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

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.     

Resolving feeding difficulties with early airway intervention in Pierre Robin Sequence

OBJECTIVES/HYPOTHESIS: To observe rates of gastrostomy tube (g-tube) placement in Pierre Robin Sequence (PRS) and to determine whether relieving airway obstruction solves feeding difficulties. STUDY DESIGN: All PRS referrals to a multidisciplinary cleft team for children at a tertiary pediatric hospital from January 1988 to June 2006 were retrospectively reviewed. METHODS: Patients were analyzed for occurrence of g-tube placement, neurologic disorders, and airway intervention including tracheotomy and mandibular distraction osteogenesis. RESULTS: Sixty-seven PRS patients were divided into two categories: 51 (76.1%) isolated PRS (iPRS) and 16 (23.9%) with additional disorders and syndromes (sPRS). Patients were then placed into two subgroups: those who received early airway intervention and those who received late or no airway intervention. Of the 51 iPRS children, 12 (23.5%) received early airway intervention, none of whom required a g-tube. There were 39 (76.5%) children who received late or no airway intervention, and 5 (12.8%) of these required g-tube placement. Of the 16 sPRS children, 8 (50%) received early airway intervention, and 7 (87.5%) of these still required a g-tube. Of the remaining 8 (50%) sPRS patients who received late or no airway intervention, 5 (62.5%) required a g-tube. CONCLUSION: In children with iPRS, feeding difficulties can be resolved with early airway intervention. Delaying airway intervention may necessitate feeding assistance because all of the iPRS children who required a g-tube fell into this category. The presence of additional disorders and syndromes further complicates treatment because most of the sPRS children required g-tubes regardless of airway intervention.     

Oropharyngeal teratomas in newborns: Management and outcome

ObjectivesCongenital teratomas of the oropharyngeal cavity are extremely rare and are associated with a high neonatal mortality rate due to severe airway obstruction. Management has been improved with progress in antenatal diagnosis. The authors describe this progress in the light of a series of 4 cases and a review of the literature.MethodsThe medical charts of four neonates treated in the department since 1995 were reviewed. The following criteria were studied: age at diagnosis, clinical and radiological features of the tumour, management at birth and outcome.ResultsAll four cases occurred in female neonates with an antenatal diagnosis in two cases, allowing preparation for endoscopy in the delivery room in one case and an EXIT procedure in the other case. Three neonates had to be intubated in the delivery room. Imaging showed invasion of the infratemporal fossa in 3 of the 4 cases. Surgical resection via various approaches to the infratemporal fossa was complete in every case. Adjuvant chemotherapy was administered in one case.ConclusionSurgery for these mostly benign tumours is very challenging and requires a multidisciplinary team. Perinatal planning allows appropriate management at birth, decreasing the risk of airway obstruction. Surgery is the mainstay of treatment of teratomas.     

Management of an infant with prenatal ultrasound diagnosis of upper airway obstruction

We report a case in which a cervical teratoma was diagnosed antenatally in the mid third trimester. In anticipation of potential upper airway obstruction, resources were mobilized to the operating room at the time of the planned cesarean section. The neonate was unable to breathe, but his airway was secured without delay. There was no evidence of cerebral anoxia initially or at one year follow-up. As prenatal diagnosis by ultrasound becomes more refined, the otolaryngologist will play an increasing role in perinatal decision making and anticipated emergencies at the time of delivery. Airway obstruction of various causes will be the most urgent problem.     

Primary aerodigestive presentations of Pierre Robin sequence/complex and predictive factors of airway type and management

Objective

To document the mode and age of primary aerodigestive presentation of Pierre Robin sequence/complex (PRS/C) children to the otolaryngologist and to explore predictive factors of upper airway type and management.

Methods

This is a retrospective cohort study conducted in a tertiary pediatric referral center. A prospective surgical database was searched for children who were diagnosed with PRS/C. Demographics, presenting complaint, secondary diagnoses, type of upper airway obstruction, secondary airway lesions, presence of cleft palate, and airway interventions were collected. Multiple linear regression analysis was performed to predict upper airway obstruction type and intervention.

Results

Seventy-seven potentially eligible patients were identified. Forty-six were included (20 females). Mean age at presentation was 20.4 ± 36.9 months (range 1–191.25 months). Twenty-three primarily presented with respiratory failure, 14 with sleep disordered breathing, and nine with swallowing dysfunction. Children with presentations other than respiratory failure were older (p = 0.004). Nineteen were syndromic. Overt cleft palate was more common in those presenting with respiratory failure (p = 0.01). The type of airway obstruction encountered and use of tracheostomy were positively predicted by the primary presenting feature of respiratory failure (p < 0.05) and male gender (p < 0.05).

Conclusion

A substantial number of PRS/C patients present later than the neonatal period with presentations other than respiratory failure. Both male gender and presentation with respiratory failure predicted a more severe airway obstruction type and the need for trachesotomy.     

Early resection and reconstruction of head and neck masses in infants with upper airway obstruction

Objective

Congenital airway obstruction can be caused by tumors or lesions arising from the neck, tongue and oral cavity. Neonates may require prolonged intubation or tracheostomy before curative resection and reconstruction. The aim of the study was to carry out surgical resection and reconstruction with locoregional flap and free bone graft in the neonatal and early infantile period for definitive management of head and neck masses and treatment of potential airway obstruction.

Methods

Newborns with obstructive head and neck masses in Queen Mary Hospital, University of Hong Kong Medical Centre between 2006 and 2009 were operated on in the neonatal period.

Results

There were one obstructive neck teratoma, two intraoral teratomas and one neuroglial heterotopia. All tumors were resected within the first 3 months of life without major complication. A local cervical cutaneous flap was first used to reconstruct the lateral pharyngeal wall defect in a neonate with a huge neck teratoma, followed by another infant with a neuroglial heterotopia. A piece of cranial bone was used for reconstruction of the skull base defect. None of them required tracheostomy or prolonged intubation. Oral feeding was resumed in the early postoperative period.

Conclusion

Surgical resection and reconstruction with locoregional flap and bone graft can be performed safely in neonatal and early infantile period as management of head and neck masses and treatment of upper airway obstruction.     

Pierre Robin sequence: An institutional experience in the multidisciplinary management of airway, feeding and serous otitis media challenges

Objectives

To evaluate the course and prognosis of airway obstruction, feeding difficulties and hearing abnormalities in patients with Pierre Robin sequence (PRS).

Methods

A retrospective review was conducted, of 69 patients with PRS, attending between 1991 and 2010 at the Children's University Hospital in Dublin. Data regarding airway management, nutritional status and hearing difficulties was collected prospectively.

Results

Airway obstruction requiring intervention other than positional therapy was seen in 39% (27) patients. Fifty nine percent (16/27) of these patients, who failed positional therapy, were successfully managed with a nasopharyngeal airway. Following failed intervention with nasopharyngeal airways, two patients had airway maintenance achieved with a successful glossopexy procedure. One patient had an adequate airway achieved with nasal continuous positive airway pressure. Eight patients (12%) required a surgical tracheostomy. Of those who required a tracheostomy, six patients had isolated PRS, one patient had PRS in association with Stickler syndrome and one patient had Nager acro-facial dystosis. Duration of tracheostomy tube ranged from 10 to 19 months, mean 13 months.Seventy percent (48 patients) required supplementary feeding in the form of nasogastric (NG) or gastrostomy tube. Forty-four patients were successfully managed with a temporary NG tube. One patient required a prolonged NG tube, and three required a gastrostomy tube. Twenty-one (30%) patients were successfully managed with a specialised Haberman bottle. Twenty-four patients (35%) who had their airway managed successfully by positional therapy, still required supplemental feeding.Thirty-one patients (45%) demonstrated a conductive hearing loss at some stage, which affected their speech and language development. Twenty-four patients (35%) required tympanostomy tube insertion once, while 7 (10%) of patients required ventilation tube insertion twice or more.

Conclusion

Airway management in the majority of PRS can be successfully achieved by conservative methods. Even in the presence of an adequate airway, many patients will require supplemental feeding. Early audiological assessment is necessary as many patients will need tympanostomy tube placement to ensure adequate speech and language development.     

Subglottic stenosis: another challenge for intubation and potential mechanism of airway obstruction in Pierre Robin Sequence

Objective

To determine the endotracheal tube (ETT) size and presence of subglottic narrowing in children less than 1 year old with Pierre Robin Sequence (PRS).

Materials and methods

We performed a retrospective review from 2005 to 2009 of infants with PRS who underwent diagnostic laryngoscopy (DL) and intubation.

Results

Fifteen children with a median age of 25 days were reviewed. All patients, except one, were born full term. Subglottic narrowing was visualized in 5 patients (33%). 73% of all patients required an ETT size smaller than what is recommended by intubation guidelines in the literature in regards to normative data based on age and weight.

Conclusion

Infants with PRS may have a higher incidence of subglottic stenosis and require a smaller ETT compared to the normal population. This pilot study warrants a larger prospective investigation to validate these findings.     

Sensation of nasal airflow compared with nasal airway resistance in patients with rhinitis

Over the past few decades there has been some controversy over the relationship between subjective assessment and objective measurement of nasal airway obstruction. To study the hypothesis that there is a close relationship between the two parameters, we analysed changes in nasal patency following histamine challenge. One hundred and two subjects with a history of allergic or non-allergic rhinitis assessed their nasal patency on a visual analogue scale during nasal histamine provocation. Active anterior rhinomanometry was performed immediately after each patient assessment. At all points, significant correlations were observed between subjective and objective assessments of nasal obstruction. Regression analysis also provided strong evidence of a close relationship between the two parameters. We conclude that rhinomanometry can be used as an objective tool in determining nasal patency.     

Distraction osteogenesis of the mandible for airway obstruction in children

OBJECTIVES: Objectives of the study were to determine the effectiveness of distraction osteogenesis of the mandible to relieve airway obstruction in children with tongue-base airway obstruction and to describe the new surgical techniques developed for use in infants and young children. STUDY DESIGN: Prospective, nonrandomized study. METHODS: Analysis of 11 children with severe airway obstruction secondary to tongue-base obstruction was performed. Patients were between 2 weeks and 5.5 years of age. All patients underwent distraction osteogenesis of the mandible. RESULTS: Twelve distraction procedures in the 11 children in the study were accomplished; decannulation or extubation was successful in all children. CONCLUSION: Distraction osteogenesis of the mandible can be used to treat tongue-base airway obstruction in appropriately selected children.     

Determining the site of airway obstruction in obstructive sleep apnea with airway pressure measurements during sleep

OBJECTIVE: To determine the site(s) of upper airway obstruction in patients with obstructive sleep apnea syndrome (OSAS) and their changes during sleep with upper airway (UA) pressure measurement; and to analyze the correspondence between the UA pressure measurement and polysomnography (PSG). METHOD: Thirty patients with OSAS underwent UA pressure measurement. The catheter with five solid-state ultraminiature sensors was inserted through the patients' upper airway to the esophagus. The sensors were located at the nasopharynx, oropharynx, tongue base, hypopharynx, and esophagus. The lower limit of UA obstruction was determined by relying on the observed pressure pattern. RESULT: 1) During inspiration, obstruction occurred associated with an increased negative inspiratory pressure inferior to the site of obstruction and a disappeared negative inspiratory pressure above the site of obstruction; 2) three patterns of obstruction were observed; 3) the site of obstruction was located at the site of the palate; 4) velopharyngeal obstruction and tongue-pharynx obstruction were all present; 5) nasopharyngeal obstruction velopharyngeal obstruction and tongue-pharynx obstruction were all present; and 6) peak inspiratory pressure differences between the normal breath and apnea were associated with the longest apnea interval (P <.05). CONCLUSION: UA pressure measurement can evaluate the site of obstruction in patients with OSAS and their changes during sleep. Peak inspiratory pressure differences between normal breath and apnea can show the severity of OSAS.