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Pierre Robin综合征是指小颌、舌向后垂和裂腭的三联征。病因不详,一种理论认为是由于胎儿在子宫内头呈屈曲位,妨碍了下颌骨向前发育,迫使成长中的舌部在腭板间的后上方生长,影响了腭板融合而形成了三联征。呼吸道阻塞是最严重的症状,应及早加以注意。患儿初起有阵发性紫绀、不安和气道阻塞,尤于仰卧位或入睡放松时明显,上述症状可在出生时即出现,或在出生后6～8周呈进行性加剧。常发生喂养困难,可导致恶病质。本病应与后鼻孔闭锁、气管食管瘘相鉴别,前者可通过鼻前孔插管,后者放入鼻饲管摄胸片来区别。作者改良了Douglas(1946)的手术方法,其操作如下:
Pierre Robin syndrome refers to the triad of the jaw, tongue, dorsal and cleft palate. Etiology is unknown, a theory that is due to the fetus in the womb was first flexion, impeding the development of the mandible forward, forcing the growth of the tongue in the upper and rear above the palatal plate growth, affecting the palate plate fusion and the formation of triple Levy Airway obstruction is the most serious symptom and should be noted as soon as possible. Early onset of children with paroxysmal cyanosis, anxiety and airway obstruction, especially in the supine position or when sleeping to relax significantly, the above symptoms may occur at birth, or 6 to 8 weeks after birth showed progressive aggravating. Frequent feeding difficulties can lead to cachexia. The disease should be after the nostril atresia, tracheal esophageal fistula phase identification, the former through the nasal cavity before intubation, the latter into the nasogastric tube to differentiate. The authors modified the surgical approach of Douglas (1946), which operates as follows: