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Clinics in Plastic Surgery Apr 2019Pierre Robin sequence consists of clinical triad of micrognathia, glossoptosis, and airway compromise with variable inclusion of cleft palate. Evaluation of airway... (Review)
Review
Pierre Robin sequence consists of clinical triad of micrognathia, glossoptosis, and airway compromise with variable inclusion of cleft palate. Evaluation of airway obstruction includes physical examination, polysomnography for obstruction events, and a combination of nasoendoscopy and bronchoscopy to search for synchronous obstructive lesions. A multidisciplinary approach is required given the high rate of syndromic disease. Management of airway obstruction and feeding starts with nonsurgical maneuvers, such as prone and lateral positioning, nasopharyngeal stenting, and continuous positive airway pressure. Surgical management includes mandibular distraction and tongue-lip adhesion. Subglottic obstruction and central sleep apnea may best be treated with tracheostomy.
Topics: Airway Obstruction; Female; Humans; Infant; Male; Mandible; Mouth; Osteogenesis, Distraction; Pierre Robin Syndrome; Polysomnography; Tracheostomy
PubMed: 30851756
DOI: 10.1016/j.cps.2018.11.010 -
The Journal of Craniofacial Surgery Mar 2018The Pierre Robin sequence (PRS) has been defined as the presence of micrognathia, glossoptosis, and respiratory obstruction in the neonatal period. Since its original... (Review)
Review
BACKGROUND
The Pierre Robin sequence (PRS) has been defined as the presence of micrognathia, glossoptosis, and respiratory obstruction in the neonatal period. Since its original description, different therapeutic approaches have been proposed obtaining different success rates, but there is no consensus about its management.
METHODS
A literature review was conducted in PubMed, Embase, and Cochrane databases, for the period of January,1985 to November, 2016. A number of 23 articles resulting from clinical studies, discussing diagnostic tests or therapeutic approaches, and directly or indirectly comparing diagnostic or treatment modalities were selected and assessed using the GRADE methodology.
RESULTS
After reviewing and analyzing the selected articles, an evidence-based algorithm for diagnosis and integral management of PRS patients was designed.
CONCLUSION
Based on the anatomical principles and natural evolution of PRS, the clinical scenario must be evaluated thoroughly as a dynamic event to develop a management sequence that minimizes morbidity and mortality and accelerates patients' reinsertion to normal life.
Topics: Airway Obstruction; Glossoptosis; Humans; Micrognathism; Pierre Robin Syndrome
PubMed: 29215441
DOI: 10.1097/SCS.0000000000004178 -
Journal of Stomatology, Oral and... Nov 2018Pierre Robin syndrome (PRS) is characterized of a triad of clinical signs: micrognathia, glossoptosis and obstruction of the upper airways frequently associated with... (Review)
Review
Pierre Robin syndrome (PRS) is characterized of a triad of clinical signs: micrognathia, glossoptosis and obstruction of the upper airways frequently associated with palatal cleft. It is a heterogenic pathological entity and it can be found as isolated disease (nsPRS) or in association with other syndromes (sPRS), with more pronounced symptoms and systemic involvement. This review aims to summarize the principal features of PRS, analysing the different aspects of the disease. Epidemiological data highlight incidence, severity and mortality of PRS; pathophysiological mechanism reports the etiology and pathogenesis of the disease distinguishing between isolated and syndromic form. Because of the clinical importance of PRS, it's fundamental to describe the features of the Robin sequence to clearly define its primary and secondary clinical signs useful to diagnosis. A complete evaluation of the syndrome allows choosing the most appropriate therapeutic treatment, opting for conservative or surgical management, in order to improve the quality of life of the patient.
Topics: Airway Obstruction; Glossoptosis; Humans; Micrognathism; Pierre Robin Syndrome; Quality of Life
PubMed: 29777780
DOI: 10.1016/j.jormas.2018.05.002 -
Anesthesia and Analgesia Aug 2014The clinical triad of micrognathia (small mandible), glossoptosis (backward, downward displacement of the tongue), and airway obstruction defines the Pierre Robin... (Review)
Review
The clinical triad of micrognathia (small mandible), glossoptosis (backward, downward displacement of the tongue), and airway obstruction defines the Pierre Robin sequence (PRS). Airway obstruction and respiratory distress are clinical hallmarks. Patients may present with stridor, retractions, and cyanosis. Severe obstruction results in feeding difficulty, reflux, and failure to thrive. Treatment options depend on the severity of airway obstruction and include prone positioning, nasopharyngeal airways, tongue lip adhesion, mandibular distraction osteogenesis, and tracheostomy. The neonate and infant with PRS require care from multiple specialists including anesthesiology, plastic surgery, otolaryngology, speech pathology, gastroenterology, radiology, and neonatology. The anesthesiologist involved in the care of patients with PRS will interface with a multidisciplinary team in a variety of clinical settings. This perioperative review is a collaborative effort from multiple specialties including anesthesiology, plastic surgery, otolaryngology, and speech pathology. We will discuss the background and clinical presentation of patients with PRS, as well as some of the controversies regarding their care.
Topics: Airway Obstruction; Anesthesia; Cooperative Behavior; Feeding Methods; Humans; Infant; Infant, Newborn; Interdisciplinary Communication; Otorhinolaryngologic Surgical Procedures; Patient Care Team; Patient Positioning; Perioperative Care; Pierre Robin Syndrome; Plastic Surgery Procedures; Respiratory Insufficiency; Treatment Outcome
PubMed: 25046788
DOI: 10.1213/ANE.0000000000000301 -
Clinics in Perinatology Dec 2018Pierre Robin sequence (PRS) is a congenital condition characterized by the presence of micrognathia, glossoptosis, and cleft palate. PRS has varying effects on airway... (Review)
Review
Pierre Robin sequence (PRS) is a congenital condition characterized by the presence of micrognathia, glossoptosis, and cleft palate. PRS has varying effects on airway patency and feeding ability and thus has a broad range of management options. The purpose of this article is to describe the nature of the background of the condition and address the previous and current trends in diagnosis and management of PRS.
Topics: Abnormalities, Multiple; Airway Management; Airway Obstruction; Female; Humans; Infant, Newborn; Male; Perinatology; Pierre Robin Syndrome; Postpartum Period; Prognosis; Plastic Surgery Procedures; Treatment Outcome; Ultrasonography, Prenatal
PubMed: 30396414
DOI: 10.1016/j.clp.2018.07.009 -
Paediatric Respiratory Reviews Mar 2023Pierre Robin Sequence (PRS) is defined by a constellation of characteristics including micrognathia, glossoptosis and airway obstruction. PRS can occur in isolation or... (Review)
Review
Pierre Robin Sequence (PRS) is defined by a constellation of characteristics including micrognathia, glossoptosis and airway obstruction. PRS can occur in isolation or can be associated with syndromes and another anomalies. Airway obstruction and feeding difficulties are the major presenting issues, and the severity of the condition ranges from mild, with minimal to no symptoms, to severe, with overt obstruction resulting in apnoeas, severe respiratory distress and cyanosis. The presence of airway obstruction can result in obstructive sleep apnoea and abnormalities in gas exchange, as well as exacerbation of already present feeding difficulties and failure to thrive, secondary to mismatch of caloric intake to energy usage associated with increased effort of breathing. Management of airway obstruction for infants with PRS varies between centres. This paper explores the surgical and non-surgical management options available, their effectiveness and pitfalls in children with PRS. Despite the pros and cons of each management option, it is evident that resource availability and multidisciplinary clinical support are key factors to successful management.
Topics: Infant; Child; Humans; Pierre Robin Syndrome; Osteogenesis, Distraction; Airway Obstruction; Sleep Apnea, Obstructive; Dyspnea; Treatment Outcome
PubMed: 35987882
DOI: 10.1016/j.prrv.2022.07.001 -
Pediatric Neurology Apr 2023The Pierre Robin Sequence presents heterogeneous symptoms, and each newborn can manifest from mild breathing and feeding difficulties to severe complications, as well as...
BACKGROUND
The Pierre Robin Sequence presents heterogeneous symptoms, and each newborn can manifest from mild breathing and feeding difficulties to severe complications, as well as a predisposition to present changes in growth and neuropsychomotor development in the first years of life.
OBJECTIVE
The aims were to evaluate and associate the neuropsychomotor development of zero- to 12-month-old children with Pierre Robin sequence (PRS) in the personal-social, fine motor-adaptive, language, and gross motor aspects.
METHODS
The subjects of the study were 17 infants of both sexes with PRS admitted to the special care unit (SCU) of a reference hospital in the interior of the state of São Paulo, Brazil, in the age range of 20 days to 263 days. Developmental assessments were performed using the Denver Development Screening Test II. The evaluations were carried out in the SCU, with duration of 30 minutes each. Statistical analysis was descriptive using the Mann-Whitney test, two-proportion equality test, and Spearman correlation. The level of significance was set at 0.05.
RESULTS
According to Denver Development Screening Test II, median 78.5 of the babies were at risk for developmental delay identified by the Denver II Test (n = 14, 82.4%). For the developmental areas analyzed by the test there was statistically significant difference in language area.
CONCLUSION
The babies aged up to 12 months with PRS in this study presented risks for delay in neuropsychomotor development in language, gross motor, fine motor-adaptive, and personal-social aspects, and this finding should be considered to set goals in family orientation and intervention.
Topics: Infant; Infant, Newborn; Male; Child; Female; Humans; Aged; Pierre Robin Syndrome; Brazil; Language; Retrospective Studies
PubMed: 36774683
DOI: 10.1016/j.pediatrneurol.2023.01.010 -
Acta Medica (Hradec Kralove) 2020Pierre Robin sequence (PRS) is characterized by the triad of retrognathia, glossoptosis, and airway obstruction. PRS may occur in isolation or in conjunction with other... (Review)
Review
Pierre Robin sequence (PRS) is characterized by the triad of retrognathia, glossoptosis, and airway obstruction. PRS may occur in isolation or in conjunction with other syndromes. Distinguishing isolated and syndromic forms of PRS helps clinicians decide the management plan. We describe two cases of PRS of Indian ethnicity and describe some of the difficulties that we faced while distinguishing isolated PRS from syndromic PRS. Both cases had a similar clinical presentation. However, one of the cases had a positive family history of congenital deafness and cleft palate, whereas the other case had apparent upper limb anomalies. These facts heightened the suspicion of an associated syndrome. However, based on the available facts and after thorough investigations, a tentative diagnosis of isolated PRS was made for both the patients. Both the cases were managed conservatively and were advised a long-term follow-up. When the associated anomalies are few, minor or concealed at birth, longitudinal follow-up of all PRS cases combined with thorough diagnostics including chromosomal analysis could help differentiate syndromic PRS from isolated PRS. Regardless, all cases of PRS require a multidisciplinary approach.
Topics: Humans; Infant; Infant, Newborn; Male; Pierre Robin Syndrome; Prone Position; Respiratory Insufficiency
PubMed: 32771075
DOI: 10.14712/18059694.2020.23 -
The British Journal of Oral &... Sep 2023Pierre Robin Sequence (PRS) is a congenital craniofacial anomaly distinguished by the presence of micrognathia, glossoptosis, and upper airway obstruction. Cleft palate... (Review)
Review
Pierre Robin Sequence (PRS) is a congenital craniofacial anomaly distinguished by the presence of micrognathia, glossoptosis, and upper airway obstruction. Cleft palate occurs in over 3/4 of patients with PRS. The wide U-shape cleft and airway dysfunction create challenges in clinical management. Currently, disputes exist on the treatment protocol and prognosis of cleft palate management among patients with PRS. This review is focused on the deformity features, intervention timing, technique selection, airway support, and outcome evaluation of cleft palate among patients with PRS, aiming to provide reference to further evolution in the management of PRS-related cleft palate.
Topics: Humans; Cleft Palate; Airway Obstruction; Pierre Robin Syndrome; Prognosis; Outcome Assessment, Health Care
PubMed: 37453893
DOI: 10.1016/j.bjoms.2023.06.003 -
Journal of Family Health Mar 2017
Review
Topics: Airway Management; Feeding Methods; Humans; Infant; Infant, Newborn; Pierre Robin Syndrome
PubMed: 29727101
DOI: No ID Found