141
PROFESSIONAL ARTICLE
Pierre Robin Sequence: treatment with nasopharyngeal tube
Copyright (c) 2022 Slovenian Medical Journal. This work is licensed under a
Creative Commons Attribution-NonCommercial 4.0 International License.
Pierre Robin sequence: treatment with nasopharyngeal 
tube
Pierre Robinova sekvenca: zdravljenje z nosno-žrelnim tubusom
Mojca Železnik,1 Andreja Eberlinc,2 Daša Gluvajić,3 Uroš Krivec,4 Jana Lozar Krivec1
Abstract
Pierre Robin sequence is described as a triad of micro- and/or retrognathia, glossoptosis, and upper airway obstruction, in 
90% the triad is associated with cleft palate. In children with the Pierre Robin sequence, the main functional problems are 
upper respiratory obstruction and feeding problems, which can be manifested by a variety of respiratory problems, and 
poor weight gain. Most patients need conservative treatment, rarely surgical treatment; only the most severe cases will 
need a tracheotomy. In Slovenia, at the Clinical Department of Neonatology, Division of Paediatrics – University Children’s 
Hospital we introduced a new method for the treatment of newborns with the Pierre Robin sequence, treatment with the 
nasopharyngeal tube. The nasopharyngeal tube overcomes the obstruction of the upper respiratory tract at the level of 
the root of the tongue and can be placed for a longer period of time, even in the home environment; we can alternately 
replace the tube from one to another nostril. The treatment of children with the Pierre Robin sequence is multidisciplinary 
and involves a neonatologist, otorhinolaryngologist, maxillofacial surgeon and a pulmonologist, while a gastroenterol-
ogist, clinical dietitian, and geneticist are included if needed. The article presents the clinical picture and the manage-
ment of children with Pierre Robin sequence, and the clinical pathway for the evaluation and treatment of the neonate 
with Pierre Robin sequence, which was introduced at the Department of Neonatology, Division of Paediatrics – University 
Children’s Hospital, University Medical Centre Ljubljana.
Izvleček
Pierre Robinova sekvenca je triada, ki jo sestavljajo mikro- in/ali retrognatija, glosoptoza in zapora zgornjih dihal, ki se ji v 
90 % pridruži palatoshiza. Pri otrocih s Pierre Robinovo sekvenco sta glavni funkcionalni težavi obstrukcija zgornjih dihal in 
oteženo hranjenje, ki se kažeta z raznolikimi dihalnimi težavami in slabim pridobivanjem telesne mase. Pri večini bolnikov 
1 Department of Neonatology, University Children’s Hospital Ljubljana, University Medical Centre Ljubljana, Ljubljana, Slovenia
2 Department of Maxillofacial and Oral Surgery, University Medical Centre Ljubljana, Ljubljana, Slovenia
3 Department of Otorhinolaryngology and Head & Neck Surgery, University Medical Centre Ljubljana, Ljubljana, Slovenia
4 Department of Paediatric Pulmology, University Children’s Hospital Ljubljana, University Medical Centre Ljubljana, Ljubljana, Slovenia
Correspondence / Korespondenca: Jana Lozar Krivec, e: jana.lozar@gmail.com
Key words: Pierre Robin sequence; neonate; airway obstruction; nasopharyngeal tube
Ključne besede: Pierre Robinova sekvenca; novorojenček; zapora dihal; nosno-žrelni tubus
Received / Prispelo: 25. 11. 2020 | Accepted / Sprejeto: 10. 5. 2021
Cite as / Citirajte kot: Železnik M, Eberlinc A, Gluvajić D, Krivec U, Lozar Krivec J. Pierre Robin sequence: treatment with nasopharyngeal 
tube. Zdrav Vestn. 2022;91(3–4):141–9. DOI: https://doi.org/10.6016/ZdravVestn.3194
eng slo element
en article-lang
10.6016/ZdravVestn.3194 doi
10.5.2021 date-received
25.11.2020 date-accepted
Pediatrics Pediatrija discipline
Professional article Strokovni članek article-type
Pierre Robin Sequence: treatment with naso-
pharyngeal tube
Pierre Robinova sekvenca: zdravljenje z nos-
no-žrelnim tubusom article-title
Pierre Robin Sequence: treatment with naso-
pharyngeal tube
Pierre Robinova sekvenca: zdravljenje z nos-
no-žrelnim tubusom alt-title
Pierre Robin sequence, neonate, airway ob-
struction, nasopharyngeal tube
Pierre Robinova sekvenca, novorojenček, zapora 
dihal, nosno-žrelni tubus kwd-group
The authors declare that there are no conflicts 
of interest present.
Avtorji so izjavili, da ne obstajajo nobeni 
konkurenčni interesi. conflict
year volume first month last month first page last page
2022 91 3 4 141 149
name surname aff email
Jana Lozar Krivec 1 jana.lozar@gmail.com
name surname aff
Mojca Železnik 1
Andreja Eberlinc 2
Daša Gluvajić 3
Uroš Krivec 4
eng slo aff-id
Department of Neonatology, 
University Children's Hospital 
Ljubljana, University Medical 
Centre Ljubljana, Ljubljana, 
Slovenia
Klinični oddelek za 
neonatologijo, Pediatrična 
klinika, Univerzitetni klinični 
center Ljubljana, Ljubljana, 
Slovenija
1
Department of Maxillofacial and 
Oral Surgery, University Medical 
Centre Ljubljana, Ljubljana, 
Slovenia
Klinika za maksilofacilano in 
oralno kirurgijo, Univerzitetni 
klinični center Ljubljana, 
Ljubljana, Slovenija
2
Department of 
Otorhinolaryngology and Head 
& Neck Surgery, University 
Medical Centre Ljubljana, 
Ljubljana, Slovenia
Klinika za otorinolaringologijo 
in cervikofacialno kirurgijo, 
Univerzitetni klinični center 
Ljubljana, Ljubljana,Slovenija
3
Department of Paediatric 
Pulmology, University Children's 
Hospital Ljubljana, University 
Medical Centre Ljubljana, 
Ljubljana, Slovenia
Služba za pljučne bolezni, 
Pediatrična klinika, Univerzitetni 
klinični center Ljubljana, 
Ljubljana, Slovenija
4
Slovenian Medical Journallovenian Medical Journal
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PEDIATRICS
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1 Introduction
Pierre Robin Sequence (PRS) is a condition character-
ized by a sequence of lower and upper jaw abnormalities 
and thus respiratory and gastrointestinal problems (1). In 
1923, the French dentist Pierre Robin described a triad 
consisting of micro- or/and retrognathia, posterior dis-
placement of the tongue into the oropharynx and hypo-
pharynx (glossoptosis), and thus the obstruction of the 
upper respiratory tract. In 90% of cases, the triad is asso-
ciated with a cleft palate (palatoschisis) (2-4) (Figure 1).
The estimated incidence ranges from one in 8,000 and 
20,000 births, depending on the PRS definition (2,3,5,6). 
Airway obstruction is a major predictor of morbidity 
and mortality. The mortality rate of all children with PRS 
ranges from 1.7% to 11.3%, and in patients with associat-
ed other abnormalities it is up to 26% (3-5).
2 Aetiology
The primary pathogenetic event leading to PRS is un-
known. Researchers believe that hypoplasia of the lower 
jaw, which occurs before the 9th week of gestation, causes 
the tongue to move up and back, which mechanically pre-
vents the fusion of the palatal sutures between the 8th and 
10th week of gestation (3,4,7,8).
PRS can manifest either as isolated abnormality in the 
development of the oropharyngeal area or in 45-80% of 
cases there are other anomalies associated, PRS being a 
part of the syndrome. Therefore, it is advisable that a clin-
ical geneticist is involved in the management of a neo-
nate with PRS, who, if necessary, performs further genetic 
tests (3-5,9). Most often, PRS is associated with Stickler’s 
syndrome, which is a connective tissue disease and also 
the main reason for retinal detachment in children (7).
3 Pathophysiology
In a child with PRS, the main functional problems 
are dynamic upper airway obstruction and feeding dis-
order, which cause breathing disorders and poor weight 
gain (2). The degree of upper airway obstruction, which 
Figure 1: Schematic presentation of a neonate with normal anatomy of the upper respiratory tract (left) and with Pierre 
Robin sequence (right).
cle palate
glossoptosis
micro- or retrognathia
upper 
airway 
obstructionoccurs primarily due to micro- or retrognathia and air-
way obstruction with the root of the tongue, is not con-
stant (5,7) (Figure 1). The tongue compressed inside the 
small lower jaw, the tongue prolapses into cleft palate, the 
lack of control over the muscles of the tongue, and the 
pressure of the tongue on the oropharynx and hypophar-
ynx further contribute to upper airway obstruction. Ob-
struction usually decreases over time, but it is not clear 
whether the cause of the improvement is faster growth of 
the lower jaw after birth or a decrease in glossoptosis with 
growth and neurological development (3,8). Additional 
factors that may contribute to airway obstruction in chil-
dren with PRS are described, such as: hypotonia, central 
sleep apnoea, laryngomalacia, tracheomalacia, and sub-
glottic stenosis (3).
The causes of feeding problems are many: from mi-
crognathia, glossoptosis, concomitant cleft palate, im-
paired mobility of the tongue, to oesophagus motility dis-
order, which causes gastroesophageal reflux (5). Increased 
work of breathing and tachypnoea negatively affect the 
coordination of sucking, swallowing and breathing. Feed-
ing problems increase the possibility of aspiration and 
pulmonary complications (5,10). Gastroesophageal re-
flux affects the balance between respiration and feeding, 
as it causes inflammation and swelling of the airways, in-
creases secretions and thus influences the mechanics of 
swallowing (3). Associated heart or other anomalies may 
further contribute to feeding problems and thus to poor 
weight gain (5).
4 Clinical picture
Depending on the degree of upper airway obstruction, 
children with PRS may have a number of signs of respi-
ratory distress that appear immediately after birth or in 
the first weeks of life (2,3). Before birth, the anomaly is 
rarely visible. Micrognathia can be seen by ultrasound or 
magnetic resonance imaging, and in the case of glossop-
tosis and swallowing disorders in the foetus, ultrasound 
can detect polyhydramnios (3,11,12).
zadoščajo konzervativni ukrepi, v redkih primerih pa je potrebno kirurško zdravljenje, v najtežjih primerih pa traheotomi-
ja. Klinični oddelek za neonatologijo Pediatrične klinike UKC Ljubljana je za zdravljenje novorojenčkov s Pierre Robinovo 
sekvenco uvedel v slovenski prostor novo metodo, tj. vstavitev nosno-žrelnega tubusa. Ta premosti zaporo zgornjih dihal v 
višini korena jezika in je ob izmenični zamenjavi strani vstavitve skozi nosnici lahko nameščen daljše obdobje tudi v doma-
čem okolju. Obravnava otrok s Pierre Robinovo sekvenco je multidisciplinarna. Pri obravnavi sodelujejo neonatolog, oto-
rinolaringolog, maksilofacialni kirurg in pulmolog. Gastroenterolog, dietetik in genetik pa so vključeni po potrebi. Članek 
predstavi klinično sliko otrok s Pierre Robinovo sekvenco, metode zdravljenja in uradno klinično pot obravnave v Sloveniji.
143
PROFESSIONAL ARTICLE
Pierre Robin Sequence: treatment with nasopharyngeal tube
1 Introduction
Pierre Robin Sequence (PRS) is a condition character-
ized by a sequence of lower and upper jaw abnormalities 
and thus respiratory and gastrointestinal problems (1). In 
1923, the French dentist Pierre Robin described a triad 
consisting of micro- or/and retrognathia, posterior dis-
placement of the tongue into the oropharynx and hypo-
pharynx (glossoptosis), and thus the obstruction of the 
upper respiratory tract. In 90% of cases, the triad is asso-
ciated with a cleft palate (palatoschisis) (2-4) (Figure 1).
The estimated incidence ranges from one in 8,000 and 
20,000 births, depending on the PRS definition (2,3,5,6). 
Airway obstruction is a major predictor of morbidity 
and mortality. The mortality rate of all children with PRS 
ranges from 1.7% to 11.3%, and in patients with associat-
ed other abnormalities it is up to 26% (3-5).
2 Aetiology
The primary pathogenetic event leading to PRS is un-
known. Researchers believe that hypoplasia of the lower 
jaw, which occurs before the 9th week of gestation, causes 
the tongue to move up and back, which mechanically pre-
vents the fusion of the palatal sutures between the 8th and 
10th week of gestation (3,4,7,8).
PRS can manifest either as isolated abnormality in the 
development of the oropharyngeal area or in 45-80% of 
cases there are other anomalies associated, PRS being a 
part of the syndrome. Therefore, it is advisable that a clin-
ical geneticist is involved in the management of a neo-
nate with PRS, who, if necessary, performs further genetic 
tests (3-5,9). Most often, PRS is associated with Stickler’s 
syndrome, which is a connective tissue disease and also 
the main reason for retinal detachment in children (7).
3 Pathophysiology
In a child with PRS, the main functional problems 
are dynamic upper airway obstruction and feeding dis-
order, which cause breathing disorders and poor weight 
gain (2). The degree of upper airway obstruction, which 
Figure 1: Schematic presentation of a neonate with normal anatomy of the upper respiratory tract (left) and with Pierre 
Robin sequence (right).
cle palate
glossoptosis
micro- or retrognathia
upper 
airway 
obstruction
After birth, upper airway obstruction is often at the 
forefront, manifested by grunting, breathing difficul-
ty, and obstructive sleep apnoea, as well as feeding dif-
ficulties. Glossoptosis and airway obstruction can be of 
varying degrees. In severe obstruction, patients may have 
inspiratory stridor or biphasic stridor, and occasionally 
even cyanosis (8,9), whereas in mild glossoptosis the air-
way may be clear in the waking state and during suck-
ling, but during sleep, obstructive sleep apnoea may oc-
cur (13). Early recognition and intervention are key to 
prevent the consequences of breathing disorders such 
as: poor weight gain, food aspiration, respiratory failure, 
persistent hypoxaemia, hypercapnia, gastroesophageal 
reflux, increased pulmonary vascular resistance, and thus 
the development of cor pulmonale condition, neurologi-
cal impairment and also death (14).
Cleft palate is present in 90% of children with PRS, 
therefore a large proportion of children have problems 
with establishing a normal feeding pattern due to subop-
timal anatomical conditions because of the communica-
tion between the oral and nasal cavities, which prevents 
the creation of intra-oral vacuum, necessary for success-
ful suckling (15-17). A recently published Slovenian study 
of children with orofacial clefts showed that almost three 
quarters of children had problems establishing feeding 
immediately after birth, and more than a seventh of them 
the problems persisted even after surgical treatment (15).
The cleft palate also affects speech development and 
speech disorders later on (articulation disorders and hy-
pernasality), more than half of children with palatoschisis 
also have recurrent otitis media or otitis media with ef-
fusion with conductive hearing loss (18). Interventions 
such as a tracheotomy in a child with PRS can have an 
additional effect on speech development (19).
5 Diagnostics
In 2020, the consensus of paediatricians and otorhi-
nolaryngologists on the recommended treatment of chil-
dren with PRS was published (20). In order to select the 
optimal treatment, in addition to the clinical assessment 
of breathing effort during wakefulness, sleep and feeding, 
it is necessary to evaluate the level of respiratory distur-
bances and determine the location of airway obstruction. 
The association between the degree of micrognathia and 
the degree of glossoptosis has not been demonstrated, 
which means that the assessment of micrognathia cannot 
predict the degree of airway obstruction (21). Before start-
ing treatment, endoscopic airway examination is advised 
to assess glossoptosis and location of airway obstruction 
(6,11). With flexible nasolaryngoscopy, the upper respi-
ratory tract and vocal cord mobility can be assessed, and 
the presence of laryngomalacia can be demonstrated. The 
disadvantage of this examination is that the child is usual-
ly restless and crying during the examination, which can 
make the assessment of the level and the degree of air-
way obstruction due to glossoptosis, which is most pro-
nounced when the child is asleep, impossible (22). Flexi-
ble nasolaryngoscopy and/or bronchoscopy in a sedated 
child provide a better assessment of the location of airway 
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obstruction, and allows additional assessment of the en-
tire airway, thus identifying any attendant abnormalities 
such as subglottic stenosis, tracheomalacia, bronchoma-
lacia and stenosis of trachea (3,11).
Using cardiorespiratory polygraphy during sleep in 
the first months after birth allows an objective evaluation 
of breathing disturbances (2,3,23). Obstructive sleep ap-
noea is extremely common in patients with PRS. A retro-
spective study by Hicks et al., which included 31 neonates 
with PRS, found moderate to severe obstructive sleep ap-
noea in 64% of neonates (6). Cardiorespiratory polygra-
phy during sleep also allows to evaluate the effectiveness 
of the measures taken in the patient and to assess the im-
provement or worsening of the level of obstruction (6,8). 
If left untreated, the obstruction usually progresses in the 
first 4–6 weeks after birth (3).
When assessing the feeding of children with PRS, it 
is important to distinguish between feeding problems 
due to upper airway obstruction and problems result-
ing from altered anatomy in the presence of cleft palate, 
swallowing disorders, aspirations, and gastroesophageal 
reflux. Additionally, feeding and growth problems may 
occur in connection with the underlying syndrome or 
other associated abnormalities (3). Clinical diagnostics 
of gastroesophageal reflux can be difficult. A 24-hour pH 
impedance testing is most commonly used to quantify 
reflux (3,24,25). Inadequate weight gain can also result 
from increased calorific needs due to increased work of 
breathing. A clinical dietitian and a speech therapist must 
be included in the management, while a personal paedia-
trician is responsible for long-term monitoring of growth 
and development. A paediatric gastroenterologist is con-
sulted in cases of severe gastroesophageal reflux disease 
and oesophageal motor dysfunction (3,11).
6 Treatment
There are many methods used to treat neonates and 
children with PRS. Although PRS is a well-known clinical 
entity, current classification systems do not consider the 
diversity of the clinical picture of neonates with PRS, that 
could allow the selection of the optimal treatment for a 
particular clinical case (3,4,26).
The protection of the airway is crucial in the treatment 
of children with PRS, since it enables easier breathing and 
feeding, and lowers the risk of food aspiration during 
oral feeding (5). A multidisciplinary approach is advised, 
which facilitate faster and more efficient identification of 
problems, prevents unnecessary interventions and en-
ables optimal care for children with PRS (3).
In more than half of the children, conservative, 
non-surgical interventions are sufficient (3). The first-
choice measure, which is successful in more than half of 
patients with mild to moderate airway obstruction, is the 
prone or side position, which allows the lower jaw and 
tongue to fall forward, moving the tongue away from the 
back of the pharynx (4).
If this measure is not successful, we decide to insert 
a nasopharyngeal tube (NPT) or use respiratory sup-
port with continuous positive airway pressure (CPAP) 
or non-invasive ventilation (NIV) (3,21). According to a 
study by Abel et al., the effectiveness of NPT in children 
with PRS with moderate to severe obstruction is 80%, 
and the use of NPT also reduces the number of required 
tracheotomies (8). In a study by Leboulanger et al., the 
use of NIV reduced the proportion of time of SpO2 be-
low 90% from an average of 14% to 1%, and also reduced 
the value of transcutaneously measured partial pressure 
of carbon dioxide (PtcCO2) from 7.6 to 4.1 kPa (9). The 
disadvantages of NIV are: the difficulty in optimal mask 
selection due to the changed facial anatomy, the difficulty 
of establishing oral feeding, and ventilation intolerance 
(9,23). When technical limitations or inability to accept 
ventilation support enable the use of CPAP or NIV, at 
least some degree of respiratory support can be achieved 
with high-flow therapy (HFT) (27).
Some centres use custom-made orthodontic plates 
(e.g. a pre-epiglottic baton plate). The plate reduces upper 
airway obstruction by resolving glossoptosis. It is used 
mainly in children with a milder form of PRS (2).
Among surgical methods of treatment, the most com-
mon are surgical attachment of the base of the tongue to 
the lower lip (glossopexy) and mandibular distraction 
osteogenesis. The first is indicated when the patient has 
severe glossoptosis, and endoscopy has ruled out sub-
glottic stenosis or other respiratory abnormalities, and it 
consists of two procedures (5,11). The secondary proce-
dure, which allows the base of the tongue to be released, 
is usually performed between 12 and 18 months of age 
(11). According to studies, the effectiveness of the meth-
od for resolving the airway obstruction is between 71% 
and 89% (4,11). Recurrence of glossoptosis may occur af-
ter adhesion release; other complications of the procedure 
include wound dehiscence, tongue swelling after surgery, 
and scarring (2,4,5).
Mandibular distraction osteogenesis consists of bi-
lateral vertical osteotomy of the hypoplastic mandibula 
followed by an external or internal distractor placement 
(2,4). The external distractor is easier to place and re-
move. Possible disadvantages of its use are the movement 
of the distractor due to the external forces and scarring at 
the site of its placement. The internal distractor is usually 
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PROFESSIONAL ARTICLE
Pierre Robin Sequence: treatment with nasopharyngeal tube
better tolerated by children, but secondary surgery under 
general anaesthesia is required for its removal. Osteoto-
my is followed by an activation phase, when the bone is 
stretched and it grows at a rate of 0.5–2 mm per day. The 
final stage is consolidation, when the newly formed bone 
is ossified (3). Until the lower jaw sufficiently grows, the 
airway must be secured with a NPT or an endotracheal 
tube, or the patient should breathe through a tracheos-
tomy (2-4). Mandibular distraction osteogenesis allows 
rapid resolution of obstruction, but, like any surgical 
treatment, has certain risks (2,4). Compared with glos-
sopexy, it allows faster establishment of oral feeding (5), 
while other authors observe poor weight gain as a result 
of dysphagia, which is not corrected after distraction (2). 
A possible complication of treatment may be damage to 
the mandibular and inferior alveolar nerve, and damage 
to the molar design, which occurs in half of the cases 
(2,4,6). Mandibular distraction osteogenesis is probably 
most effective when micrognathia and glossoptosis are at 
the forefront. However, in the case of multilevel airway 
obstruction, distraction will not be effective and patient 
will require a tracheotomy despite elongation of the lower 
jaw (6).
Tracheotomy does not correct basic PRS abnormali-
ties, but it is the only method that efficiently resolves air-
way obstruction. It is an invasive method and is used in 
children with PRS when: other methods of treatment are 
not successful, in very severe airway obstruction, in mul-
tilevel airway obstruction, and in patients with associated 
anomalies (2,3,23,28). In children with tracheotomy the 
hospitalization is longer. In 65% of cases there are com-
plications associated with tracheotomy, which can also 
be life-threatening (19,28). The most common compli-
cations are: bleeding, pneumothorax, decannulation, tra-
cheitis, tracheal stenosis, and, exceptionally, even sudden 
death (2,3).
90% of children with PRS have palatoschisis, which 
does not require specific treatment in the first months of 
life, except for an adapted bottle and an adapted feeding 
position (15). According to the current recommenda-
tions, an isolated cleft palate is treated by maxillofacial 
surgeons after the first year of life (29,30).
Regardless of the treatment method, 38–62% of neo-
nates with PRS have such severe feeding difficulties that a 
gastric tube must be inserted, either orally or nasally (3). 
The use of gastric tube is recommended in all patients 
with feeding difficulties and it does not interfere with the 
decision for discharge into home care. It is usually need-
ed for a transitional period (3). The gastric tube provides 
additional benefits by further opening the airway, as it al-
lows the tongue to be displaced away from the posterior 
pharyngeal wall, and by reducing air swallowing during 
feeding, which can cause an increase in gastric pressure 
and thus contribute to gastroesophageal reflux. Placing 
the baby in an upright position is recommended to pre-
vent reflux. If this measure is not sufficient, other anti-
reflux measures, including the introduction of a proton 
pump inhibitor, may be advised (3,10). Children with as-
sociated syndromic disease or other anomalies and neu-
rological disorders may have chronic feeding problems 
that require gastrostomy tube insertion (3,11).
After discharge from the hospital, the child’s paedia-
trician plays an important role in following a child with 
PRS by paying particular attention to breathing and feed-
ing problems. He/she needs to monitor the child’s growth 
and development. In children with cleft palate, special at-
tention must be paid to hearing and speech development 
(2,3,8,22). In most cases, children with PRS have a good 
prognosis, and timely prevention of hypoxic events allows 
them normal cognitive development (3).
Despite the progress in management, analyses of the 
results of different ways of treating children with PRS are 
lacking, so the approach to a child with PRS depends on 
the experience and strategy of specific centre. Howev-
er, the international recommendations of paediatricians 
and otorhinolaryngologists on the management of chil-
dren with PRS from 2020 allow several management ap-
proaches (20).
7 Pierre Robin Sequence treatment with a 
nasopharyngeal tube
Until now, the Department of Neonatology has used 
conservative treatment methods in neonates with PRS 
and mild to moderate signs of airway obstruction, such 
as prone or side position and insertion of a gastric tube or 
CPAP therapy. In patients with severe airway obstruction, 
tracheotomy was used, while glossopexy and mandibu-
lar distraction osteogenesis were not used in agreement 
with maxillofacial surgeons due to a number of possible 
side effects. In 2020, based on positive experiences of for-
eign centres, we introduced NPT treatment as a possible 
method of maintaining a free airway.
Table 1 shows the number of children born with schi-
sis, palatoschisis, and PRS between 2016 and 2020 in Slo-
venia. According to the five-year period, the incidence of 
PRS in Slovenia is 1.2 per 10,000 live births. Of the 12 
children born with PRS, seven children needed a gastric 
tube, two children born in the last year were managed 
with NPT, and one child required a tracheotomy.
After preparing the patient, the NPT is inserted 
through the nose, without general anaesthesia, so that 
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the distal tip of the NPT is positioned behind the root 
of the tongue, but above the epiglottis (2,3,6). The inten-
tion of the insertion of NPT is to overcome the airway 
obstruction at the level of the oropharynx or root of the 
tongue and facilitate breathing and feeding. A properly 
inserted NPT allows the child to breathe through both, 
the tube and the free nostril, as the tongue is moved away 
from the posterior pharyngeal wall due to the inserted 
NPT (3) (Figure 2). Given the way in which obstruction 
is resolved, the use of NPT is probably the most effective 
method when the cause of airway obstruction is micro- or 
retrognathia and glossoptosis (2).
The depth of NPT insertion is determined by measur-
ing the distance between the nostril and the ear tragus 
of the patient or by direct visualisation of the NPT tip 
with a flexible endoscope. The insertion depth can also 
be determined using the tables that determine the size 
Legend: No. – number; PRS – Pierre Robin sequence.
Year of 
birth
No. of 
children born 
with schisis
No. of 
children 
born with 
palatoschisis
No. of 
children 
born with 
PRS
2016 27 10 3
2017 26 12 0
2018 33 12 3
2019 22 9 3
2020 25 11 3
Table 1: Number of all children born with schisis, 
palatoschisis and Pierre Robin sequence between 2016 and 
2020 in Slovenia.
Figure 2: Schematic presentation of the inserted 
nasopharyngeal tube.
nasopharyngeal 
tube
epiglottis
and depth of the insertion of the NPT according to body 
weight (11,14,31). After the first insertion of the NPT, the 
correct position can be confirmed by lateral neck X-ray in 
the neutral position of the head (8) (Figure 3).
The child has an NPT inserted 24 hours a day for weeks 
or months. Discharge from the hospital to the home en-
vironment is possible when parents learn to remove and 
replace, aspirate, and fix the NPT, as well as to handle a 
child with an inserted NPT (3). Masters et al. anticipate 
changing the NPT every 2–4 days at first and later on ev-
ery 5–7 days (14). On average, children need NPT for 3–4 
months or until the airway obstruction is relieved due to 
enlargement of the lower jaw which is the result of rapid 
growth after birth, stimulated mainly by suckling (3,6,11).
NPT treatment can have complications which are, 
nevertheless, rare. Complications include blockage of the 
NPT with secretions, aspiration of gastric contents due 
to incorrect positioning, inadvertent removal of the NPT, 
damage to the skin, cartilage and mucosa of the nose, and 
the formation of nasal stenosis (2,3).
At the Department of Neonatology, based on our own 
experience and professional reports, we have developed 
a protocol for the treatment of neonates with PRS with 
NPT (Figure 4). Indications for NPT insertion are: clini-
cally perceived airway obstruction and signs of increased 
work of breathing at rest, significant sleep desaturations 
or obstructive episodes detected by the cardiorespiratory 
Figure 3: Lateral X-ray image of the head in the neutral 
position; the tip of the inserted nasopharyngeal tube is at 
the level of the epiglottis; the position of the nasopharyngeal 
tube should be corrected.
147
PROFESSIONAL ARTICLE
Pierre Robin Sequence: treatment with nasopharyngeal tube
polygraphy, feeding problems and poor weight gain, and 
increased work of breathing with hypercapnia despite 
the neonate being in the prone1 or side position. Contra-
indications for the insertion of NPT are damage to the 
nose and throat, obstruction of the nasal cavity, septal 
deviation, blood clotting disorders, and additional air-
way obstruction below the epiglottis. Before the insertion 
of NPT, cardiorespiratory polygraphy while sleeping is 
performed. The size and approximate depth of the NPT 
insertion is determined using tables. The first insertion 
of NPT is performed in sedation during a flexible naso-
laryngoscopy, which, in addition to assessing the upper 
respiratory tract, nasal patency, vocal cord mobility and 
location of airway obstruction, enables accurate insertion 
of the NPT just above the epiglottis, between the base of 
the tongue and the posterior pharyngeal wall. After in-
sertion of the NPT, a lateral neck X-ray is performed in 
the neutral position of the head. If necessary, the depth 
of insertion of the NPT is corrected to ensure the tube 
insertion 1 cm above the epiglottis (Figure 3).
After insertion of the NPT, vital signs are continuous-
ly monitored, especially SpO2, and a cardiorespiratory 
polygraphy is performed during sleep. Patients are bot-
tle-fed using special bottles and nipples for patients with 
schisis, and if necessary, a gastric tube is inserted. Evalu-
ation of feeding or oromotor functions is performed ac-
cording to NOMAS® (Neonatal Oral - Motor Assessment 
Scale) (32). Antireflux measures are taken in all patients, 
such as upright position and pre-thickened formula milk 
or feed thickener. If these measures are not sufficient, a 
proton pump inhibitor is introduced. After insertion of 
the NPT, the patient is still placed in a prone or side po-
sition. We recommend changing the NPT every 1-2 days 
Figure 4: Clinical pathway for treatment of neonate with Pierre Robin sequence.
Legend: NPT – nasopharyngeal tube; CPAP – continuous positive airway pressure; NIV – non-invasive ventilation; HFT – high-
flow therapy.
SpO2 monitoring and 
cardiorespiratory 
polygraphy 
during sleep
increased work of 
breathing only in 
supine position
without increased 
work of breathing 
at rest or 
during feeding
airway endoscopy 
and 
NPT insertion
discharge into home care repeated endoscopy, decision 
on the appropriateness of 
respiratory support 
(CPAP/NIV or HFT)
other methods of 
surgical treatment 
– e.g. tracheotomy
side position
increased work of 
breathing at rest 
or during feeding
SpO2 monitoring and 
cardiorespiratory 
polygraphy 
during sleep
SpO2 monitoring and 
cardiorespiratory 
polygraphy during 
sleep
abnormal
abn
orm
al
no
rm
al
no
rm
al
no
rm
al
ab
no
rm
al
1The prone sleeping position is a risk factor for sudden, unexpected 
infant death, so the patient should be monitored while in this position.
148
PEDIATRICS
Zdrav Vestn | March – April 2022 | Volume 91 | https://doi.org/10.6016/ZdravVestn.3194
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at first, then on every 3-5 days, alternately in one or the 
other nostril. If necessary, the size of the NPT is changed 
as the child grows.
During hospitalization, parents are taught to re-
move and replace the NPT, aspirate the tube and upper 
respiratory tract, and feed the child by gastric tube if nec-
essary. Parents are taught basic resuscitation procedures 
and are made aware of emergency measures in case of suf-
focation or food aspiration. Before discharge to the home 
environment, we inform the child’s paediatrician and the 
visiting nursing service about the patient’s condition and 
special medical needs and care. We introduce the child to 
the pulmonologist, as the infants continue their manage-
ment at the Department for Pulmology of the University 
Children’s Hospital, University Medical Centre Ljubljana 
(Figure 5).
8 Conclusion
PRS is a rare, life-threatening clinical condition. It 
requires timely identification and action. At the Depart-
ment of Neonatology, a new method for the treatment 
of children with PRS by inserting NPT was introduced, 
which by resolving the obstruction at the level of the 
pharinx and base of the tongue allows maintaining a free 
airway in children with PRS.
Conflict of interest
None declared.
Figure 5: Photograph of a child with a nasopharyngeal tube 
inserted (the child’s parents have agreed to publishing the 
article containing their child’s photograph).
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Pierre Robin Sequence: treatment with nasopharyngeal tube
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