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The Journal of Craniofacial Surgery Jun 2020Pierre Robin sequence is the constellation of micrognathia, glossoptosis, and tongue-based airway obstruction. When airway obstruction is severe, feeding, growth, and... (Review)
Review
Pierre Robin sequence is the constellation of micrognathia, glossoptosis, and tongue-based airway obstruction. When airway obstruction is severe, feeding, growth, and respiratory demise are at risk. Neonatal mandibular distraction osteogenesis is a technique which improves tongue-based airway obstruction and avoids tracheostomy in patients with severe expressions of Pierre Robin sequence. Its efficacy in relieving airway obstruction is well documented, and it has become the surgical intervention of choice at many craniofacial centers. However, this is an uncommon procedure which can be performed within the first weeks of life, offering little space for a learning curve. The success of neonatal distraction and avoidance of complications is highly dependent on proper surgical technique. This report provides a brief overview of the disease, details the technique of the senior surgeon with captioned videos, describes the protocol used at our institution and reports long-term outcomes with a case description.
Topics: Airway Obstruction; Humans; Infant, Newborn; Larynx; Learning Curve; Mandible; Osteogenesis, Distraction; Pierre Robin Syndrome
PubMed: 32209938
DOI: 10.1097/SCS.0000000000006343 -
International Journal of Pediatric... Apr 2015Pierre Robin sequence is not a rare condition and paediatric specialists caring for respiratory related issues are likely to encounter cases in their practice. There... (Review)
Review
Pierre Robin sequence is not a rare condition and paediatric specialists caring for respiratory related issues are likely to encounter cases in their practice. There have been a few recent reviews on the topic, mostly focusing on the surgical interventions performed for cases with severe airway obstruction. In the present review, we will highlight the different challenges that remain today in the global evaluation of infants afflicted with this condition through a thorough review of the medical literature, giving the clinician a full scope of the disease and of the various management options. The need for an improved objective evaluation of airway obstruction and for a better classification will be emphasized. We are therefore proposing a novel classification scheme that will better account for respiratory and feeding difficulties in these infants. Finally, many knowledge gaps persist regarding this condition, underlining the necessity for further research both in the genetic field and regarding the outcome of therapy.
Topics: Airway Obstruction; Child; Humans; Infant; Lip; Male; Osteogenesis, Distraction; Pierre Robin Syndrome; Polysomnography; Tongue
PubMed: 25704848
DOI: 10.1016/j.ijporl.2015.01.035 -
Neonatal Network : NN Aug 1999The label Pierre Robin sequence is given to infants presenting with a triad of specific congenital anomalies: micrognathia, glossoptosis, and cleft palate. However, this... (Review)
Review
The label Pierre Robin sequence is given to infants presenting with a triad of specific congenital anomalies: micrognathia, glossoptosis, and cleft palate. However, this label should be considered the first, not the final, step in the diagnostic process. In approximately 80 percent of newborns with Pierre Robin sequence, the triad of anomalies is part of an underlying genetic condition. This article reviews the variable etiologies of and general clinical considerations for Pierre Robin sequence. To illustrate how clinical management might vary based on the identification of an underlying condition, three case examples of neonates with Pierre Robin sequence and different underlying genetic conditions are presented.
Topics: Female; Humans; Infant, Newborn; Information Services; Internet; Male; Neonatal Nursing; Pedigree; Pierre Robin Syndrome; Self-Help Groups
PubMed: 10693474
DOI: 10.1891/0730-0832.18.5.13 -
American Journal of Medical Genetics.... Sep 2023Pierre Robin sequence (PRS) is frequently co-occurring with other non-PRS congenital anomalies. The types and the prevalence of anomalies co-occurring with PRS vary in...
Pierre Robin sequence (PRS) is frequently co-occurring with other non-PRS congenital anomalies. The types and the prevalence of anomalies co-occurring with PRS vary in the reported studies. The aims of this report was to study the types and the prevalence of the anomalies co-occurring with PRS in a well-studied population northeastern France. The types and the prevalence of anomalies co-occurring in cases with PRS were ascertained in all terminations of pregnancy, stillbirths and live births in 387,067 births occurring consecutively during the period 1979-2007 in the area covered by our registry of congenital anomalies which is population-based, 89 cases of PRS were registered during the study period with a prevalence of 2.29 per 10,000 births, 69.7% of the cases had associated non-PRS anomalies. Chromosomal abnormalities were present in 10 (11.2%) cases including three 22 q11.2 deletion. Non-chromosomal recognizable conditions were diagnosed in 27 cases (30.3%) including 10 Stickler syndrome, 8 Treacher Collins syndrome, 3 cases with short stature and 6 other syndromes. Multiple congenital anomalies (MCA) were present in 25 cases (28.1%). The most frequent MCA were in the ear, face and neck (35 out of 98 anomalies, 35.7%), cardiovascular (18 anomalies, 18.4%), musculoskeletal (11 anomalies, 11.2%), central nervous (7 anomalies, 7.1%), urinary (6 anomalies, 6.1%), and eye (6 anomalies, 6.1%) system. The high prevalence of associated anomalies justifies a thorough screening for other congenital anomalies in cases with PRS.
Topics: Pregnancy; Female; Humans; Pierre Robin Syndrome; Abnormalities, Multiple; Chromosome Aberrations; Connective Tissue Diseases; France
PubMed: 37477275
DOI: 10.1002/ajmg.a.63344 -
Clinics in Plastic Surgery Apr 2014The neonatal morbidity and mortality associated with Pierre Robin sequence has decreased with improved nonsurgical and surgical methods for airway protection. With the... (Review)
Review
The neonatal morbidity and mortality associated with Pierre Robin sequence has decreased with improved nonsurgical and surgical methods for airway protection. With the introduction of distraction osteogenesis and the understanding that improved outcomes occur with a systematic approach to patient management that includes mandibular distraction for appropriate candidates, the proportion of severely micrognathic patients requiring tracheostomies can be decreased. The challenge to institutions is to implement and optimize systematic methods for evaluation and treatment of these neonates to achieve timely, consistent, and cost-effective outcomes.
Topics: Humans; Infant, Newborn; Mandible; Osteogenesis, Distraction; Pierre Robin Syndrome; Tracheostomy
PubMed: 24607189
DOI: 10.1016/j.cps.2013.12.007 -
Otolaryngologic Clinics of North America Jun 2012This article reviews the various treatment methods for airway obstruction and feeding difficulty in infants with Pierre Robin Sequence (PRS), and highlights the benefits... (Review)
Review
This article reviews the various treatment methods for airway obstruction and feeding difficulty in infants with Pierre Robin Sequence (PRS), and highlights the benefits and limitations of early mandibular distraction osteogenesis in particular as a way of managing both airway obstruction and feeding difficulty in these children.
Topics: Airway Management; Airway Obstruction; Blood Gas Analysis; Child; Gastrostomy; Humans; Laryngoscopy; Lip; Mandible; Osteogenesis, Distraction; Patient Selection; Physical Examination; Pierre Robin Syndrome; Polysomnography; Tongue; Tracheotomy
PubMed: 22588044
DOI: 10.1016/j.otc.2012.03.007 -
International Journal of Pediatric... Aug 2015To review early clinical manifestations of Pierre Robin sequence (PRS) and their management during the first year of life in the University Hospitals Leuven. (Review)
Review
OBJECTIVES
To review early clinical manifestations of Pierre Robin sequence (PRS) and their management during the first year of life in the University Hospitals Leuven.
METHODS
Retrospective series of 48 patients with PRS born between 2001 and 2011 and treated at a tertiary referral hospital. Review of the current literature about management of respiratory and breathing difficulties in the early life of PRS patients.
RESULTS
Of our cleft palate patients 15.3% presented with PRS. A syndrome was diagnosed in 14.6%, associated anomalies without a syndromic diagnosis in 56.3% and isolated PRS in 29.2% of the cases. Mortality rate directly related to PRS was 2.1%. Respiratory difficulties were observed in 83.3% and feeding difficulties in 95.6% of the patients. Respiratory problems were addressed in a conservative way in 75%, in a non-surgical invasive way in 42.5% and in a surgical way in 12.5%. A statistically significant relationship between the association of a syndrome or other anomalies, and a higher need for resuscitation and invasive treatment were found (chi-square test, p-values=0.019 and 0.034). Feeding difficulties were managed conservatively in 91.3%, invasively in 80.4% and surgically in 15.2%.
CONCLUSIONS
PRS is frequently associated with other abnormalities or syndromes. Therefore routine screening for associated anomalies in neonates with PRS is recommendable. Respiratory and feeding complications are highly frequent and possibly severe, particularly in patients with associated anomalies or syndromes, and should be recognized and addressed appropriately in an early stage. There is a potential role for the nasopharyngeal airway in reducing the need for the more traditional surgical interventions for respiratory problems.
Topics: Airway Obstruction; Cleft Palate; Deglutition Disorders; Humans; Infant; Infant, Newborn; Pierre Robin Syndrome; Prevalence; Retrospective Studies; Tertiary Care Centers
PubMed: 26092549
DOI: 10.1016/j.ijporl.2015.05.012 -
Oral and Maxillofacial Surgery Clinics... May 2020Patients with Pierre-Robin sequence recalcitrant to nonsurgical intervention have historically required tracheostomy. Mandibular distraction provides a predictable... (Review)
Review
Patients with Pierre-Robin sequence recalcitrant to nonsurgical intervention have historically required tracheostomy. Mandibular distraction provides a predictable alternative to tracheostomy. Orthodontic perioperative interventions should be considered, including overcorrection, placement of temporary anchorage devices, elastics, and molding the regenerate. Mandibular distraction can be technically difficult and may cause complications. Performed correctly, mandibular distraction provides patients with a better quality of life than tracheostomy.
Topics: Airway Obstruction; Child; Humans; Infant; Mandible; Osteogenesis, Distraction; Pierre Robin Syndrome; Quality of Life; Treatment Outcome
PubMed: 32247440
DOI: 10.1016/j.coms.2020.01.012 -
Seminars in Fetal & Neonatal Medicine Dec 2021There are a wide range of surgical and maxillofacial options to alleviate upper airway obstruction in children with craniofacial disorders. The nasopharyngeal prong... (Review)
Review
There are a wide range of surgical and maxillofacial options to alleviate upper airway obstruction in children with craniofacial disorders. The nasopharyngeal prong (NPP) is a simple idea where the airway obstruction arising from the posteriorly placed tongue secondary to a small mandible can be overcome quickly and without resorting to more invasive surgical procedures. The role of the NPP is of particular interest in Robin sequence (RS). RS describes a congenital anomaly with retrognathia (often with associated U-shaped cleft palate) where in some children the tongue and small jaw can significantly impact on airway patency with upper airway obstruction. The NPP is a modified endotracheal tube, of ideal diameter and cut to a desired length that can bypass the airway obstruction and regain patency to the upper airway. RS has a natural history of mandibular growth and resolution of the symptoms with time and thus the NPP presents a medium term solution precluding the need in selected children of more permanent and potentially unnecessary surgical procedures. The role of the NPP in other craniofacial disorders (either as a temporary solution or longer term option) requires further research.
Topics: Airway Obstruction; Child; Cleft Palate; Humans; Mandible; Pierre Robin Syndrome; Tongue
PubMed: 34602353
DOI: 10.1016/j.siny.2021.101285 -
Clinics in Plastic Surgery Jul 2021Pierre Robin sequence is defined by the clinical triad: mandibular hypoplasia, glossoptosis, and airway obstruction. Mandibular distraction osteogenesis (MDO) is a... (Review)
Review
Pierre Robin sequence is defined by the clinical triad: mandibular hypoplasia, glossoptosis, and airway obstruction. Mandibular distraction osteogenesis (MDO) is a standard treatment of Robin sequence associated with severe airway obstruction and is the only intervention that directly corrects the underlying anatomic pathologic condition. Compared with tongue-lip adhesion, MDO has demonstrated more success in treating airway obstruction in infants with Pierre Robin sequence, including patients with syndromic diagnoses and concomitant anomalies. This article provides a current, comprehensive review of neonatal mandibular distraction and offers treatment guidelines based on a combined surgical experience of more than 400 patients.
Topics: Airway Obstruction; Humans; Infant, Newborn; Mandible; Orthognathic Surgical Procedures; Osteogenesis, Distraction; Pierre Robin Syndrome; Postoperative Complications; Treatment Outcome
PubMed: 34051891
DOI: 10.1016/j.cps.2021.03.005