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International Journal of Oral and... Dec 2023The purpose of this article is to report the author's technique for using patient-specific distractors for customized distraction osteogenesis of the mandible in...
The purpose of this article is to report the author's technique for using patient-specific distractors for customized distraction osteogenesis of the mandible in patients with Pierre Robin sequence and upper airway obstruction. The advantages of virtual planning and patient-specific plates in other aspects of craniomaxillofacial surgery, such as orthognathic and reconstructive surgery, have been reported previously. Similar to patient-specific plates, the theorized advantages of patient-specific distractors in infants with Robin sequence and upper airway obstruction include increased accuracy, decreased operating time, and less morbidity to vital anatomic structures such as the inferior alveolar nerve and developing tooth buds. This technique is novel in using patient-specific distractors in the craniomaxillofacial skeleton.
Topics: Humans; Infant; Pierre Robin Syndrome; Osteogenesis, Distraction; Airway Obstruction; Mandible; Treatment Outcome; Retrospective Studies
PubMed: 37532615
DOI: 10.1016/j.ijom.2023.07.006 -
The Cleft Palate-craniofacial Journal :... Mar 2024Assess the impact of prenatal diagnosis of Robin sequence (RS) on parental experience during gestation and early infancy.
OBJECTIVE
Assess the impact of prenatal diagnosis of Robin sequence (RS) on parental experience during gestation and early infancy.
DESIGN
Prospective case-control study. An online survey was administered via email to 44 parents representing 34 unique patients with RS.
PARTICIPANTS
Parents of children diagnosed with RS and who received mandibular distraction at our tertiary care children's hospital. Participants were separated by the timing of RS diagnosis into prenatal and control postnatal groups.
MAIN OUTCOME MEASURES
Effects of timing of diagnosis on parents' preparation, caregiver support, education about the condition, stress, and overall mental health.
RESULTS
Complete responses were received from 44 parents representing 34 unique patients (50% response rate): prenatal, = 17; postnatal, = 27. Prenatal diagnosis improved parents' satisfaction regarding time to prepare for treatment ( = .001), stress of uncertainty about their child's health ( = .018), and stress about the operation(s) their child would need ( = .001). Both the prenatal (82%) and postnatal (78%) groups reported a negative impact on mental health based on diagnosis timing. All parents in the prenatal group preferred having received a prenatal diagnosis and the majority of the postnatal group (85%) would have preferred to have received the diagnosis prenatally.
CONCLUSIONS
Prenatal diagnosis of RS provided tangible benefits for parents by allowing them to mentally prepare, make plans for delivery and treatment, and become educated about the condition. Parents in both groups reported a negative impact on their mental health based on diagnosis timing and the majority of parents consistently preferred prenatal diagnosis.
Topics: Child; Pregnancy; Female; Humans; Case-Control Studies; Pierre Robin Syndrome; Prenatal Diagnosis; Parents; Surveys and Questionnaires
PubMed: 36217734
DOI: 10.1177/10556656221130833 -
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 Annals of Otology, Rhinology, and... Jul 2024to investigate the ability of mandibular distraction osteogenesis (MDO) to avoid gastrostomy tube (G-tube). (Review)
Review
Pre-Operative Characteristics Helping to Avoid Gastrostomy Tube After Mandibular Distraction in Neonates With Pierre-Robin Sequence: A Institutional Case-Series and Review of the Literature.
OBJECTIVE
to investigate the ability of mandibular distraction osteogenesis (MDO) to avoid gastrostomy tube (G-tube).
DATA SOURCES
PubMed, EBSCOhost, Cochrane, and Embase.
REVIEW METHODS
We retrospectively reviewed the number of MDO cases performed at our institution for patients with Robin Sequence (RS) over the past 10 years. In our institutional review, patients were excluded if they had a G-tube already placed at the time of surgery. We also performed a systematic review of the literature. Articles were excluded if they did not detail feeding outcomes after MDO, or if MDO was performed on patients that did not have RS.
RESULTS
In our systematic review, 12 articles were included that comprised a total of 209 neonates with RS that underwent MDO. A total of 174 (83.3%) patients avoided a G-tube once MDO was performed. A total of 14 patients met the inclusion criteria at our institution. Of the 14 RS patients, 9 (64%) avoided having a G-tube placed and all (14/14) avoided tracheostomy. The average birth weight of patients avoiding a G-tube was 3.11 kg compared to 2.25 kg ( = .045) in the group requiring a G-tube. In the group avoiding a G-tube, the average weight at time of operation was 3.46 kg compared to 2.83 kg ( = .037) in the group requiring a G-tube.
CONCLUSION
MDO may be considered as a surgical option to prevent G-tube placement for neonates with non-syndromic RS who have difficulty with PO feeding but whose airway obstruction is not severe enough to require respiratory support. Based on our institutional experience, a minimum weight of 3.00 kg correlated with higher success rates of PO intake and avoiding a G-tube.
Topics: Humans; Pierre Robin Syndrome; Infant, Newborn; Gastrostomy; Osteogenesis, Distraction; Retrospective Studies; Mandible; Male; Female; Enteral Nutrition; Intubation, Gastrointestinal
PubMed: 38712740
DOI: 10.1177/00034894241249547 -
The Journal of Craniofacial SurgeryTracheostomy is the definitive treatment for airway management in severe cases of craniofacial-associated upper airway obstruction, like the Pierre-Robin sequence, but...
BACKGROUND
Tracheostomy is the definitive treatment for airway management in severe cases of craniofacial-associated upper airway obstruction, like the Pierre-Robin sequence, but is associated with significant morbidity. The purpose of this study was to examine tracheostomy-associated morbidities and mortalities in craniofacial patients to identify opportunities to improve clinical care and patient prognosis.
METHODS
The study was a retrospective review of pediatric craniofacial patients who were tracheostomized between 2016 and 2022. Data regarding their demographics, craniofacial diagnoses, endoscopic airway anomalies, intubation grade of view classification, tracheostomy-related complications, and causes of mortality were analyzed.
RESULTS
Sixteen of the 17 tracheostomized pediatric patients had the Pierre-Robin sequence, with 5 of those patients having an additional syndromic craniofacial diagnosis. Additional airway anomalies were found in 82.4% of the patients. The mean length of hospital stay after tracheostomy was 4.08 months. Infection was the most common complication, observed in 94.1% of patients, followed by stomal granulation in 76.5% of patients. Two mortalities were observed: one following the compassionate removal of ventilator support and the other following the accidental dislodgment of the tracheostomy tube.
CONCLUSIONS
Tracheostomy-related complications were observed in all craniofacial patients in this group. Compared with the general pediatric population, tracheostomized craniofacial patients may endure longer hospital stays and greater stomal granulation rates. Mandibular distraction osteogenesis may allow for tracheostomy avoidance in these patients, and future research should focus on comparing the long-term complication rates and outcomes between tracheostomy mandibular distraction osteogenesis in this challenging patient population.
Topics: Humans; Child; Infant; Treatment Outcome; Tracheostomy; Pierre Robin Syndrome; Airway Obstruction; Retrospective Studies; Morbidity; Osteogenesis, Distraction; Mandible; Postoperative Complications
PubMed: 37639682
DOI: 10.1097/SCS.0000000000009627 -
The Journal of Craniofacial Surgery May 2024To investigate the growth patterns of the maxillomandibular complex in preadolescent patients with Pierre-Robin sequence (PRS).
Growth Patterns of the Maxillomandibular Complex in Preadolescent Patients With Pierre-Robin Sequence Using Cluster Analysis and Longitudinal Follow-Up Cephalometric Data.
OBJECTIVE
To investigate the growth patterns of the maxillomandibular complex in preadolescent patients with Pierre-Robin sequence (PRS).
METHODS
The samples consisted of 20 preadolescent PRS patients who had cleft palate and did not undergo growth-modification therapy or surgical intervention [6 boys and 14 girls; mean age of lateral cephalograms taken, 8.8 y (T1) and 13.7 y (T2)]. According to k-means cluster analysis, four clusters were defined over 3 major groups at T1: (1) Both very retrusive maxilla and mandible group: Cluster-4 [n=4, very large gonial angle, very low facial height ratio (FHR)] and Cluster-1 (n=5, small gonial angle, normal FHR); (2) Retrusive maxilla and very retrusive mandible group (Cluster-3, n=7, normal gonial angle, very low FHR); and (3) Both normal maxilla and mandible group (Cluster-2, n=4, very large gonial angle, low FHR). Seven angular and ratio variables [sella-nasion-A point (SNA), sella-nasion-B point (SNB), A point-nasion-B point (ANB), saddle angle, gonial angle, mandibular-body-length/anterior-cranial-base-length (MBL/ACBL), and FHR] at T1 and T2 and growth change from T1 to T2 were investigated.
RESULTS
At T1, SNA, SNB, saddle angle, gonial angle (all P<0.05), and FHR (P<0.01) showed significant difference among 4 clusters. At T2, SNA, SNB and gonial angle (all P<0.05) still showed significant difference among 4 clusters. During T1 to T2, there was no significant change in variables at each cluster except an increase in MBL/ACBL in Cluster-1 and FHR in Cluster-3 (all P<0.05).
CONCLUSIONS
Preadolescent PRS patients exhibited diverse skeletal phenotypes at T1, which did not change significantly from their original skeletal patterns by growth during T1 to T2.
PubMed: 38771200
DOI: 10.1097/SCS.0000000000010187 -
The Journal of Craniofacial Surgery Sep 2023The authors aim to report a rare sequela following neonatal mandibular distraction osteogenesis (MDO) involving delayed onset sublingual swelling. They performed a...
The authors aim to report a rare sequela following neonatal mandibular distraction osteogenesis (MDO) involving delayed onset sublingual swelling. They performed a retrospective chart review of 3 patients who presented with delayed onset sublingual edema following neonatal MDO. The 3 patients presented at 2, 4, and 12 months following MDO for micrognathia secondary to Robin sequence with intermittent sublingual swelling associated with sialorrhea and feeding difficulties. There was no associated recent illness, fevers, or purulent drainage. All 3 children underwent magnetic resonance imaging which demonstrated asymmetric sublingual gland edema. The edema was located on the left sublingual gland in 2 children and was bilateral in the third. The symptoms continue to recur 25.5±3.3 months (range, 22.3-28.9) postoperatively and all are being managed conservatively. Chronic delayed onset intermittent sublingual edema is a possible long-term complication following neonatal MDO and further studies should explore the incidence and management of this finding.
Topics: Infant, Newborn; Child; Humans; Infant; Retrospective Studies; Osteogenesis, Distraction; Airway Obstruction; Treatment Outcome; Neoplasm Recurrence, Local; Mandible; Pierre Robin Syndrome
PubMed: 37497798
DOI: 10.1097/SCS.0000000000009554 -
Acta Paediatrica (Oslo, Norway : 1992) Dec 2023We aimed to investigate the developmental outcome of children with Robin sequence (RS) for whom continuous positive airway pressure was the main strategy to release...
AIM
We aimed to investigate the developmental outcome of children with Robin sequence (RS) for whom continuous positive airway pressure was the main strategy to release upper airway obstruction.
METHODS
We included children with isolated RS or RS associated with Stickler syndrome who were aged 15 months to 6 years. We used the French version of the Child Development Inventory and calculated the developmental quotient (DQ) for eight different domains and the global DQ (DQ-global). We searched for determinants of risk of delay.
RESULTS
Of the 87 children, for 71%, the developmental evolution was within the norm (DQ-global ≥86 or ≥-1 SD), 29% were at high risk of delay (DQ-global <86 or <-1 SD), and only 3% were at very high risk of delay (DQ-global <70 or <-2 SD). The DQs for expressive language and language comprehension were lower in our study population than the general population, but an improvement was noticed with the children's growth.
CONCLUSION
Risk of a developmental delay was not greater for children with the most severe respiratory phenotype than the others. Children whose mothers had low education levels were more at risk than the others.
Topics: Female; Humans; Child; Infant; Pierre Robin Syndrome; Paris; Child Development; Hearing Loss, Sensorineural; Mothers
PubMed: 37786287
DOI: 10.1111/apa.16979 -
The Cleft Palate-craniofacial Journal :... Nov 2023Prior research suggests that children with cleft palate (CP) are at increased risk of obstructive sleep-disordered breathing (SDB). However, few studies differentiate...
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.
PATIENTS/PARTICIPANTS
A total of 145 children who underwent primary CP repair from 2014 to 2021.
MAIN OUTCOME MEASURE
Post-palatoplasty SDB is defined as parent-reported symptoms and/or evidence of obstructive sleep apnea (OSA).
RESULTS
Median age at palatoplasty was 11.1 [IQR 10.2-13.6] months. Most patients (61.4%) had nonsyndromic CP, 26.9% had a syndrome, and 11.7% had RS. Children with syndromic CP and RS had more post-palatoplasty SDB symptoms (56.4% vs 58.8% vs 30.3%, = .006) and higher rates of OSA (25.6% vs 29.4% vs 5.6%, = .001) compared to children with nonsyndromic CP after palatoplasty. Children with syndromic CP and RS had nearly 3 to 4 higher odds of post-palatoplasty SDB than children with nonsyndromic CP (adjusted odds ratio [aOR] 2.88, 95% CI 1.29-6.47, = .010; aOR 3.73, 95% CI 1.19-11.70, = .024).
CONCLUSION
This study showed that children with CP experience higher rates of SDB after palatoplasty than the general pediatric population. Within the cohort, children with syndromic CP and isolated RS were more likely to have obstructive sleep disorders than nonsyndromic children after palatoplasty. Clinicians should counsel caregivers accordingly and closely monitor these groups for SDB after palate repair.
Topics: Child; Humans; Infant; Cleft Palate; Retrospective Studies; Sleep Apnea Syndromes; Sleep Apnea, Obstructive; Pierre Robin Syndrome
PubMed: 35642260
DOI: 10.1177/10556656221105203 -
The Cleft Palate-craniofacial Journal :... Nov 2023Mandibular distraction osteogenesis (MDO) may improve airway grade in patients with Robin Sequence (RS), but little is known about the response of the oropharyngeal...
PURPOSE
Mandibular distraction osteogenesis (MDO) may improve airway grade in patients with Robin Sequence (RS), but little is known about the response of the oropharyngeal airway to the distraction process in cases of tongue base obstruction (TBAO). This study used drug-induced sleep endoscopy (DISE) to evaluate the impact of MDO on the oropharynx.
METHODS
RS patients with severe obstructive sleep apnea (OSA) were prospectively enrolled, and underwent DISE prior to MDO, and at the time of distractor removal. Laryngoscopy views, glossoptosis degree, polysomnography (PSG) results, oxygen saturations and airway measurements were compared pre- and post-MDO.
RESULTS
Twenty patients met inclusion criteria. At the time of distractor placement, a grade II laryngoscopic view was most frequently observed (63%), and one patient (5%) had a grade I view. Median obstructive apnea hypopnea index (OAHI) improved after MDO (49.1 [30.2-74.0] to 9.1, [3.9-18.0], ≤ .001). Median oxygen saturation nadir also improved (preoperative 69% [60-76] to 85% [82-91], ≤ .001). At distractor removal, mean laryngoscopic view improved (≤ .002) with no views that were grade 3 or higher. Median intraoperative oropharyngeal width improved, (3.1 mm [2.8-4.4] to 6.0 mm [4.4-6.8], ≤ .021), as did median cephalometric anteroposterior oropharyngeal width (3.5 mm [2.7-4.1] to 6.3 mm [5.6-8.2], ≤ .002).
CONCLUSION
Following MDO, RS patients with TBAO have an approximate doubling of oropharyngeal width and an improvement in laryngoscopic grade. These findings likely contribute to improved oxygenation, OAHI and ease of intubation.
PubMed: 37915226
DOI: 10.1177/10556656231204517