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Early postnatal tracheostomy for airway compromise is associated with high morbidity and cost. In certain patients with tongue-base airway obstruction (TBAO), mandibular distraction osteogenesis may be preferred. We present a comprehensive analysis of surgical, airway, and cephalometric outcomes in a large series of neonatal patients with TBAO. A r...
Citations
... Tracheostomy avoidance was comparable with that of other studies with a similar syndromic sample proportion. [17][18][19] The patients in this study experienced significant improvements in AHI and oxygen nadir on polysomnography, representing a successful relief of tongue-based airway obstruction. 20,21 Feeding outcomes in this study are less clear. ...
... 20,21 Feeding outcomes in this study are less clear. Achievement of full oral feeds was considerably lower than similar studies 18,22 and literature reviews. 10,19 While most subjects were able to be discharged without the need for enteral feeding devices, nearly one third of patients were discharged with a GT. ...
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.
... Children with isolated RS had superior weight gain than children with syndromic RS following surgery. Goldstein (2015) reported on 20 patients (74%) who transitioned from nasogastric or gastrostomy tubes to complete oral feeding after 4 or 11 months postdistraction, respectively. 8 Three of the ones that did not improve in feeding, had a failed MDO with respect to breathing. ...
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.
... 3,4 With MDO, the forward movement of the mandible and subsequent repositioning of the tongue and pharyngeal muscles help relieve physical obstruction at the level of the tongue base. [5][6][7] There is currently a paucity of data on how the magnitude of mandibular advancement influences outcomes. We hypothesize that the amount of relief, quantified by changes in apnea-hypopnea index (AHI) scores, is related to the degree of distraction-a crucial variable often dictated by surgeon preference. ...
Purpose
Upper airway obstruction seen in Robin Sequence (RS) is commonly treated with mandibular distraction osteogenesis (MDO). The purpose of this study is to evaluate the impact of distraction distance on sleep study outcomes in patients with obstructive sleep apnea (OSA) secondary to RS.
Materials and Methods
A retrospective cohort study was conducted for patients with isolated RS who underwent MDO at Children's Hospital Los Angeles between January 2006-September 2021. The predictor variable was distraction distance (maximal distraction using a 30 mm device versus sub-maximal distraction), and the primary outcome variable was OSA scores. Relationships between covariates, including demographics, preoperative sleep variables, and postoperative OSA outcomes using polysomnography, were also analyzed. Descriptive statistics and tests of statistical significance were performed using SPSS (version 28.0), including Student’s t-test, proportions testing, multiple linear regression, and correlation analysis.
Results
Seventy-one patients met inclusion criteria (39.4% female, 60.6% male). Average age at MDO was 3.0 ± 10.2 months. Fifty-six patients were distracted maximally with a 30 mm distractor, while the remaining 15 patients experienced shorter distraction due to distractor limitations (25 mm distractor), persistent infection or family request. Looking at absolute values of postoperative sleep study variables, there were no significant differences between patients who were maximally and sub-maximally distracted across AHI, highest carbon dioxide, lowest oxygen saturation, and oxygen requirement. However, both cohorts demonstrated significant improvements in lowest oxygen saturation, AHI, highest carbon dioxide level, and highest oxygen requirement compared to their pre-distraction levels. Compared with patients distracted <30 mm, maximal distraction had a significantly greater improvement in apnea-hypopnea index (AHI) when controlling for preoperative sleep study variables (p=0.047).
Conclusion
Patients with isolated RS who have more severe OSA experienced greater improvements in AHI, oxygen requirement, and oxygen saturation after MDO. Two-thirds of patients no longer had oxygen requirements after MDO. Our results suggest that MDO is helpful in treating patients with RS regardless of distraction distance. However, our study provides evidence that increasing the distraction distance may further improve AHI, which is particularly beneficial to patients with a significant preoperative AHI.
... Across all 19 studies, 729 patients with RS were enrolled, among whom FND was documented in 52 patients. (Frawley et al., 2013;Goldstein et al., 2015;Lam et al., 2014;Lin et al., 2007;Mahrous Mohamed et al., 2011;Murage et al., 2014;Zellner et al., 2017) (Martín-Masot et al., 2018); and 2 studies documented FND without noting the duration of palsy (Allam et al., 2011;Mao et al., 2019). Ten of 19 publications (47.4%) documented marginal mandibular branch (MMB) dysfunction (Allam et al., 2011;Frawley et al., 2013;Konaşet al., 2016;Lin et al., 2007;Mahrous Mohamed et al., 2011;Scott et al., 2011;Senders et al., 2010;Steinberg et al., 2016;Tibesar et al., 2010;Zellner et al., 2017), and 9 studies did not document which branch of the facial nerve was injured. ...
... Eight studies used internal devices (Allam et al., 2011;Frawley et al., 2013;Goldstein et al., 2015;Hammoudeh et al., 2012;Mahrous Mohamed et al., 2011;Murage et al., 2014;Steinberg et al., 2016), 3 used external devices (Scott et al., 2011;Senders et al., 2010;Tibesar et al., 2010), and 2 studies used both types of device (Lin et al., 2007;Martín-Masot et al., 2018); 6 studies did not document the type of device (Hu et al., 2001;Lam et al., 2014;Mao et al., 2019;Murage et al., 2014;Zellner et al., 2017). Distraction rate ranged from 1.00 mm/day to 2.00 mm/day (median: 1.00 mm/day, mean 1.37 mm/day) and the total distraction length ranged from 13.00 mm to 22.3 mm (median: 18.1 mm, mean 18.5 mm). ...
... al., 2013;Goldstein et al., 2015;Hammoudeh et al., 2012;Hu et al., 2001;Lam et al., 2014; Mahrous Mohamed et al., 2011;Mao et al., 2019;Murage et al., 2014), 5 were cohort retrospective(Konaşet al., 2016;Scott et al., 2011;Senders et al., 2010;Steinberg et al., 2016;Tibesar et al., 2010), 2 were case series(Lin et al., 2007;Mudd et al., 2012), and the remaining 4 studies were case control(Tahiri et al., 2015), retrospective case control(Zellner et al., 2017), observational descriptive(Martín-Masot et al., 2018), and combined retrospective and prospective(Allam et al., 2011). ...
Objective
Robin Sequence (RS), characterized by micrognathia, glossoptosis, and upper airway obstruction, is an increasingly recognized diagnosis. An effective surgical intervention is mandibular distraction osteogenesis (MDO). This study analyzes published evidence regarding facial nerve dysfunction (FND) associated with MDO.
Design and Setting
According to PRISMA guidelines, a systematic review was carried out with databases queried in June 2019 using MESH terms, or equivalent terms, as follows: “distraction osteogenesis” and “Robin Sequence”. A review of original Spanish and English articles, were included. Outcome measures included the prevalence of FND; the affected branches; the rate of permanent vs. transient FND; the use of an internal vs. external device; the daily distraction rate; and finally, the overall distraction length. Subsequently, a meta-analysis was conducted to collate results regarding the prevalence of FND and the factors associated with it.
Results
Of 239 unique studies identified, 19 studies with 729 patients met inclusion criteria; 52 patients developed FND after MDO. A random-effects meta-analysis yielded a pooled prevalence of FND of 6.40%, with moderately heterogeneous studies (I2 = 41%, τ2 = 0.006). Marginal mandibular nerve involvement was most commonly noted. Nine studies reported transient FND, six permanent, one both, and two unspecified. Internal distractors were used in 8 studies and external in 3 and both in 2. Distraction rate was 1.00 to 2.00 mm/day and total distraction length ranged from 13.00 to 22.3 mm. Sample size was the only parameter inversely associated with rate of FND (p = 0.04).
Conclusion
This analysis of FND associated with MDO for patients with RS demonstrates a lack of consistent documentation. MDO-associated FND does not appear to be uncommon, and permanent dysfunction can occur. This review underscores the importance of thorough documentation to elucidate the mechanism of FND.
... This phase proceeds until an effective improvement of the respiratory distress is obtained, even if many authors describe the positive effect of a slight overcorrection in the clinical practice. [41][42][43][44][45][46] Only after a consolidation period can the devices be removed. ...
After a failed mandibular osteodistraction, the wrong positioned mandible of a few patients with Pierre Robin sequence returned in the most functional position and regained a proper symmetry, without external intervention. The study aims to explain this self-adjustment and introduce the floating bone phenomenon.
The inclusion criteria were severe micrognathia, Fast and Early Mandibular Distraction Osteogenesis protocol, postoperative mandibular wrong positioning, presurgery, immediate postsurgery, and long-term computed tomography scan. Five patients were included. The considered parameters were the distance between mandibular dental centerline and midsagittal facial axis, the rotation of the mandibular body, the magnitude of elongation, and the lowering of the mandibular body.
Three patients went from a decentralization >4 mm in the activation phase to a normalization of the said value in the follow-up. In the same period, the interincisal point of 2 patients moved respectively from 0.5 mm on the left and 0.8 mm on the right to 1.2 mm and 1.6 mm on the right, respectively. The rotation of the mandibular body was meanly 25.6° among all patients. The mean value of the distraction was 14.1 mm. A difference of about 4.4 mm between the left and the right side was measured. The lowering of the mandible varied between 2.8 and 12.6 mm.
All patients improved their symmetry. Four of them improved in all the measured parameters, while 1 patient presented a worsening in the decentralization of the interincisal point.
The floating bone phenomenon could break new grounds in the management of patients with Pierre Robin sequence.
... In that study, the mean age of low-weight patients was 23 days, whereas the mean age of controls was 2.7 years. Multiple other studies have reported clinical success with neonatal distraction; however, their results were not directly compared to those of older infants (Goldstein et al., 2015;Scott et al., 2011). Although early intervention appears to be safe and beneficial when performed at experienced centers, it is important to realize that this approach has not been universally adopted. ...
Objective:
The purpose of this study was to determine whether performing mandibular distraction osteogenesis (MDO) during the neonatal period increased inpatient complications as measured through health-care burden.
Materials and methods:
This was a retrospective cohort study of the Kids' Inpatient Database from 2000 to 2011. Infants receiving MDO prior to 12 months of age were included. The primary study predictor was distraction age, classified as either neonatal or non-neonatal. Secondary predictors were patient demographics, hospitalization characteristics, diagnoses, and procedures. The outcomes were the number of procedures performed, postoperative length of stay (pLOS), hospital charges, and the discharge transfer rate. Outcomes were compared between the primary predictors using χ2 and independent 2-sample t tests. Multiple linear and logistic regression models were created using clinically relevant predictors to assess the independent effect of neonatal age on each outcome.
Results:
The study sample contained 102 patients, of who 50 (49.0%) were distracted in the neonatal period. Neonatal MDO patients were more likely to have a cleft palate (86.0% vs 55.8%; P < .001) and present with feeding difficulties (38.0% vs 19.2%; P = .036) that were treated through total parenteral nutrition (26.0% vs 9.6%; P = .030) but otherwise did not have significantly different characteristics compared to non-neonatal patients. The multiple regression models confirmed that neonatal age did not influence any of the study outcomes, although other secondary predictors were found to influence the pLOS, hospital charges, and number of inpatient procedures.
Conclusions:
Neonatal MDO was not associated with increased complications. At experienced centers, neonatal status should not be considered a contraindication to treatment.
... Mandibular distraction osteogenesis (MDO) has become the first-line operation in many centers for infants with airway obstruction associated with (Pierre) Robin sequence (RS) who have failed non-invasive management 1 . This procedure boasts high rates for successful resolution of obstructive sleep apnea (OSA) and avoid-ance of tracheostomy [2][3][4][5][6][7][8][9][10][11][12] . Long-term complications associated with the position of the osteotomies, such as injury to developing teeth and the inferior alveolar nerve, however, are common 1,[13][14][15] . ...
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±96days were included. Maxillofacial computed tomography scans were obtained 15±7days 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±15.6days 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.
... It is particularly useful for mandible elongations, which are being applied nowadays with younger patients, and even to neonates for treating micrognathia with airway obstruction because it is apparently very safe. 7,8 It is also a very important reconstructive tool exhibiting good and stable results for treating maxillary hypoplasia in patients with cleft lip and palate. 2,9 One disadvantage usually highlighted is the need for two operating procedures, as devices should be removed at the end of the treatment. ...
Maxillary segmental defects are a reconstructive challenge particularly when the anterior arch is involved. Missing bone, mucosa, and teeth should be replaced; fistulae closure should be guaranteed by bone and mucosa continuity; stable functional occlusion should be achieved; and facial aesthetics restored. These defects resulting from tumor excisions, if left untreated, will cause additional problems related to interposed scar and collapse of lip and nose. Immediate reconstruction should avoid these problems and should be considered when safe tumor-free margins are obtained. This study describes the treatment of such a defect resulting from a myxoma excision, with trifocal distraction requiring only one additional surgery. Internal distractors were fixed bilaterally for transport of two segmental discs created by inverted L osteotomies at the same surgical time of tumor excision. A second surgery was performed after central contact between transport discs was achieved, for device removal and creation of a dynamic system to regenerate molding and compression-focus development. This was accomplished by internal fixation of one side and central fixation of a full-open distractor to act as compression device when reversely activated. Central perfect adaptation between transported segments and good-quality attached mucosa lining the alveolar crest were determinant to obtain a stable functional and aesthetic result.
... 25 at present, MDo is the only accepted treatment modality that directly addresses micrognathia in infants. 26 a recent systematic review summarized evidence from 66 studies on MDo in the treatment of airway obstruction in children with micrognathia. 25 They found that primary MDo for the relief of upper airway obstruction was successful in preventing tracheostomy in 95% of cases. ...
Obstructive sleep apnea in infants is a distinct entity that needs to be considered separately to older children. This review article summarizes risk factors associated with infant obstructive sleep apnea, clinical presentation, evaluation and management options for this condition, which differs somewhat in this younger population.
... MDO ¼ mandibular distraction osteogenesis; TLA ¼ tongue-lip adhesion; SPRFM ¼ subperiosteal release of the floor of the mouth. (Cohen et al., 1998;Morovic and Monasterio, 2000;Denny et al., 2001;Izadi et al., 2003;Chigurupati et al., 2004;Mandell et al., 2004;Monasterio et al., 2004;Sorin et al., 2004;Wittenborn et al., 2004;Burstein and Williams, 2005;Denny and Amm, 2005;Roy and Patel, 2006;Mitsukawa et al., 2007;Burstein, 2008;Dauria and Marsh, 2008;Gifford et al., 2008;Genecov et al., 2009;Lee and Kim, 2009;Looby et al., 2009;Pinheiro Neto et al., 2009;Sadakah et al., 2009;Shen et al., 2009;Franco et al., 2010;Gozu et al., 2010;Miloro, 2010;Cheng et al., 2011;Chowchuen et al., 2011;Farina et al., 2011;Kolstad et al., 2011;Mahrous Mohamed et al., 2011;Olson et al., 2011;Scott et al., 2011;Breugem et al., 2012;Hammoudeh et al., 2012;Hong et al., 2012;Mudd et al., 2012;Pluijmers et al., 2012;Schoemann et al., 2012;Sesenna et al., 2012;Andrews et al., 2013;Handley et al., 2013;Cascone et al., 2014;Rachmiel et al., 2014;van Lieshout et al., 2014;Goldstein et al., 2015;Ching et al., in press), 18 on TLA (Freed et al., 1988;Augarten et al., 1990;Bull et al., 1990;Argamaso, 1992;Gilhooly et al., 1993;Hoffman, 2003;Kirschner et al., 2003;Denny et al., 2004;Schaefer et al., 2004;Huang et al., 2005;Evans et al., 2006;Cozzi et al., 2008;Bijnen et al., 2009;Rogers et al., 2011;Abramowicz et al., 2012;Mann et al., 2012;Sedaghat et al., 2012;Mokal et al., 2014), three on SPRFM (Caouette-Laberge et al., 1996;Breugem et al., 2008;Caouette-Laberge et al., 2012), and three publications reporting results of both TLA and MDO (Al-Samkari et al., 2010;Papoff et al., 2013;Flores et al., 2014). The 70 retained publications originated from 19 countries (one study from each Brazil, China, Egypt, India, Japan, Korea, Mexico, Poland, Saudi Arabia, Taiwan, Thailand, and Turkey; two from Australia and Chile; three from Canada, Israel, Italy, and The Netherlands; and 41 from the United States). ...
... Three studies reported complete resolution of obstructive sleep apnea but did not provide objective data. Only six studies reported sufficiently detailed data to enable calculation of the number of infants with significant preoperative obstruction who had persistent significant obstruction postoperatively (Burstein and Williams, 2005; Cheng et Hammoudeh et al., 2012;Sedaghat et al., 2012;Cascone et al., 2014;Goldstein et al., 2015). Data was obtained from the authors for two additional studies (Farina et al., 2011;Flores et al., 2014). ...
... Pre-and postintervention results are presented in Tables 3 and 4. Published standards for polysomnography were reported to be used by the authors of all publications providing detailed results except one. The most commonly available data was the AHI (which includes central and obstructive apneas), with only two publications reporting the obstructive apnea-hypopnea index (OAHI; Sedaghat et al., 2012;Goldstein et al., 2015). Most results concerned nonsyndromic PRS. ...
Background:
Tongue-lip adhesion (TLA), mandibular distraction osteogenesis (MDO), and subperiosteal release of the floor of the mouth (SPRFM) are the most commonly performed surgical procedures to treat severe airway obstruction in infants born with Pierre Robin sequence (PRS).
Objectives:
To determine the rate of failure of each type of procedure, in terms of mortality and the need for tracheostomy, and to determine what proportion of infants have significant airway obstruction postoperatively as determined by polysomnography (PSG) and compare the data by procedure type.
Method:
A comprehensive literature search (1981 through June 2015) was done of the National Library of Medicine database using PubMed. Extracted data included diagnosis, type of surgery, and outcome including mortality, need for postoperative tracheostomy and details of PSG. Persistence of significant airway obstruction was defined as an apnea-hypopnea index >15 events/hour on PSG.
Results:
Both mortality rate and need for tracheostomy were low for all procedures. Many studies lacked sufficient detail to identify significant airway obstruction postoperatively. In studies with sufficient data, MDO was associated with the lowest percentage of significant airway obstruction postprocedure (3.6%) compared to 50% for infants who underwent TLA. Insufficient PSG data was available for patients who were treated with SPRFM.
Conclusions:
There is a paucity of objective PSG data to definitively assess postoperative airway outcomes for PRS. MDO appears to be the most effective technique based on the available PSG data. Standardized use of PSG may lead to better identification and treatment of patients at risk for suboptimal airway outcomes postoperatively.
