In this section
Pierre Robin Sequence (PRS) occurs in approximately 1 in 6,000 babies in Victoria. It is a congenital condition comprised of a triad of characteristics including micrognathia, glossoptosis, and upper airway obstruction. Upper airway obstruction is a result of micrognathia and glossoptosis. A cleft palate is a common finding in babies with PRS, however a recent international consensus was that micrognathia, glossoptosis and upper airway obstruction should be the defining features in diagnosing PRS, and a cleft palate is not considered a definitive feature. Given that the airway obstruction occurs at the base of the tongue, the main treatment aim is moving the tongue forward to alleviate the obstruction. The use of a nasopharyngeal tube has proven to be a highly effective method in alleviating upper airway obstruction in babies with Pierre Robin Sequence.
The aim of this guideline is to provide medical and nursing staff with a framework for the insertion and management of a modified NPT in neonates with PRS, to relieve upper airway obstruction.
A thorough respiratory assessment should be carried out on patients at commencement of shift, with cares, and when clinically indicated. Patients with PRS may be observed to have breathing movements but not actually have adequate air entry for adequate oxygenation and ventilation. This is why auscultation with a stethoscope is a vital component of respiratory assessment for PRS patients to ensure transmission of breaths.
Symptoms of respiratory compromise may not present immediately, and airway obstruction may be intermittent. Clinical signs may include, stridor, laboured breathing, apnoea, cyanosis, desaturations, bradycardia, irritability, and restlessness. Additional signs and symptoms may include difficulty feeding and poor weight gain. Poor weight gain may be an indication of chronic airway obstruction as the infants energy expenditure is increased secondary to airway obstruction and greater work of breathing.
The optimal efficiency of a nasopharyngeal airway to maintain airway patency is significantly dependent on both internal diameter of the airway and the position of the distal tip. Selection of the size (diameter) of the ETT and length to place it at the nostril is outlined in the table below. This table should be utilized as a guide, and a thorough respiratory assessment should be conducted during insertion to ensure that the NPT is sitting at a length that bypasses the obstructing tongue. An estimate length can also be obtained by measuring from the tip if the nose to the ear tragus, then adding an additional 0.5cm.
The ideal position of an NPT should not extended past the epiglottis, and should sit approximately 1cm above. If the airway is sized too short, it may fail to alleviate obstruction, and if the length is too long it can stimulate and aggravate the cough and gag reflex.
Weight of neonate (kg)
Length of ETT (cm)
Diameter of ETT (mm)
2.5 - 3.5
A member of the medical team or senior member of nursing staff (if approved by medical team) should insert the initial NPT, and nurses may insert subsequent NPTs as required. A lateral neck x-ray should be obtained post insertion to confirm the position of the initial NPT. The position should be adjusted if necessary
according to the x-ray. If respiratory distress persists despite an appropriately positioned NPT, Continuous Positive Airway Pressure (CPAP) or endotracheal intubation should be considered. Please note when administering CPAP, single prong mode must be utilised, and the length should be the same length as the
NPT so that the level of tongue base
obstruction is bypassed.
Following successful insertion of an NPT, this should be documented in EMR as an LDA. Any new NPT insertions should be documented as a new LDA, and previous NPT’s removed.
Any patient with an NPT insitu should also have a laminated bedside card, which clearly states the size, length, and date the NPT was last changed.
Ensure that two health practitioners (one to insert the NPT, the other to provide comfort measures to the neonate) are present for the insertion of the NPT, and that the medical team/AUM is aware that the procedure is occurring. The initial NPT should
only be inserted after a thorough assessment of the neonate by a member of the medical team, and discussion with the consultant.
Prior to insertion, administer sucrose as per RCH nursing clinical guideline (
Sucrose oral for procedural pain management in infants), gather all required equipment, and perform hand hygiene.
1. There are two strands that will be taped to the face, and the thin blue strand will be trimmed and taped to the nose (see picture below). Please ensure the blue strand is trimmed and secured to the patient nose (see bottom picture), and not their forehead.
This helps to minimise movement and stabilise the NPT
2. Apply comfeel to each cheek and the nose to preserve skin integrity, before insertion and taping.
3. Apply lubricant to the tip of the NPT. Insert the tube into the nostril, and once passed through the choana, continue insertion slowly and carefully, until misting is seen in the tube, which will occur on exhalation. Secure the tube by placing the tape directly
over the straps on the cheeks and nose. Apply the tape as close to the nare as possible to prevent mobility of the tube after securing. Make sure that the nares are still pink, and monitor for any signs of pressure areas.
4. If insertion of a NGT is required, insert this into the other nostril, and secure onto the Leukoplast tape with Tegaderm/Opsite.
5. Ensure a lateral neck x-ray (with patient's head in neutral position) is taken after initial NPT insertion. If alteration is required, re-cut a new NPT according to the modified length required from the x-ray. Refer to x-ray below which shows a patient with an NPT in situ. The NPT
is sitting just above the epiglottis and below the posterior tongue, therefore relieving the obstruction.
In the first 10 days post insertion of the NPT, it should be changed every 2-4 days or PRN if secretions are affecting tube patency. More frequent occlusions may occur during this time from the trauma of initial insertion. After this period it should be routinely changed every 5-7 days, with alternating nostrils utilised. If the NPT is required over long-term, size and length may need adjusting according to patient's growth. Further lateral neck x-rays are only required if clinically indicated.
Other indications to change tube:
Ensure spare modified NPT of same size, and one size smaller, with tapes pre-cut are available at the bedside at all times in case of accidental removal.
A thorough respiratory assessment is necessary at shift commencement, and if patient's condition changes. Respiratory assessment should always include auscultation of patient's chest with a stethoscope to assess for air entry with breathing movements.
The presence of stridor indicates obstructed breathing, however absence of stridor does not indicate a patent airway.
Continuous pulse oximetry monitoring must be in place for all infants with an NPT inserted for upper airway obstruction. Cardio-respiratory monitoring is important in the first 10 days post insertion of an NPT, but if obstructive episodes have ceased, continuous pulse oximetry may be sufficient after discussion with medical team. If obstructive episodes are still occurring, or the NPT is removed for a trial period, continue with full cardio-respiratory monitoring in addition to the continuous pulse oximetry.
Continuous close observation for respiratory distress and obstructive episodes is essential, as respiratory monitoring may pick up breathing movements, but cannot measure adequate airway airflow.
Suction should be carried out prn and should never exceed more than 0.5cm beyond the tip of the NPT. Lavage by instillation of normal saline is not supported due to the risk of aspiration. If the NPT is suspected to be blocked, the tube will need to be changed.
The evidence table for Nasopharyngeal Tube (NPT) modified for Pierre Robin Sequence (PRS) can be viewed here.
Please remember to
read the disclaimer.
The development of this nursing guideline was coordinated by Emma Lewis, Clinical Support Nurse, Butterfly, and approved by the Nursing Clinical Effectiveness Committee. Updated August 2020.