■皮埃尔·罗宾序列(PRS)是一种罕见而复杂的疾病,通常需要来自各个学科的完整医疗团队的合作,以挽救具有这种基因突变的婴儿的生命。以三种临床表现为特征:舌下垂,小颌畸形,和腭裂.治疗主要包括释放上呼吸道阻塞和增加营养,使婴儿过上正常的生活。唇舌粘连程序已在医学文献中被确定为解决与PierreRobin序列相关问题的推荐方法。这种方法在这种情况下被成功采用。
方法:2.5公斤,新生儿舌头位置异常,无法正常母乳喂养和喂养,没有任何医疗,家庭,或者社会历史。经过检查,发现婴儿的舌头位于后部,小颌畸形,和腭裂,导致皮埃尔·罗宾序列的诊断(图。1,2).婴儿手术的准备工作已经开始。婴儿仅通过静脉喂养,并提供氧气面罩25天,直到完成所有必要的咨询,并确认婴儿已准备好进行手术和全身麻醉。手术计划包括将舌头连接到下唇以增强舌头的肌肉力量,解决后位置问题,推迟上颚修复直到1.5岁。
■PRS是一种临床实体,其特征是下颌骨发育不全(小颌)三联征,舌下垂(低渗,缩回的舌头)和呼吸道阻塞,需要多学科团队进行初步评估,管理和维护护理。TLA是增加口咽端口横截面积的一种简单有效的方法。
结论:处理PierreRobin序列中的气道阻塞涉及多种因素,也没有一个通用的治疗方法可以解决所有的病例。适当的气道管理策略和喂养计划对于每个PRS患者至关重要。我们的评论强调,TLA是一种简单的外科手术,短期并发症最少或没有。当需要缓解时,TLA应被视为主要的手术干预措施。
UNASSIGNED: Pierre Robin Sequence (PRS) is a rare and complex condition that often necessitates the collaboration of a full medical team from various disciplines to save the lives of babies with this genetic mutation, characterized by three clinical manifestations: glossoptosis, micrognathia, and cleft palate. Treatment primarily involves freeing upper airway obstructions and enhancing nutrition to allow the babies to lead a normal life. The lip-tongue adhesion procedure has been identified in medical literature as the recommended approach to addressing the issues associated with Pierre Robin sequence, and this method was successfully adopted in this case.
METHODS: 2.5 kg, a newborn male baby with an abnormal position of the tongue and the inability to breastfeed and feed normally, without any medical, family, or social history. Following an examination, it was discovered that the baby had a posterior position of the tongue, micrognathia, and a cleft palate, leading to a diagnosis of Pierre Robin Sequence (Figs. 1, 2). Preparations for the baby\'s surgery have commenced. The baby was solely fed intravenously and provided with an oxygen mask for 25 days until all necessary consultations were completed and the baby\'s readiness for surgery and general anesthesia was confirmed. The surgical plan involved attaching the tongue to the lower lip to enhance the tongue\'s muscular strength, addressing the posterior position issue, and delaying the palate repair until the age of 1.5 years.
UNASSIGNED: PRS is a clinical entity characterized by the triad of mandibular hypoplasia (small jaw), glossoptosis (hypotonic, retracted tongue) and respiratory obstruction that require a multidisciplinary team for initial evaluation and management and maintenance care. TLA is a simple and effective procedure for increasing the cross-sectional area of oropharyngeal port.
CONCLUSIONS: Handling airway obstruction in Pierre Robin Sequence involves various factors, and there is no universal treatment that can address all cases. Appropriate airway management strategies and feeding programs are essential for each individual with PRS. Our review highlights that TLA is a straightforward surgical procedure with minimal or no short-term complications. TLA should be considered as the primary surgical intervention when relief is needed.