Marañés Gálvez C, Martínez Plaza A, Fernández Valadés R, Liceras Liceras E, Martín Cano F, Cortés Sánchez R, Martínez Tellería A, Cabello A, García Medina B
Unidad Multidisciplinar de Fisuras Labiopalatinas, Servicio de Cirugía Pediátrica, Hospital Universitario Virgen de las Nieves, Granada.
Cir Pediatr. 2011 Apr;24(2):102-8.
Craniofacial malformations (Pierre-Robin sequence, Treacher-Collins syndrome, Nager syndrome, etc.) are frequently associated to severe mandibular hypoplasia, which can cause upper airway obstruction by retroposition of the base of the tongue in the posterior pharyngeal space. Most of the patients respond to postural treatment. In prone decubitus position, it may be necessary to monitor oxygen saturation, insert a nasopharyngeal tube and even an endotracheal one. In more severe cases with prolonged and frequent pauses of apnea, tracheostomy may be necessary, but it is associated with high morbidity and sometimes mortality. In the last two years, in the Multidisciplinary Cleft Lip and Palate Unit of the Hospital Virgen de las Nieves, 4 children with severe obstructive apnea secondary to severe mandibular hypoplasia have been treated with mandibular distraction osteogenesis, this procedure being effective in the resolution of the condition. It has avoided tracheostomy, it has lengthened the jaw in a period of 2-3 weeks. During this time, the obstructive respiratory problems and also swallowing problems have disappeared. The esthetic results were excellent and the complications, for the moment, minimum.
颅面畸形（如皮埃尔 - 罗宾序列征、特雷彻 - 柯林斯综合征、纳格尔综合征等）常与严重的下颌骨发育不全相关，这可通过舌根后移至咽后间隙导致上呼吸道梗阻。大多数患者对体位治疗有反应。在俯卧位时，可能有必要监测血氧饱和度，插入鼻咽管甚至气管内插管。在更严重的病例中，若呼吸暂停持续时间长且频繁发作，可能需要进行气管切开术，但这会带来高发病率，有时甚至导致死亡。在过去两年中，在拉斯涅韦斯圣母医院的多学科唇腭裂治疗单元，4例因严重下颌骨发育不全继发严重阻塞性呼吸暂停的患儿接受了下颌骨牵张成骨治疗，该方法有效解决了这一病症。它避免了气管切开术，在2至3周内延长了下颌骨。在此期间，阻塞性呼吸问题以及吞咽问题均消失。美学效果极佳，目前并发症最少。
