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目的探讨Pierre Robin序列征患儿的临床特点及随访情况,为合理诊治及随访提供依据。方法回顾性分析2011年1月至2014年12月本院足月新生儿重症监护病房收治的Pierre Robin序列征患儿的临床资料,总结患儿一般资料、临床表现、辅助检查、治疗、转归和随访情况。结果 11例Pierre Robin序列征患儿均具有小下颌畸形、腭裂、舌后坠及喂养困难、呼吸困难等典型临床特征,主要合并症有颅面部畸形、心血管畸形、听力损伤、染色体疾病等,11例患儿临床分级1级4例,2级3例,3级4例,按临床分级给予综合治疗,其中2例死亡,3例放弃治疗患儿出院后2例死于严重呛咳、肺炎及严重营养不良,1例存活。6例病情好转出院的患儿中,2例失访,4例随访患儿中2例生长发育指标与正常同龄儿童相仿,2例生长发育较正常同龄儿童落后,腭裂严重的1例患儿3个月时行腭裂修复术和下颌骨牵拉骨成形术。结论掌握Pierre Robin序列征的临床特征及诊断要点有助于早期诊断本病,按本病分级制定个体化治疗方案能有效改善患儿预后。
Objective To investigate the clinical features and follow-up of children with Pierre Robin syndrome and to provide a basis for reasonable diagnosis and follow-up. Methods The clinical data of Pierre Robin sequence from January 2011 to December 2014 in our hospital full-term neonatal intensive care unit were retrospectively analyzed. The general information, clinical manifestations, auxiliary examination, treatment and outcome of children were summarized And follow-up situation. Results Eleven patients with Pierre Robin sequence had typical clinical features such as small mandibular deformity, cleft palates, tongue falling and feeding difficulties, dyspnea and so on. The main complications were craniofacial deformity, cardiovascular malformations, hearing impairment and chromosomal diseases. 11 cases of children with grade 1 in 4 cases, grade 2 in 3 cases and grade 3 in 4 cases were classified according to clinical classification, of which 2 died and 3 gave up treatment. Two patients died of severe cough and pneumonia after discharge And severe malnutrition, 1 case survived. Of the 6 children whose condition improved after discharge, 2 patients were lost to follow-up. The growth and development of 2 children in 4 cases were similar to those of normal children. Two children whose growth and development were lagging behind normal children and one case of severe cleft palate Month cleft palate repair and mandibular traction osteoplasty. Conclusion To master the clinical features and diagnostic points of Pierre Robin sequence will help early diagnosis of this disease. To formulate individualized treatment plans according to the disease grade can effectively improve the prognosis of children.