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Pierre Robin sequence (PRS) is a condition consisting of three essential components: micrognathia or retrognathia, cleft palate, and glossoptosis. It can be part of multiple congenital anomalies. We present the case and outcome of a 3-month-old clinically stable patient who has PRS with Dandy-Walker variant – which is a rare presentation in the literature – with a large right hemispheric brain abscess, treated with multiple minimally-invasive surgical drainage procedures with adjuvant antibiotics.

Background: Pierre Robin sequence (PRS) is a rare congenital anomaly. Respiratory disorders and feeding difficulties represent the main burden. Objective: The aim of this study was to investigate the epidemiology of PRS using a cohort of cases from EUROCAT, the European network of population-based registries of congenital anomalies. Methods: We analysed cases of PRS born in the period 1998-2017 collected by 29 population-based congenital anomaly registries in 17 different countries. We calculated prevalence estimates, prenatal detection rate, survival up to 1 week, and proportions of associated anomalies. The effect of maternal age was tested using a Poisson regression model. Results: Out of 11 669 155 surveyed births, a total of 1294 cases of PRS were identified. The estimate of the overall prevalence was 12.0 per 100 000 births (95% CI 9.9, 14.5). There was a total of 882 (68.2%) isolated cases, and the prevalence was 7.8 per 100 000 births (95% CI 6.7, 9.2). A total of 250 cases (19.3%) were associated with other structural congenital anomalies, 77 cases (6.0%) were associated with chromosomal anomalies and 77 (6.0%) with genetic syndromes. The prenatal detection rate in isolated cases was 12.0% (95% CI 9.8, 14.5) and increased to 16.0% (95% CI 12.7, 19.7) in the sub-period 2008-2017. The prevalence rate ratio of non-chromosomal cases with maternal age ≥35 was higher than in cases with maternal age

Background: Pierre Robin sequence (PRS) patients have an increased risk of difficult intubation due to anatomical airway abnormalities, and intubation simulation with a three-dimensional (3D) printed airway model before anesthesia may facilitate safe airway management. Case: We describe the case of a 6.5-year-old boy with a history of PRS (a triad of micrognathia, glossoptosis, and airway obstruction), tracheostomy, and subglottic fibrosis who required general anesthesia. Preparation for this potentially difficult intubation included estimation of endotracheal tube size using a 3D printed airway model derived from 3D computed tomography of the airway, which enabled successful endotracheal intubation via video laryngoscopy.Conclusions: If general anesthesia is necessary in patients with dysmorphic features such as PRS and there is a history of tracheal pathology, the possibility of difficult intubation should always be considered and simulation of endotracheal intubation using a 3D printed model of the airway can be helpful clinically in such situations.

IntroductionThe Pierre-Robin sequence (PRS) is a pattern of congenital facial abnormalities comprising micrognathia, glossoptosis, and airway obstruction. Associated spinal pathologies have rarely been reported with PRS.MethodsWe explore the molecular genetic basis of this association through a systematic review of spinal disease in patients with PRS. We also present an illustrative case of a PRS patient with tethered cord in the setting of chromosome 10q terminal deletion.ResultsOur systematic literature review of spinal disease in patients with PRS revealed several patterns in the underlying genetic syndromes causing these conditions to co-occur. These principles are illustrated in the case of a 6-month-old female with PRS and a 14.34-Mb terminal deletion of chromosome 10q, who was found to have a sacral dimple during a routine outpatient checkup. Magnetic resonance imaging of the spine revealed a lumbar syrinx associated with tethered spinal cord. Surgical de-tethering was undertaken, with subsequent improvement in motor function and decrease in the size of the syrinx. The deletion of chromosome 10q in our patient had not previously been described in association with tethered cord or PRS.ConclusionSpinal pathologies are understudied contributors to disease burden in patients with PRS. The range of predisposing syndromes and mutations in patients with both PRS and spinal disorders remains poorly characterized but may be more defined than previously conceived. Clinical screening is most critical during neonatal and adolescent developmental periods with continued neurological assessment. This study emphasizes the need for early genetic testing and counseling in this patient population, in parallel with research efforts to develop molecular classifications to guide clinical management.

Objective Pierre Robin Sequence (PRS) is a heterogeneous pathological condition characterized by the coexistence of micrognathia, glossoptosis, and cleft palate, resulting in upper airway tract obstruction. Among the treatment modalities, the orthodontic approach is one part of the comprehensive care of those patients and will be present in the treatment modalities during all the growth period of the child. Methods All patients with PRS observed in the period 2013-2017 were treated with a definite functional approach. The results were retrospectively analyzed with regard to functional outcome, total treatment time, and number of plates provided for a single patient. Results In all the patients, the indicated treatment protocol has been applied as early impression and plate supply, stimulation of bottle feeding with the use of the plate, eventual substitution of the plate if no more adequate to the transverse and sagittal growth of the palate, and continuing the use until the surgical closure of the cleft. All the patients showed a positive outcome to the proposed treatment approach, evaluated with regard to the incidence of feeding improvement and weight gain, to the limit for the surgical phase, in the absence of adverse effects. Conclusion The use of a functional obturator plate, removing functional alterations to mandibular growth, reduces and, in some cases, eliminates the need for surgical intervention. As also stated in the literature, if despite the presence of the plate nutritional problems persist, immediate different surgical approaches, mainly mandibular osteodistraction, become necessary.

IntroductionThe Pierre-Robin sequence (PRS) is a pattern of congenital facial abnormalities comprising micrognathia, glossoptosis, and airway obstruction. Associated spinal pathologies have rarely been reported with PRS.MethodsWe explore the molecular genetic basis of this association through a systematic review of spinal disease in patients with PRS. We also present an illustrative case of a PRS patient with tethered cord in the setting of chromosome 10q terminal deletion.ResultsOur systematic literature review of spinal disease in patients with PRS revealed several patterns in the underlying genetic syndromes causing these conditions to co-occur. These principles are illustrated in the case of a 6-month-old female with PRS and a 14.34-Mb terminal deletion of chromosome 10q, who was found to have a sacral dimple during a routine outpatient checkup. Magnetic resonance imaging of the spine revealed a lumbar syrinx associated with tethered spinal cord. Surgical de-tethering was undertaken, with subsequent improvement in motor function and decrease in the size of the syrinx. The deletion of chromosome 10q in our patient had not previously been described in association with tethered cord or PRS.ConclusionSpinal pathologies are understudied contributors to disease burden in patients with PRS. The range of predisposing syndromes and mutations in patients with both PRS and spinal disorders remains poorly characterized but may be more defined than previously conceived. Clinical screening is most critical during neonatal and adolescent developmental periods with continued neurological assessment. This study emphasizes the need for early genetic testing and counseling in this patient population, in parallel with research efforts to develop molecular classifications to guide clinical management.

Introduction Abnormalities of the epiglottis and hyoid bone have been described in association with Pierre Robin Sequence (PRS). Embryologically, the epiglottis stems between the third and fourth branchial arches, while the hyoid bone develops from the second and third arches. The body of the hyoid bone completes its development by 4 months of age, and in fact can be seen on imaging in the majority of children after 9 months of age. Case We present a case of an 18-year-old male known for PRS, who underwent a tracheostomy within a week of life and multiple airway evaluations since. Complete absence of his epiglottis was noted early on. Numerous computed tomographies (CTs) preformed during his lifetime with three-dimensional reconstruction of facial bones; however, only reported bony anomalies of the mandible and zygomatic arches. In a retrospective review of imaging by his surgeons and the center's radiologist, the body of the hyoid bone was found to be clearly absent from the earliest CT at 2 years of age. His greater cornua; meanwhile, remain present leading into soft tissue and potentially cystic content in the midline. Discussion These anomalies bring up the question of an underreported association within PRS patients considering their close embryological origins. Aplasia of the epiglottis is well reported independently, but there have been very few studies on hyoid anomalies in this population. A larger series is needed to evaluate the frequency as well as potential clinical impact of these abnormalities in unison within the PRS population.

The aim of this retrospective study is to determine the predictive factors of postoperative dyspnea in infants with Pierre Robin sequence (PRS). Forty children with PRS, who underwent general anesthesia, were retrospectively analyzed. The patient’s physiological status and anesthesiology data were collected accordingly, demographic characteristics including age, gender, height and weight at surgery, weight gain, preoperative airway status, tracheal intubation route, American Society of Anesthesiologists grading and airway Cormack–Lehane classification. Weight gain, dyspnea before the operation, Cormack–Lehane grade distribution showed a significant difference between patients with and without postoperative dyspnea (p = 0.0175, p = 0.0026, and p = 0.0038, respectively). Incompetent weight gain was identified as a predictor (p = 0.0371) of PRS postoperative dyspnea through the binary logistic regression model. In conclusion, this study established an early alerting model by monitoring the weight gain, dyspnea before the operation, Cormack–Lehane grade as potential combinations to predict the risk of postoperative dyspnea for PRS.

Mandibular distraction osteogenesis (MDO) is an effective treatment for tongue-based airway obstruction in infants with severe Pierre Robin sequence (PRS). Most infants receiving MDO require postoperative mechanical ventilation (MV) to assist breathing. Optimal MV time for each individual patient and factors influencing the time must be identified to guide clinical decision-making. A retrospective analysis was performed on 75 infants with PRS receiving MDO from November 2016 to August 2018. Twenty-six were females and 47 were males. Data extracted from the hospital information system included sex, age, weight, history of preterm labor, preoperative pulmonary infection, laryngomalacia/tracheomalacia, laryngoscope exposure classification, anesthesia duration, operation duration, postoperative treatment site, situation of distraction, postoperative complications and MV duration. Statistical analyses were conducted to investigate the potential associations of these factors with MV time. Seventy-three PRS syndrome patients received anesthesia for MDO device procedures were considered eligible for study. Patient sex, history of preterm labor, preoperative pulmonary infection, laryngomalacia/tracheomalacia, laryngoscopy exposure difficulty, postoperative treatment site (neonatal or pediatric intensive care unit), ventilator-associated pneumonia, age, weight, anesthesia duration, and operation duration had no significant influence on postsurgical MV time (P > .05). Amount of distraction at the time of extubation had statistically significant influence on postoperative MV time (P

Background Pierre Robin Sequence (PRS) patients are known for their triad of micrognathia, glossoptosis, and airway obstruction. Their airway can be a challenge even for the most experienced pediatric anesthesiologist. Case presentation We report the case of a 9 day old 3.5 kg boy diagnosed with PRS, cleft palate, and a vallecular cyst with severe upper airway obstruction. The combination of PRS, cleft palate and the presence of vallecular cyst made this a cascade reaction of difficult airway. Due to his unique anatomy, we didn’t appreciate how difficult his airway was until multiple attempts with high-tech equipment failed. Ultimately it was the finger guide intubation, this old technique without any equipment, that rescued this patient from lose of airway. Conclusions The boy was successfully rescued by finger guided intubation. Finger guide intubation should be added to the anesthesiologist’s newborn rescue intubation training.

Pierre Robin sequence (PRS) may lead to life-threatening respiratory and feeding disorders. With the aim to analyse the association of the severities of retrognathia and glossoptosis with those of respiratory and feeding disorders, we retrospectively studied a series of 50 infants with retrognathia, glossoptosis, cleft palate, and airway obstruction. The patients were managed from birth to at least 6 years of age by a single pediatric team at the Armand Trousseau Hospital in Paris within a 12 years period (2000–2012). Retrognathia and glossoptosis were graded in the neonatal period according to a specific clinical examination. Ventilation assistance was required for 32/50 (64%) patients, and enteral feeding for 41/50 (82%). The grades of retrognathia and glossoptosis and the severity of respiratory disorders did not differ between patients with isolated PRS and syndromic PRS. Severe respiratory disorders were more common and long-lasting feeding (>12 months) was more frequently required in patients with syndromic PRS compared with isolated PRS (42 vs. 13%, p = 0.04 and 42 vs. 4%, p < 0.01 respectively). Using univariate analysis, neurological impairments and laryngomalacia were associated with severe respiratory disorders [Odds ratio (OR) 5.0, 95% CI 1.3–19.6; and OR 14.6, 95% CI 1.3–161.4; p < 0.05] as well as with long-lasting feeding (>12 months) disorders (OR 18.6, 95% CI 3.9–89.2 and OR 20.4, 95% CI 3,4–122.8; p < 10−2). Syndromic SPR status was also associated with severe respiratory disorders (OR 4.9, 95% CI 1–32.5; p < 0.05). Using multivariate analysis, only syndromic PRS status was predictive for severe respiratory disorders (adjusted OR 8, 95% CI 1.47–44.57; p < 0.05); and only neurological impairments remained a significant risk for long lasting feeding disorders (>12 months) (adjusted OR 21.72, 95% CI 3.4–138.63; p < 10−2). The grades of retrognathia and glossoptosis were not predictive factors for the severity of respiratory and feeding disorders.Conclusion: In children with PRS, the severity of clinical conditions may not correlate with anatomic variables but rather with laryngeal abnormalities, neurological impairement and syndromic PRS status.

Pierre Robin Sequence is a congenital pathology defined by the triad micrognathia, glossoptosis and often a U-shaped cleft of soft palate. Newborns affected by airways obstruction may necessitate more invasive options: tongue-lip adhesion, tracheostomy and mandibular distraction osteogenesis. The authors analyzed the effect of fast and early mandibular osteodistraction on deciduous dental development in patients affected by Pierre Robin Sequence. Analysis of the patients treated for severe form was performed by a team composed by maxillofacial surgeons and dentists. Five patients were included for the analysis: before and long term clinical and radiological assessments were considered. All patients underwent fast and early mandibular osteodistraction; two years follow up computed tomography and panorex reconstructions showed bone consolidation, 33 of 35 teeth analyzed before ostedistraction are present after distraction protocol; no positional changes were detected at the follow up analysis either deciduous teeth and molar permanent buds. No deformities regarding molar buds were detected. In conclusion external mandibular distractor devices have been associated with dental injuries and facial scaring. Even though, the dental complications identified can not be unambiguously connected to the external distractor devices.

Background Pierre Robin sequence (PRS) refers to the association of micrognathia, glossoptosis, and airway obstruction. Cases with severe dyspnea due to upper airway obstruction immediately after birth are very rare. We here report two cases with PRS who developed severe dyspnea due to morphological abnormality immediately after birth and were rescued by fiberoptic nasotracheal intubation. Case presentation The patient in case 1 had micrognathia and cleft palate, and his tongue protruded into the nasal cavity via a cleft palate. His invaginated tongue was considered an extreme type of glossoptosis and he was diagnosed as Pierre Robin sequence. The patient in case 2 also had micrognathia and cleft palate same as case 1 and accompanied some anomalad. Her chromosome analysis confirmed a diagnosis of 1p36 deletion syndrome and she diagnosed as 1p36 deletion syndrome complicated with Pierre Robin sequence. In both cases, tongue protruded into the nasal cavity via a cleft palate occupied pharynx and nasal cavity, resulting in severe dyspnea. Only the backside of the tongue was visible by laryngoscopy and oropharyngeal intubation was impossible. Therefore, fiberoptic nasotracheal intubation was done to secure the airway for resuscitation. Conclusion We conclude that extreme type of glossoptosis in PRS concludes tongue invaginated into nasal cavity which have not reported before and that such cases require resuscitation by fiberoptic intubation immediately after birth. As such, neonatologists should obtain the skill of fiberoptic intubation.

Purpose To describe the speech of a patient with Pierre Robin Sequence (PRS) and severe speech disorders before and after participating in an Intensive Speech Therapy Program (ISTP). Methods The ISTP consisted of two daily sessions of therapy over a 36-week period, resulting in a total of 360 therapy sessions. The sessions included the phases of establishment, generalization, and maintenance. A combination of strategies, such as modified contrast therapy and speech sound perception training, were used to elicit adequate place of articulation. The ISTP addressed correction of place of production of oral consonants and maximization of movement of the pharyngeal walls with a speech bulb reduction program. Therapy targets were addressed at the phonetic level with a gradual increase in the complexity of the productions hierarchically (e.g., syllables, words, phrases, conversation) while simultaneously addressing the velopharyngeal hypodynamism with speech bulb reductions. Results Re-evaluation after the ISTP revealed normal speech resonance and articulation with the speech bulb. Nasoendoscopic assessment indicated consistent velopharyngeal closure for all oral sounds with the speech bulb in place. Conclusion Intensive speech therapy, combined with the use of the speech bulb, yielded positive outcomes in the rehabilitation of a clinical case with severe speech disorders associated with velopharyngeal dysfunction in Pierre Robin Sequence.

Temporomandibular joint ankylosis (TMJA) is a severe disorder described as an intracapsular union of the disc-condyle complex to the temporal articular surface with bony fusion. The management of this disability is challenging and rarely based on surgical and rehabilitation protocols. We describe the treatment in two young adults affected by Goldenhar syndrome and Pierre Robin sequence with reankylosis after previous surgical treatments. There are three main surgical procedures for the treatment of TMJA: gap arthroplasty, interpositional arthroplasty, and joint reconstruction. Various authors have described reankylosis as a frequent event after treatment. Treatment failure could be associated with surgical errors and/or inadequate intensive postoperative physiotherapy. Surgical treatment should be individually tailored and adequate postoperative physiotherapy protocol is mandatory for success.

Catel-Manzke syndrome is a rare autosomal recessive disorder characterized by Pierre Robin sequence with hyperphalangy and clinodactyly of the index finger. Recently, homozygous or compound heterozygous pathogenic variants in TGDS have been discovered to cause Catel-Manzke syndrome. Here, we describe a 12-month-old male with molecularly confirmed Catel-Manzke syndrome who presented with Pierre Robin sequence (but without cleft palate) and hyperphalangy, and we compare his phenotype with the seven previously described patients with pathogenic variants in TGDS. Our patient is on the severe end of the phenotypic spectrum, presenting with respiratory complications and failure to thrive. Furthermore, our finding of a homozygous p.Ala100Ser pathogenic variant in our patient supports that it is a common mutation in Catel-Manzke syndrome.

Purpose Newborns with Pierre Robin sequence (PRS) and syndromic micrognathia show microgenia and glossoptosis, which cause reduction of the airway and breathing difficulty from birth. Our goal is to analyze quantitative and qualitative volumetric changes before and after fast and early mandibular osteodistraction (FEMOD) and to compare radiological data. Methods The sample was composed of 4 patients, who satisfied inclusion criteria for completeness of data. Computed tomography pre- and post-operation were performed, then a volumetric assessment was made with Dolphin Imaging. Polysomnography was performed before and after FEMOD. Results Pre- and post-operative CT scan data were compared. The analysis of all three sections showed a significant increase of volumetric parameters. The retroglossal volume average increase was 346%, and the retropalatal volume average increase was 169%. These data matched the improvement recorded by polysomnography. Conclusions The data confirm FEMOD as an efficient treatment to improve airways and breathing problem in patients affected by Pierre Robin sequence and syndromic micrognathia. The three-dimensional volume rendering could be a useful method to evaluate and quantify the increase in airways volume.

Toriello and Carey [1988: Am J Med Genet 31:17-23] first described a syndrome with component manifestations of corpus callosum agenesis, unusual facial appearance, Robin sequence, and other anomalies. This was termed the Toriello-Carey syndrome by Lacombe et al. [1992: Am J Med Genet 42:374-376]. Since then, 11 reports describing 16 additional children have been published; in addition, we have had the opportunity to review over 30 unpublished cases. However, for various reasons, only 25 of the unpublished patients were included in this review. Based on this total, we can begin to better delineate this syndrome, as well as provide some information on natural history. (C) 2003 Wiley-Liss, Inc.

Pierre Robin sequence (PRS) is an aetiologically distinct subgroup of cleft palate. We aimed to define the critical genomic interval from five different 5q22-5q31 deletions associated with PRS or PRS-associated features and assess each gene within the region as a candidate for the PRS component of the phenotype. Clinical array-based comparative genome hybridisation (aCGH) data were used to define a 2.08 Mb minimum region of overlap among four de novo deletions and one mother-son inherited deletion associated with at least one component of PRS. Commonly associated anomalies were talipes equinovarus (TEV), finger contractures and crumpled ear helices. Expression analysis of the orthologous genes within the PRS critical region in embryonic mice showed that the strongest candidate genes were FBN2 and PHAX. Targeted aCGH of the critical region and sequencing of these genes in a cohort of 25 PRS patients revealed no plausible disease-causing mutations. In conclusion, deletion of ?2 Mb on 5q23 region causes a clinically recognisable subtype of PRS. Haploinsufficiency for FBN2 accounts for the digital and auricular features. A possible critical region for TEV is distinct and telomeric to the PRS region. The molecular basis of PRS in these cases remains undetermined but haploinsufficiency for PHAX is a plausible mechanism.

Pierre Robin Sequence (PRS) is a congenital abnormality characterized by mandibular hypoplasia, glossoptosis and often secondary palate cleft. It may be an isolated or part of a most complicated syndrome. The genetic syndrome that most frequently co-occurs is Stickler syndrome characterized by skeletal abnormalities, joint pain, congenital myopia and retinal detachment. The authors describe their fast and early mandibular osteodistraction (FEMOD) protocol in severe cases of PRS airway obstruction.

The laryngeal mask airway (LMA) Classic™ and Air-Q® are supralaryngeal devices used for airway management in routine and difficult pediatric airways. We describe a novel two-stage technique of insertion of the LMA Classic™ awake prior to induction of anesthesia, to assure oxygenation and ventilation, and after induction removal and placement of the Air-Q® for intubation using the flexible fiberoptic bronchoscope. The LMA Classic's™ pliable design and relatively small size allow it to be easily placed in awake infants. In contrast, the Air-Q® is an excellent device for intubation because of its larger internal diameter and removable 9 mm adapter. Our goal was to reduce unpredictability and potentially increase the safety of induction of anesthesia and intubation in infants with Pierre Robin sequence. By using these devices in a two-stage approach we created a technique for consistent oxygenation, ventilation, and intubation in these infants.

Introduction This problem-based learning (PBL) case contains an unusual association of two conditions. Specifically, it highlights the challenges inherent in safely anesthetizing an uncooperative infant with a potentially difficult airway and full stomach, and highlights the conflicting demands each complication places on the optimal anesthesia technique. This example encourages consideration of the clinical and practical anesthetic implications of two relatively uncommon conditions of early infancy in isolation, then to build upon this foundation by considering the impact that each has on the management of the other in the very rare instances where they may co-exist. Many of the principles so considered are generalizable to similar (but not identical) clinical dilemmas. Methods The material is presented as a narrative case history which poses several key questions crucial to the elucidation of optimal ongoing management. Each of these is expanded upon in the subsequent discussion along with included references and figures. Results This resource was originally presented as an abstract, and then as a PBL to faculty participants at a national pediatric anesthesia conference. It received uniformly enthusiastic evaluation from attendees and is worthy of a wider audience. Discussion The case is intended to stimulate group discussion and should complement relevant clinical experience and self-directed learning. It is primarily aimed at physician-trainees in a pediatric anesthesia fellowship program. The resource also provides an overview of the broader topics of anesthetic management of infantile pyloric stenosis and Pierre Robin sequence as well as current thinking about the optimal approach to managing general anesthesia in an infant so afflicted.

Pierre Robin Sequence is a congenital condition involving a combination of micrognathia and glossoptosis with or without a cleft palate. Feeding problems are often associated with cleft anamoly which make it difficult for the infant to maintain adequate nutrition. Here we present a case of 58-day-old neonate with Pierre Robin sequence, in whom we contructed a palatal obturtor for feeding.

INTRODUCTIONA Tübingen palate plate and early cleft closure for successful airway management is described in a newborn with Pierre Robin sequence.PRESENTATION OF CASEA three-day-old newborn with an acute airway obstruction underwent primary treatment with a Tübingen soft palate plate.DISCUSSIONAfter stabilization of the airway, the cleft soft palate was closed after three months.CONCLUSIONThe Tübingen soft palate plate proved to be a very satisfactory treatment for the infant.

Congenital cervico-thoracic kyphosis has been encountered in a girl with Pierre Robin sequence. The constellation of the spine malformation complex such as incomplete development of the vertebral bodies associated with defective ossification of the cervico-thoracic pedicles causing effectively the development of complete spinal cord injury at the kyphotic level of C7/T1 were present. Congenital kyphosis secondary to vertebral body hypoplasia has not been reported in connection with Pierre Robin sequence., GMS German Medical Science; 9:Doc06; ISSN 1612-3174

Pierre Robin sequence (PRS) or anomalad is one of the most readily recognized presentation, and results of the first brachial arch malformation. PRS presents a classic triad of micrognathia, glossoptosis, and cleft palate. Infants with PRS can present with varied problems, some of them emergencies. However, in infants with a complete cleft palate, most commonly encountered problems are with feeding. This article describes the clinical and laboratory procedures for construction of a feeding plate in a neonate with PRS due to the presence of a cleft palate.

Neonates with Pierre Robin Sequence (PRS) usually present with varying degrees of upper airway obstruction and difficulty feeding. Early treatment is important for such children in order to prevent impaired cognitive development resulting from hypoxemic episodes. Various procedures aimed at widening the pharyngeal space have been proposed, including prone position, tongue-lip adhesion, mandibular traction, non-invasive ventilation and palatal plates. Mandibular distraction osteogenesis (MDO) using external or internal devices has become increasingly popular as an alternative treatment option when other medical or surgical techniques do not prove to be satisfactory. This review summarizes current evidence on the effectiveness of MDO in infants with PRS. Because of a lack of studies comparing this treatment with other procedures, general recommendations cannot be drawn and treatment of infants with PRS still requires individualization.