Case report of Pierre Robin sequence with severe upper airway obstruction who was rescued by fiberoptic nasotracheal intubation.
Case report of Pierre Robin sequence with severe upper airway obstruction who was rescued by fiberoptic nasotracheal intubation.
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DOI：
10.1186/s12871-017-0336-0
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发表时间：
2017-03-14
影响因子：
2.2
通讯作者：
Yamada Y
中科院分区：
文献类型：
--
作者：
Takeshita S;Ueda H;Goto T;Muto D;Kakita H;Oshima K;Tainaka T;Ono T;Kazaoka Y;Yamada Y
Pierre Robin序列（PRS）是指小颌畸形、舌下垂和气道阻塞的相关性。出生后立即因上呼吸道阻塞而引起严重呼吸困难的病例非常罕见。我们在此报告两例呼吸窘迫综合征患者，他们在出生后立即因形态异常而出现严重的呼吸困难，并经纤维鼻气管插管抢救。例1为小颌畸形伴腭裂，舌经腭裂突入鼻腔。他的舌内陷被认为是一种极端类型的舌下垂，他被诊断为皮埃尔罗宾序列。例2患者与例1相同，也有小颌畸形和腭裂，并伴有一定程度的腭裂。她的染色体分析证实了1p36缺失综合征的诊断，她被诊断为1p36缺失综合征合并Pierre Robin序列。在这两种情况下，舌头突出到鼻腔通过腭裂占据咽和鼻腔，导致严重的呼吸困难。喉镜下只能看到舌的背面，口咽插管是不可能的。因此，进行了光纤经鼻气管插管，以确保气道复苏。我们的结论是，极端类型的舌下垂在PRS包括舌陷入鼻腔，以前没有报道，这种情况下，需要复苏的纤维光学插管出生后立即。因此，外科医生应掌握纤维插管的技能。
Pierre Robin sequence (PRS) refers to the association of micrognathia, glossoptosis, and airway obstruction. Cases with severe dyspnea due to upper airway obstruction immediately after birth are very rare. We here report two cases with PRS who developed severe dyspnea due to morphological abnormality immediately after birth and were rescued by fiberoptic nasotracheal intubation. The patient in case 1 had micrognathia and cleft palate, and his tongue protruded into the nasal cavity via a cleft palate. His invaginated tongue was considered an extreme type of glossoptosis and he was diagnosed as Pierre Robin sequence. The patient in case 2 also had micrognathia and cleft palate same as case 1 and accompanied some anomalad. Her chromosome analysis confirmed a diagnosis of 1p36 deletion syndrome and she diagnosed as 1p36 deletion syndrome complicated with Pierre Robin sequence. In both cases, tongue protruded into the nasal cavity via a cleft palate occupied pharynx and nasal cavity, resulting in severe dyspnea. Only the backside of the tongue was visible by laryngoscopy and oropharyngeal intubation was impossible. Therefore, fiberoptic nasotracheal intubation was done to secure the airway for resuscitation. We conclude that extreme type of glossoptosis in PRS concludes tongue invaginated into nasal cavity which have not reported before and that such cases require resuscitation by fiberoptic intubation immediately after birth. As such, neonatologists should obtain the skill of fiberoptic intubation.