Short-term condylar and glenoid fossa changes in infants with Pierre Robin sequence undergoing mandibular distraction osteogenesis

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.     

Imaging characteristics of the mandible and upper airway in children with Robin sequence and relationship to the treatment strategy

Some patients with Robin sequence (RS) may respond to conservative therapy, while others require surgery; however, there are currently no objective anatomical criteria to gauge surgical indication. The purpose of this study was to explore the imaging characteristics of the mandible and upper respiratory tract in children with RS and examine how individual morphometric parameters influence the treatment strategy. Cone beam computed tomography (CBCT) images were obtained from 57 children with RS. Twenty parameters were measured as potential surgical indicators. The children were divided into two groups according to surgical requirement: a non-surgical group (26 children) and a surgical group (31 children who underwent mandibular osteodistraction). Differences in the imaging parameters were compared between the groups. The mandibular ramus was shorter bilaterally (left, P = 0.047; right, P = 0.027) and the mandibular body was longer bilaterally (left, P = 0.030; right, P = 0.025) in the surgical group when compared to the non-surgical group. In addition, the mandibular angle bilaterally (left, P = 0.023; right, P = 0.003) and the cross-sectional area at the epiglottis tip (P = 0.010) were smaller in the surgical group. Compared to RS patients receiving conservative treatment, children requiring surgery have specific anatomical features of the mandible and upper airway that can be used as surgical indications.     

Precise osteotomies for mandibular distraction in infants with Robin sequence using virtual surgical planning

Mandibular distraction osteogenesis (MDO) has become the first-line operation in many centers for the management of obstructive sleep apnea (OSA) in infants with (Pierre) Robin sequence (RS) not relieved by non-surgical approaches. Preoperative virtual surgical planning (VSP) may improve precision and decrease complications for this operation. This article reports a retrospective study of RS infants who underwent MDO for OSA using preoperative VSP and three-dimensionally printed cutting guides performed by one surgeon. Seventeen subjects who had MDO at a mean age of 87 ± 96 days were included. Maxillofacial computed tomography scans were obtained 15 ± 7 days prior to MDO. Osteotomy designs included linear (n = 4, 23.5%), inverted-L (n = 11, 64.7%), and multi-angular (n = 2, 11.8%). Cutting guides were used successfully and osteotomies were created as planned in all cases. Devices were removed 67 ± 15.6 days after placement. Bone formation in the distraction gap was seen in all cases at device removal. All patients had successful airway outcomes. There were no major and four minor complications during the follow-up period of 458 ± 267 days. In conclusion, MDO is a successful procedure for the management of OSA associated with RS in infants, and VSP facilitates its precise design and execution.     

Early weight gain in infants with Robin sequence after mandibular distraction

This retrospective cohort study was performed to assess weight gain in infants with Robin sequence (RS) treated by mandibular distraction osteogenesis (MDO). The primary outcome variable was average daily weight gain for the following time periods: (1) birth to MDO (T1), (2) MDO to distractor removal (T2), (3) distractor removal to 6 months later (T3), and (4) 6 months to 12 months following distractor removal (T4). Published growth curves were used for comparison. Differences were assessed using the Wilcoxon matched-pairs signed rank test. Twenty-two infants were included in the study. During T1, the infants had 9.47 ± 12.61 g/day less weight gain than expected (P = 0.001). However, for T2, T3, and T4, the infants demonstrated 3.48 ± 6.17 g/day (P = 0.028), 2.19 ± 4.47 g/day (P = 0.030), and 1.83 ± 3.25 g/day (P = 0.028) more weight gain than expected. Feeding tube use resulted in improved weight gain during T1 (P < 0.001), but was associated with poorer weight gain in T3 (P = 0.003) and T4 (P = 0.001). In conclusion, infants with RS treated by MDO demonstrated poorer weight gain relative to their peers between birth and the MDO operation. However, from the MDO procedure to 12 months post-distractor removal, infants who had MDO showed faster weight gain than their age-matched peers.     

A new way to anchor the external device in mandibular distraction: three case reports with a Pierre Robin sequence

Pierre Robin sequence is a pathology derived from alteration in the first and second branchial arch. Patients have breathing problems due to micrognathia and glossoptosis, causing severe upper airway obstruction. One surgical treatment is distraction osteogenesis. Three patients with Pierre Robin sequence (case 1, 3 months old; cases 2 and 3, 1 month old) with severe upper airway obstruction requiring mechanical ventilator assistance, underwent mandibular distraction osteogenesis prematurely with a new anchoring system, thus avoiding tracheostomy and its consequences. An intraoral approach was used to avoid scarring. A new anchoring device with transfixing Kirschner wire in the proximal (mandibular ramus) and distal segment (chin zone) was used. This diminishes the risk of distractor device displacement, guaranteeing optimal stability. A more anterior installation reduces the risk of damaging tooth buds in the mandibular body and the inferior alveolar nerve. The more anterior the fixation, the more horizontal the distraction vector becomes. The position and stability of the device are crucial. In these three patients the placement of two transfixing Kirschner wires using an intraoral approach showed good results and stability during the period of distraction and consolidation, with optimal results on the upper airway, avoiding tracheostomy.     

Condylar positions before and after bilateral mandibular distraction osteogenesis in children with Pierre Robin sequence

This study aimed to analyze the changes in mandibular morphology after bilateral mandibular distraction osteogenesis (BMDO) in children with Pierre Robin sequence. The positions of the condyles were analyzed in reconstructed three-dimensional craniomaxillofacial images obtained for 18 children before and 8–12 weeks after BMDO. Differences between pre- and postoperative parameters were assessed using paired t-tests. After surgery, a significant decrease in superior joint space was detected (P < 0.05), but no change in anterior joint space or posterior joint space was observed. The ratio of the distance between gonia and distance between condylion points (GoL–GoR/CoL–CoR) (P < 0.001) and the distances between the condyle and midsagittal plane (P < 0.001) increased after surgery, while the condylar horizontal angle decreased (P < 0.05). No change in condylar vertical angle was noted. After BMDO, the condyle displayed an outward and upward shift, as well as outward rotation along the proximal segment. The mandible body exhibited forward movement with a more significant opening range. These changes were consistent with the extent of the newly formed bone tissue and the improvement in coordination and appearance of the children’s facial structures. The long-term effects of changes in condylar position on the development of the maxillofacial structures needs to be studied further.     

Outcomes after tongue–lip adhesion or mandibular distraction osteogenesis in infants with Pierre Robin sequence and severe airway obstruction

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.     

Mandible traction with wires for the treatment of upper airway obstruction caused by Pierre Robin sequence in Chinese infants: Preliminary findings

Pierre Robin sequence (PRS) is a congenital abnormality that may cause upper airway obstruction requiring surgical intervention. This preliminary study aimed to examine the feasibility and effectiveness of mandible traction with wires for the treatment of upper airway obstruction caused by PRS in Chinese infants. Measures of interest included transcutaneous oxygen saturation before and after surgery, duration of surgery and traction, complications, and CT findings. Seven infants were included in the study (mean birth weight: 2485 g, range: 2405–2570 g); four were born preterm and three were born full term. Mean age at surgery was 13.7 days (range: 2–28 days), mean duration of surgery was 16.6 min (range: 13–25 min) and mean duration of traction was 26.6 days (range: 21–35 days). Mean follow-up was 6.2 months (range: 1–11 months). No infant experienced severe complications. All infants experienced increases in transcutaneous oxygen saturation after surgery. Mean transcutaneous oxygen saturation was 82% before surgery and 98% after surgery. Follow-up morphology of the mandible was excellent. There was no upper airway obstruction, and short-term growth and development were satisfactory. These preliminary findings suggest that mandibular traction with wires may be an effective treatment for upper airway obstruction caused by PRS in Chinese infants.     

Weight gain in infants with Pierre Robin sequence

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.     

Tracheostomy versus mandibular distraction osteogenesis in infants with Robin sequence: a comparative cost analysis

Many treatments have been described for infants with Robin sequence and severe respiratory distress, but there have not been many comparative studies of outcome and cost-effectiveness. The aim of this study was to compare the cost and complications of two common interventions – mandibular distraction osteogenesis and tracheostomy. Nine patients with isolated Robin sequence (mandibular distraction osteogenesis, n = 5, and tracheostomy, n = 4) were included in the analyses. Predetermined costs and complications were obtained retrospectively from medical records and by questionnaires to the parents over a 12-month period. Overall direct costs (admission to hospital, diagnostics, surgery, and homecare) were 3 times higher for tracheostomy (€105.523 compared with €33.482, p = 0.02). Overall indirect costs (absence from work) were almost 5 times higher (€2.543 compared with €543, p = 0.02). There was a threefold increase in overall total cost/patient (both direct and indirect) for tracheostomy (€108.057 compared with 34.016, p = 0.02) and 4 times more complications were encountered. This study shows that mandibular distraction osteogenesis in infants diagnosed with Robin sequence costs significantly less and results in fewer complications than tracheostomy, and this contributes to our current knowledge about the ideal approach for infants with Robin sequence and might provide a basis for institutional protocols in the future.     

Respiratory distress in Pierre Robin sequence: an experience with mandible traction by wires

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.     

Management of airway and feeding difficulties in a neonate with Pierre Robin sequence: a case report

Pierre Robin sequence is a congenital disorder that affects neonates, and is characterised by the classic triad of micrognathia, glossoptosis, and cleft of the secondary palate. We present the case of a child with Pierre Robin sequence who had severe respiratory distress and feeding problems, and describe a simple technique for forward traction of the mandible to relieve the respiratory distress and activate the tongue.     

Long-term speech outcome in patients with Robin sequence after cleft palate repair and tongue-lip adhesion: A 21-year retrospective analysis

The purpose of this study was to assess the effect of tongue−lip adhesion (TLA) on the long-term speech and articulation outcomes of patients with Robin sequence (RS) after cleft palate repair. Outcomes were compared to those in patients with RS who required positioning alone and to patients with isolated cleft palate (ICP).All consecutive patients with RS (with or without TLA) versus isolated cleft palate (ICP) who underwent cleft palate repair were retrospectively reviewed. Speech and articulation included all assessments between the age of 3–6 years. Secondary speech operations, velopharyngeal insufficiency (VPI), hypernasality, and articulation errors by cleft-type characteristics (CTC), including 4 categories (passive), non-oral, anterior-oral, and posterior-oral. A total of 41 RS patients and 61 ICP patients underwent repair with sufficient follow-up. Of them, 23 patients underwent a TLA at median age of 12 days. Rates of hypernasality (p = 0.004), secondary speech operations (p = 0.004), and posterior oral CTC (p = 0.042) were higher in RS compared to ICP. Isolated RS had speech outcomes similar to those of ICP; however, syndromic RS patients needed more secondary speech operations compared to isolated RS (p = 0.043). TLA-RS patients did not demonstrate differences in speech outcomes or any CTCs (all p > 0.05) compared to non−TLA-RS patients, except for the anterior oral CTC (74% TLA-RS vs 28% non−TLA-RS, p = 0.005).Within the limitations of the study, it seem that TLA does not affect long-term velopharyngeal function in patients with RS. However, TLA-RS patients demonstrated higher rates of anterior-oral CTC, which might be related to a different positioning of the tongue after TLA. Every effort should be taken to treat patients with RS conservatively instead of with TLA because of this demonstrated a negative effect on one type of articulation error. However, if conservative therapy fails, a TLA is still a valuable adjunct in the treatment of RS, and cleft speech pathologists who treat such patients should be more aware of this phenomenon in order to improve long-term articulation outcomes.     

Predictors of speech outcomes in children with Pierre Robin sequence

Backgound

Pierre Robin sequence (PRS) has worse speech outcomes than isolated cleft palate. We aimed to search for possible associations of phonological outcomes with PRS status (isolated vs syndromic), clinical severity, soft palate muscles deficiency, or surgical procedure.

The effect of mandibular distraction osteogenesis on airway obstruction and polysomnographic parameters in children with Robin sequence

Introduction

The optimal surgical technique for the management of patients with Robin Sequence (RS) has not been established. One of the most commonly used surgical techniques, mandibular distraction osteogenesis (MDO), is still controversial because of its potential risks and the lack of clear evidence of its efficacy.

Effects of maxillary distraction osteogenesis on the upper-airway size and nasal resistance in subjects with cleft lip and palate

OBJECTIVES: To investigate the short- and long-term effects of maxillary distraction osteogenesis (DOG) on the upper-airway size and nasal resistance in nine patients with cleft lip and palate (CLP). STUDY DESIGN: Changes in the upper-airway size were measured by using lateral cephalometric radiographs taken immediately before and after DOG, and 1 year later. Nasal resistance was measured with a rhinomanometer. An analysis of variance was used to establish statistical significance. Spearman correlation coefficient was used to evaluate the relationship between changes in the cross-sectional area of the upper airway and nasal resistance in association with DOG. RESULTS: Immediately after DOG, the anteroposterior dimension of the superior part of the upper airway was significantly increased (p < 0.01) and nasal resistance was significantly decreased (p < 0.05). Moreover, the cross-sectional area of the total upper airway was significantly increased (p < 0.01). There was a significant correlation between the increase in the upper-airway cross-sectional area and the reduction in nasal resistance (p < 0.05). The upper-airway size was significantly augmented (p < 0.05) and nasal resistance was significantly reduced (p < 0.05) at 1 year after DOG compared with immediately before DOG. CONCLUSION: An increase in the upper-airway size and a reduction in nasal resistance occurred after maxillary DOG in patients with CLP, and these changes were stable after 1 year.     

青少年单侧不完全性唇腭裂植骨术后上颌骨及上气道的CBCT研究

目的 评估青少年单侧不完全性唇腭裂患者术后上颌骨及上气道的三维形态结构特征,并探索二者之间的相关性.方法 选取8～12岁青少年单侧不完全性唇腭裂术后患者30例及与年龄、性别相匹配的非唇聘裂骨性Ⅰ类错畸形患者30例,拍摄CBCT影像并对其上颌骨及上气道进行三维结构参数的测量分析,分析唇腭裂组上颌骨及上气道三维结构参数的...     

Effects of orthognathic surgery on pharyngeal airway and respiratory function during sleep in patients with mandibular prognathism

The aim of this study was to determine changes in overnight respiratory function and craniofacial and pharyngeal airway morphology following orthognathic surgery. The subjects were 40 patients in whom mandibular prognathism was corrected by orthognathic surgery: a one-jaw operation in 22 patients and a two-jaw operation in 18 patients. Morphological changes were studied using cone beam computed tomography immediately before surgery and at more than 6 months after surgery, and the apnoea–hypopnoea index (AHI) was measured with a portable polysomnography system. Pharyngeal airway volume was decreased significantly after surgery, especially in the one-jaw operation group. AHI was not changed significantly after surgery in either group, although AHI in one patient in the one-jaw operation group was increased to 19 events/h. There was no significant change in pharyngeal airway morphology in that patient, but he was obesity class 1 and was 54 years old. In conclusion, some patients who are obese, have a large amount of mandibular setback, and/or are of relatively advanced age may develop sleep-disordered breathing after mandibular setback; a two-jaw operation should therefore be considered in skeletal class III patients who have such risks because it decreases the amount of pharyngeal airway space reduction caused by mandibular setback surgery.