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1.
ObjectiveTo determine weight gain during treatment with the modified palatal plate (MPP) in infants with isolated and syndromic Pierre Robin Sequence (PRS) suffering from micrognathia, upper airway obstruction (UAO), and failure to thrive (FTT), the authors conducted a retrospective study of infants treated with the MPP.MethodsThe main outcome measure was infant weight (g) for up to three months after birth. Demographic and outcome data (associated syndromes, comorbidities, presence of cleft lip or palate, intubation attempts, tracheotomy and cleft repair) were collected.Results14 children born January 2010 - December 2019 were included. The majority (86%) of infants showed highly significant weight gain (p < 0.001) within a 3-month period (mean pretreatment weight 3147 g with a SD of 425 g vs mean weight at three months 4435 g with a SD of 635 g). Syndromic PRS was found in 7% of infants. 43% of nonsyndromic PRS patients were found to have other congenital anomalies. Genetic testing showed normal karyotypes in 93% of infants and a microdeletion in 7% of infants. 21% of infants required tracheotomy, but no patients required mandibular distraction (MDO) or tongue-lip adhesion (TLA) to relieve UAO.ConclusionPRS infants treated with the MPP showed highly significant weight gain within a 3-month period and did not require mandibular surgery for early airway management, but faster gain of weight might have implications for strategies to perform surgery at an earlier point in time.  相似文献   

2.
Management of infants with Pierre Robin sequence.   总被引:2,自引:0,他引:2  
OBJECTIVE: Several methods of treating babies with Pierre Robin sequence have been described since the condition itself was first documented in 1923. The main aim of treatment has been to relieve upper airway obstruction. Treatment methods used range from positioning of the baby to invasive surgery. The aim of this article was to describe the assessment, treatment, and monitoring methods used for babies referred with Pierre Robin sequence (PRS). SETTING/PATIENTS: From December 1995 to May 2000, 22 consecutive patients were admitted to Birmingham Children's Hospital with PRS. Their airway and nutritional status were assessed and continuously monitored. INTERVENTIONS: Treatment concentrated on the relief of airway obstruction with a nasopharyngeal airway (NPA) and nutritional support of the babies until they grew out of their respiratory and feeding difficulties. MAIN OUTCOME MEASURES: Outcome measures were oxygen saturation, growth of the babies, and the need for surgery. RESULTS: All babies were managed successfully with an NPA and nutritional support. No baby required surgery, and the majority showed good weight gain. CONCLUSION: Relieving airway obstruction by NPA is an effective and safe treatment for babies with PRS until they have grown out of their respiratory and feeding difficulties. It avoids the need for surgery and can be used on neonatal wards using the monitoring described.  相似文献   

3.
The objective was to review and compare outcomes after tongue–lip adhesion (TLA) and mandibular distraction osteogenesis (MDO) in infants with severe breathing difficulties related to Pierre Robin sequence (PRS). A single-centre retrospective (2002–2012) study was carried out; 18 infants with severe breathing difficulties related to PRS resistant to conservative treatment, who underwent TLA or MDO to correct airway obstruction, were enrolled. The primary outcome measures were successful weaning from respiratory support and resumption of full oral feeding. Nine underwent TLA and nine MDO. Eight of the nine infants who underwent MDO and all those treated with TLA were successfully weaned from respiratory support. After discharge, residual respiratory distress was diagnosed more commonly after TLA than after MDO (6/9 vs 1/9, P = 0.050). Infants resumed oral feeding sooner after MDO than after TLA (mean days after surgery to full oral feeds 44 ± 24 vs 217 ± 134, P < 0.003). The length of hospital stay was longer for infants treated with MDO than for those treated with TLA. The rate of complications was similar. Infants with severe airway obstruction related to PRS can benefit safely from either TLA or MDO. Although MDO lengthens the time to discharge, this option stabilizes airway patency of infants with PRS more efficiently and achieves full oral feeding more rapidly than TLA.  相似文献   

4.
Surgical treatment of tumours in the anterior mandible and surrounding tissues may result in defects which can be restored by a fibula free flap. The upper airway may change during this process. The purpose of this retrospective study was to evaluate upper airway changes after fibula free flap reconstruction. A total of 37 patients who underwent anterior mandibulectomy and fibula free flap reconstruction between 2012 and 2020 were recruited. Patients with benign and malignant tumours involving the anterior mandible were included. Spiral computed tomography was performed 1 week preoperatively, 1 week postoperatively, and at> 1 year (range 12–23 months) after surgery. Cross-sectional areas and volumes of the upper airway were measured. Data were analysed by two-way analysis of variance. The upper airway in the malignant tumour group showed an increasing trend, especially at the soft palate and tongue base levels (P < 0.01). In the benign tumour group, the upper airway showed no significant changes. The location of the minimum cross-sectional area moved downwards in both groups, and the area increased in the malignant tumour group during long-term follow-up. Upper airway obstruction is less likely to occur in the long term after surgical resection of anterior mandible malignancies and fibula free flap reconstruction.  相似文献   

5.
Congenital retrognathia and glossoptosis characterize isolated Pierre Robin sequence (iPRS); the small mandible and its retracted position cause retrodisplacement of the tongue and reduction of the oropharyngeal airway. These neonates may be affected by airway obstruction, feeding difficulties, failure to thrive, and chronic hypoxaemia. To solve the respiratory problems secondary to glossoptosis, various treatments have been described including prone positioning, a nasopharyngeal tube, glossopexy, and mandibular distraction. Over the last 28 years, the authors have treated 118 neonates and infants affected by iPRS by carrying out traction of the mandible using two parasymphyseal wires, positioned under local anaesthesia. All the procedures were successful, with no major complication. The patients’ respiratory problems and apnoea disappeared suddenly after beginning traction.  相似文献   

6.
OBJECTIVE: This unit has reported management of infants with Pierre Robin Sequence (PRS) and upper airway obstruction using nasopharyngeal airways and nutritional support until enough growth takes place for the infant to thrive. There was a mean hospital stay of 60 days. This long in-patient stay prompted review of our management protocols and consideration of treatment at home. This paper reports our experience of managing infants with PRS at home using a nasopharyngeal airway and nasogastric feeding tube and reviews whether such management reduces in-patient stay while remaining safe and effective. DESIGN: Retrospective review of cases referred over a 3.5-year period. Comparison is made with the unit's previously published results. PATIENTS: Thirteen PRS infants were referred to the West Midlands Regional Cleft service and required transfer to Birmingham Children's Hospital for specialist assessment and airway control. INTERVENTIONS: The parents of 12 infants underwent training to manage the airway and feeding tube. Treatment then continued at home. OUTCOME MEASURES: In-patient episode, rate of weight gain, and complication rate were used. Results: The median hospital stay was 19.5 days compared to 54 days previously. The median rate of weight gain was 34 g/d. There were no complications or readmissions. CONCLUSION: This series demonstrates the revised management protocol followed has reduced in-patient stays and remained effective, with infants continuing to thrive after discharge home, and has a low complication rate.  相似文献   

7.
The purpose was to evaluate short-term changes in condylar and glenoid fossa morphology in infants with Pierre Robin sequence (PRS) undergoing early (age <4 months) mandibular distraction osteogenesis (MDO) for the management of severe airway obstruction. Computed tomography data from infants with PRS who had MDO were compared to those of age-matched control infants without facial skeletal dysmorphology. Surface/volume, linear, and angular measurements of the condyle and glenoid fossa were obtained and compared between infants with PRS and controls. Eleven infants with PRS met the inclusion criteria. There were five female and six male subjects with a mean age at the time of MDO of 41 ± 32 days. Prior to MDO, PRS mandibles had a smaller condylar articulating surface area and volume than age-matched control mandibles, with a more laterally positioned condylar axis (P  0.05). Following MDO, there were significant increases in condylar articulating surface area and volume, approaching those of normal controls, with further lateral translation of the condylar axis (P  0.05). Condyle and glenoid fossa morphology is largely normalized following early MDO in infants with PRS. The condylar axis translates laterally as a result of MDO; this change is not observed with mandibular growth in infants without PRS.  相似文献   

8.
Procedures to relieve airway obstruction in Pierre Robin sequence (PRS) include glossopexy, mandibular distraction, and tracheostomy. Previously, it has been suggested that the imbalance of the genioglossus insertion on the mandible could be responsible for the micrognathia, tongue elevation, and glossoptosis seen in PRS. We performed a retrospective study to determine the usefulness of subperiosteal release of the floor of the mouth in PRS with airway problems. Between 1991 and 2005, 14 consecutive patients with PRS were treated with subperiosteal release of the floor of the mouth performed at the British Columbia's Children's Hospital. There were 8 boys and 6 girls. Mean age at surgery was 15 weeks (range, 1-68 weeks). Postoperative follow-up ranged from 8 months to 13 years. Seven patients (4 boys and 3 girls) were successfully treated, and a tracheostomy was prevented. Fifty-five percent of the syndromic patients needed a tracheostomy, whereas 1 patient (33%) with isolated PRS needed a tracheostomy. The most common syndrome associated with PRS was Stickler syndrome. Five (72%) of the 7 patients who required a tracheostomy were boys. If conservative measures fail to relieve supraglottic airway obstruction seen in patients with PRS, a subperiosteal release of the floor of the mouth can be an effective intervention in certain patients. It is a simple intervention with few complications and little morbidity. More investigations are mandatory to precisely define patient characteristics ideal for this intervention.  相似文献   

9.
Congenital craniofacial malformations such as Pierre Robin sequence or Treacher Collins syndrome are associated with mandibular micrognathia, resulting in obstructive sleep apnea (OSA) due to a decreased pharyngeal airway; in severe cases this leads to tracheostomy dependence. We present a series of 18 patients in whom we performed mandibular lengthening using internal distraction devices to relieve airway obstruction. Seven were tracheostomy-dependent and 11 were respiratory distressed without tracheostomy. The mandible was distracted at a rate of 1 mm per day. Following 3 months of consolidation for bony maturation, the distraction devices were removed. Results demonstrated forward mandibular elongation of a mean 22 mm (range 20–25 mm) and an increase in SNB angle and in pharyngeal airway. All patients with tracheostomies were decannulated, and there was an improved airway with resolution of signs and symptoms of OSA and elimination of oxygen requirement in all patients. We conclude that mandibular distraction using internal devices is a useful and comfortable method for younger children to expand the mandible forward and increase the pharyngeal airway.  相似文献   

10.
The purpose of this retrospective longitudinal cephalometric study was to analyze size, growth, and relations of the mandible, tongue, and airway in the isolated, nonsyndromic Pierre Robin Sequence (PRS) infant. The objective was to understand better the early morphologic changes that occur in these structures. The data were obtained from lateral cephalometric radiographs taken in the first 2 years of life on 17 PRS infants, 26 isolated cleft palate (CP), and 26 normal control (N) infants. Mean values of each variable were compared using a univariate analysis of variance. A multivariate discriminant function analysis (DFA) was also used to characterize group differences. The three groups were distinct throughout the period of study. Differences were greater at the earliest age and the distinction was greater between the PRS and N infants with the CP infant in between, but having more similarity to the PRS infant. Initially, the PRS infant had a shorter tongue and mandibular length, narrower airway, smaller tongue area and the hyoid position was more posterior and inferior as compared to N. The tongue shape and position were specific to the PRS infants. This group distinction diminished with age and resulted from an increased mandibular growth rate in the PRS infant. These findings support the hypothesis of "partial mandibular catch-up growth" in the PRS infant. The increased growth rate in the PRS infant improved the airway dimension, which might be partly responsible for the natural resolution of the respiratory distress. This increased growth rate did not allow for the various structures to reach values equal to normal.  相似文献   

11.
Pierre Robin sequence (PRS) is a triad of micrognathia, glossoptosis, and cleft palate that results in an obstruction of the airway on inspiration and impeding feeding. The tongue of infants with PRS fall back toward the posterior pharyngeal wall (glossoptosis) due to receding chin produced by mandibular micrognathia (small jaw) or retrognathia. This causes a serious condition with potentially severe, life-threatening airway obstruction. If untreated, this problem can lead to exhaustion, cardiac failure, and ultimately death, especially during the early months of life. Actually, in the majority of PRS infants, these symptoms can be managed by placing the infant in the prone position until adequate growth of the jaw occurs. If this type of treatment fails, the infant then should be considered for other conservative therapies or surgical interventions. This paper reviews surgical interventions such as tongue-lip adhesion, mandibular traction, mandibular distraction, tracheotomy and conservative orthodontic approaches, and presents a baby treated successfully with an orthodontic appliance.  相似文献   

12.
PURPOSE: The purpose of this study was to evaluate mandibular lengthening by distraction osteogenesis (DO) to achieve decannulation of micrognathic children with "permanent" tracheostomies. PATIENTS AND METHODS: Using a retrospective chart review, patients were included who had 1 ) airway compromise/tracheostomy, 2 ) micrognathia, 3 ) polysomnography-documented obstructive apnea, and 4 ) mandibular advancement using DO. Excluded were 1 ) adults, 2 ) neonates without tracheostomy, and 3 ) patients with central apnea. Patient age, past medical history, age at tracheostomy, and distraction protocol were documented. Oxygen saturation, posterior airway space (in millimeters), and sella-nasion-B point (SNB) angle were recorded. The distraction protocol consisted of a latency of 48 hours and a rate of 1 mm/day. RESULTS: There were 5 children, aged 2 to 14 years, who received a tracheostomy between ages 2 and 36 months for airway obstruction. All patients underwent bilateral mandibular distraction using semiburied, unidirectional devices. The average latency was 58 hours, the rate was 1 mm/day, the duration of fixation was 40 to 60 days, and the magnitude of advancement was 23 mm. Healing was evaluated by clinical, radiologic, and ultrasound examinations. No complications were experienced. Mean follow-up was 3.2 years. Postdistraction sleep studies demonstrated no obstructive apneic events and a mean oxygen saturation of 98% (preoperative, 76%, P < .005). Cephalometric values improved: posterior airway space 4 to 14 mm; SNB 66 degrees to 72 degrees ( P < .005 for both variables). Four of the 5 patients have been successfully decannulated to date. CONCLUSIONS: The results of this preliminary study indicate that mandibular advancement by DO is a potentially viable treatment option for tracheostomy-dependent children with upper airway obstruction secondary to micrognathia.  相似文献   

13.
目的:评估用正颌外科技术矫治9例颞颌关节强直所致睡眠呼吸暂停综合征的效果。方法:采用患侧关节成形,下颌升支、体部的“L”型半层截骨及健侧矢状截骨术,将患侧下颌升支加高固定,下颌前部整体前移。结果:9例颞颌关节强直后睡眠呼吸暂停综合征患者术后张口度达2.5~3.5cm,连续血氧饱和度最低值由术前的58%提高至术后的95%以上,唾眠呼吸暂停综合征得以治愈。结论:颞颌关节强直后睡眠呼吸暂停综合征患者通过正颌外科手术可以在关节成形的同时,解决患者下颌后缩的缺陷,解除上气道狭窄,从而缓解或纠正患者的低氧血症。  相似文献   

14.
The objectives of this study were to analyse the changes in airway cross-sectional areas and volumes due to surgical movements of the jaws and to identify any possible correlation with the direction of the movements. Fifty-seven participants, aged 18–28 years (mean 20.8 years) at surgery, were followed up for 12 months postoperatively. Pre- and postoperative measurements of the facial region obtained from computed tomography in a supine position were analysed according to the surgical movements and changes in the upper airways. Intra-rater reliability was assessed. Comparisons and correlations of jaw movements, changes in airway volume, and body mass index (BMI) were made. The cohort showed a significant change between the pre- and postoperative measurements for areas associated with the anterior nasal spine (P = 0.013), posterior nasal spine (P = 0.049), uvula (P = 0.006), and epiglottis (P = 0.046). Additionally, a correlation was found between the airway volume change and the change in mandible position (correlation coefficient 0.324, P = 0.014). All participants were non-smokers, and no correlation was observed between BMI and the upper airway volume. Changes in the upper airway can be expected following surgical movements of the jaws. A correlation was shown between a sagittal direction of the movements and the changes in the airways. Patients with obstructive sleep apnoea who are indicated for surgical movements of the jaws are expected to benefit from orthognathic surgery.  相似文献   

15.

Background

Severe obstructive sleep apnoea (OSA) is a life threatening condition associated with Pierre Robin sequence (PRS) due to mandibular micrognathia and glossoptosis. Often these patients require tracheostomy at an early age which has high morbidity. Distraction osteogenesis (DO) is an accepted method of treatment for patients with hypoplastic mandible to achieve mandibular lengthening without need for a bone graft. It has also been used in respiratory distressed neonates and infants to avoid tracheostomy.

Case report

An eight month old baby, a diagnosed case of PRS with severe OSA and recurrent episodes of aspiration pneumonia and on nasogastric tube feeding since birth was referred to us for evaluation and possibility of therapeutic augmentation of the mandible by DO. After a thorough clinico-radiological assessment the child was operated for bilateral extraoral placement of horizontal corpus distractor. A total distraction of 12 mm was carried out and consolidation of callus was monitored by USG. Postoperatively the patient was followed up for 12 months. Presently she has normal respiratory and feeding function without any episode of aspiration pneumonia.

Conclusion

Mandibular corpus DO is a safe and effective technique that can be applied to predictably relieve severe upper airway obstruction in selected PRS cases. In order to avoid the limitations of alternative surgical procedures and the tracheostomy-associated morbidity, DO should be considered among the routine treatment modalities.
  相似文献   

16.
Segmental mandibular advancement (SMA) consists of a combination of bilateral sagittal split osteotomy, anterior subapical osteotomy with extraction of the first premolars, and genioplasty, to allow an extended advancement of the mandible for the improvement of tongue base obstruction in moderate-to-severe obstructive sleep apnoea (OSA) and to minimize any unfavourable aesthetic change due to the large jaw advancement. The aim of this pilot study was to evaluate the surgical outcomes and complications following SMA in OSA patients. Twelve patients (nine male, three female) underwent SMA as part or whole of their skeletal advancement procedure for OSA. The apnoea–hypopnoea index (AHI) improved from a mean± standard deviation 42.4 ± 22.0/hour preoperatively to 9.0 ± 17.4/hour at 1 year postoperative. Surgical success (50% reduction in AHI) was achieved in 11 of the 12 patients (91.7%) at 1 year postoperative, while seven patients (58.3%) attained surgical cure (AHI<5/hour). The lowest oxygen saturation (LSAT) increased from a mean 73.3% preoperatively to 78.7% at 1 year postoperative. The airway volume increased from a mean 2.4 ± 1.7 cm3 at baseline to 6.7 ± 3.5 cm3 at 1 year postoperative (P < 0.001). No major complication occurred. This pilot study showed that SMA appears to be safe and effective as part or whole of the skeletal advancement surgery for moderate-to-severe OSA.  相似文献   

17.
This paper aims to review the utility of I- gel as a successful airway management device for infants with Pierre robin sequence (PRS) undergoing glossopexy. A prospective study was conducted on PRS neonates. The algorithm followed was putting a ‘Tongue traction stitch’ followed by the following sequence – two trials with direct laryngoscope intubation, two attempts with fiberoptic endoscope intubation followed by insertion of I-gel? to manage difficult airway during glossopexy procedure. 6 patients were intubated with direct laryngoscope, 12 patients were intubated with fibreoptic endoscope and the rest 13 patients were intubated using I-gel?. Successful management of difficult airway was achieved with this airway management protocol during glossopexy and nil postoperative complications were encountered. Within the limitations of the study it seems that, I-gel? is a relevant alternative toprovide a reliable and secure airway access to carry out glossopexy procedure in such patients.  相似文献   

18.
The aim of this study was to evaluate volumetric changes of the posterior airway space (PAS) following bimaxillary surgery using a high oblique sagittal split osteotomy (HSSO) of the mandibular ramus.The cone beam CTs of Class II and Class III patients taken before (T0) and 6–12 months after surgery (T1) were analyzed using 3D software (Mimics® Innovation Suite 18.0). The PAS was divided into three segments (superior, middle, inferior) by three planes parallel to the Frankfurt horizontal plane intersecting at the posterior nasal spine, the velum palatinum and the epiglottis. Total (TPAS) and partial volumes (SPAS = superior, MPAS = middle, IPAS = inferior) were calculated.For the 25 Class II patients, a highly significant increase (p<0.001) of the total, middle and inferior airway space (TPAS: +33.6%, MPAS: +43.1%, IPAS: +55.9%) was found, while the increase of the upper airway space was statistically not significant (+5.4%, p = 0.074). For the 28 Class III patients, the total, middle and inferior airway space increased statistically insignificantly (TPAS: +4.6%, p = 0.265, MPAS: +2.7%, p = 0.387, IPAS: +2.8%, p = 0.495), while the increase of the upper airway space was statistically significant (+9.7%, p = 0.010).Bimaxillary orthognathic surgery using the HSSO technique led to a significant increase of PAS for Class II patients and could conserve the PAS for Class III patients.  相似文献   

19.
Diagnosing and comanaging patients with obstructive sleep apnea syndrome   总被引:8,自引:0,他引:8  
BACKGROUND: Obstructive sleep apnea syndrome, or OSAS, is a common, but underdiagnosed, disorder that potentially is fatal. It is characterized by repetitive episodes of complete or partial upper airway obstruction leading to absent or diminished airflow into the lungs. These episodes usually last 10 to 30 seconds and result in loud snoring, a decrease in oxygen saturation, and chronic daytime sleepiness and fatigue. The obstruction is caused by the soft palate, base of the tongue or both collapsing against the pharyngeal walls because of decreased muscle tone during sleep. Potentially fatal systemic illnesses frequently associated with this disorder include hypertension, pulmonary hypertension, heart failure, nocturnal cardiac dysrhythmias, myocardial infarction and ischemic stroke. CLINICAL IMPLICATIONS: The classic signs and symptoms of OSAS may be recognizable by dental practitioners. Common findings in the medical history include daytime sleepiness, snoring, hypertension and type 2 diabetes mellitus. Common clinical findings include obesity; a thick neck; excessive fat deposition in the palate, tongue (enlarged) and pharynx; a long soft palate; a retrognathic mandible; and calcified carotid artery atheromas on panoramic and lateral cephalometric radiographs. CONCLUSIONS: Dentists cognizant of these signs and symptoms have an opportunity to diagnose patients with occult OSAS. After confirmation of the diagnosis by a physician, dentists can participate in management of the disorder by fabricating mandibular advancement appliances and performing surgical procedures that prevent recurrent airway obstruction.  相似文献   

20.
Mandibular distraction has proven to be a valuable tool for lengthening the hypoplastic mandible and relieving airway obstruction in infants. Numerous devices have been developed to achieve the desired mandibular lengthening. Complications including poor vector control, need to mold regenerate, facial scarring, external pin loosening, and bulky hardware have been associated with previous devices. In an attempt to circumvent some of these problems, the senior author developed an internal curvilinear device (Osteomed Corporation, Dallas, TX), which is applicable to the infant mandible. The aim of this paper is to describe the use of this distractor as well as its indications and outcomes.Twelve micrognathic infants (ages range from 9 days to 8 months) who underwent mandibular distraction between March 2005-May 2006 at Lucile Packard Children's Hospital were included in the study. Preoperative workup included an evaluation by a multidisciplinary team including a pediatric otolaryngologist, neonatal intensivist, pediatric pulmonologist, occupational therapist, and craniofacial surgeon. Pre and postoperative maxillomandibular discrepancy, sleep study, feeding evaluation, and three-dimensional computerized tomography scans were compared.All patients tolerated the distraction process well to completion without postoperative complication, except for one patient who had temporary facial nerve weakness, which resolved in 2 months. All patients with obstructive apnea had the obstructive component improved. The last six patients had pre and postoperative polysomnograms to document the improvement. Two patients with neurologic impairment had persistent central apnea. One nonsyndromic patient with inability to feed and feeding-related airway obstruction was taking complete oral feeds 2 weeks after distraction.Mandibular distraction with an internal curvilinear device is effective at relieving airway obstruction in micrognathic infants, while avoiding some previously reported complications.  相似文献   

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