pierre robin children

BACKGROUND: The Pierre Robin Sequence presents heterogeneous symptoms, and each newborn can manifest from mild breathing and feeding difficulties to severe complications, as well as a predisposition to present changes in growth and neuropsychomotor development in the first years of life. OBJECTIVE: The aims were to evaluate and associate the neuropsychomotor development of zero- to 12-month-old children with Pierre Robin sequence (PRS) in the personal-social, fine motor-adaptive, language, and gross motor aspects...

Dysphagia in Robin Sequence can be present in varying degrees, requiring multidisciplinary management and specific swallowing assessment by a specialist. Most studies published to date have evaluated only respiratory outcomes, and the available evidence on the improvement of swallowing is questionable. To conduct a systematic review and meta-analysis of studies evaluating swallowing in children with Robin Sequence before and after airway clearance procedures. The research question was developed based on the PICO strategy...

OBJECTIVE: Stickler Syndrome (SS) is an inherited collagenopathy characterized by heterogenous orofacial, ocular, auditory, and skeletal abnormalities. The orofacial manifestations are variable and some patients present with cleft palate and velopharyngeal insufficiency (VPI). The incidence of VPI in SS is poorly studied and no studies have compared the incidence of VPI between Type I (COL2A1) and Type II (COL11A1) SS. The objective of this study is to compare the incidence of VPI between SS subtypes and discuss the surgical techniques used to treat them...

Background: Pierre Robin sequence (PRS) is a congenital craniofacial deformity characterized by micrognathia, glossoptosis and airway obstruction. Some affected neonates are born with severe life-threatening upper airway obstruction that requires surgery. If without timely treatment, it is possible to cause not only organ damage and developmental abnormalities but also early newborn mortality. Case presentation: In this report, a 51-hours-old neonate was diagnosed with PRS, who had severe upper airway obstruction and required surgery...

Purpose: The aims of this study were, in a cohort of children with neonatal opioid withdrawal syndrome (NOWS), (a) to report 1-year neurodevelopmental outcomes and specifically characterize speech, language, and hearing outcomes and (b) to report the prevalence of cleft lip and/or cleft palate. Method: This prospective observational cohort study includes newborns with confirmed in utero opioid exposure who received pharmacological treatment for NOWS. During 1-year-old developmental visits, we administered standardized assessments (Bayley Scales of Infant and Toddler Development-Third Edition [Bayley-III] or Developmental Assessment of Young Children-Second Edition [DAYC-2]-due to COVID-19 restrictions)...

BACKGROUND: Humidification is an important process in clinical oxygen therapy. We aimed to evaluate the effects and safety of humidified versus nonhumidified low-flow oxygen therapy in children with Pierre-Robin syndrome. METHODS: This study was an open-label, single-centered randomized controlled trial (RCT) with a parallel group design. The study protocol has been registered in Chinese Clinical Trial Registry (ChiCTR1900021584). The children were randomized to the humidified versus nonhumidified groups...

This study aimed to document the complication status of infants with orofacial clefts born between 2011 and 2014 in Japan. This was a descriptive study using data from the Japan Environment and Children's Study. Among 103,060 pregnancies, 248 infants with orofacial clefts were included (livebirth, 239; stillbirth, 4; miscarriage, 5). The items of interest were complication status of orofacial clefts: isolated (typical orofacial clefts only); multi-malformed (orofacial clefts with unrelated major defects); syndromic (orofacial clefts with a syndrome or a chromosomal defect)...

To describe perioperative feeding performance in infants with Robin sequence (RS) who underwent mandibular distraction osteogenesis (MDO). A retrospective study of infants that underwent MDO from May 2010 to December 2019. Tertiary pediatric hospital. A total of 40 patients underwent MDO and 20 met inclusion criteria. Of the included infants, 6 had an associated syndrome and 80% were male. Time to full oral feeds, rate of G-tube placement, and change in weight percentile following MDO. Average oral intake prior to MDO was 22...

Pierre Robin Sequence (PRS) is defined by a constellation of characteristics including micrognathia, glossoptosis and airway obstruction. PRS can occur in isolation or can be associated with syndromes and another anomalies. Airway obstruction and feeding difficulties are the major presenting issues, and the severity of the condition ranges from mild, with minimal to no symptoms, to severe, with overt obstruction resulting in apnoeas, severe respiratory distress and cyanosis. The presence of airway obstruction can result in obstructive sleep apnoea and abnormalities in gas exchange, as well as exacerbation of already present feeding difficulties and failure to thrive, secondary to mismatch of caloric intake to energy usage associated with increased effort of breathing...

BACKGROUND: It has been shown that children with Pierre Robin sequence (PRS) have a higher risk of difficult intubation before surgery. When mask ventilation or tracheobronchial intubation is expected to be challenging, flexible bronchoscopy (FB) is advantageous in airway safety when it is used to guide tracheobronchial intubation (TI). AIM: To evaluate the complications of TI using FB in children with PRS and explore the effect of nursing services on postoperative complications...

INTRODUCTION: As part of surgical interventions in pediatric patients, children with craniofacial malformations and syndromes are presented in the field of oral and maxillofacial surgery, anesthesia and also in all other clinical disciplines. In particular, the Pierre Robin sequence in the clinical context leads to a situation albeit a rare one, which should be given high attention in preoperative, intraoperative and postoperative care. MATERIAL AND METHODS: In a retrospective analysis from 1993 to 2020 in the Department of Oral and Maxillofacial Surgery at the University Hospital Halle (Saale), a total of 54 patients were identified with syndromic changes and a need for surgical treatment...

OBJECTIVE: Describe the intelligence quotient (IQ) of children with Pierre Robin sequence (PRS). DESIGN: Prospective cohort study. SETTING: Neurodevelopmental follow-up clinic within a hospital. PATIENTS: Children with PRS (n = 45) who had been in the Neonatal Intensive Care Unit (NICU) were classified by a geneticist into 3 subgroups of isolated PRS (n = 20), PRS-plus additional medical features (n = 8), and syndromic PRS (n = 17) based on medical record review and genetic testing...

OBJECTIVE: To characterize the prevalence and presentation of laryngomalacia and efficacy of supraglottoplasty (SGP) in a cohort of patients with Pierre Robin Sequence (PRS). DESIGN: Retrospective cohort study. SETTING: Tertiary-care children's hospital. PATIENTS, PARTICIPANTS: Consecutive patients with PRS born between January 2010 and June 2018. MAIN OUTCOME MEASURES: Chart review included demographics, comorbid airway obstruction including laryngomalacia, timing of surgical interventions, clinical symptoms, sleep study data, and modified barium swallow study data...

OBJECTIVE: Prior research suggests that children with cleft palate (CP) are at increased risk of obstructive sleep-disordered breathing (SDB). However, few studies differentiate the effects of CP repair on SDB based on syndrome status. The goal of this study was to evaluate differences in SDB after palatoplasty among children with nonsyndromic CP, syndromic CP, and isolated Robin sequence (RS). DESIGN: Retrospective chart review. SETTING: Tertiary academic children's hospital...

BACKGROUND: The NEonate and Children audiT of Anaesthesia pRactice IN Europe (NECTARINE) prospective observational study reported critical events requiring intervention during 35.2% of 6542 anesthetic episodes in 5609 infants up to 60 weeks postmenstrual age. The United Kingdom (UK) was one of 31 participating countries. METHODS: Subgroup analysis of UK NECTARINE cases (12.8% of cohort) to identify perioperative critical events that triggered medical interventions...

INTRODUCTION: Robin sequence (RS) is a leading cause of obstructive sleep apnea (OSA) in newborns. Most studies have focused on understanding anatomic factors leading to OSA and changes in apnea-hypopnea index (AHI) on polysomnography (PSG) beyond the neonatal period. This study aims to define age-related OSA features between patients with RS, without RS and healthy controls using PSG-based analyses of respiratory arousal responses and gas-exchange parameters. DESIGN: Retrospective comparison of PSG features in a total of 48 children encompassing three groups: (a) infants with RS (n = 24, <1-year old), (b) non-RS older children (1-2 years old) with severe OSA (obstructive AHI (OAHI) of ≥10 events; n = 12), and (c) control infants and children (0-2 years old) without sleep apnea (OAHI ≤1...

Objective: By comparing and studying the changes of food intake, weight, body length, BMI, blood albumin level and other indicators of infants with Pierre-Robin Sequence (PRS) before and after Bilateral Mandible Distraction Osteogenesis operation, to explore the effect of distraction osteogenesis on PRS patients about the improvement of nutrition status. Methods: The children with PRS who admitted to the Oral and Maxillofacial Surgery Department of Guangzhou Women and Children's Medical Center from July 2015 to December 2020 were selected...

INTRODUCTION: Obstructive sleep apnea (OSA) is a well-recognized complication of velopharyngeal insufficiency (VPI) surgery, but studies assessing OSA by means of a respiratory polygraphy (PG) are scarce. The aim of the study was to evaluate the incidence of new-onset OSA after posterior flap pharyngoplasty (PFP). MATERIALS: The postoperative PG of children with VPI who had a normal preoperative PG were analyzed. RESULTS: Eighteen patients (mean age, 9...

BACKGROUND: Pierre Robin sequence (PRS) is a heterogeneous condition involving retro(micro)gnathia, glossoptosis and upper airway obstruction, very often with posterior cleft palate. Patients with PRS, either isolated or associated with Stickler syndrome have good intellectual prognosis. Nevertheless, the quality of life in adolescence and the phonatory and morphological outcomes are rarely analysed. We assessed the phonatory and morphological outcomes of 72 cognitively unimpaired adolescents with PRS, studied their oral (COHIP-SF19), vocal (VHI-9i) and generic quality of life (QoL; KIDSCREEN-52), and searched for determinants of these outcomes...

OBJECTIVE: Airway obstruction in newborns with Pierre Robin sequence (PRS) may be managed with tongue-lip adhesion (TLA), mandibular distraction osteogenesis (MDO), or tracheostomy to prevent airway compromise when conservative airway interventions fail or are contraindicated based on the type of obstruction present. Unfortunately, some of these procedures have the potential to affect a child's speech and feeding development. METHOD: The authors retrospectively reviewed the records of all children with PRS treated at our institution in the last 25 years...