Pierre Robin 综合征17例临床分析

Clinical analysis of 17 cases of Pierre Robin syndrome

目的：探讨Pierre Robin综合征患儿的临床特征及诊治经验，为临床提供诊治依据。方法回顾性分析2008年9月至2013年8月本院新生儿中心收治的17例Pierre Robin综合征患儿的临床诊治资料，总结Pierre Robin综合征的临床特点及诊治要点。结果⑴17例Pierre Robin综合征患儿均具有小颌畸形、腭裂或高腭弓、舌下坠及喂养困难等典型临床特征；⑵17例患儿按临床分级给予综合治疗，其中3例死亡（1例死于严重肺炎，2例放弃治疗患儿出院后死于严重呛咳，合并肺炎及严重营养不良）；⑶14例存活者中2例失访，12例患儿获随访，其中5例在生后1年生长发育指标与正常同龄儿童相仿，7例出院后存在不同程度喂养困难，生长发育落后于正常同龄儿童。结论临床医生深刻认识Pierre Robin综合征的临床特征及诊断要点，可早期诊断本病，按本病的分级制订个体化的治疗方案处理，并将喂养技能传授给家长，能有效改善患儿的预后。

Objective To explore the clinical characteristics of Pierre Robin syndrome and the experience of its diagnosis and therapy, and provide the accurate diagnosis and treatment of Pierre Robin syndrome .Methods Seventeen cases of Pierre Robin syn-drome from September 2008 to August 2013 were enrolled in this study .The clinical data were analyzed retrospectively to explore the clinical characteristics and treatment of Pierre Robin syndrome .Results Seventeen cases of Pierre Robin syndrome had typical clini-cal features, including micrognathia, cleft palate or high palatine arches, glossoptosis, and feeding difficulties.The babies were given corresponding treatment according to their clinical grading. Among those babies, three cases died , including one dying of severe pneu-monia in hospital, and the other two dying of severe pneumonia and malnutrition after giving up treatment .Among 14 survivors, 12 ba-bies were followed up , including 5 babies who achieved optimal growth and development when they were one year old , and the other 7 babies who had feeding difficulties in varying degrees after discharge , and lagged behind normal children in the growth and develop-ment.Conclusions Early diagnosis, accurate classification, and individualized treatment plan for Pierre Robin syndrome might im-prove the prognosis of children with this type of disease .