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1.
J Craniofac Surg ; 35(5): 1379-1382, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38861322

RESUMO

BACKGROUND: The hypoplastic mandible in the congenital condition Pierre Robin sequence (PRS) displaces the base of the tongue posteriorly, which results in upper airway obstruction (UAO) that can potentially be corrected with mandibular distraction osteogenesis (MDO). Jaw thrust (JT) is routinely performed during evaluation of the airway; similar to MDO, it projects the mandible and tongue anteriorly to open the airway. The authors demonstrate that JT can be used as a criterion to predict successful MDO outcomes in infants with PRS. METHODS: The study was a single-center, retrospective chart review of infants diagnosed with PRS between 2016 and 2023. Data regarding their demographics, comorbid diagnoses, JT success, airway anomalies, laryngeal grade of view, apnea-hypopnea index, and perioperative course were statistically analyzed. RESULTS: Of the 16 patients included in the study, 11 had successful relief of their airway obstruction with JT and proceeded with MDO. The unsuccessful JT group had significantly greater proportions of females, birth prematurity, gastrostomies, tracheostomies, and longer hospital stays. In the successful JT group, both the mean laryngeal grade of view ( P =0.029) and mean apnea-hypopnea index ( P =0.025) improved significantly post-MDO. Post-MDO tracheostomy was also avoided in all but 1 patient who was not previously tracheostomized. CONCLUSIONS: There is no widely accepted algorithm to guide craniofacial surgeons on the optimal intervention for relieving UAO in infants with PRS. In our institutional experience, patients whose preoperative JT relieved UAO also successfully relieved UAO with MDO. In patients with PRS, JT may be a useful criterion for selecting appropriate candidates for MDO.


Assuntos
Obstrução das Vias Respiratórias , Mandíbula , Osteogênese por Distração , Síndrome de Pierre Robin , Humanos , Osteogênese por Distração/métodos , Feminino , Estudos Retrospectivos , Masculino , Síndrome de Pierre Robin/cirurgia , Obstrução das Vias Respiratórias/cirurgia , Lactente , Resultado do Tratamento , Mandíbula/cirurgia , Mandíbula/anormalidades , Recém-Nascido
2.
Br J Oral Maxillofac Surg ; 62(6): 511-522, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38845304

RESUMO

The purpose of this systematic review and meta-analysis was to determine the most effective and least morbid surgical technique for relieving retroglossal airway obstruction in infants with Robin sequence (RS). The study adhered to PRISMA guidelines and included 25 studies (24 cohorts and one case series) that investigated interventions for airway improvement, including conservative measures, tongue-lip adhesion (TLA), mandibular distraction osteogenesis (MDO), and tracheostomy. The primary outcome variable was complication rate, while predictor variable was the use of interventions for airway improvement. Results showed that conservative measures were the preferred initial management strategy in most studies, while TLA was recommended for infants with mild obstruction, and MDO or tracheostomy was reserved for severe cases. Only complications could be analysed via meta-analysis due to data heterogeneity, revealing that tracheostomy had a summary odds ratio of 5.39 in favour of TLA, while MDO had a ratio of 2.8 over TLA, and the complication rates were similar between MDO and tracheostomy. If conservative measures fail, the study recommends mandibular distraction as the preferable technique for stable airway improvement. If the infant is unsuitable for distraction, tongue-lip adhesion may serve as an alternative, while tracheostomy should be reserved for cases of severe multi-level obstruction. The authors propose that large-scale, multicentre trials comparing long-term outcomes are required to establish definitive guidelines.


Assuntos
Obstrução das Vias Respiratórias , Osteogênese por Distração , Síndrome de Pierre Robin , Humanos , Síndrome de Pierre Robin/cirurgia , Síndrome de Pierre Robin/complicações , Obstrução das Vias Respiratórias/cirurgia , Obstrução das Vias Respiratórias/etiologia , Lactente , Osteogênese por Distração/métodos , Traqueostomia , Complicações Pós-Operatórias , Resultado do Tratamento , Língua/cirurgia , Lábio/cirurgia , Mandíbula/cirurgia
3.
Br J Oral Maxillofac Surg ; 62(6): 551-558, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38902108

RESUMO

Treatment of children with Pierre Robin sequence (PRS) having a hypoplastic mandible and upper airway distress after birth may consist of external distraction devices. Shape anomalies of the permanent molars and positional changes due to surgery have been documented. The aim of this study is to compare the long-term effects (>5 years) on the growth pattern of PRS-patients treated with an external mandibular distraction device with no-surgery cases and to investigate the dental development or damage. A retrospective cohort study was performed. PRS-patients with and without surgery were included. A digital cephalometric analysis was made to evaluate the growth pattern of the mandible between groups as well as with normal values. Nine of 19 patients underwent an external mandibular distraction. All children were extubated after 4-5 days with no signs of respiratory distress. Screw and device loosening presented in one patient. The articular and sellar angles were significantly larger and smaller, respectively, in the Surgery group. Mandibular distraction surgery might result in a 'growth boost' compared to the No-surgery group. No significant difference in dental development was found. Mandibular distraction osteogenesis is an effective way of relieving severe upper airway obstruction.


Assuntos
Cefalometria , Mandíbula , Osteogênese por Distração , Síndrome de Pierre Robin , Humanos , Síndrome de Pierre Robin/cirurgia , Osteogênese por Distração/métodos , Osteogênese por Distração/instrumentação , Estudos Retrospectivos , Feminino , Mandíbula/cirurgia , Masculino , Criança , Pré-Escolar , Lactente , Resultado do Tratamento , Obstrução das Vias Respiratórias/cirurgia
4.
Ann Otol Rhinol Laryngol ; 133(7): 679-685, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38712740

RESUMO

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 (P = .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 (P = .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.


Assuntos
Gastrostomia , Osteogênese por Distração , Síndrome de Pierre Robin , Humanos , Síndrome de Pierre Robin/cirurgia , Recém-Nascido , Gastrostomia/métodos , Osteogênese por Distração/métodos , Estudos Retrospectivos , Mandíbula/cirurgia , Masculino , Feminino , Nutrição Enteral/métodos , Intubação Gastrointestinal/métodos
5.
Ann Plast Surg ; 92(6S Suppl 4): S382-S386, 2024 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-38725108

RESUMO

OBJECTIVE: Mandibular distraction osteogenesis (MDO) is rapidly becoming a standard of care for management of patients with severe Pierre Robin sequence. The tongue is brought forward to alleviate airway obstruction. This study will look at an institutional, multisurgeon experience with MDO over 10 years. DESIGN: A retrospective chart review was conducted. SETTING: All patients who underwent MDO at the authors' institution from 2012 to 2022 were included. Three craniofacial surgeons performed all interventions. PATIENTS: Demographics, preoperative and postoperative respiratory and feeding status, and distraction data were collected for 27 patients meeting inclusion criteria. MAIN OUTCOME MEASURES: Primary outcomes were avoidance of a gastrostomy tube, avoidance of a tracheostomy, discharge from hospital on room air, and complications. A significance value of 0.05 was utilized. RESULTS: The average age at MDO was 135 days, mean activation phase was 13.6 days, mean distraction length was 14.9 mm, and mean consolidation phase was 64.2 days. A longer activation phase was associated with discharge with a gastrostomy tube and a shorter activation phase was associated with discharge on full oral feeds. The ability to discharge on room air was associated with a shorter latency phase, shorter activation phase, and decreased distance of distraction. CONCLUSIONS: The goal of MDO is to achieve full oral feeds with no respiratory support. Several different latency periods were used in this study, and a short latency period was demonstrated to be safe.


Assuntos
Mandíbula , Osteogênese por Distração , Síndrome de Pierre Robin , Humanos , Síndrome de Pierre Robin/cirurgia , Síndrome de Pierre Robin/complicações , Osteogênese por Distração/métodos , Estudos Retrospectivos , Feminino , Masculino , Lactente , Resultado do Tratamento , Mandíbula/cirurgia , Obstrução das Vias Respiratórias/cirurgia , Obstrução das Vias Respiratórias/etiologia
6.
Int J Pediatr Otorhinolaryngol ; 180: 111964, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38714046

RESUMO

OBJECTIVE: Investigate an association between upper airway obstruction (UAO) management in Robin Sequence (RS) and need for bilateral myringotomy and tubes (BMT). METHODS: Retrospective chart review of RS patients treated at a tertiary free-standing pediatric hospital from 1995 to 2020 was performed. Patients were grouped based on airway management: conservative, tracheostomy, tongue-lip adhesion (TLA), and mandibular distraction osteogenesis (MDO). Demographic data, cleft palate (CP) association, numbers of BMT and ear infections, and audiogram data including tympanograms were collected. One-way ANOVA and Chi-square/Fisher's exact tests were used to compare continuous and categorical data, respectively. Multivariable regression analysis was used to compare BMT rates between treatment groups. RESULTS: One hundred forty-eight patients were included, 70.3 % of which had CP. Most patients (67.6 %) had at least one BMT; 29.1 % required two or more BMT. The rate of BMT was higher in patients with CP compared to those with intact palates (p = 0.003; 95 % CI 1.30-3.57) and those treated with tracheostomy (p = 0.043; 95 % CI 1.01, 2.27). Surgically managed patients were more likely to have hearing loss (67.5 % vs. 35.3 %, p = 0.017) and ear infections (42.1 % vs. 20.0 %, p = 0.014) pre-compared to post-procedure for airway management. CONCLUSION: Most RS patients require at least 1 set of BMT. Those with CP and/or treated with tracheostomy had a higher likelihood of needing BMT. Rate of hearing loss and ear infection was higher in surgically managed RS patients. Patients with RS and overt CP require a statistically higher number of BMTs compared to those with either submucous cleft palate or intact palate.


Assuntos
Obstrução das Vias Respiratórias , Ventilação da Orelha Média , Síndrome de Pierre Robin , Humanos , Síndrome de Pierre Robin/complicações , Síndrome de Pierre Robin/cirurgia , Masculino , Estudos Retrospectivos , Obstrução das Vias Respiratórias/cirurgia , Obstrução das Vias Respiratórias/etiologia , Feminino , Ventilação da Orelha Média/métodos , Lactente , Traqueostomia , Pré-Escolar , Manuseio das Vias Aéreas/métodos , Osteogênese por Distração/métodos , Criança
7.
Ann Plast Surg ; 92(6): 653-657, 2024 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-38718326

RESUMO

BACKGROUND: Pierre Robin sequence (PRS), characterized by micrognathia, glossoptosis, cleft palate, and obstructed airways, is one of the craniofacial conditions challenging various approaches to managing compromised airways, ranging from conservative measures to invasive airway surgery. This study was conducted to identify predictive risk factors for tracheostomy in the PRS. METHODS: A retrospective chart review was conducted at a tertiary referral hospital in Thailand. Children diagnosed with PRS from January 2010 to December 2021 were enrolled. Univariate and multivariate analysis methods were used to identify the risk factors. RESULTS: Sixty-five patients with PRS were identified in electronic medical records, but 6 were excluded. Thirteen of the remaining 59 patients underwent tracheostomy. There were no significant differences in sex, preterm gestational age, cleft palate, associated syndromes, chromosome abnormalities, or cardiac or neurological involvement between patients who received tracheostomy and those who did not. However, patients with oropharyngeal dysfunction who received tracheostomy tended to use a nasogastric tube or percutaneous gastrostomy 92.3% of the time ( P = 0.043). In addition, patients with abnormal airways from endoscopy were more likely to undergo tracheostomy (odds ratio, 2.17; 95% confidence interval [CI], 1.20-3.90). Interestingly, patients with a sum of Apgar scores at 1 and 5 minutes <15 were more likely to undergo tracheostomy (adjusted odds ratio, 9.91; 95% CI, 1.32-74.52). Furthermore, patients with at least 3 identified comorbidities were more likely to undergo tracheostomy (adjusted odds ratio, 11.34; 95% CI, 1.16-111.15). CONCLUSIONS: The need for tracheostomy depends on comorbidities, Apgar scores, and abnormal airway anatomy. Feeding methods can become more complex with tracheostomy. Prognostic risk factors can help guide treatment and counseling for health care workers and caregivers.


Assuntos
Síndrome de Pierre Robin , Centros de Atenção Terciária , Traqueostomia , Humanos , Síndrome de Pierre Robin/cirurgia , Masculino , Tailândia , Estudos Retrospectivos , Feminino , Prognóstico , Recém-Nascido , Lactente , Fatores de Risco , Medição de Risco
8.
Clin Oral Investig ; 28(5): 247, 2024 Apr 11.
Artigo em Inglês | MEDLINE | ID: mdl-38602599

RESUMO

OBJECTIVES: There is a paucity of information about the possible risk factors that could identify patients with Robin sequence (RS) who are more prone to developing obstructive airway complications after palate closure. This study aimed to compare the respiratory complication rates in patients with RS and isolated cleft palate (ICP). MATERIALS AND METHODS: In this retrospective study, we reviewed the medical records of 243 consecutive patients with RS and ICP who were treated at Amsterdam University Medical Centers over the past 25 years. We collected preoperative data on previous treatment, diagnostic findings, surgical technique, weight, and presence of congenital anomalies. RESULTS: During cleft palate closure, patients with RS were older (11.9 versus 10.1 months; p = 0.001) and had a lower gestational age than those with ICP (37.7 versus 38.5 weeks; p = 0.002). Patients with RS had more respiratory complications (17 versus 5%; p = 0.005), were more often non-electively admitted to the pediatric intensive care unit (PICU) (13 versus 4.1%; p = 0.022), and had a longer hospital stay duration (3.7 versus 2.7 days; p = 0.011) than those with ICP. The identified risk factors for respiratory problems were a history of tongue-lip-adhesion (TLA) (p = 0.007) and a preoperative weight of < 8 kg (p = 0.015). Similar risk factors were identified for PICU admission (p = 0.015 and 0.004, respectively). CONCLUSIONS: The possible risk factors for these outcomes were a low preoperative weight and history of TLA. Closer postoperative surveillance should be considered for patients with these risk factors. CLINICAL RELEVANCE: Identifying risk factors for respiratory complications could provide clinicians better insight into their patients and allows them to provide optimal care for their patients.


Assuntos
Fissura Palatina , Síndrome de Pierre Robin , Humanos , Lactente , Fissura Palatina/cirurgia , Hospitalização , Síndrome de Pierre Robin/complicações , Síndrome de Pierre Robin/cirurgia , Estudos Retrospectivos , Língua
9.
J Craniomaxillofac Surg ; 52(6): 792-797, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38644093

RESUMO

Congenital craniofacial malformations play an important role in upper airway obstruction. One of the main causes is mandibular hypoplasia which is present in the pierre robin sequence. Mandibular distraction osteogenesis (MDO) is one of the most commonly used treatments for the resolution of upper airway obstruction in patients that do not respond to a conservative treatment. We performed a long term follow up of syndromic and non-syndromic patients with pierre robin sequence where the lateral xrays were studied before surgery (T1), after MDO (T2) and long-term follow-up (T3, at least 5 years). Possible complications of the surgery were also studied through panoramic x-rays and clinical controls. The results evidenced an increase of mandibular length comparing T1-T2 and a good stability during the long-term follow-up. The Sd patients presented smaller mandible dimensions. We had not found any complications during the follow-up. The scars outcomes are modest, but none of the patients requested a surgical correction. Mandibular distraction osteogenesis is a treatment to be considered in patients with upper airway obstruction that do not respond to conservative treatments. The results of the surgery are stable and the complications in experienced hands are low.


Assuntos
Obstrução das Vias Respiratórias , Mandíbula , Osteogênese por Distração , Síndrome de Pierre Robin , Humanos , Osteogênese por Distração/métodos , Obstrução das Vias Respiratórias/cirurgia , Mandíbula/cirurgia , Mandíbula/anormalidades , Masculino , Feminino , Síndrome de Pierre Robin/cirurgia , Síndrome de Pierre Robin/complicações , Síndrome de Pierre Robin/diagnóstico por imagem , Pré-Escolar , Criança , Seguimentos , Radiografia Panorâmica , Lactente , Adolescente , Resultado do Tratamento
10.
World J Pediatr ; 20(6): 581-589, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38578561

RESUMO

BACKGROUND: Various conservative and surgical approaches exist to treat Robin sequence (RS), but their effects on facial profile and mandibular catch-up growth are unclear. A functional treatment concept, used in our centre for 25 years, includes an individualized palatal plate with a velo-pharyngeal extension and intensive feeding training. METHODS: We performed a prospective study to objectively describe facial profiles in infants with RS treated with the above concept. Infants with isolated RS were admitted to our tertiary perinatal and national referral centre for craniofacial malformations between May 2018 and Nov 2019. Infants with RS received 3D-photographs during clinically indicated visits. Healthy controls were recruited from Dec 2018 to Sep 2019 and received 3D-photographs every 3 months. The digitally measured jaw index (JI), defined as alveolar overjet (O) x maxillary arch (U)/mandibular arch (L), and the soft tissue reference points A'-point, Nasion', B'-point angle (ANB'), describing the relative position of maxilla to mandible, were evaluated. Linear mixed models were used to examine time trajectories in JI and ANB'. RESULTS: A total of 207 3D images, obtained in 19 infants with RS and 32 controls, were analysed. JI and ANB' decreased over time in both groups [for JI - 0.18 (95% CI - 0.25 to - 0.10); for ANB': - 0.40° per month [(95% CI - 0.48 to - 0.32)]] but remained lower in controls [for JI - 2.5 (95% CI - 3.2 to - 1.8); for ANB'-1.7° (95% CI - 2.4 to - 1.0)]. Also, the ANB' model showed a significant effect of the interaction term diagnosis x age. CONCLUSIONS: Based on longitudinal 3D images, we describe changes in objective parameters of facial profile in infants with and without RS during the first year of life. Our findings indicate catch-up growth in infants treated for RS. Video Abstract.


Assuntos
Síndrome de Pierre Robin , Humanos , Estudos Prospectivos , Síndrome de Pierre Robin/diagnóstico por imagem , Masculino , Feminino , Lactente , Imageamento Tridimensional , Face/anatomia & histologia , Estudos de Casos e Controles , Recém-Nascido
11.
J Craniomaxillofac Surg ; 52(4): 514-521, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38448335

RESUMO

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 (SpO2) 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.


Assuntos
Obstrução das Vias Respiratórias , Fissura Palatina , Síndrome de Pierre Robin , Lactente , Recém-Nascido , Humanos , Criança , Fissura Palatina/cirurgia , Síndrome de Pierre Robin/cirurgia , Estudos Retrospectivos , Manuseio das Vias Aéreas , Nasofaringe , Obstrução das Vias Respiratórias/cirurgia
12.
J Clin Sleep Med ; 20(6): 995-997, 2024 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-38305773

RESUMO

The author recently observed a case involving a 12-year-old sighted girl who exhibited symptoms typical of non-24-hour sleep-wake rhythm disorder (N24SWD). This disorder, more commonly found in blind individuals, presents a unique challenge when diagnosed in those with vision. Several interventions can be attempted, ranging from behavioral adjustments to light therapy. Although melatonin has been noted for its effectiveness in realigning the patient's sleep-wake cycle, the use of ramelteon, a melatonin receptor agonist, has seldom been reported in managing N24SWD. However, this case illuminates the potential of ramelteon as another therapeutic option for sighted individuals with N24SWD. Further study is suggested to determine the potential of ramelteon in managing this disorder among sighted individuals of varying age groups. CITATION: Huang C-H. Managing non-24-hour sleep-wake rhythm disorder with ramelteon in a 12-year-old girl with Pierre Robin sequence and developmental delay: a case report. J Clin Sleep Med. 2024;20(6):995-997.


Assuntos
Deficiências do Desenvolvimento , Indenos , Síndrome de Pierre Robin , Transtornos do Sono do Ritmo Circadiano , Humanos , Feminino , Criança , Indenos/uso terapêutico , Transtornos do Sono do Ritmo Circadiano/tratamento farmacológico , Transtornos do Sono do Ritmo Circadiano/terapia , Deficiências do Desenvolvimento/complicações , Síndrome de Pierre Robin/complicações , Polissonografia
13.
J Clin Sleep Med ; 20(7): 1059-1067, 2024 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-38372169

RESUMO

STUDY OBJECTIVES: Sleep laboratory polysomnography is the gold standard for obstructive sleep apnea (OSA) diagnosis in infants, but its access remains limited. Oximetry-capnography is another simple and widely used tool that can provide information on the presence of desaturations and alveolar hypoventilation. However, its reliability is debated. This study aimed at examining its use in determining OSA severity in infants. METHODS: This retrospective study was conducted in a sleep unit in a tertiary hospital in infants < 4 months old with clinical signs of OSA or Pierre Robin sequence who underwent a 1-night polysomnography coupled with oximetry-capnography. RESULTS: Among the 78 infants included (median [interquartile range] age: 61 [45-89] days at polysomnography), 44 presented with Pierre Robin sequence and 34 presented with isolated airway obstruction. The clinical, sleep, and respiratory characteristics were not significantly different between the 2 subgroups. In the entire cohort, 63.5% had severe OSA. The median obstructive apnea-hypopnea index was 14.5 (7.4-5.9) events/h, peripheral oxygen saturation (SpO2) was 97.4% (96.5-98.1%), and transcutaneous carbon dioxide pressure (PtcCO2) was 41.1 mmHg (38.3-44.9). The optimal threshold to predict an obstructive apnea-hypopnea index > 10 events/h was 6 events/h for an oxygen desaturation index ≥ 3% (sensitivity, 95.7%; specificity, 51.9%) and 2 events/h for an oxygen desaturation index ≥ 4% (sensitivity, 95.7%; specificity, 48.1%). CONCLUSIONS: Whereas transcutaneous capnography does not appear to be sufficient in predicting severe OSA in infants < 4 months old with Pierre Robin sequence or clinical signs of OSA, oximetry may be a useful alternative for the screening of severe OSA in infants in the absence of polysomnography. CITATION: Gyapay R, Ioan I, Thieux M, et al. Gas exchange parameters for the prediction of obstructive sleep apnea in infants. J Clin Sleep Med. 2024;20(7):1059-1067.


Assuntos
Oximetria , Polissonografia , Apneia Obstrutiva do Sono , Humanos , Apneia Obstrutiva do Sono/diagnóstico , Apneia Obstrutiva do Sono/fisiopatologia , Estudos Retrospectivos , Masculino , Feminino , Lactente , Polissonografia/métodos , Oximetria/métodos , Capnografia/métodos , Recém-Nascido , Reprodutibilidade dos Testes , Síndrome de Pierre Robin/complicações , Síndrome de Pierre Robin/diagnóstico , Troca Gasosa Pulmonar/fisiologia
16.
J Craniofac Surg ; 35(1): 163-167, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37934950

RESUMO

INTRODUCTION: Robin sequence (RS) is a congenital clinical condition characterized by micrognathia, glossoptosis, and respiratory distress. Conservative methods could be responsible for releasing feeding and respiratory impairment but little information about mandibular growth is known in long-term follow-up. OBJECTIVE: Assessing the longitudinal behavior of the facial profile of individuals with isolated RS who underwent conservative micrognathia treatment using photographs during the whole craniofacial growth. METHODS: Photographs of the right facial profile of 100 patients were used (50 individuals with isolated RS and 50 individuals without craniofacial anomaly). The individuals with RS were evaluated at 3 different times (T1: infant, T2: mixed dentition, T3: permanent dentition) by measuring the facial convexity angle (FCA; G.Sn.Pog´). A comparison between T3 and control group (C), individuals without craniofacial anomalies and in permanent dentition, was also performed, checking the FCA, nasolabial angle (Ls.Sn.Cm), mentolabial fold (Li.Si.Pog´), facial inferior third (Sn.Gn´.C) angles and the ratio between middle anterior facial height and lower anterior facial height. RESULTS: The T3 group showed an increased angle of facial convexity and increased facial inferior third angle and middle anterior facial height/lower anterior facial height ratio compared with the control group. In the longitudinal evaluation of individuals with isolated RS, significant differences were identified between T1 and T2 groups and T1 and T3 groups showing that the increased facial convexity was higher in the infants and that did not change significantly between the phases of mixed and permanent dentition. CONCLUSIONS: RS showed increased facial convexity in all phases evaluated, but their convexity decreased with growth. When compared with individuals without craniofacial anomalies, the individuals continue to exhibit retrognathism in the permanent dentition. The lack of a mandible projection has led to a considerable number of orthognathic surgeries for the correction of discrepancies.


Assuntos
Micrognatismo , Síndrome de Pierre Robin , Lactente , Humanos , Síndrome de Pierre Robin/terapia , Cefalometria , Seguimentos , Mandíbula/diagnóstico por imagem
17.
J Craniomaxillofac Surg ; 52(1): 55-58, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37884434

RESUMO

The study aimed to evaluate the mid-term effect of MDO in children with Robin sequence (RS). In this case series, 13 patients with RS who underwent MDO were followed up for more than 5 years. Data were collected using clinical history and physical examination. Polysomnography was performed and endoscopic evaluations of the airway was performed if patients still presented obstructive signs of upper airways and/or dysphagia. The patients' clinical signs improved in the mid-term after versus before MDO (inspiratory noise, 92,3% vs 30,8%; apnea, 84,6% vs 7,7%; cyanosis, 76,9% vs 0%; desaturations, 69,2% vs 0%; and suprasternal/intercostal retractions, 61,5% vs 0%; p < 0.05). Statistically significant improvement was noted in the following polysomnographic parameters evaluated in the pre and postoperative mid-term: apnea-hypopnea index, total sleep time and desaturation index (p < 0.05). Within the limitations of the study it seems that MDO is an effective surgical option for children with RS, not only in the short term as previously demonstrated, but also in the mid-term.


Assuntos
Obstrução das Vias Respiratórias , Osteogênese por Distração , Síndrome de Pierre Robin , Criança , Humanos , Lactente , Polissonografia , Estudos Retrospectivos , Síndrome de Pierre Robin/cirurgia , Apneia , Resultado do Tratamento , Mandíbula/cirurgia , Obstrução das Vias Respiratórias/diagnóstico , Obstrução das Vias Respiratórias/etiologia , Obstrução das Vias Respiratórias/cirurgia
18.
J Craniofac Surg ; 35(1): 279-361, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37811988

RESUMO

A European guideline on Robin Sequence was developed within the European Reference Network for rare and/or complex craniofacial anomalies and ear, nose, and throat disorders. The guideline provides an overview of optimal care provisions for patients with Robin Sequence and recommendations for the improvement of care.


Assuntos
Anormalidades Craniofaciais , Doenças Faríngeas , Síndrome de Pierre Robin , Humanos , Síndrome de Pierre Robin/diagnóstico , Síndrome de Pierre Robin/cirurgia , Nariz , Anormalidades Craniofaciais/diagnóstico , Anormalidades Craniofaciais/cirurgia
19.
J Craniofac Surg ; 35(1): 6-9, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37622565

RESUMO

INTRODUCTION: While the literature is replete of clinical studies reporting on the Robin sequence (RS), population-based analyses are scarce with significant variability within the literature in terms of reported incidence, demographic parameters, and outcomes. The authors have conducted a 20-year population-based analysis to guide clinical practice. METHODS: A birth cohort was created from the available datasets in the Healthcare Cost and Utilization Project-Kids' Inpatient Database (HCUP-KID; 2000-2019). Robin sequence patients were identified and further stratified by syndromic status. Incidence, demographic parameters, and outcomes including mortality and tracheostomy rates were computed. A subset analysis comparing the isolated and syndromic cohorts was conducted. Data was analyzed through a χ 2 or t test. RESULTS: The incidence of RS was 5.15:10,000 (95% CI: 4.99-5.31) from a birth cohort of 7.5 million. Overall, 63.3% of the cohort was isolated RS and 36.7% had syndromic RS. Robin sequence patients had a significantly higher rate of cardiac (25.9%) and neurological (8.6%) anomalies compared with the general birth cohort and were most commonly managed in urban teaching hospitals ( P <0.0001). The pooled mortality and tracheostomy rates were 6.6% and 3.6%, respectively. Syndromic status was associated with a longer length of hospital stay (27.8 versus 13.6 d), tracheostomy rate (6.2% versus 2.1%), and mortality (14.1% versus 2.2%) compared with isolated RS ( P <0.0001). CONCLUSIONS: The true incidence of RS is likely higher than previously reported estimates. Isolated RS patients have a low associated mortality and tracheostomy rate and are typically managed in urban teaching hospitals. Syndromic status confers a higher mortality rate, tracheostomy rate, and length of stay compared with nonsyndromic counterparts.


Assuntos
Síndrome de Pierre Robin , Humanos , Estados Unidos/epidemiologia , Estudos Retrospectivos , Síndrome de Pierre Robin/epidemiologia , Síndrome de Pierre Robin/cirurgia , Síndrome de Pierre Robin/complicações , Incidência , Tempo de Internação , Pacientes Internados
20.
J Craniofac Surg ; 35(1): 154-157, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37603896

RESUMO

Life-threatening airway obstruction is a major concern in patients with Pierre-Robin sequence. Tongue-lip adhesion (TLA) has been used to manage airway obstruction. The authors present the case of a female neonate with Pierre-Robin sequence who presented with airway obstruction and a cleft palate. She underwent a TLA procedure with modified tongue base suspension (TBS). Endoscopy was used to check and control the traction of the tongue base to enable unobstructed self-ventilation. Positive outcomes including improved O2 saturation and weight gain were noted. The effectiveness of TLA was enhanced by using TBS with real-time endoscopy to evaluate the oropharyngeal airway space required to alleviate airway obstruction. The use of endoscopy enabled us to check and determine how much the tongue base should be tracted by manipulating the tongue anteriorly and posteriorly. The authors report transoral endoscopy-assisted TLA and modified TBS.


Assuntos
Obstrução das Vias Respiratórias , Laringe , Síndrome de Pierre Robin , Recém-Nascido , Humanos , Feminino , Síndrome de Pierre Robin/cirurgia , Língua/cirurgia , Endoscopia , Resultado do Tratamento
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