초록
왜소악, 설하수, 구개열의 세 가지 특징적 증상과 더불어 간헐적인 청색증, 흉곽의 함몰, 기도 폐색에 의한 호흡 곤란과 수유곤란 등의 임상소견을 나타낸 3례의 Pierre Robin sequence 환아에서, 혀의 위치를 교정하기 위한 구강저골막하박리술과 혀를 전방으로 위치시켜 호흡 곤란과 기도 폐색을 예방하기 위한 설구순접합술과 동시에 시행하고 약 1년 경과 후 설구순분리술을 시행한 결과 호흡 곤란과 수유장애의 해소, 혀의 전방 재위치, 체중 증가 및 하악골의 성장 증진 등 만족할 만한 결과를 얻었다. 구강저골막하박리술과 구순접합술 동시 시술은 Pierre Robin sequence 환아에서 기도 폐색의 외과적 치료를 위한 간단하고도 신뢰할만한 방법이라고 사료된다.
The deformities of micrognathia and glossoptosis in the newborn are frequently associated with a cleft palate, which is known as Pierre Robin sequence. Upper airway obstruction is the most serious problem in these patients. Treatment of Pierre Robin sequence includes either positional or surgical intervention. Mild cases are often managed in the prone position. However, when the patient fails to thrive due to chronic upper airway obstruction, or severe respiratory distress ensures despite positional treatment, surgical intervention is mandatory to relieve the obstruction. We experienced three infants with Pierre Robin sequence who showed a symptom triad of micrognathia, glossoptosis, and cleft palate. Intermittent cyanosis, depression of the chest, respiratory difficulty and feeding problems were also observed. To relieve severe upper airway obstruction caused by micrognathia and glossoptosis, we simultaneously performed modified tongue lip adhesion (TLA) and a subperiosteal release of the floor of the mouth (SRFM). Respiratory and feeding difficulties were relieved, the tongue positioned anteriorly, body weight increased, and mandibular growth improved. Simultaneous TLA and SRFM may constitute a simple and reliable method for surgical treatment of airway obstruction in patients with Pierre Robin sequence.
