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The Cleft Palate-craniofacial Journal :... Feb 2024To describe perioperative feeding performance in infants with Robin sequence (RS) who underwent mandibular distraction osteogenesis (MDO). A retrospective study of...
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 22.1% of individual goal feeds. Among the 15 (75%) children that did not require G-tube placement, mean time to full oral feeds after MDO was 11 days ± 5.7 days, with 80% of infants reaching full oral feeds within 2 weeks after extubation. The proportion of G-tube placement in patients with a syndrome was higher than in isolated RS (-0.6; 95% CI: -1.0, -0.2). Mean percentages of weight-for-age percentile decreased during the first 3 months after the procedure. This was followed by a mean upturn in weight starting after the third month after MDO with a recovery to preoperative mean weight-for-age percentiles by 6 months after surgery. This study suggests that infants with RS may achieve full oral feeds despite poor feeding performance before MDO. Infants with syndromic RS are more likely to require G-tube. These findings may be used to inform G-tube discussion and offer a timeline to work toward goal oral feeds for infants with RS after MDO.
Topics: Infant; Humans; Male; Child; Female; Retrospective Studies; Pierre Robin Syndrome; Osteogenesis, Distraction; Treatment Outcome; Mandible; Airway Obstruction
PubMed: 36128745
DOI: 10.1177/10556656221127542 -
The Kobe Journal of Medical Sciences Nov 2023The purpose of this study was to provide the experience of a single center with mandibular distraction osteogenesis (MDO) in Pierre Robin Sequence (PRS) patients. A...
The purpose of this study was to provide the experience of a single center with mandibular distraction osteogenesis (MDO) in Pierre Robin Sequence (PRS) patients. A longitudinal research analysis was conducted to identify PRS patients who underwent MDO at Vietnam National Children's Hospital between 2019 and 2021. The following criteria were used to determine inclusion: 1) those pediatric patients with PRS who were not well handled with conservative therapy, 2) those who received MDO with internal mandibular distractors, and 3) no previous treatment elsewhere. Demographic data, postoperative complications, and surgical results were all evaluated. The inclusion criteria were met by 73 patients. There were no difficulties associated with our distraction strategy. The majority of individuals with tracheostomies were successfully decannulated, and the remainder were able to avoid tracheostomies. Using MDO in PRS is an effective technique to avoid future airway issues. The success rate was lower and the complication rate higher for patients who had a tracheotomy before distraction and for those who underwent distraction at an age older than 2 months. The presence of laryngomalacia, gastric reflux disease, cardiac abnormalities, and GI anomalies did not increase the likelihood of MDO failure in PRS patients.
Topics: Humans; Infant; Child; Osteogenesis, Distraction; Pierre Robin Syndrome; Vietnam; Retrospective Studies; Treatment Outcome; Mandible
PubMed: 38018223
DOI: 10.24546/0100485260 -
The Cleft Palate-craniofacial Journal :... Feb 20241. To assess the skeletal class occlusion and lateral cephalometry in children with isolated cleft palates (non-Robin sequence) and 2. to identify associations between...
OBJECTIVES
1. To assess the skeletal class occlusion and lateral cephalometry in children with isolated cleft palates (non-Robin sequence) and 2. to identify associations between these findings and pre-palatoplasty cleft palate measurements.
STUDY DESIGN
Retrospective cohort study.
SETTING
North American Institutional Tertiary Paediatric Center.
PATIENTS
Our cleft database was reviewed, and patients were included if they had an isolated cleft palate without a Robin Sequence diagnosis, had a Furlow palatoplasty and had available per operative cleft palate measurements and available lateral cephalogram between 6 and 8 years old. Thirty-two patients matched to inclusion criteria.
INTERVENTION
Furlow's Palatoplasty.
MAIN OUTCOME AND MEASURES
Cleft size at palatoplasty, cephalometric measurements and skeletal occlusal classes were analysed. ANOVA was used to test the association between cephalometric measurements and occlusal classes. Results are presented as means with a 95% confidence interval. The association between cleft measurements and cephalometric parameters was tested with Spearman Correlation (r).
RESULTS
The skeletal occlusal outcome at 7 years old for this series of patients was: Class I: 19%; Class II: 59% and Class III: 22%. No single cleft measurement at palatoplasty was predictive of the skeletal occlusal outcome. A larger hard palate cleft was associated with a shorter antero-posterior maxilla.
CONCLUSIONS
The skeletal occlusal class outcomes were similar to those found in a previous study in the literature. The occlusal prognosis appears to be better than in patients with Robin Sequence or with an associated cleft lip. No preoperative measurement was found to be associated with the occlusal outcome.
PubMed: 38389267
DOI: 10.1177/10556656241236078 -
European Annals of Otorhinolaryngology,... Jun 2018Tongue-lip adhesion may be used to relieve obstructive sleep apnoea in infants with Pierre Robin sequence (PRS), but only a few studies have objectively evaluated its...
OBJECTIVE
Tongue-lip adhesion may be used to relieve obstructive sleep apnoea in infants with Pierre Robin sequence (PRS), but only a few studies have objectively evaluated its efficacy. The purpose of this study was to evaluate the results of tongue-lip adhesion by polysomnography.
MATERIEL AND METHODS
A single-centre retrospective study was conducted in infants with PRS treated by tongue-lip adhesion from 2004 to 2015, in whom at least laryngotracheal endoscopy and polysomnography were performed. The variables collected were the syndromic diagnosis, demographic data, respiratory management before tongue-lip adhesion, symptoms, and additional airway interventions. Obstructive sleep apnoea was classified into 3 groups according to severity. Polysomnography was performed one month after tongue-lip adhesion. Statistical analysis was performed with Wilcoxon signed-rank test with a limit of statistical significance of P<0.005.
RESULTS
Thirty-seven subjects in whom tongue-lip adhesion was performed at a mean age of 45 days (8 to 210 days) were included. Thirty-one patients had isolated PRS and 6 patients had associated anomalies. All patients had confirmed severe obstructive sleep apnoea. All patients required respiratory support prior to surgery: 8 intubated patients, 15 patients with noninvasive ventilation and 14 patients with nasopharyngeal airways. Eight patients had bradycardia before tongue-lip adhesion. All parameters were improved on postoperative polysomnography: oxygen saturation, hypercapnia, apnoea-hypopnoea index, bradycardia (P<0.005). Only 8 patients had persistent severe obstructive sleep apnoea and required tracheostomy (n=5) or noninvasive ventilation (n=3). No significant correlation was observed between treatment success and any predictive variables.
CONCLUSION
Tongue-lip adhesion improved airway obstruction in all infants with PRS and resolved obstructive sleep apnoea in 29 patients. However, 8 patients required additional airway interventions.
Topics: Female; Humans; Infant; Infant, Newborn; Lip; Male; Pierre Robin Syndrome; Polysomnography; Retrospective Studies; Sleep Apnea, Obstructive; Tongue; Treatment Outcome
PubMed: 29195837
DOI: 10.1016/j.anorl.2017.11.002 -
PloS One 2023Airway management can be challenging in neonates and infants. The Pierre Robin sequence (PRS) is a condition characterized by micrognathia, glossoptosis and airway...
An approach to difficult airway in infants: Comparison of GlideScope® Spectrum LoPro, GlideScope® Spectrum Miller and conventional Macintosh and Miller blades in a simulated Pierre Robin sequence performed by 90 anesthesiologists.
BACKGROUND
Airway management can be challenging in neonates and infants. The Pierre Robin sequence (PRS) is a condition characterized by micrognathia, glossoptosis and airway obstruction. The airway management of these patients poses great challenges for anesthesiologists and pediatricians alike. To date, there has been no direct comparison of the hyperangulated GlideScope® Spectrum LoPro (GLP), the straight GlideScope® Spectrum Miller (GSM), a conventional Macintosh (MC) and a conventional Miller blade (ML) in patients with PRS.
METHODS
For this purpose, 90 anesthesiologists (43 with limited experience, 47 with extensive experience) performed orotracheal intubation on an Air-Sim® Pierre Robin X manikin using GLP, GSM, MC and ML in randomized order. 'Time-to-vocal-cords', 'time-to-intubate', 'time-to-ventilate', the severity of oral-soft-tissue-trauma and the subjective evaluation of each device were recorded.
RESULTS
A significantly faster and better view of the vocal cords and lower oral-soft-tissue-trauma was achieved using the GLP (p<0.001). Though, there were no significant differences in the 'time-to-intubate' or 'time-to-ventilate'. The highest intubation success rate was found with GSM and the lowest with GLP (GSM 100%, ML 97.8%, MC 96.7%, GLP 93.3%). When using the videolaryngoscopes, there were no undetected esophageal intubations but in six cases prolonged attempts of intubation (>120s) with the GLP. In the sub-group with extensive experience, we found significantly shorter intubation times for the GSM and ML. The GLP was the tool of choice for most participants, while the conventional MC received the lowest rating.
CONCLUSIONS
Videolaryngoscopy leads to increased safety for the prevention of undetected esophageal intubation in the airway management in a PRS manikin. Hyperangulated blades may ensure a good and fast view of the vocal cords and low oral-soft-tissue-trauma but pose a challenge during the placement of the tube. Specific skills and handling seem to be necessary to ensure a safe tube placement with this sort of blades.
Topics: Infant, Newborn; Humans; Infant; Pierre Robin Syndrome; Anesthesiologists; Laryngoscopes; Intubation, Intratracheal; Airway Management; Manikins; Soft Tissue Injuries; Laryngoscopy
PubMed: 37535590
DOI: 10.1371/journal.pone.0288816 -
Frontiers in Physiology 2023The main clinical manifestations of patients with Pierre Robin sequence (PRS) include micrognathia, the glossoptosis and dyspnoea. The difficulty of tracheal intubation...
The main clinical manifestations of patients with Pierre Robin sequence (PRS) include micrognathia, the glossoptosis and dyspnoea. The difficulty of tracheal intubation (TI) in such patients is increased. The purpose of the study was to evaluate the reliability and efficacy of the PRS simulator. A PRS simulator was developed by using 3-dimensional (3D) printing technology under computer-aided design. A total of 12 anaesthesiologists each trained 5 times for TI on the PRS Training Simulator-1 and recorded the simulation time. After the training, they were randomly divided into three groups with a total of 12 nontrained anaesthesiologists, and the simulation was completed on PRS Simulator-2, 3 and 4. The simulation time was recorded, and the performance was evaluated by three chief anaesthesiologists. Then, all 24 anaesthesiologists completed the questionnaire. A PRS simulator developed by 3D printing was used to simulate the important aspects of TI. The average number of years worked was 6.3 ± 3.1 years, and 66.7% were female. The time for the 12 anaesthesiologists to complete the training gradually decreased ( < 0.01). Compared with the trained anaesthesiologists, the simulation time of TI in the nontrained anaesthesiologists was much longer (all < 0.01). In addition, the simulation performance of the trained anaesthesiologists was relatively better (all < 0.01). The reliability and efficacy of the PRS simulator is herein preliminarily validated, and it has potential to become a teaching and training tool for anaesthesiologists.
PubMed: 38374871
DOI: 10.3389/fphys.2023.1292523 -
Head & Face Medicine May 2023Robin sequence (RS) is a congenital condition characterized by micrognathia, glossoptosis and upper airway obstruction. Diagnosis and treatment are characterized by...
BACKGROUND
Robin sequence (RS) is a congenital condition characterized by micrognathia, glossoptosis and upper airway obstruction. Diagnosis and treatment are characterized by heterogeneity, resulting in a lack of uniformly collected data.
METHODS
We have set up a prospective, observational, multicenter, multinational registry aimed at obtaining routine clinical data from RS patients receiving different treatment approaches and enabling an assessment of outcomes obtained through different therapeutic approaches. Patient enrolment has started in January 2022. Disease characteristics, adverse events and complications depending on the different diagnostic and treatment approaches and their effects on neurocognition, growth, speech development and hearing outcome are evaluated using routine clinical data. In addition to characterizing the patient population and comparing outcomes achieved with different treatment approaches, the registry will evolve to focus on endpoints such as quality of life and long-term developmental status.
DISCUSSION
This registry will provide data on different treatment approaches collected during routine care with diverse framework conditions and will allow assessing diagnostic and therapeutic outcomes of children with RS. These data, urgently demanded by the scientific community, may contribute to refining and personalizing existing therapeutic approaches and increase knowledge about the long-term outcome of children born with this rare condition.
TRIAL REGISTRATION
DRKS00025365.
Topics: Child; Humans; Multicenter Studies as Topic; Pierre Robin Syndrome; Prospective Studies; Quality of Life; Registries; Treatment Outcome; Observational Studies as Topic
PubMed: 37210548
DOI: 10.1186/s13005-023-00364-3 -
AJNR. American Journal of Neuroradiology Mar 2019Midnasal stenosis is a poorly defined entity that may be a component of other conditions of nasal obstruction contributing to respiratory distress in infants. We sought...
BACKGROUND AND PURPOSE
Midnasal stenosis is a poorly defined entity that may be a component of other conditions of nasal obstruction contributing to respiratory distress in infants. We sought to establish whether midnasal vault narrowing is a component of well-defined syndromes of nasal narrowing, such as bilateral choanal atresia and pyriform aperture stenosis, and to characterize the nasal anatomy of patients with syndromic craniosynostosis.
MATERIALS AND METHODS
A convenience sample of patients with pyriform aperture stenosis, bilateral choanal atresia, and Apert and Crouzon syndromes with maxillofacial CT scans was identified. Patients with Pierre Robin Sequence were used as controls. Nasal measurements were performed at the pyriform aperture, choana, and defined midnasal points on axial and coronal CT scans. Intra- and interrater reliability was quantified with the intraclass correlation coefficient. tests with Bonferroni adjustment were used to assess differences from controls.
RESULTS
The study included 50 patients: Eleven had pyriform aperture stenosis, 10 had Apert and Crouzon syndromes, 9 had choanal atresia, and 20 were controls. Measurements in patients with pyriform aperture stenosis and Apert and Crouzon syndromes were narrower than those of controls at all measured points ( < .001). Measurements in patients with choanal atresia were only narrow in the posterior half of the nose ( < .001). The intra- and interrater reliability of midnasal and pyriform measurements was very good to excellent (intraclass correlation coefficient > 0.87). The choanal measurement was good (intraclass correlation coefficient = 0.76-0.77).
CONCLUSIONS
Pyriform aperture stenosis, Apert and Crouzon patients were narrower at all measured points compared to controls. Bilateral choanal atresia patients were only narrower in the posterior half of the nose. More research is needed to evaluate the clinical implications of these radiographic findings.
Topics: Benchmarking; Constriction, Pathologic; Female; Humans; Infant; Infant, Newborn; Male; Nose; Nose Diseases; Reproducibility of Results; Tomography, X-Ray Computed
PubMed: 30765383
DOI: 10.3174/ajnr.A5980 -
Drugs Aug 2017Ibuprofen is the most widely used non-steroidal anti-inflammatory drug (NSAID) for the treatment of inflammation, mild-to-moderate pain and fever in children, and is the... (Review)
Review
Ibuprofen is the most widely used non-steroidal anti-inflammatory drug (NSAID) for the treatment of inflammation, mild-to-moderate pain and fever in children, and is the only NSAID approved for use in children aged ≥3 months. Its efficacy and safety profile have led to its increasing use in paediatric care, even without medical prescription. However, an increase of suspected adverse reactions to ibuprofen has been noted in concomitance with the raised, often medically unsupervised, consumption of the drug. The purpose of this work was a critical review of the paediatric literature over the last 15 years on side effects and adverse events associated with ibuprofen, in order to highlight circumstances associated with higher risks and to promote safe and appropriate use of this drug. The literature from 2000 to date demonstrates that gastrointestinal events are rare, but (when they occur) include both upper and lower digestive tract lesions. Dehydration plays an important role in triggering renal damage, so ibuprofen should not be given to patients with diarrhoea and vomiting, with or without fever. Likewise, ibuprofen should never be administered to patients who are sensitive to it or to other NSAIDs. It is contraindicated in neonates and in children with wheezing and persistent asthma and/or during varicella. Most of the analysed studies reported adverse events when ibuprofen was being used for fever symptoms or flu-like syndrome. Ibuprofen should not be used as an antipyretic, except in rare cases. Ibuprofen remains the drug of first choice in the treatment of inflammatory pain in children.
Topics: Abnormalities, Multiple; Age Factors; Anti-Inflammatory Agents, Non-Steroidal; Asthma; Cerebellar Diseases; Chickenpox; Child; Fever; Humans; Ibuprofen; Inflammation; Intellectual Disability; Pain; Pierre Robin Syndrome
PubMed: 28597358
DOI: 10.1007/s40265-017-0751-z -
Frontiers in Pediatrics 2021By 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)...
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. 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. All patients accepted bilateral mandible distraction osteogenesis surgery, and the pre- and post-operative indicators were recorded, such as food intake, weight, length, blood albumin level and others. BMI was calculated based on the indicators mentioned above, and comparative statistical analysis was performed. 1. All patients were fed with whole milk before the first surgery, and the average calorie per kg was 91.8 kcal/kg, significantly lower than the standard (100-150 kcal/kg), suggesting the overall nutritional intake of PRS patients is low; 2. tests for independent samples were used to analyze the pre-operative and post-operative indicators. The WFA percentile increased from 14.16 ± 2.17 to 15.01 ± 1.85% ( = 0.0048), WFA score increased from -2.40 ± 0.18 to -1.90 ± 0.14 after the surgery ( = 0.0010), LFA percentile increased from 20.04 ± 3.48 to 33.67 ± 4.29% ( = 0.0098), LFA z score increased from -2.09 ± 0.19 to -1.42 ± 0.23 ( = 0.0009), BMI score increased from -1.95 ± 0.22 to -1.39 ± 0.16 ( = 0.0408), ALB raised from 37.06 ± 0.51 to 42.85 ± 0.30 g/L ( < 0.001), which indicating that the physique of patients improved after the distraction osteogenesis surgery, mainly was reflected by the lifting of weight and length growth curves; the body shape also improved, indicating that the patients' nutrition status after the surgery is also improved. Bilateral mandible distraction osteogenesis surgery has a positive effect on the nutrition status of children with PRS. This effect is mainly reflected by the improvements of the body physical indicators after surgery.
PubMed: 34778154
DOI: 10.3389/fped.2021.771333