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

Abstract

Introduction

Neonatal upper airway obstruction secondary to micrognathia can be managed with conservative or surgical interventions. Traditionally, severe upper airway obstruction was managed with a tracheostomy. Although tracheostomy may be life saving, it is associated with high rates of complications and can lead to developmental problems. More recently, mandibular distraction osteogenesis has been utilized to relieve micrognathia associated airway obstruction.

Methods

A clinical narrative review of the current literature was performed to evaluate the efficacy of mandibular distraction osteogenesis in neonates with Pierre Robin sequence.

Objectives

(1) To evaluate whether mandibular distraction osteogenesis can relieve the upper airway obstruction in micrognathic neonates and (2) to discuss and increase the awareness of various issues surrounding neonatal mandibular distraction procedures including preoperative workup, distraction protocols, and complications.

Results

Mandibular distraction osteogenesis can be a safe and effective intervention in neonates diagnosed with Pierre Robin sequence with severe micrognathia and airway obstruction. Interestingly, in patients with additional complex syndromes, the airway obstruction was not consistently alleviated.

Conclusion

When conservative measures fail, mandibular distraction osteogenesis should be considered to obviate the need for a tracheostomy in newborns with micrognathia associated upper airway obstruction.

Introduction

Neonatal upper airway obstruction secondary to micrognathia was first widely described by Pierre Robin in 1934 [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). Additional craniofacial syndromes were later recognized to be sometimes associated with PRS. Most notably, they include Treacher Collins syndrome, Nager syndrome and hemifacial microsomia [2].

Micrognathia can potentially cause upper airway obstruction in the neonatal patient due to posterior tongue collapse and physical obstruction of the oropharyngeal and hypopharyngeal regions. Although vast majority of the children born with micrognathia or PRS are either asymptomatic or can be treated with conservative management, some patients may have significant respiratory and/or feeding issues, necessitating more aggressive interventions [3], [4], [5].

Conservative therapy for micrognathia associated airway and feeding problems include various positioning strategies and feeding techniques, as well as placement of nasopharyngeal airways and application of nasal continuous positive airway pressure [4], [5], [6], [7]. As the neonate grows, most patients will demonstrate catch-up mandibular growth and attain adequate strength and coordination, such that airway obstruction no longer becomes a problem. A relatively large case series of children with non-syndromic PRS demonstrated that less than 10% required invasive surgical intervention (distraction and/or tracheostomy) [4], [5], [6]. Yet, not all patients will respond to these non-invasive interventions.

Severe neonatal upper airway obstruction secondary to PRS that fails conservative management demands urgent medical attention. Traditionally, tracheostomy has been the most effective and definitive treatment option for these patients [8]. Tracheostomy, however, is associated with frequent morbidity, high cost, and occasional mortality [9], [10], [11]. As well, decannulation may take several years and there is significant negative psychological impact on the caregivers and family members of a child with tracheostomies [8]. Therefore, other methods of airway management in this patient population are required.

Distraction osteogenesis of the mandible is a relatively new treatment option in children with PRS [12]. The process of gradually lengthening the mandible can lead to correcting the posterior tongue base position, which can relieve the pharyngeal airway obstruction. Initially, reports of mandibular advancement during the first few days of life were limited [13], [14] but many more studies can be found in the recent literature [6], [15], [16], [17], [18], [19], [20]. The objective of this clinical narrative review is to discuss the efficacy of distraction osteogenesis in the neonatal patient. More specifically, the effectiveness in relieving the airway obstruction in neonates with PRS, along with other issues surrounding this surgical technique, such as improvements in feeding and pre- and post-operative investigations will be discussed. In addition, the technical and surgical refinements and future direction for mandibular distraction osteogenesis will be briefly explored.