Tomáz Francisnele Maria de Aquino Fraporti, Borges Alvaro Henrique, Borba Alexandre Meireles, Volpato Luiz Evaristo Ricci
Department of Dentistry, University of Cuiabá, Cuiabá, MT, Brazil.
Ann Maxillofac Surg. 2017 Jan-Jun;7(1):104-107. doi: 10.4103/ams.ams_20_16.
Pierre Robin sequence (PRS) is characterized by the triad micrognathia, glossoptosis, and airway obstruction, commonly associated with cleft palate. This study reports the case of a patient with characteristics consistent with the diagnosis of PRS in the 1 week of life. To stabilize the airway, oro-tracheal intubation was performed without success, followed by tracheostomy and glossopexy and after a small improvement in symptoms, it was decided to perform the mandibular distraction osteogenesis (MDO). At the age of 6, her maxillofacial development was within expectations, with patent airway, the presence of deciduous teeth, and without neurological impairment. The MDO described in this case allowed patient's respiratory and feeding improvement, thus avoiding episodes of cyanosis, allowing timely removal of the tracheostomy and glossopexy, and execution of palatoplasty at the right time. While it is uncertain whether MDO will replace the possible need for future orthognathic surgery, treatment greatly improved the quality of life of the patient.
皮埃尔·罗宾序列征（PRS）的特征为小颌畸形、舌后坠和气道阻塞三联征，常伴有腭裂。本研究报告了1例出生1周时具有符合PRS诊断特征的患者。为稳定气道，进行了经口气管插管但未成功，随后进行了气管切开术和舌固定术，症状稍有改善后，决定进行下颌骨牵引成骨术（MDO）。6岁时，她的颌面发育符合预期，气道通畅，有乳牙，且无神经功能障碍。本病例中描述的MDO改善了患者的呼吸和进食情况，从而避免了发绀发作，得以适时拔除气管造口管和解除舌固定术，并适时进行腭裂修复术。虽然不确定MDO是否会取代未来可能需要的正颌手术，但该治疗大大提高了患者的生活质量。
