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Annals of Plastic Surgery Apr 2024Pharyngeal flap (PF) surgery is effective at improving velopharyngeal sufficiency, but historical literature shows a concerning prevalence rate of obstructive sleep...
BACKGROUND
Pharyngeal flap (PF) surgery is effective at improving velopharyngeal sufficiency, but historical literature shows a concerning prevalence rate of obstructive sleep apnea (OSA), reported as high as 20%. Our institution has developed a protocol to minimize risk of postoperative obstructive complications and increase safety of PF surgery. We hypothesize that (1) preoperative staged removal of significant adenotonsillar tissue along with (2) multiview videofluoroscopy to guide patient-specific surgical approach via appropriately sized PFs can result in excellent speech outcomes while limiting occurrence of OSA.
METHODS
This was a retrospective chart review of all patients with velopharyngeal insufficiency (VPI) (aged 2-20 years) seen at the University of Rochester from 2015 to 2022 undergoing PF surgery to correct VPI. Nasopharyngoscopy was used for surgical planning and airway evaluation. Patients with tonsillar and adenoid hypertrophy underwent staged adenotonsillectomy at least 2 months before PF. Multiview videofluoroscopy was used to identify anatomic causes of VPI and to determine PF width. Patients underwent polysomnography and speech evaluation before and at least 6 months after PF surgery.
RESULTS
Forty-one children aged 8.5 ± 4.1 years (range, 4 to 18 years) who underwent posterior PF surgery for VPI were identified. This included 10 patients with 22q11.2 deletion and 4 patients with Pierre Robin sequence. Thirty-nine patients had both pre- and postoperative speech data and underwent both a pre- and postoperative sleep study. Polysomnography showed no significant difference in obstructive apnea-hypopnea index after posterior PF surgery (obstructive apnea-hypopnea index preop, 1.3 ± 1.2 events per hour; postop, 1.7 ± 2.1 events per hour; P = 0.111). Significant improvements in speech outcome were seen in patients who underwent PF (modified Pittsburgh score preop, 11.52 ± 1.37; postop, 1.09 ± 2.35; P < 0.05).
CONCLUSIONS
Use of preoperative staged adenotonsillectomy as well as patient-specific PF dimensions results in effective resolution of VPI and a low risk of OSA.
Topics: Child; Humans; Speech; Retrospective Studies; Critical Pathways; Pharynx; Velopharyngeal Insufficiency; Sleep Apnea, Obstructive; Postoperative Complications; Treatment Outcome
PubMed: 38556656
DOI: 10.1097/SAP.0000000000003859 -
The Journal of Craniofacial Surgery Mar 2024Pierre-Robin Sequence (PRS) is a congenital abnormality characterized by micrognathia, glossoptosis, and variable severity upper airway obstruction. Clear management...
PURPOSE
Pierre-Robin Sequence (PRS) is a congenital abnormality characterized by micrognathia, glossoptosis, and variable severity upper airway obstruction. Clear management algorithms are lacking, particularly the indications for surgical versus nonsurgical intervention. The authors reviewed the management of these children in Queensland.
METHODS
All children diagnosed with PRS at Queensland Children's Hospital from April 2014 to October 2019 were identified (n=45), and their charts were retrospectively reviewed. Three management patterns emerged: prone/lateral positioning, nasopharyngeal airway (NPA) use, and surgery (tracheostomy or mandibular distraction).
RESULTS
Most children (n=30; 67%) were managed successfully nonsurgically with an NPA (median age of insertion 0.25 mo, median duration 5.0 mo). Of these, 12 patients (40%) also required supplemental oxygen. The median age of NPA cessation was 5.5 months, with oxygen therapy ceasing at a median 8.25 months, upon which no further support was required. The remaining majority (n=13; 29%) of children were managed without an NPA, using positioning alone (10/13; 77%) or positioning combined with supplemental oxygen (1/13), CPAP (1/13), or both adjunct measures (1/13). Only 2 patients underwent surgical intervention. Feeding supplementation using nasogastric tube was necessary in 78% of patients for a median duration of 4 months. Cleft palate co-existed in all but one patient.
CONCLUSION
Management of upper airway obstruction in PRS children is variable between units. Over a 5-year period, 96% of children with PRS were successfully managed without surgical intervention at the Queensland Children's Hospital. These findings contrast with some other literature and may suggest that more careful consideration of surgical intervention in PRS patients is prudent.
PubMed: 38534164
DOI: 10.1097/SCS.0000000000010083 -
The Cleft Palate-craniofacial Journal :... Mar 2024Compare the feeding management practices in infants with cleft palate with and without Pierre Robin sequence (PRS) and determine if specific feeding difficulties or...
OBJECTIVES
Compare the feeding management practices in infants with cleft palate with and without Pierre Robin sequence (PRS) and determine if specific feeding difficulties or interventions predict delayed palate repair.
DESIGN
Retrospective cross-sectional study.
SETTING
Seventeen cleft palate teams contributed data.
PATIENTS
414 infants were included in this study: 268 infants with cleft palate only and 146 infants with cleft palate and PRS.
PROCEDURES
Data were collected via parent interview and electronic health records.
MAIN OUTCOME MEASURES
Outcomes for the primary objective included categorical data for: history of poor growth, feeding therapy, milk fortification, use of enteral feeding, and feeding difficulties. The outcome for the secondary objective was age in months at primary palate repair.
RESULTS
Infants with PRS had a significantly higher prevalence of feeding difficulties (81% versus 61%) and poor growth (29% versus 15%) compared to infants with cleft palate only. Infants with PRS received all feeding interventions-including feeding therapy, milk fortification, and enteral feeding-at a significantly higher frequency. Infants with PRS underwent primary palate repair at a mean age of 13.55 months (SD = 3.29) which was significantly ( < .00001) later than infants with cleft palate only who underwent palate repair at a mean age of 12.05 months (SD = 2.36). Predictors of delayed palate repair included diagnosis of PRS as well as Hispanic ethnicity and a history of poor growth.
CONCLUSIONS
These findings can be used to establish clinical directives focused on providing early, multimodal feeding interventions to promote optimal growth and timely palate repair for infants with PRS.
PubMed: 38490219
DOI: 10.1177/10556656241239766 -
Journal of Cranio-maxillo-facial... Apr 2024The objective of this study was to report outcomes of early cleft palate repair in infants born with Robin sequence (RS). A retrospective case series in a tertiary...
The objective of this study was to report outcomes of early cleft palate repair in infants born with Robin sequence (RS). A retrospective case series in a tertiary referral paediatric hospital was carried out, examining a consecutive series of 69 infants born with RS and cleft palate. A minimally invasive approach was taken to upper airway obstruction, with liberal nasopharyngeal airway (NPA) and non-invasive ventilation (NIV) use, guided by sleep studies. The palate was repaired between 6 and 9 months with a modified Malek technique. The most frequently used airway adjunct (59.4% of patients) was an NPA and the median duration of use was 5.6 months. All patients underwent a modified Malek cleft palate repair at a median of 7 months of age. Overnight oximetry demonstrated higher mean oxygen saturation (SpO) across the group from initial neonatal admission to discharge (median 96.5% (interquartile range [IQR] 95-98%) vs 97.45% (IQR 96.5-98%) (P = 0.2, N = 34). Of those with a cardiorespiratory polysomnogram, the obstructive apnoea-hypopnea index (OAHI) was significantly lower postoperatively (5.9 vs 2.8, P = 0.028). This study supports the use of non-surgical airway strategies and early cleft palate repair in infants born with RS and cleft palate.
Topics: Infant; Infant, Newborn; Humans; Child; Cleft Palate; Pierre Robin Syndrome; Retrospective Studies; Airway Management; Nasopharynx; Airway Obstruction
PubMed: 38448335
DOI: 10.1016/j.jcms.2024.02.006 -
Indian Journal of Otolaryngology and... Feb 2024Pierre Robin Sequence (PRS), a rare congenital disorder, is a triad of micrognathia, glossoptosis, and tongue based airway obstruction (TBSO). It may occur as isolated...
Pierre Robin Sequence (PRS), a rare congenital disorder, is a triad of micrognathia, glossoptosis, and tongue based airway obstruction (TBSO). It may occur as isolated anomaly (iPRS) or as a part of a syndrome (sPRS), like that seen in association with Stickler Syndrome. Approximately 20% of children with PRS have congenital heart diseases. To the best of our knowledge this case of a one-day old infant is the first one to be reported as having two heart defects; patent ductus arteriosus and patent foramen ovale in Pierre Robbin Sequence child.
PubMed: 38440550
DOI: 10.1007/s12070-023-04307-x -
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 -
Breastfeeding Medicine : the Official... Mar 2024Infants with significant retrognathia often have difficulty forming a latch adequate to establish exclusive breastfeeding. This article describes the use of at-breast...
Infants with significant retrognathia often have difficulty forming a latch adequate to establish exclusive breastfeeding. This article describes the use of at-breast supplementers (ABSs) to facilitate extended breastfeeding relationships, even when supplementation is necessary for growth. Two cases are described where infants with severe retrognathia initially struggled with weight gain necessitating supplementation but were able to ultimately exclusively feed at-breast with the use of ABSs. While the two cases differed in the form of supplemental milk used and duration of ABS use, both dyads breastfed for beyond 2 years. Feeding solely at the breast and subsequent extended breastfeeding may be possible even for infants who require supplementation, including those with anatomical or functional challenges such as retrognathia. The ABS is a relatively simple system that may be beneficial for difficulties such as poor latch and low milk supply. More awareness and education is needed so that clinicians consider supporting dyads with this approach.
Topics: Infant; Female; Humans; Animals; Breast Feeding; Retrognathia; Allergens; Milk
PubMed: 38377616
DOI: 10.1089/bfm.2023.0268 -
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 -
The Cleft Palate-craniofacial Journal :... Feb 2024The objective was to evaluate the readability of easily accessible parent-directed information concerning Robin Sequence (RS) online, compared to the American Medical...
The objective was to evaluate the readability of easily accessible parent-directed information concerning Robin Sequence (RS) online, compared to the American Medical Association (AMA)-recommended sixth grade (age 11-12) readability level. A Google search of the term "Pierre Robin Sequence information" was performed. The first ten websites were evaluated using six commonly used readability formulas. Sample texts from three websites were 'translated' by the authors, with the aim of achieving a sixth grade readability level. The following outcomes were used: Automated Readability Index (ARI), Coleman Liau Index (CLI), Gunning Fog Score, Simple Measure of Gobbledygook (SMOG), Flesch Kincaid Grade Level (FKGL), and Flesch Reading Ease (FRE) score. The mean pooled grade level of the top 10 included websites was 12.1 (age 17-18). The overall FRE Index was 45.8, which is equivalent to a College-grade reading level. The mean grade level by each test used was: Flesch-Kincaid Grade Level 11.6 (age 16-17), Gunning Fog Score 13.3 (age 18+), SMOG 10.0 (age 14-15), Coleman-Liau Index 13.8 (age 18+), and ARI 12.0 (age 17-18). The author-translated resources achieved pooled mean grade levels of 6.3-6.5. Parent-directed online materials concerning RS have a readability in excess of the AMA-recommended sixth grade reading level. Even though the condition is complex, more readable resources are achievable. Coproduction of parent-directed resources in association with public an patient involvement (PPI) contributors is encouraged.
PubMed: 38373442
DOI: 10.1177/10556656241234587 -
The Cleft Palate-craniofacial Journal :... Feb 2024To identify weight gain trends of infants with Robin sequence (RS) treated by the Stanford Orthodontic Airway Plate treatment (SOAP).
OBJECTIVE
To identify weight gain trends of infants with Robin sequence (RS) treated by the Stanford Orthodontic Airway Plate treatment (SOAP).
DESIGN
Retrospective longitudinal cohort study.
SETTING
Single tertiary referral hospital.
PATIENTS
Eleven infants with RS treated with SOAP.
INTERVENTIONS
Nonsurgical SOAP.
MAIN OUTCOME MEASURES
Body weight, Weight-for-age (WFA) Z-scores, and WFA percentiles at birth (T0), SOAP delivery (T1), SOAP graduation (T2), and 12-months old (T3).
RESULTS
Between T0 and T1, the weight increased but the WFA percentile decreased from 36.5% to 15.1%, and the Z-score worsened from -0.43 to -1.44. From T1 to T2, the percentile improved to 22.55% and the Z-score to -0.94. From T2 to T3, the percentile and the Z-scores further improved to 36.59% and -0.48, respectively.
CONCLUSIONS
SOAP provided infants experiencing severe respiratory distress and oral feeding difficulty with an opportunity to gain weight commensurate with the WHO healthy norms without surgical intervention.
PubMed: 38373407
DOI: 10.1177/10556656241233239