Mandibular distraction osteogenesis in children with Pierre Robin sequence: Impact on health-related quality of life

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Abstract

Background

Upper airway obstruction in children with Pierre Robin sequence (PRS) may be severe enough to require surgical intervention. Although many studies have demonstrated the effectiveness of mandibular distraction osteogenesis (MDO) in relieving airway obstruction, no study has reported health-related quality of life (QOL) outcomes.

The objective of the present study was to conduct the first health-related QOL assessment post-MDO.

Methods

The Glasgow Children's Benefit Inventory (GCBI) questionnaire was distributed in a retrospective manner to the caregivers of all children who had undergone MDO at the authors’ institutions between January 2007 and December 2010. Patients who had other major surgical procedures were excluded.

Results

The response rate was 84% (21 of 25 questionnaires completed). The total mean GCBI score was +54 (SD, 19.5), which demonstrated a subjective overall benefit in health-related QOL post-MDO. All of the domains within the GCBI also scored in the positive range, indicating domain specific improvements in health-related QOL. There were no significant differences in the GCBI scores between syndromic-PRS patients and isolated PRS patients; similar results were observed between tracheostomy patients and no tracheostomy patients.

Summary

In treating children with PRS and severe upper airway obstruction, MDO resulted in a subjective overall benefit in health-related QOL in our study population.

Introduction

Upper airway obstruction secondary to micrognathia was first widely described by Pierre Robin [1]. He described a constellation of findings, which included micrognathia, glossoptosis, and in some patients, cleft palate. These findings are now commonly referred to as Pierre Robin sequence (PRS).

Micrognathia can cause upper airway obstruction due to posterior tongue collapse and physical obstruction of the oropharyngeal and hypopharyngeal regions. Although the majority of children born with micrognathia or PRS can be treated with conservative management, some newborns may have significant respiratory and feeding problems, necessitating more aggressive interventions [2], [3], [4]. Traditionally, tracheostomy has been the most effective and definitive treatment for these patients [5]. Tracheostomy, however, is associated with frequent morbidity and occasional mortality [6], [7], [8].

Mandibular distraction osteogenesis (MDO) is a relatively new treatment option in children with PRS, which has been shown to be very effective in relieving the upper airway obstruction by gradually lengthening the mandible [9], [10], [11], [12], [13], [14], [15].

Although MDO has been shown to be an effective and safe alternative to tracheostomy, no study has assessed the health-related quality of life (QOL) outcomes.

Health-related QOL is difficult to precisely define but it is generally agreed to be a multidimensional item, which considers physical, psychological, and social aspects of daily life [16]. Today, health-related QOL is considered by many to be a very important measure in the evaluation of a medical therapy, particularly in surgical interventions where outcomes may affect many aspects of daily life [17].

In this study, the effects of MDO on health-related QOL outcomes are reported in a series of children with PRS and micrognathia associated airway obstruction.

Section snippets

Study methods

All children with PRS and severe upper airway obstruction that underwent MDO at the authors’ institutions from January 2007 to December 2010 were included (N = 25; mean age at surgery of 60 days; 9 girls and 16 boys). Children in whom MDO was performed with other major surgical procedures were excluded from the study; patients who underwent simultaneous minor procedures that were deemed to be of no significance, such as bronchoscopies, were not excluded.

To measure the health-related QOL, the

Patient characteristics

Of the 25 total patients, 21 children with severe upper airway obstruction due to micrognathia and PRS who underwent MDO completed the questionnaire (response rate of 84%). Of the four non-respondents, two were not proficient in English, one refused to participate, and one was lost to follow-up.

Briefly, newborns were considered to have severe airway obstruction if they were intubated at birth and later failed extubation and/or presented with significant oxygen desaturations with signs of

Discussion

Although many articles have demonstrated the effectiveness of MDO in relieving micrognathia associated upper airway obstruction [10], [11], [12], [13], [14], [15], no study has analyzed the health-related QOL outcomes post-MDO. Current research of new medical or surgical interventions should not only include clinical evaluations but the impact on a patient's daily life should also be part of the overall effectiveness assessment. In order to achieve this goal, many studies have utilized

Summary

To the authors’ knowledge, this is the first study to assess the health-related QOL outcomes in patients who underwent MDO, and in our study population, there was a clear subjective overall benefit in health-related QOL post-MDO.

Conflicts of interest

The authors have no financial disclosure or conflicts of interest.

This material has never been published and is not currently under evaluation in any other peer-reviewed publication.

References (22)

  • P. Hong

    A clinical narrative review of mandibular distraction osteogenesis in neonates with Pierre Robin sequence

    Int. J. Pediatr. Otorhinolaryngol.

    (2011)
  • P. Hong et al.

    Feeding and mandibular distraction osteogenesis in children with Pierre Robin sequence: a case series of functional outcomes

    Int. J. Pediatr. Otorhinolaryngol.

    (2012)
  • P. Robin

    Glossoptosis due to atresia and hypotrophy of the mandible

    Am. J. Dis. Child.

    (1934)
  • L. Caouette-Laberge et al.

    The Pierre Robin sequence: review of 125 cases and evolution of treatment modalities

    Plast. Reconstr. Surg.

    (1994)
  • R.B. Schaefer et al.

    Airway management in patients with isolated Pierre Robin sequence during the first year of life

    J. Craniofac. Surg.

    (2003)
  • R.B. Schaefer et al.

    To distract or not to distract: an algorithm for airway management in isolated Pierre Robin sequence

    Plast. Reconstr. Surg.

    (2004)
  • S.M. Tomaski et al.

    Airway obstruction in the Pierre Robin sequence

    Laryngoscope

    (1995)
  • I.T. Singer et al.

    Developmental sequelae of long-term infant tracheostomy

    Dev. Med. Child Neurol.

    (1989)
  • C.W. Lewis et al.

    Tracheotomy in pediatric patients

    Otolaryngol. Head Neck Surg.

    (2003)
  • A. Zeitouni et al.

    Tracheostomy in the first year of life

    J. Otolaryngol.

    (1993)
  • A.T. Ow et al.

    Meta-analysis of mandibular distraction osteogenesis: clinical applications and functional outcomes

    Plast. Reconstr. Surg.

    (2008)
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