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.     

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.     

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.     

A comparison of airway interventions and gastrostomy tube placement in infants with Robin sequence

The purpose of this study was to evaluate feeding impairment following non-operative or operative management of airway obstruction in a large series of infants with Robin sequence (RS) by rate of G-tube placement. A retrospective study was conducted at Boston Children’s Hospital including 225 patients (47.1% female) with RS treated between 1976 and 2018. Subjects were grouped by intervention required for successful management of airway obstruction: non-operative only (n = 120), tongue–lip adhesion (TLA, n = 75), mandibular distraction osteogenesis (MDO, n = 21), or tracheostomy (n = 9). The operative group had a higher rate of G-tube placement (58.1%) than the non-operative group (28.3%, P < 0.0001). Subjects in the TLA and tracheostomy groups had higher odds of G-tube placement than subjects in the MDO group: odds ratio (OR) 5.5 (95% confidence interval (CI) 1.8–17.3, P = 0.004) and OR 27.0 (95% CI 3.2–293.4, P = 0.007), respectively. Syndromic patients and those with gastrointestinal anomalies also had higher odds of G-tube placement: OR 3.5 (95% CI 1.7–7.2, P = 0.001) and OR 5.9 (95% CI 1.6–21.0, P = 0.007), respectively. Infants with RS who require an airway operation and those with a syndromic diagnosis or gastrointestinal anomalies are more likely to require placement of a G-tube. Of the operative groups, MDO was associated with the lowest G-tube rate, compared to TLA and tracheostomy.     

A systematic review on the outcome of mandibular distraction osteogenesis in infants suffering Robin sequence

Objectives

Mandibular distraction osteogenesis (MDO) has been successfully applied in infants suffering Robin sequence (RS) with severe upper airway obstruction, but no comparative studies for the different types of MDO exist to date. The objective of the current study was to systematically review the published data considering this matter, providing a fundament for protocols and a more conscious treatment strategy for infants with RS in the near future.

Material and methods

For the period from January 1966 to January 2012, the Pubmed, EMBASE, and Cochrane Library databases were searched. Abstracts were screened based on predetermined selection criteria. Relevant full-text articles were retrieved. The articles were analyzed on the type of MDO used, preoperative workup, patient characteristics, postoperative outcome, and complications.

Results

The search yielded 109 articles. After checking abstracts and full texts on predetermined inclusion and exclusion criteria, 12 studies (four describing external MDO, five internal MDO, and three both types) were extracted for further analyses.

Conclusion

Internal MDO seems very feasible in infants suffering RS, minimizing side effects such as hypertrophic scarring, nerve damage, and extensive care needs, although the indications for usage are more limited compared to the external device. Corresponding protocols and long-term outcome studies are needed to make a better comparison and the use and indication of the different types of distraction even more distinct.

Clinical relevance

A base for a guideline to support the choice of a designated operative management for neonates with RS is provided, hereby obviating possible complications of the different types of MDO in the future.     

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.     

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.

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.     

Mechanisms of airway obstruction in Robin sequence: implications for treatment.

Though the problems associated with Robin sequence may be numerous, especially if the primary cause of the sequence is a multiple anomaly syndrome, the most acute problems in affected newborns is upper airway obstruction. Until recently it has been tacitly assumed that glossoptosis is always the cause of the airway obstruction. More recent evidence has shown that the sources of airway obstruction are multiple and the cause of apnea heterogeneous. The purpose of this paper is to report the mechanisms of upper airway obstruction in 53 infants with Robin sequence. The use of flexible fiber optic endoscopy to specify treatment is discussed in detail.     

Management of infants with Pierre Robin sequence.

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.     

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.     

Mandibular morphology and distraction osteogenesis vectors in patients with Robin sequence

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.     

Glossopexy for upper airway obstruction in Robin sequence.

A modified glossopexy is described with results of the operation in 24 patients who were specifically selected based on nasopharyngoscopic examination of the upper airway. Only patients with documented glossoptosis on endoscopy were selected for glossopexy. The procedure is designed to use two points of attachment for the tongue, one at the mandibular alveolus and the other at the lower lip. The genioglossus is also released to lengthen the tongue. All patients had resolution of their upper airway obstruction. There was only one partial dehiscence. The glossopexies were all released at the time of palate repair, usually before the first birthday.     

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.     

Swallowing disorders in Pierre Robin sequence: its correction by distraction

Pierre Robin sequence is characterized by micro-gnathia, glossoptosis, feeding difficulties, and upper respiratory obstruction, which are frequently complicated by bronchial aspiration and pulmonary infection. Gastroesophageal reflux is also common in these patients. To assess the results of mandibular distraction, a study was performed in 18 patients to detect swallowing disorders associated with apnea episodes and gastroesophageal reflux. Polysomnography, barium pharyngoscopy, determination of blood gases, and esophageal pH measurements were undertaken before and 4 months after distraction osteogenesis. Bilateral corticotomies, followed by distraction with external devices, were performed, achieving 7 to 19 mm of elongation (mean = 12 mm). Gastroesophageal reflux was found in 83% of cases associated with apnea episodes, but it disappeared after distraction osteogenesis. Mean preoperative oxygen saturation was 72%, and it was 93% afterward. The preoperative apnea index was 18.3, and the preoperative 8.5 hypopnea index was 8.5; both disappeared. Pharyngeal transit time became less than 1 second after treatment. Abnormal tongue movements and barium stasis in the pharyngeal recess and in the trachea were eliminated in all the patients.     

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.     

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.