea as well as avoiding tracheostomy. It mentions that further studies must be initiated before MDO can be considered as the standard of care.

The article presents a study on the effectiveness of sodium supplementation in increasing weight gain among babies with grade 3 Pierre Robin sequence in England. It notes that the findings reveal that improvements were observed in babies' weight with low urinary sodium following oral sodium supplementation. The significant increase in weight allow babies with grade 3 Pierre Robin sequence to thrive. The study also found out that urinary sodium tests are essential in monitoring urinary sodium.

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Abstract: Objective: To evaluate if there is a difference in mandibular distraction osteogenesis (MDO) treatment success rates and adverse outcomes in newborns, early infants, and older pediatric patients. Design: Retrospective medical review spanning a nine-year period. Ten newborn (≤35 days old), five early infant (36 days–5 months) and eight older pediatric (>5 months) patients underwent MDO for treatment of micrognathia with a severe tongue-based obstruction. Success was defined as avoidance of tracheostomy or CPAP, and decannulation of patients with tracheotomies. Postoperative complications were grouped into minor and major. Results: MDO successfully treated 90% of newborns, 100% of early infants and 100% of older pediatric patients. There was no difference in the rates of success (p =0.48), minor (p =1.00) and major (p =1.00) postoperative complications between newborns and early infants. Older pediatric patients had no treatment failures, tended to have fewer minor (p =0.18) and significantly fewer major (p= 0.04) postoperative complications compared to younger patients. The distractor pin mobility (9%) and scar revisions (13%) were uncommon. Conclusions: Mandibular distraction osteogenesis is a reliable method for relieving severe tongue-based obstructions in pediatric patients. When comparing newborns and early infant patients, treatment success rates and the occurrence of complications were not found to be different. Older pediatric patients had no treatment failures, and tended to have fewer postoperative complications compared to younger patients. [Copyright &y& Elsevier]

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. Main Outcome Measure: Intubation grade. Results: The relative risk of a difficult intubation in the cleft lip, cleft palate without the Pierre Robin sequence, cleft lip-palate, and cleft palate with Pierre Robin sequence groups was 0, 2.7, 10, and 23%, respectively. The infants born with the Pierre Robin sequence had a statistically significant higher intubation grade. The degree of difficulty was increased in cases with early airway and feeding problems (p < .0001). Within the group of cleft palate patients without any lip malformation, a wider cleft was associated with a higher intubation grade with statistical significance (p = .0323). Conclusions: Infants born with Pierre Robin sequence have a statistically significantly higher risk of difficult intubation. Within this group, of all the studied factors, a clinical history of early airway and feeding problems was the best predictor of a difficult endotracheal intubation. In cleft palate patients without any cleft lip, larger width of the cleft is also a significant predictor. [ABSTRACT FROM AUTHOR]

Abstract: 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. Fifty nine percent (16/27) of these patients, who failed positional therapy, were successfully managed with a nasopharyngeal airway. Following failed intervention with nasopharyngeal airways, two patients had airway maintenance achieved with a successful glossopexy procedure. One patient had an adequate airway achieved with nasal continuous positive airway pressure. Eight patients (12%) required a surgical tracheostomy. Of those who required a tracheostomy, six patients had isolated PRS, one patient had PRS in association with Stickler syndrome and one patient had Nager acro-facial dystosis. Duration of tracheostomy tube ranged from 10 to 19 months, mean 13 months. Seventy percent (48 patients) required supplementary feeding in the form of nasogastric (NG) or gastrostomy tube. Forty-four patients were successfully managed with a temporary NG tube. One patient required a prolonged NG tube, and three required a gastrostomy tube. Twenty-one (30%) patients were successfully managed with a specialised Haberman bottle. Twenty-four patients (35%) who had their airway managed successfully by positional therapy, still required supplemental feeding. Thirty-one patients (45%) demonstrated a conductive hearing loss at some stage, which affected their speech and language development. Twenty-four patients (35%) required tympanostomy tube insertion once, while 7 (10%) of patients required ventilation tube insertion twice or more. Conclusion: Airway management in the majority of PRS can be successfully achieved by conservative methods. Even in the presence of an adequate airway, many patients will require supplemental feeding. Early audiological assessment is necessary as many patients will need tympanostomy tube placement to ensure adequate speech and language development. [Copyright &y& Elsevier]

Abstract: The aim: The aim of this work was to assess the multislice CT scan for analysis of the craniofacial anatomic changes in Pierre Robin sequence both predistraction and postdistraction, and to assess the use of unidirectional internal distractors in this patient group. Patients & methods: The study involved 11 patients. Their age at the time of distraction ranged from 2 to 7 months. Six were females and 5 were males. All had retromicrognathia, glossoptosis and obstructive sleep apnoea. All were diagnosed clinically and by CT scan. All were managed by distraction osteogenesis and were followed postoperatively by multislice CT. Results: The distance between the base of the tongue and the posterior pharyngeal wall increased by a mean of 141%, and the total mandibular length increased by a mean of 26%. The increase in the distance between the hyoid bone and the posterior pharyngeal wall increased by a mean of 42% .The distance between the hyoid bone and the genoid process increased by a mean of 9%. Conclusion: Multislice CT scan was found to be a practical imaging technique to evaluate the morphologic changes in the airway and the mandible after distraction osteogenesis. It rules out the need for other traditional methods. Owing to the plasticity and malleability of the infant mandible that allow for sufficient bone remodelling, unidirectional internal distractors achieved a satisfactory maxillomandibular relationship which was tolerated by the infants and accepted by the parents. [Copyright &y& Elsevier]

The article presents a study on the severity and prevalence of obstructive sleep apnea among infants with Pierre Robin syndrome. The results of the study shows that the severity of obstructive sleep apnea is 55%. It suggests that polysomnography must be performed because of the high incidence of obstructive sleep apnea in infants.

Abstract: Introduction: Neonatal upper airway obstruction secondary to micrognathia can be managed with conservative or surgical interventions. Traditionally, severe upper airway obstruction was managed with a tracheostomy. Although tracheostomy may be life saving, it is associated with high rates of complications and can lead to developmental problems. More recently, mandibular distraction osteogenesis has been utilized to relieve micrognathia associated airway obstruction. Methods: A clinical narrative review of the current literature was performed to evaluate the efficacy of mandibular distraction osteogenesis in neonates with Pierre Robin sequence. Objectives: (1) To evaluate whether mandibular distraction osteogenesis can relieve the upper airway obstruction in micrognathic neonates and (2) to discuss and increase the awareness of various issues surrounding neonatal mandibular distraction procedures including preoperative workup, distraction protocols, and complications. Results: Mandibular distraction osteogenesis can be a safe and effective intervention in neonates diagnosed with Pierre Robin sequence with severe micrognathia and airway obstruction. Interestingly, in patients with additional complex syndromes, the airway obstruction was not consistently alleviated. Conclusion: When conservative measures fail, mandibular distraction osteogenesis should be considered to obviate the need for a tracheostomy in newborns with micrognathia associated upper airway obstruction. [Copyright &y& Elsevier]

Pierre Robin Sequence is a congenital condition involving a combination of micrognathia and glossoptosis with or without a cleft palate. Feeding problems are often associated with cleft anamoly which make it difficult for the infant to maintain adequate nutrition. Here we present a case of 58-day-old neonate with Pierre Robin sequence, in whom we contructed a palatal obturtor for feeding. [ABSTRACT FROM AUTHOR]

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. Thirty-five (10.9%) of the 320 infants born between January 1, 1996, and March 31, 2006, identified using the criteria listed above were managed with nasopharyngeal airway (NPA) during infancy. Interventions: Use of NPA. Main Outcome Measure: Summary statistics describing the distribution of the infants' demographic characteristics, duration, and timing of their NPA placement, need for tracheotomy, feeding interventions, and death. Results: Of the 35 patients included in this case series, 60% (21) were male. Eighteen (51.4%) patients had the diagnosis of PRS, 13 (37.1%) had secondary cleft palate and other craniofacial anomalies, and four (11.4%) had branchial anomalies and micrognathia (nonsyndromic or syndromic). Thirty-one children (88.6%) were born at term. Mean and median age at initial NPA placement was 3.2 and 1.3 weeks, respectively; median duration of NPA was 8.0 weeks. Nine children received tracheotomies. Feeding tubes were required in 85.7% of patients. Two children died; however, neither death was attributed to airway obstruction or the use of NPA. Conclusions: NPA is one option in the management of patients with craniofacial anomalies and airway obstruction. The majority of nonsyndromic PRS patients treated with NPA during infancy did not require airway intervention beyond NPA. [ABSTRACT FROM AUTHOR]

Abstract: 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. Over the last 28 years, the authors have treated 118 neonates and infants affected by iPRS by carrying out traction of the mandible using two parasymphyseal wires, positioned under local anaesthesia. All the procedures were successful, with no major complication. The patients’ respiratory problems and apnoea disappeared suddenly after beginning traction. [Copyright &y& Elsevier]

Abstract: Stickler syndrome is a group of rare genetic conditions (incidence, 1/7500 births) related to mutations in the collagen genes. Both the mutations and the clinical features vary widely across affected patients. The main manifestations are craniofacial birth defects, bone and joint symptoms, ocular abnormalities, and hearing loss. Stickler syndrome may be revealed at birth (25% of cases) by a combination of cleft palate, retrognathism, and micrognathism known as Pierre Robin sequence, which may cause neonatal respiratory problems. The ocular abnormalities include severe myopia, abnormalities of the vitreous, and a high risk of retinal detachment (60% of cases), which may cause blindness (4% of cases). Severe hearing loss with onset in early childhood may impair performance at school. Osteoarthritis (75% of patients) with onset before 30 years of age is a severe manifestation that causes chronic hip and low back pain and functional impairments. Joint replacement surgery is often required. The risk associated with multiple anesthesias is highest in patients with craniofacial defects. The bone status may deserve to be evaluated, as the combination of genetic abnormalities and physical impairments may promote bone loss. Clinicians should be cognizant of Stickler syndrome so that they can detect the disease in patients and their family members, prevent functional impairments, organize a multidisciplinary management strategy, and arrange for genetic counseling. [ABSTRACT FROM AUTHOR]

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. Patients: Seven patients with significant respiratory and feeding difficulties between 0 and 6 months of age. Both patients with nonsyndromic PRS and patients with syndromic PRS were included. Interventions: The type of respiratory tract obstruction was defined by nasopharyngoscopy. Patients with type 1 obstruction received a plate with an epiglottic spur; whereas, patients with obstruction type 2, 3, or 4 received a plate with a pharyngeal tube. Results: All patients were successfully treated with orthopedic appliances alone. Under plate therapy they showed good oxygen saturation and could consequently be better nourished orally. Conclusions: The presented novel method is a noninvasive technique in treatment of infants with UAO. [ABSTRACT FROM AUTHOR]

Objective: To evaluate oral feeding capacity, the swallowing process, and risk for aspiration, both clinically and during fiberoptic endoscopic evaluation of swallowing, in infants with isolated Robin sequence treated exclusively with nasopharyngeal intubation and feeding facilitating techniques. Design: Longitudinal and prospective study. Setting: Hospital de Reabilitação de Anomalias Craniofaciais, University of São Paulo, Bauru, Brazil. Patients: Eleven infants with isolated Robin sequence, under 2 months of age, treated with nasopharyngeal intubation. Interventions: Feeding facilitating techniques were applied in all infants throughout the study period. The infants were evaluated clinically and through fiberoptic endoscopic evaluation of swallowing at first, second, and, if necessary, third week of hospitalization (T1, T2, T3). The mean volume of ingested milk was registered during clinical evaluation, and events were registered during feeding. Results: The respiratory status of all infants was improved after nasopharyngeal intubation; 72% of them presented risk for aspiration during fiberoptic endoscopic evaluation of swallowing at T1. This risk was less frequent when thickened milk was given to the infants and at subsequent evaluations (T2 and T3). Conclusions: Nasopharyngeal intubation aids in stabilizing the airway in isolated Robin sequence, but it does not relate directly to feeding. The risk for aspiration was present in most of the infants, mainly during the first week of hospitalization, and improved within a few weeks, after the use of feeding facilitating techniques. [ABSTRACT FROM AUTHOR]

Neonates with Pierre Robin sequence (PRs) suffer from varying degrees of airway obstruction and feeding difficulties, the courses of which differ from patient to patient, due to mandibular deficiency. We aimed to evaluate the course and prognosis of upper airway obstruction in 20 newborns with PRs. Among 15 isolated and 5 syndromic cases, 7 patients fell into Group I, 5 into Group II and 8 into Group III, respectively, according to the clinical classification system proposed by Caouette-Laberge. The 12 patients in Groups I and II were treated with positioning and gavage feeding, whereas the 6 patients in Group III underwent bilateral mandibular distraction. Decannulation or avoidance of tracheostomy was achieved in all of them. A patient who had a significant comorbidity was managed with tracheostomy and one patient expired due to pulmonary problems. When conservative measures fail, mandibular distraction osteogenesis should be considered to obviate tracheostomy in newborns with micrognathia. [ABSTRACT FROM AUTHOR]

The article discusses a study on airway management for neonates and infants with severe micrognathia and upper airway obstruction who underwent mandibular distraction (MD) osteogenesis at the Children's Hospital at Westmead in Sydney, New South Wales from 2004 to 2007. Pierre Robin sequence (PRS) was found in six patients. Acute cyanotic episodes with cerebral hypoxia could reportedly result from inadequate treatment. The benefit of a long-term tracheostomy to infants with severe micrognathia is noted.

Context: The disorder currently accepted as Pierre Robin syndrome/anomaly/sequence (PRS) has been plagued by controversy ever since initially being described. Controversy exists not only about the appropriate terminology and etiopathogenesis of the disorder but also about its management. Clinical findings and treatment outcomes of a large database of 266 PRS cases were compared with the current state of knowledge in the scientific literature, relating to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch‑up growth, midfacial hyperplasia, and the early management. Aims of Part 2: Contribute to the sparse scientific knowledge about pathogenesis and involved genetics. Subjects and Methods: An analysis of this large database was conducted focusing on genetic involvement, family history, and the incidence of additional syndromes. Results: Beside of differences related to clinical signs of dyspnea, feeding problems and mortality rates, various concomitant syndromes, and genetic abnormalities were found in cases of Fairbairn–Robin triad (FRT) and Siebold–Robin sequence (SRS), in addition to differences in relation to clinical signs of dyspnea, feeding problems, and mortality rates. Conclusion: Multiple FRT cases presented with various concomitant syndromes and genetic abnormalities, but only one type occurred in two SRS cases. The latter presented a significantly different mortality rate when compared to the FRT subgroup. [ABSTRACT FROM AUTHOR]

Objective: To study the prevalence of abnormal gastroesophageal reflux in infants with Robin sequence who had severe respiratory obstruction treated with nasopharyngeal intubation and to evaluate the efficacy of nonsurgical treatment. Design: Longitudinal prospective study. Setting: Hospital de Reabilitação de Anomalias Craniofaciais, University of São Paulo, Brazil. Patients: Twenty infants with severe isolated Robin sequence treated with nasopharyngeal intubation. Interventions: We performed 24-hour esophageal pH monitoring on each child at 2, 4, and 6 months of age. Respiratory and feeding status were evaluated. We considered abnormal gastroesophageal reflux as reflux index values above the 95th percentile of the Vandenplas reference for normal children. Results: The prevalence of reflux index above the 95th percentile at the first exam was 6/20, a value significantly higher than the reference (5/103, p , .01). At the second and third exams, reflux index values were decreased. Ninety percent of the infants showed improvement of respiratory difficulty and developed oral feeding capacity. Conclusions: The prevalence of abnormal gastroesophageal reflux is higher in infants with severe cases of Robin sequence than in normal infants. Nonsurgical procedures improved respiratory and feeding difficulties of most of these infants. [ABSTRACT FROM AUTHOR]

The triad of glossoptosis, micrognathia, and cleft palate characterizes the Pierre Robin sequence (PRS). Complications can be severe and the clinical manifestations are very heterogeneous, but airway obstruction is almost present and may be life threatening. To avoid tracheostomy or allow for early decannulation in severely affected children, external or biorebsorbable internal distraction devices can be applied to repair mandibular deficiences. We report a case of a children submitted to bilateral mandibular distraction with external nonresorbable devices. After optimal lengthening of mandibles, laringotracheoplasty was performed to close the tracheostomy with improvement of respiratory pattern and quality of life this child. Biorebsorbable internal distraction devices offers some advantages, but complications include device failure to support resistance. Distraction osteogenesis has become a safe, less-invasive procedure with a negligible risk of infection making it effective to substitute tracheostomy as first choice in treatment of patients with Pierre Robin Sequence and life threatening airway obstruction. [ABSTRACT FROM AUTHOR]

Objective: To provide a detailed description of the nasopharyngeal intubation (NPI) technique and photographs, which should be helpful for those who may need to perform it for treating the airway obstruction in Robin sequence. Design: To describe and illustrate the NPI technique and the necessary considerations for its application. Setting: Hospital de Reabilitação de Anomalias Craniofacial of University of São Paulo, Brazil. Result: The NPI procedure involves the use of a whitish, Portex, number 3.0 or 3.5, silicone tube, introduced 8 cm deep into the infant's nostril and fixed with Micropore tape. The tube is to be removed at least twice a day for proper hygiene (with running water, detergent, and swabs) and should be changed every 7 days. This procedure is taught to the children's parents or caretakers by the nurse during hospitalization. Conclusion: The technique is so simple that it can be performed by the parents themselves, allowing continuation of the treatment at home. [ABSTRACT FROM AUTHOR]

Objective: The objective of this study was to explore the prevalence, range of reported symptoms, and clinical risk factors of obstructive sleep apnea in preschool children with cleft lip and/or palate. Design: Questionnaires were distributed to parents/guardians of all children from birth to 5 years of age who were followed by the cleft clinic. Results: Questionnaire data and cleft classification were available for 248 children, with a mean age of 33.4 months. Obstructive sleep apnea was identified in 31.4% of the children. Only 29.5% of children with obstructive sleep apnea had undergone an investigation of these symptoms. The three most common symptoms reported in children with a questionnaire diagnosis of obstructive sleep apnea were (1) ''heavy or loud breathing,'' (2) ''easily distracted,'' and (3) ''on the go'' or ''driven by a motor.'' The only clinical risk factor associated with a questionnaire diagnosis of obstructive sleep apnea was the presence of a syndrome (χ2 = 3.5, p = .05). There were no significant differences in risk of obstructive sleep apnea by age, cleft classification, and surgical status. Conclusion: Preschool children with cleft lip and/or palate have a risk of obstructive sleep apnea that is as much as five times that of children without cleft. Obstructive sleep apnea appears to be underrecognized in this group of children. Further research is needed to investigate important risk factors for obstructive sleep apnea in children with cleft lip and/or palate. [ABSTRACT FROM AUTHOR]

Objective: To describe birth prevalence of rare craniofacial anomalies and associations with antenatal and perinatal factors.Study Design: All live and stillbirths in Western Australia between 1980 and 2010 were identified from the Western Australian Birth Registrations and the Midwives Notification System (also provides information on antenatal and perinatal factors). Rare craniofacial anomalies (craniosynostosis, craniofacial microsomia, and others [Pierre Robin, Van der Woude, and Treacher Collins syndrome]) were ascertained from the Western Australian Register of Developmental Anomalies and linked to other data sources. Trends in prevalence, adjusted for sex and Indigenous status, were investigated by Poisson regression and presented as annual percent change (APC). Strengths of association of related factors were assessed using multivariable log-binomial regression adjusted for sex, Indigenous status, birth year, socioeconomic disadvantage, and remoteness and reported as risk ratios with 95% CIs.Results: There was a temporal increase in prevalence of metopic synostosis (APC 5.59 [2.32-8.96]) and craniofacial microsomia (Goldenhar syndrome) (APC 4.43 [1.94-6.98]). Rare craniofacial anomalies were more likely among infants born preterm, as twins or greater-order multiples, with growth restriction, to older parents, to mothers undertaking fertility treatments, and with pre-existing medical conditions, specifically epilepsy, diabetes, or hypothyroidism. Prenatal identification of rare craniofacial anomalies was uncommon (0.6%).Conclusions: Our findings indicate a steady increase over time in prevalence of metopic synostosis and craniofacial microsomia (Goldenhar syndrome). Possible associations of fertility treatments and pre-existing maternal medical conditions with rare craniofacial anomalies require further investigation. [ABSTRACT FROM AUTHOR]

Objective: Distraction osteogenesis is a good technique to correct micrognathia and to prevent tracheostomy in patients with Pierre Robin sequence. However,the airway must be maintained during the distraction period. The purpose of this study was to determine the usefulness of temporary tongue-lip traction during the initial period of mandibular distraction in Pierre Robin sequence patients with severe airway problems requiring operative procedures. Patients: Three Pierre Robin sequence patients between 4 months and 3 years of age requiring surgical procedures to correct recurrent and severe pulmonary complications. Method: All patients underwent distraction osteogenesis of themandible. During surgery,a deep one-tension suture was used to apply traction to the tongue. When the patient gained control of the upper airway during the initial period of distraction and micrognathia was corrected,th e traction suture was removed. Results: The patients were followed for a mean of 5.4 years. No patients complained of severe pulmonary complications,and tracheostomy was avoided. The lower face area was increased in all three patients. Conclusion: In severe Pierre Robin sequence cases,temporary tongue-lip traction was a good adjunct to distraction osteogenesis because this method avoided tracheostomy. [ABSTRACT FROM AUTHOR]

The article reviews the physical findings on Stickler syndrome including differentiation between similar syndromes. A systematic review of physical findings is presented along with treatment options. It is noted that Stickler syndrome is an inherited autosomal dominant disease of the collagen due to the mutations in the genes that direct development of type II collagen.

Objective: Pierre Robin sequence (PRS) involves the triad of micrognathia, glossoptosis, and cleft palate. Neonates with PRS suffer from two problems--airway obstruction and feeding difficulties--but the severity of these problems varies greatly. The resultant varying definitions of PRS have resulted in inconsistent management of those babies given the diagnosis. This in turn makes comparison of published data and communication between clinicians difficult. Historically at Birmingham Children's Hospital, the cleft team only identified babies presenting with cleft palate and severe respiratory distress as ''true'' PRS. These babies were admitted to the high-dependency neonatal ward for airway assessment and management. Babies with a mild degree of respiratory obstruction but who were managed successfully at home were not classified as having PRS despite needing considerable input from the Cleft Clinical Nurse Specialist. A system of classifying babies with PRS based on the severity of their symptoms and signs and treatment required was therefore devised and is described in this paper. Results: Since implementation, this new classification of babies with PRS into three grades has clarified care pathwaysand enhanced communication between the Cleft Clinical Nurse Specialists and other health professionals involved with the care of these babies. [ABSTRACT FROM AUTHOR]

Summary: Objective: Evaluate parental perceptions associated with tracheostomy morbidity and quality of life in the management of Pierre Robin Sequence (PRS). Study Design: Retrospective review/survey. Methods: 42 Pierre Robin patients were identified, records were reviewed and airway assessments evaluated relative to airway compromise. Twenty patients had undergone tracheostomy. Perceptions of quality of life/morbidity related to tracheostomy were assessed using parental surveys. Results: 31/41 (76%) patients participated in the survey. 15/31 (48%) of survey participants required tracheostomy and were decannulated after a mean of 28 months. Of the patients who had undergone tracheostomy, 10/15 (67%) had isolated Pierre Robin (iPRS) and the remaining 5/15 (33%) had syndromes associated with Pierre-Robin (sPRS). 9/10 (90%) iPRS and 4/5 (80%) sPRS families’ expectations were met regarding expected duration of tracheostomy although 3/5 (60%) sPRS, and 8/10 (80%) iPRS described the overall experience as difficult. Of the 2/15 patient''s families who were dissatisfied 1 patient had iPRS and the other sPRS. 9/15 (60%) required multiple (≥3) hospitalizations. 3/13 (23%) reported airway problems after decannulation and 2/15 (13%) remained tracheostomy dependent at the time of survey. Prolonged tracheostomy duration represented a significant parental concern. Conclusions: A subset of patients required extended duration of tracheostomy; some continued to have airway problems after decannulation and/or distraction. Although some patients benefit from early mandibular distraction other Pierre Robin patients have multi-level obstruction requiring additional therapies and often tracheostomy. Parental concerns and perceptions relative to tracheostomy have not been adequately studied for Pierre Robin airway obstruction. Of those responding to this survey, the majority of parents’ expectations were met regarding tracheostomy. Of those whose expectations were not met, it seems that better pre-intervention counseling regarding length of tracheostomy tube dependence, as well as a discussion about potential complications and hospitalizations frequently associated with prolonged tracheostomy, may lead to improved parental expectations. [Copyright &y& Elsevier]

Pierre Robin sequence (PRS) and the associated anatomical and functional issues have been well documented in literature. Treatment of neonates born with PRS varies depending on the severity of their respiratory status and their inability to orally feed. The purpose of this article is to discuss PRS and to describe the Cincinnati Children''s Hospital Medical Center integrated team approach in assessing and evaluating the infants'' ability to maintain a functional respiratory system, orally feed, and support appropriate weight gain. The focus on neonatal mandibular distraction is described because of the recent success in airway correction for the neonatal population. A primary focus on the current clinical protocol used at Cincinnati Children''s Hospital Medical Center to evaluate and treat oral feeding before, during, and after neonatal mandibular distraction will be presented. This article highlights the need for future research to establish evidence-based practice guidelines for oral feeding with this neonatal population. [Copyright &y& Elsevier]

Airway management in infants with Pierre Robin sequence (PRS) is often challenging. Advancement of the tongue base using mandibular distraction in neonates can avoid tracheotomy and is increasingly propagated. The osteotomies can be performed via intra- and extraoral approaches. Nowadays, for precise bone cutting, piezosurgical devices have been introduced in maxillofacial surgery, which we used for osteotomies via the intraoral approach in the 7-week-old patient presented in this study. Instead of a preoperative CT scan, a 3D data set was generated preoperatively in the operating theatre using a 3D C-arm system. After bilateral transcutaneous pin placement and osteotomies, a second 3D data set was acquired. Primary and secondary reconstructions clearly visualized the patient’s bone despite the low level of ossification in newborns and the presence of a large amount of metal implants. In view of the low level of radiation exposure going along with the 3D C-arm imaging, a follow-up examination after removal of the distractors was performed. With this report of a 7-week-old female infant with the diagnosis of PRS, we want to extend the surgical and diagnostic armamentarium for the treatment of infants with PRS. [ABSTRACT FROM AUTHOR]

Abstract: Background/Purpose: The study describes a safe and least aggressive method to resolve airway obstruction in children born with a Pierre Robin sequence (PRS). Methods: In a retrospective study, we analyzed the assessment of airway obstruction at birth and for the following months. The definition of PRS was based on the anatomical anomaly triad cleft palate, micro/retrognathia, and glossoptosis with some degree of airway obstruction. We defined 3 categories of children depending on their difficulties of breathing or eating at birth. Results: From 1984 to 2004, 48 children were born in our hospital with a diagnosis of PRS. There were 32 children with nonsyndromic PRS (nsPRS) and 16 with syndromic PRS (sPRS): respectively, 40% (13) and 32% (5) had slight respiratory and/or feeding problems; 26% (8) and 56% (9), isolated feeding difficulties; 34% (11) and 12% (2), severe respiratory and feeding problems. Pharyngeal tube was used in 8 children with nsPRS and in 2 with sPRS. Neonatal surgery was not necessary. Primary palatoplasty was performed at almost the same time as for the patients with isolated cleft palate. Conclusions: Children born with PRS have a good prognosis at birth provided that adequate respiratory support is given using either positive airway pressure mask or pharyngeal tube. [Copyright &y& Elsevier]

Dental trauma secondary to a syncopal episode occurs rarely in the pediatric population. Although the majority of these events occurs in patients with a benign medical history, a subset of these cases occur in patients with a genetic predisposition to cardiac arrhythmia. Long QT syndrome, characterized by prolonged cardiac ventricular repolarization, represents one genetic condition in which patients sustain events of seizure, syncope, and sudden cardiac death in response to precipitating physical, mental, or emotional stressors. In this case report, extensive dental injuries occurred after an episode of cardiac syncope in a patient with a dual presentation of long QT syndrome and Pierre Robin sequence. The purpose of this paper was to highlight the potentially malignant course of symptomatic long QT syndrome and emphasize the importance of warning sign recognition and multidisciplinary medical management of pediatric patients with this condition. [ABSTRACT FROM AUTHOR]

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Pierre Robin sequence is a triad of micrognathia, glossoptosis, and cleft palate that results in upper airway obstruction and feeding problems. It is a serious condition with potentially severe, life-threatening airway obstruction. Approaches for treatment of airway obstruction among neonates with Pierre Robin sequence include prone positioning of the infant, tongue-lip adhesion, mandibular distraction, and tracheostomy. This case report describes a modified nutrition plate and a baby treated using this appliance. [ABSTRACT FROM AUTHOR]

A wide spectrum of anomalies can be associated with Pierre Robin sequence. This report presents a 3-day-old infant with micrognathia, U-shaped cleft palate, low-set right ear with microtia, glossoptosis, esophageal atresia, and right congenital radioulnar synostosis. The association of congenital radioulnar synostosis and esophageal atresia with Pierre Robin sequence has not been previously described. [ABSTRACT FROM AUTHOR]

Objective: The Pierre Robin Sequence (PRS) is subgroup of the cleft palate population. As with the etiology of cleft lip or palate, the etiology of PRS is generally unknown. Some factors are suggestive of a genetic basis for PRS. The purpose of this study was to compare genetic information on PRS avail- able in the literature and in a cytogenetic database to facilitate focused genetic studies of PRS. Design: After searching Medline for "pierre robin and genetics," the Mendelian Cytogenetics Network database for "robin" and "pierre robin," and two reviews from the Human Cytogenetics Database for "cleft palate" and "micrognathia," a comparison of the data and a search in Online Mendelian Inheritance in Man (OMIM) Gene Map was performed to identify relevant candidate genes. Results: The findings revealed consistency to a certain degree to loci 2q24.1-33.3, 4q32-qter, 11q21-23.1, and 17q21-24.3. A search in the OMIM Gene Map provided many candidate genes for PRS in these regions. The GAD67 on 2q31, the PVRL1 on 11q23-q24, and the SOX9 gene on 17q24.3-q25.1 are suggested to be of particular importance. Conclusion: Candidate loci and a few potential candidate genes for PRS are proposed from the present study. This may enable researchers to focus their effort in the studies of PRS. [ABSTRACT FROM AUTHOR]

Summary: Objective: To evaluate the course and prognosis of airway obstruction and feeding difficulty in the Pierre Robin sequence (PRS). Methods: Retrospective review of 60 patients with PRS between 1993 and 2002 at the University of California, Davis Medical Center. Patients were placed into diagnostic subgroups: (1) Isolated PRS; (2) Syndromic PRS (known syndrome with PRS); (3) Unique PRS (unique anomalies with PRS). Data regarding severity, duration, and management of airway obstruction and feeding difficulty were collected. Results: Airway obstruction requiring intervention beyond positional therapy was seen in 28% isolated, 42% syndromic, and 58% unique PRS. One-third of patients who failed positional therapy were temporarily stabilized with a nasopharyngeal airway or endotracheal intubation. The remaining two-thirds of patients, who failed positional therapy required a surgical airway procedure. Four patients underwent mandibular distraction osteogenesis, resulting in successful decannulation or avoidance of tracheostomy. Thirteen patients underwent tracheostomy; mean duration of tracheostomy-dependence was 17.0 months in Isolated PRS and 31.7 months in Unique PRS (p <0.01). Successful decannulation by age of 3 years was confirmed in 85% of patients who underwent tracheostomy. Tube feeding was required in 53% Isolated, 67% Syndromic, and 83% Unique PRS. Forty-two percent of PRS patients with a successful positional airway still demonstrated feeding difficulty. Short-term (0–3 months) and intermediate (4–18 months) tube feeding was more commonly required in Isolated and Syndromic PRS, while long-term (beyond 18 months) gastrostomy tube feeding was more commonly required in Unique PRS (p <0.01). By 3 years of age, a successful oral diet was seen in 91% Isolated, 92% Syndromic, and 78% Unique PRS. Conclusions: Diagnostic subgroups based on the presence of additional anomalies help families and physicians in understanding the severity and duration of feeding and airway difficulty in PRS. Two-thirds of PRS patients who fail positional therapy may ultimately require a surgical airway procedure. Feeding difficulty can be present in the absence of clinically significant airway obstruction. Families and physicians should be encouraged that by 3 years of age, most patients were successfully taking an oral diet without airway obstruction. [Copyright &y& Elsevier]

Objective: This study was undertaken to describe the sonographic features of fetal glossoptosis in the Pierre Robin sequence. Study design: Fetal sonography was prospectively performed in 8000 consecutive pregnancies at 14 to 24 weeks' gestation. In addition we retrospectively reevaluated ultrasound recordings of 4 fetuses from other hospitals, in which the diagnosis of Pierre Robin sequence was overlooked at 22 weeks' gestation. Glossoptosis was defined as a posteriorly displaced tongue that never reached the anterior mandibular alveolar ridge while watching the fetal profile. Micrognathia, which is a component of the sequence, was subjectively defined. Results: Glossoptosis with micrognathia was detected in 2 fetuses in the prospective group at 14 and 15 weeks' gestation. Both pregnancies were terminated; the diagnosis was confirmed in 1 case where postmortem examination was performed. There were no false-negative diagnoses in the other 7.998 fetuses. Glossoptosis and micrognathia were observed in the 4 retrospective cases. Conclusion: Sonographic identification of glossoptosis with fetal micrognathia suggests the possibility of Pierre Robin sequence. [ABSTRACT FROM AUTHOR]

Unlabelled: We report an infant suffering from Pierre Robin sequence complicated by gastro-oesophageal reflux and failure to thrive, which were resistant to conservative therapy and a hemifundoplication. Gastro-oesophageal reflux was accompanied by supraventricular tachycardia, treated with propafenone. Tachycardia may be present in Pierre Robin sequence as a consequence of cardiac parasympathetic imbalance. The patient recovered completely from the gastro-oesophageal reflux and tachycardia after redo-fundoplication (Nissen) and a hiatoplasty were performed.Conclusion: This case shows that a thorough search for gastro-oesophageal reflux is indicated in each case of Pierre Robin sequence with failure to thrive. [ABSTRACT FROM AUTHOR]

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Summary: We present an 8-day-old female with two admissions for respiratory failure. On the first admission, the diagnosis of Pierre Robin sequence (PRS) and laryngomalacia was made after assessment with chest radiography, echocardiography, and flexible fiberoptic laryngoscopy. Four days after discharge, the child presented with stridor and respiratory distress, and a new cardiac murmur was noted after admission. Repeat echocardiography, with confirmatory direct laryngobronchoscopy, revealed a double aortic arch (DAA) with distal tracheal compression. This case illustrates the necessity of a complete otolaryngic evaluation, including direct laryngobronchoscopy, to search for a synchronous airway lesion in any neonate with severe respiratory distress associated with stridor. [Copyright &y& Elsevier]

Summary: Introduction:: Obstructive sleep apnea syndrome (OSAS) commonly appears in patients with Pierre Robin sequence (PR). Polysomnography (PS) is the gold standard for the diagnosis of OSAS. Videonasopharyngoscopy (VNP) is the best diagnostic tool for visualizing the vocal tract and detecting structural abnormalities which can be associated with OSAS. Objective:: The purpose of this paper is to study whether VNP can be useful for identifying severe OSAS in a population of children with PR. Materials and methods:: Fifty-two children with PR who were present with sleep-disordered breathing were studied. All the parents completed a questionnaire concerning the children''s sleeping habits and sleep complaints before consultation. Each child underwent a general pediatric examination and an evaluation of craniofacial features and upper airway permeability. In all children, a PS was performed. Also, all children underwent a VNP. Results:: The diagnosis of OSAS was confirmed by PS in 31 patients. VNP showed 87% sensitivity and 100% specificity for the detection of OSAS. VNP findings showed a significant correlation with apnea–hypopnea index, arousal index, snoring time, percentage of sleep time spent at saturation of oxygen &#60;90% and a significant inverse correlation with total sleep time, sleep efficiency and the mean saturation of oxygen during sleep. Conclusion:: This study shows that in children with PR, airway obstruction as detected by VNP seems to be a risk factor for OSAS. VNP appears to be a safe and reliable tool for the evaluation of sleep-disordered breathing in children with PR. [Copyright &y& Elsevier]