Airway management in Pierre Robin sequence

doi:10.1016/S0194-5998(98)70232-3

Otolaryngology - Head and Neck Surgery

Volume 118, Issue 5, May 1998, Pages 630-635

Presented at the Annual Meeting of the American Academy of Otolaryngology–Head and Neck Surgery, Washington, D.C., Sept. 29–Oct. 2, 1996.

https://doi.org/10.1016/S0194-5998(98)70232-3 Get rights and content

Abstract

There is a great deal of controversy regarding the long-term management of airway obstruction in children with Pierre Robin sequence. All 23 pediatric otolaryngology fellowship programs were surveyed to determine their current practice patterns. Issues that were addressed included methods of evaluation of airway obstruction, including polysomnography, choices for home care in children in whom observation and positioning treatment failed, and complications of the various treatment modalities. The lack of unanimity among the respondents mirrors the controversy found in the literature. Though tracheotomy appears to be favored as the method of long-term management, responses from those surveyed often were based on emotions as much as on scientific data. This report details those findings and offers suggestions to clinicians involved with the management of patients with Pierre Robin sequence with airway compromise. (Otolaryngol Head Neck Surg 1998;118:630-5.)

Section snippets

METHODS AND MATERIAL

In December 1994, a survey of pediatric otolaryngology fellowship training programs in the United States and Canada was conducted by telephone interviews and a data collection form to determine how the airway is managed in children with PRS. One form was sent to each institution and a consensus was obtained from the practitioners at each center. Twenty-three surveys were distributed with 100% response.

Nine questions were used to clarify the general guidelines used in the treatment of patients

RESULTS

All 23 fellowship training programs are involved in the airway management of children with PRS. As can be seen from the graph in Fig. 1, most services evaluate between 6 and 10 patients per year with this condition.

. Number of patients treated per year per program.

When asked whether sleep studies were indicated for most patients with PRS, only 9 (39%) of 23 believed this to be the case. Of those nine who obtained sleep studies routinely, one performed polysomnography on all children before

DISCUSSION

A survey was conducted to evaluate the current management techniques used by pediatric otolaryngologists in the treatment of patients with PRS with airway obstruction. These results are presented.

Before we discuss the relative advantages and disadvantages of the different means of airway management in PRS, it is important to understand the pathophysiologic background of airway obstruction in these children. Several potential physiologic factors in airway obstruction have been described,

References (14)

A Augarten et al.
Management of upper airway obstruction in the Pierre Robin syndrome
Br J Oral Maxillofac Surg
(1990)
TS Olson et al.
Early home management of patients with Pierre Robin sequence
Int J Pediatr Otorhinolaryngol
(1990)
B Benjamin et al.
Management of airway obstruction in the Pierre Robin sequence
Int J Pediatr Otorhinolaryngol
(1991)
U Frohberg et al.
Surgical treatment of Robin sequence and sleep apnea syndrome: case report and review of the literature
J Oral Maxillofac Surg
(1993)
SM Tomaski et al.
Airway obstruction in the Pierre Robin sequence
Laryngoscope
(1995)
RJ Shprintzen et al.
Upper airway obstruction and the Robin sequence
Anesthesiol Clin
(1992)
AE. Sher
Mechanisms of airway obstruction in Robin sequence: implications for treatment
Cleft Palate Craniofac J
(1992)

There are more references available in the full text version of this article.

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Sleep architecture in Pierre-Robin sequence: The effect of mandibular distraction osteogenesis
2016, International Journal of Pediatric Otorhinolaryngology
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Conservative approaches to PRS patients with upper airway obstruction include prone positioning, a nasopharyngeal airway (NPA), and continuous positive airway pressure (CPAP). There is mixed literature regarding efficacy and tolerability of NPA and CPAP for long-term management of infant upper airway obstruction [9,10]. Those patients with severe obstructive symptoms are less likely to tolerate long-term conservative measures, and are appropriate surgical candidates [9].
Pierre-Robin Sequence (PRS), a triad of micro/retrognathia, glossoptosis, and upper airway obstruction, usually in conjunction with a cleft palate is frequently associated with significant morbidity. Mandibular distraction osteogenesis (MDO) is an effective treatment modality to address retroglossal upper airway obstruction by increasing the anterior-posterior diameter of the infant airway. Although MDO has been shown to improve the apnea-hypopnea index (AHI) in children with PRS, the consequences of MDO on other aspects of infant sleep, including hypercapnea, hypoxia, the REM to Non-REM ratio, as well as its effect on central and mixed apneas has not been investigated with an adequate sample size.
To characterize the effect of MDO on key components of sleep architecture in infants with PRS.
Charts from 32 infants with PRS that were addressed with MDO at our tertiary-care children's hospital were retrospectively reviewed. Of these, 26 infants (57.7% male; mean age = 4.1 weeks, SD = 5.0) had pre- and post-operative polysomnograms (PSG). Paired samples t-tests were used to compare pre- and post- MDO sleep architecture mean score differences.
Among the 26 infants, 73.1% demonstrated severe pre-MDO sleep apnea (AHI > 10). Several aspects of sleep architecture were found to improve post-operatively. Significant improvements were found in AHI (30.3 vs. 8.7; t = 4.1, p < 0.001), obstructive apneas (79.3 vs. 5.8; t = 4.0, p < 0.001), hypopneas (48.1 vs. 22.1; t = 2.2, p = 0.040), time spent below 90% SpO2 (3.9% vs. 0.7%; t = 3.3, p = 0.003), and lowest SpO2 nadir (75.4% vs. 82.9%; 3.4, p = 0.002). In addition, a marginally significant improvement was found for mixed apnea (6.3 vs. 1.6; t = 1.99, p = 0.058).
MDO improves several sleep architecture parameters in this sample of infants with PRS. Statistically significant improvement was seen in obstructive apneas, hypopneas, AHI, obstructive AHI, and several indicators of hypoxia during sleep.
Mandibular distraction osteogenesis for the management of upper airway obstruction in children with micrognathia: A systematic review
2016, International Journal of Oral and Maxillofacial Surgery
Mandibular distraction osteogenesis (MDO) is increasingly used for neonates and infants with upper airway obstruction secondary to micrognathia. This systematic review was conducted to determine the effectiveness of MDO in the treatment of airway obstruction. The databases searched included PubMed, Embase, Scopus, and grey literature sources. The inclusion criteria were applied to identify studies in children with clinical evidence of micrognathia/Pierre Robin sequence (PRS) who had failed conservative treatments, including both syndromic and non-syndromic patients. Overall 66 studies were included in this review. Primary MDO for the relief of upper airway obstruction was found to be successful at preventing tracheostomy in 95% of cases. Syndromic patients were found to have a four times greater odds of failure compared to those with isolated PRS. The most common causes of failure were previously undiagnosed lower airway obstruction, central apnoea, undiagnosed neurological abnormalities, and the presence of additional cardiovascular co-morbidities. MDO was less effective (81% success rate) at facilitating decannulation of tracheostomy-dependent children (P < 0.0001). Failure in these patients was most commonly due to severe preoperative gastro-oesophageal reflux disease, swallowing dysfunction, and tracheostomy-related complications. The failure rate was higher when MDO was performed at an age of ≥24 months. More studies are needed to evaluate the long-term implications of MDO on facial development and long-term complications.
Robin sequence: A European survey on current practice patterns
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Citation Excerpt :
Since the first, original description of RS by Pierre Robin in 1923, both the definition and management of RS have been topics of debate. Previous surveys on this condition carried out among American Pediatric Otolaryngology Fellowship programs, members of the American Cleft Palate–Craniofacial Association, and members of Dutch and Belgian cleft palate teams all found a widespread lack in uniformity (Myer Iii et al. 1998; Breugem and Mink van der Molen 2009; Breugem and Courtemanche 2010; Collins et al. 2013; Basart et al. 2015). This first European survey on both the definition and management confirms these previous findings by revealing a large variety in current practice patterns within European clinics.
To provide an overview of current practice patterns with regard to Robin sequence (RS) patients in Europe, a survey was conducted among European clinicians. This online survey consisted of different sections assessing characteristics of the respondent and clinic, definition, diagnosis, treatment, and follow-up. In total, surveys from 101 different European clinics were included in the analysis, and 56 different RS definitions were returned. The majority (72%) of the respondents used a sleep study system to determine the severity of the airway obstruction. A total of 63% used flexible endoscopy and 16% used rigid endoscopy in the diagnostic process. Treatment of the airway obstruction differed considerably between the different countries. Prone positioning for mild airway obstruction was the treatment modality used most often (63%). When prone positioning was not successful, a nasopharyngeal airway was used (62%). Surgical therapies varied considerably among countries. For severe obstruction, mandibular distraction was performed most frequently. Three-quarters of the respondents noted the presence of catch-up growth in their patient population. This first European survey study on definition and management of RS shows that there are considerable differences within Europe. Therefore, we would encourage the establishment of national (and international) guidelines to optimize RS patient care.
Mandible traction with wires for the treatment of upper airway obstruction caused by Pierre Robin sequence in Chinese infants: Preliminary findings
2014, Journal of Cranio-Maxillofacial Surgery
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.

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^☆: From the Department of Otolaryngology and Maxillofacial Surgery (Drs. Myer, Cotton, Willging, and Shott), Children’s Hospital Medical Center, Cincinnati; and the Ear, Nose and Throat Surgical Group (Dr. Reed), Jackson.

^☆☆: Reprint requests: Charles M. Myer III, MD, Department of Otolaryngology, Children’s Hospital Medical Center, 3333 Burnet Ave., Cincinnati, OH 45229-3039.

^★: 0194-5998/98/$5.00 + 0 23/1/82446

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Airway management in Pierre Robin sequence☆,☆☆,★

Abstract

Section snippets

METHODS AND MATERIAL

RESULTS

DISCUSSION

Br J Oral Maxillofac Surg

Int J Pediatr Otorhinolaryngol

Int J Pediatr Otorhinolaryngol

J Oral Maxillofac Surg

Airway obstruction in the Pierre Robin sequence

Laryngoscope

Upper airway obstruction and the Robin sequence

Anesthesiol Clin

Mechanisms of airway obstruction in Robin sequence: implications for treatment

Cleft Palate Craniofac J