Pierre Robin序列征(PRS)是一种病因复杂发病机制尚不明确的先天性发育畸形。主要表现为小下颌、腭裂、舌后坠伴气道狭窄,可导致患儿出生后呼吸及喂养困难,该病病死率较高,严重威胁新生儿的生命安全。本文患儿于孕30周左右早产且出生后...Pierre Robin序列征(PRS)是一种病因复杂发病机制尚不明确的先天性发育畸形。主要表现为小下颌、腭裂、舌后坠伴气道狭窄,可导致患儿出生后呼吸及喂养困难,该病病死率较高,严重威胁新生儿的生命安全。本文患儿于孕30周左右早产且出生后即出现呼吸困难,后经抢救无数后死亡,现回顾性分析PRS胎儿磁共振表现及相关文献复习,对该病的发病机制、病因、临床诊断及治疗措施进行深入了解,提高大家对该病的认识,从而改善患儿出生后生存情况。展开更多
The triad of micrognathia, glossoptosis and airway obstruction originally described in 1923 by Pierre Robin, is known as Robin sequence (or Pierre robin sequence “PRS”). PRS is characterized by micrognathia (small a...The triad of micrognathia, glossoptosis and airway obstruction originally described in 1923 by Pierre Robin, is known as Robin sequence (or Pierre robin sequence “PRS”). PRS is characterized by micrognathia (small and symmetrical receded mandible), glossoptosis (tongue of variable size falls backwards into the post pharyngeal wall), and cleft palate (U or V shaped). We report a case of 2 hours old newborn presented with micrognathia, retrognathia, and glossoptosis and absent anterior two thirds of tongue.展开更多
Treacher Collins syndrome is caused by the maldevelopment of the first and second branchial arches. It is characterized by mandibulofacial dysostosis and has the following features: outward and downward obliquity of l...Treacher Collins syndrome is caused by the maldevelopment of the first and second branchial arches. It is characterized by mandibulofacial dysostosis and has the following features: outward and downward obliquity of lateral canthi, colobomas of the lateral 1/3 of. the lower eyelids, flattening of malar bone, absence of zygomatic arches, a small and receding chin, sometimes accomapanied with small ears and cleft palate. In 8 cases, the authors reconstructed malar hypoplasia and micrognathia with bone graft and corrected the palpebral fissures. The results of this procedure are satisfactory.展开更多
Patients with Treacher Collins Syndrome (TCS) present unique airway management problems for anesthesiologists due to mandibular micrognathia, the small oral aperture, and temporomandibular joint anomalies. We describe...Patients with Treacher Collins Syndrome (TCS) present unique airway management problems for anesthesiologists due to mandibular micrognathia, the small oral aperture, and temporomandibular joint anomalies. We describe the case of a pediatric TCS patient with limited mouth opening who experienced severe airway obstruction during deep inhalation anesthesia (sevoflurane following i.v. midazolam) for routine dental work. When difficult airway management is expected, intubation of conscious patients is a well-recognized technique in adults;however, it is rarely appropriate for pediatric patients who usually do not cooperate. According to general anesthesia algorithms for pediatric patients with difficult airways, in most pediatric patients, tracheal intubation is performed after the induction of general anesthesia and some authors have reported the usefulness of LMA for maintaining airway patency in patients with TCS. However, in our case LMA could not be used because of severe limitation of mouth opening. In addition, the LMA is so bulky that it is impossible to insert a LMA into patients with narrow airway anatomy. We initially planned to carry out fiber-optic intubation while awake and under sedation if the airway patency could not be secured after the induction of anesthesia. The patient was sedated properly with midazolam and sevoflurane, and awake fiberoptic intubation was performed uneventfully. Our experience in this case highlighted that careful planning of backup contingencies is important in achieving fiberoptic intubation and maintaining airway patency in pediatric TCS patients with limited mouth opening, and that awake intubation can be successful even in pediatric patients.展开更多
Pierre Robin sequence(PRS)is a triad of micrognathia,glossoptosis,and cleft palate that results in an obstruction of the airway on inspiration and impeding feeding.The tongue of infants with PRS fall back toward the p...Pierre Robin sequence(PRS)is a triad of micrognathia,glossoptosis,and cleft palate that results in an obstruction of the airway on inspiration and impeding feeding.The tongue of infants with PRS fall back toward the posterior pharyngeal wall(glossoptosis)due to receding chin produced by mandibular micrognathia(small jaw)or retrognathia.This causes a serious condition with potentially severe,life-threatening airway obstruction.If untreated,this problem can lead to exhaustion,cardiac failure,and ultimately death,especially during the early months of life.Actually,in the majority of PRS infants,these symptoms can be managed by placing the infant in the prone position until adequate growth of the jaw occurs.If this type of treatment fails,the infant then should be considered for other conservative therapies or surgical interventions.This paper reviews surgical interventions such as tongue-lip adhesion,mandibular traction,mandibular distraction,tracheotomy and conservative orthodontic approaches,and presents a baby treated successfully with an orthodontic appliance.展开更多
文摘The triad of micrognathia, glossoptosis and airway obstruction originally described in 1923 by Pierre Robin, is known as Robin sequence (or Pierre robin sequence “PRS”). PRS is characterized by micrognathia (small and symmetrical receded mandible), glossoptosis (tongue of variable size falls backwards into the post pharyngeal wall), and cleft palate (U or V shaped). We report a case of 2 hours old newborn presented with micrognathia, retrognathia, and glossoptosis and absent anterior two thirds of tongue.
文摘Treacher Collins syndrome is caused by the maldevelopment of the first and second branchial arches. It is characterized by mandibulofacial dysostosis and has the following features: outward and downward obliquity of lateral canthi, colobomas of the lateral 1/3 of. the lower eyelids, flattening of malar bone, absence of zygomatic arches, a small and receding chin, sometimes accomapanied with small ears and cleft palate. In 8 cases, the authors reconstructed malar hypoplasia and micrognathia with bone graft and corrected the palpebral fissures. The results of this procedure are satisfactory.
文摘Patients with Treacher Collins Syndrome (TCS) present unique airway management problems for anesthesiologists due to mandibular micrognathia, the small oral aperture, and temporomandibular joint anomalies. We describe the case of a pediatric TCS patient with limited mouth opening who experienced severe airway obstruction during deep inhalation anesthesia (sevoflurane following i.v. midazolam) for routine dental work. When difficult airway management is expected, intubation of conscious patients is a well-recognized technique in adults;however, it is rarely appropriate for pediatric patients who usually do not cooperate. According to general anesthesia algorithms for pediatric patients with difficult airways, in most pediatric patients, tracheal intubation is performed after the induction of general anesthesia and some authors have reported the usefulness of LMA for maintaining airway patency in patients with TCS. However, in our case LMA could not be used because of severe limitation of mouth opening. In addition, the LMA is so bulky that it is impossible to insert a LMA into patients with narrow airway anatomy. We initially planned to carry out fiber-optic intubation while awake and under sedation if the airway patency could not be secured after the induction of anesthesia. The patient was sedated properly with midazolam and sevoflurane, and awake fiberoptic intubation was performed uneventfully. Our experience in this case highlighted that careful planning of backup contingencies is important in achieving fiberoptic intubation and maintaining airway patency in pediatric TCS patients with limited mouth opening, and that awake intubation can be successful even in pediatric patients.
文摘Pierre Robin sequence(PRS)is a triad of micrognathia,glossoptosis,and cleft palate that results in an obstruction of the airway on inspiration and impeding feeding.The tongue of infants with PRS fall back toward the posterior pharyngeal wall(glossoptosis)due to receding chin produced by mandibular micrognathia(small jaw)or retrognathia.This causes a serious condition with potentially severe,life-threatening airway obstruction.If untreated,this problem can lead to exhaustion,cardiac failure,and ultimately death,especially during the early months of life.Actually,in the majority of PRS infants,these symptoms can be managed by placing the infant in the prone position until adequate growth of the jaw occurs.If this type of treatment fails,the infant then should be considered for other conservative therapies or surgical interventions.This paper reviews surgical interventions such as tongue-lip adhesion,mandibular traction,mandibular distraction,tracheotomy and conservative orthodontic approaches,and presents a baby treated successfully with an orthodontic appliance.
