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The Cleft Palate-craniofacial Journal :... Jun 2023Controversy remains regarding optimal management of Pierre Robin sequence (PRS). The goal of this study was to compare airway and feeding outcomes in infants with PRS...
BACKGROUND
Controversy remains regarding optimal management of Pierre Robin sequence (PRS). The goal of this study was to compare airway and feeding outcomes in infants with PRS who underwent surgical intervention, specifically mandibular distraction osteogenesis (MDO) or tongue-lip adhesion (TLA), or who had conservative management (CM) without surgery.
METHODS
All consecutive patients treated for PRS at a pediatric academic medical center, with at least one year follow-up, were included. Patients who underwent tracheostomy as an index procedure were excluded. Patients were divided into those who underwent MDO, TLA or CM. Feeding status and data from initial and follow-up polysomnograms were collected. Comparisons between groups were made using the Kruskal-Wallis test, followed by Mann-Whitney pairwise comparison with a Bonferroni correction, when appropriate.
RESULTS
67 neonates were included. 19 underwent TLA, 29 underwent MDO and 19 underwent CM. The proportions of syndromic patients were similar between groups. Patients undergoing CM had the lowest baseline AHI (9.1), but there were no significant differences between TLA (20.1) and MDO (25.4). At follow-up, the three groups had similar mean AHI (MDO 1.3, TLA 4.2, CM 4.5). A similar proportion of patients achieved AHI 5 or less (TLA 89.5%, MDO 96.6%, CM 84.2%). At one year, there were no significant differences in weight percentiles or in risk of failure-to-thrive between groups. One patient from the TLA group required a tracheostomy.
CONCLUSION
The three treatment modalities achieved high airway and feeding success rates. All three modalities should have a place in the armamentarium of the craniofacial surgeon.
Topics: Infant, Newborn; Infant; Humans; Child; Treatment Outcome; Pierre Robin Syndrome; Retrospective Studies; Mandible; Osteogenesis, Distraction; Airway Obstruction
PubMed: 35098759
DOI: 10.1177/10556656221076345 -
The Journal of Craniofacial Surgery Jun 2023Pierre Robin Sequence (PRS) is characterized by micrognathia, glossoptosis, cleft palate, and airway distress. The aims of initial treatment are the improvement of...
BACKGROUND
Pierre Robin Sequence (PRS) is characterized by micrognathia, glossoptosis, cleft palate, and airway distress. The aims of initial treatment are the improvement of airway and feeding. There are many therapeutic options, including conservative techniques (prone positioning and nasopharyngeal tube) and invasive procedures (mandibular distraction and tracheostomy). In our center, initially conservative treatment is the rule and many patients have been treated with nasopharyngeal tube.
OBJECTIVE
The aim was to analyze of the clinical evolution of respiratory distress in infants with PRS submitted to nonsurgical treatment.
METHODS
A retrospective and observational descriptive evaluation was carried out with 56 patients with the PRS at Centro de Atendimento Integral ao Fissurado Labiopalatal (CAIF). 21 patients were selected to a transversal phase.
RESULTS
The treatment has started in an average age of 1.5 months (±2.09) and 17 (94.4%) had respiratory distress at birth. Polysomnographic exams showed an average apnea/hypopnea index of 0.93, an average number of central apnea/hour of 0.3, an average number of obstructive apnea of 0.6 and an average oxygen saturation of 92%. There was the predominance of esthetic profile in class II with 16 (88.9%) patients in this group, and orthodontic profile in class II with 15 (83.3%) patients.
CONCLUSION
The conservative treatment has presented remarkable results in the treatment of respiratory distress in bearers of PRS with a decrease of obstructive sleep events considering the growth of patient and the development of mandibular growth as well.
Topics: Infant; Infant, Newborn; Humans; Pierre Robin Syndrome; Retrospective Studies; Conservative Treatment; Airway Obstruction; Sleep Apnea, Obstructive; Dyspnea; Respiratory Distress Syndrome; Treatment Outcome; Osteogenesis, Distraction
PubMed: 37220722
DOI: 10.1097/SCS.0000000000009090 -
Turkish Journal of Orthodontics Sep 2019Pierre Robin Sequence (PRS) is a heterogeneous pathological condition characterized by the coexistence of micrognathia, glossoptosis, and cleft palate, resulting in...
OBJECTIVE
Pierre Robin Sequence (PRS) is a heterogeneous pathological condition characterized by the coexistence of micrognathia, glossoptosis, and cleft palate, resulting in upper airway tract obstruction. Among the treatment modalities, the orthodontic approach is one part of the comprehensive care of those patients and will be present in the treatment modalities during all the growth period of the child.
METHODS
All patients with PRS observed in the period 2013-2017 were treated with a definite functional approach. The results were retrospectively analyzed with regard to functional outcome, total treatment time, and number of plates provided for a single patient.
RESULTS
In all the patients, the indicated treatment protocol has been applied as early impression and plate supply, stimulation of bottle feeding with the use of the plate, eventual substitution of the plate if no more adequate to the transverse and sagittal growth of the palate, and continuing the use until the surgical closure of the cleft. All the patients showed a positive outcome to the proposed treatment approach, evaluated with regard to the incidence of feeding improvement and weight gain, to the limit for the surgical phase, in the absence of adverse effects.
CONCLUSION
The use of a functional obturator plate, removing functional alterations to mandibular growth, reduces and, in some cases, eliminates the need for surgical intervention. As also stated in the literature, if despite the presence of the plate nutritional problems persist, immediate different surgical approaches, mainly mandibular osteodistraction, become necessary.
PubMed: 31565690
DOI: 10.5152/TurkJOrthod.2019.18057 -
Oral and Maxillofacial Surgery Clinics... May 2020Patients with Pierre-Robin sequence recalcitrant to nonsurgical intervention have historically required tracheostomy. Mandibular distraction provides a predictable... (Review)
Review
Patients with Pierre-Robin sequence recalcitrant to nonsurgical intervention have historically required tracheostomy. Mandibular distraction provides a predictable alternative to tracheostomy. Orthodontic perioperative interventions should be considered, including overcorrection, placement of temporary anchorage devices, elastics, and molding the regenerate. Mandibular distraction can be technically difficult and may cause complications. Performed correctly, mandibular distraction provides patients with a better quality of life than tracheostomy.
Topics: Airway Obstruction; Child; Humans; Infant; Mandible; Osteogenesis, Distraction; Pierre Robin Syndrome; Quality of Life; Treatment Outcome
PubMed: 32247440
DOI: 10.1016/j.coms.2020.01.012 -
The Cleft Palate-craniofacial Journal :... Jun 2020To identify concepts and constructs important to parents of children with Pierre Robin Sequence (PRS).
OBJECTIVE
To identify concepts and constructs important to parents of children with Pierre Robin Sequence (PRS).
DESIGN
Qualitative study.
SETTING
All children received some care at a tertiary hospital with additional care at outside facilities. Interviews were conducted in nonclinical locations, including remote locations.
PARTICIPANTS
Parents of children <5 years old with a diagnosis of PRS. Prior treatments included observation, positioning, nasal trumpet, mandibular distraction osteogenesis, tracheostomy, and gastrostomy.
INTERVENTION
Semi-structured interviews with individuals (4) and with groups (focus groups, 4) were conducted using open-ended questions and non-leading prompts. Transcripts were analyzed with iterative open and axial coding. Concepts and constructs were identified and refined into codes and central themes. Interviews were conducted until thematic saturation was achieved.
RESULTS
Sixteen parents were interviewed. Their experiences were coded into 5 main themes, which can be summarized as: (1) child's symptoms/well-being, (2) parents' grief/isolation, (3) family stress, (4) relationships with providers, and (5) psychological and technical growth. Difficulty with feeding, weight gain, and breathing problems were core physical issues described by participants with associated intense fear. Participants described frustration from not only lack of care coordination, slow diagnoses, and poor communication but also gratitude for providers who served as advocates. Participants described gradual development of knowledge/competencies.
CONCLUSIONS
Families of children with PRS have experiences that profoundly affect their lives. Child's physical symptoms/well-being and parents' psychosocial well-being provide content for a future PRS-specific quality-of-life instrument. Concepts that emerged also provide a framework to improve parents' experience and enhance their children's quality of care.
Topics: Child; Child, Preschool; Humans; Mandible; Osteogenesis, Distraction; Parents; Pierre Robin Syndrome; Qualitative Research
PubMed: 32174155
DOI: 10.1177/1055665620910331 -
Frontiers in Pediatrics 2022The aim of the study was to report our experience with placed allogenic acellular bone matrix and mandibular distraction osteogenesis in Pierre Robin sequence (PRS), and...
OBJECTIVE
The aim of the study was to report our experience with placed allogenic acellular bone matrix and mandibular distraction osteogenesis in Pierre Robin sequence (PRS), and explore the role of distraction in the osteogenesis of acellular bone.
MATERIALS AND METHODS
A total of 428 neonates with severe PRS managed with placing allogenic acellular bone and bilateral mandibular distraction osteogenesis were included in the study. The procedure included using oblique-shaped osteotomy, fixing bilateral mandibular distractor, instantly extending a 4-6 mm gap, and placing allogenic acellular bone into the gap. The length of allogenic acellular bone was 4-5 mm. Although the surgical techniques, distraction, and consolidation periods were similar, the allogenic acellular bone matrix we placed was quite different from the traditional distraction. With the technology we used, tracheal intubation could be immediately removed, thus quickly improving breathing conditions compared to traditional methods after the surgery. The jaw extending and oral feeding could begin on the 5th day. The jaw was extended 0.6 mm twice a day until the mandible was overcorrected by 20%.
RESULTS
All 428 cases included in this study were successfully extubated after the operation, and the difficulty in breathing was instantly relieved. Total mandibular distraction was 15-20 mm. Oral feeding was started at 6 h to 6 days postoperatively, while hospital stay ranged from 18 to 20 days postoperatively. No major complications were reported. Medium to long-term results was good. Mandibular distractors were removed after 3 months.
CONCLUSIONS
Bilateral mandibular distraction osteogenesis combined with placing allogenic acellular bone in the neonate are safe and accurate procedures, which are the primary treatment options for cases of severe PRS. It can be considered that the tension of distraction can promote osteogenesis in acellular bone and thus improve distractive effect of the mandible.
PubMed: 35676894
DOI: 10.3389/fped.2022.890156 -
The Journal of Craniofacial Surgery Jun 2020Pierre Robin sequence is the constellation of micrognathia, glossoptosis, and tongue-based airway obstruction. When airway obstruction is severe, feeding, growth, and... (Review)
Review
Pierre Robin sequence is the constellation of micrognathia, glossoptosis, and tongue-based airway obstruction. When airway obstruction is severe, feeding, growth, and respiratory demise are at risk. Neonatal mandibular distraction osteogenesis is a technique which improves tongue-based airway obstruction and avoids tracheostomy in patients with severe expressions of Pierre Robin sequence. Its efficacy in relieving airway obstruction is well documented, and it has become the surgical intervention of choice at many craniofacial centers. However, this is an uncommon procedure which can be performed within the first weeks of life, offering little space for a learning curve. The success of neonatal distraction and avoidance of complications is highly dependent on proper surgical technique. This report provides a brief overview of the disease, details the technique of the senior surgeon with captioned videos, describes the protocol used at our institution and reports long-term outcomes with a case description.
Topics: Airway Obstruction; Humans; Infant, Newborn; Larynx; Learning Curve; Mandible; Osteogenesis, Distraction; Pierre Robin Syndrome
PubMed: 32209938
DOI: 10.1097/SCS.0000000000006343 -
International Journal of Oral and... Apr 2023The aims of this cohort study were to compare the mandibular morphology between patients with Robin sequence (RS) and controls, and to examine the effects of mandibular...
The aims of this cohort study were to compare the mandibular morphology between patients with Robin sequence (RS) and controls, and to examine the effects of mandibular distraction osteogenesis (MDO) using different vectors. Measurements of the mandibles of 80 patients with RS and 46 controls aged< 90 days were made using computed tomography. The data were compared among isolated RS patients (n = 58), syndromic RS patients (n = 22), and controls. Patients with RS exhibited significantly shorter ramus and body lengths and larger symphyseal angles than controls (all P < 0.001). Patients with isolated RS had shorter body lengths (P < 0.001), while syndromic patients had shorter ramus and body lengths (both P < 0.001) than controls. Seventy RS patients underwent MDO. Pre-MDO (n = 37) and post-MDO (n = 29) mandibular measurements were compared between patients undergoing MDO with a vertical vector and those undergoing MDO with a horizontal vector. Polysomnography data from part of the cohort highlighted the effectiveness of both vectors. MDO with a horizontal vector conferred 11% and 36% increases in ramus and body length, respectively, while these increases were 34% and 27.5%, respectively, with a vertical vector. MDO with a vertical vector was effective in lengthening ramus and body components and should be considered in the presence of ramus hypoplasia.
Topics: Humans; Infant; Cohort Studies; Retrospective Studies; Pierre Robin Syndrome; Osteogenesis, Distraction; Mandible; Polymers; Treatment Outcome; Airway Obstruction
PubMed: 35985910
DOI: 10.1016/j.ijom.2022.07.007 -
Paediatric and Perinatal Epidemiology Sep 2021Pierre Robin sequence (PRS) is a rare congenital anomaly. Respiratory disorders and feeding difficulties represent the main burden.
BACKGROUND
Pierre Robin sequence (PRS) is a rare congenital anomaly. Respiratory disorders and feeding difficulties represent the main burden.
OBJECTIVE
The aim of this study was to investigate the epidemiology of PRS using a cohort of cases from EUROCAT, the European network of population-based registries of congenital anomalies.
METHODS
We analysed cases of PRS born in the period 1998-2017 collected by 29 population-based congenital anomaly registries in 17 different countries. We calculated prevalence estimates, prenatal detection rate, survival up to 1 week, and proportions of associated anomalies. The effect of maternal age was tested using a Poisson regression model.
RESULTS
Out of 11 669 155 surveyed births, a total of 1294 cases of PRS were identified. The estimate of the overall prevalence was 12.0 per 100 000 births (95% CI 9.9, 14.5). There was a total of 882 (68.2%) isolated cases, and the prevalence was 7.8 per 100 000 births (95% CI 6.7, 9.2). A total of 250 cases (19.3%) were associated with other structural congenital anomalies, 77 cases (6.0%) were associated with chromosomal anomalies and 77 (6.0%) with genetic syndromes. The prenatal detection rate in isolated cases was 12.0% (95% CI 9.8, 14.5) and increased to 16.0% (95% CI 12.7, 19.7) in the sub-period 2008-2017. The prevalence rate ratio of non-chromosomal cases with maternal age ≥35 was higher than in cases with maternal age <25 for total (PRR 1.26, 95% CI 1.05, 1.51) and isolated cases (PRR 1.33, 95% CI 1.00, 1.64). Survival of chromosomal cases (94.2%) and multiple anomaly cases (95.3%) were lower than survival of isolated cases (99.4%).
CONCLUSIONS
This epidemiological study using a large series of cases of PRS provides insights into the epidemiological profile of PRS in Europe. We observed an association with higher maternal age, but further investigations are needed to test potential risk factors for PRS.
Topics: Abnormalities, Multiple; Europe; Female; Humans; Maternal Age; Pierre Robin Syndrome; Pregnancy; Prevalence; Registries
PubMed: 34132407
DOI: 10.1111/ppe.12776 -
The Cleft Palate-craniofacial Journal :... Jul 2020To investigate airway morphology changes in patients with Pierre Robin sequence (PRS) pre-/post-mandibular distraction osteogenesis (MDO) and to compare morphologic...
OBJECTIVE
To investigate airway morphology changes in patients with Pierre Robin sequence (PRS) pre-/post-mandibular distraction osteogenesis (MDO) and to compare morphologic changes to age-matched controls.
DESIGN
Retrospective case-control study.
SETTING
Urban, academic, tertiary medical center.
PATIENTS, PARTICIPANTS
Fifteen patients with PRS after MDO to relieve upper airway obstruction (UAO) (2008-2018); age-matched controls for post-MDO patients.
INTERVENTIONS
Mandibular distraction osteogenesis, curvilinear internal mandibular distractors.
MAIN OUTCOME MEASURES
(1) Physiologic improvement after MDO (apnea-hypopnea index; minimum oxygen saturation); (2) airway size (volume, surface area, length, mean/minimum cross-sectional area), shape (lateral:anterior-posterior ratio, cross-sectional area ratios, uniformity, sphericity), and changes with MDO; and (3) post-MDO airway size, shape versus age-matched controls.
RESULTS
Airway size increased after MDO (volume, = .01; surface area, = .02; length, = .01), as did cross-sectional area (mean, = .02; minimum, = .02; minimum retropalatal, = .05, mid-retroglossal, = .02). Post-MDO PRS airways were larger than controls (volume, < .01; surface area, < .01; length, < .01, cross-sectional area, = .03). Airway shape remained nonuniform and flat post-MDO; control airways were round. Two syndromic patients required repeat MDO and had subphysiologic post-MDO airway cross-sectional area. Post-MDO PRS patients with supraphysiologic cross-sectional area along the entire airway had no UAO recurrence.
CONCLUSIONS
In this small, heterogenous patient sample, MDO increases airway size, may preferentially affect the retropalatal airway, and often results in supraphysiologic airway dimensions. These retropalatal changes may be important in relieving severe UAO in patients with PRS. Generalizability of our results is limited by small cohort size and patient heterogeneity.
Topics: Airway Obstruction; Case-Control Studies; Humans; Infant; Mandible; Osteogenesis, Distraction; Pierre Robin Syndrome; Retrospective Studies; Treatment Outcome
PubMed: 31964165
DOI: 10.1177/1055665619900624