[Mandibular distraction osteogenesis in patients with craniofacial malformation]. / Distracción mandibular osteogénica en pacientes con malformaciones craneofaciales.

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.

Distraction osteogenesis for obstructive apneas in patients with congenital craniofacial malformations.

Infants with congenital craniofacial malformations often have associated severe mandibular hypoplasia causing obstruction of the hypopharynx by retroposition of the base of the tongue into the posterior pharyngeal airway. Management depends on the severity of the airway obstruction. Most cases can be managed by prone positioning until the infant outgrows the problem at 3 to 6 months of age. In more critical cases, monitoring of oxygen saturation, temporary placement of a nasopharyngeal tube, and placement of an endotracheal tube will be useful procedures. Tracheotomy is an effective method for more severe cases, but longstanding tracheotomies result in high morbidity and occasional mortality. Mandibular distraction was performed in seven patients, ranging in age from 1 to 18 months, with critical obstructive apnea secondary to mandibular hypoplasia characterized by an apnea/hypopnea index greater than 20 apneas per hour and oxygen saturation below 80 percent. Two patients were tracheotomized previously. Mandibular lengthening, from 16 to 25 mm on the left side and from 10 to 22 mm on the right, was achieved in 21 to 25 days. Improvement of airway obstruction parameters was measured on polysomnograms and lateral cephalograms. Mandibular lengthening by gradual distraction is a successful method for young patients with severe mandibular hypoplasia causing critical obstructive apneas. Avoidance of tracheotomy or early decannulation in previously tracheotomized patients is a great advantage for patients with congenital craniofacial malformation.

Síndrome de compresión traqueal y apnea refleja causado por arteria innominada anómala (AIA) / Tracheal compression and reflex apnea syndrome caused by anomalus innominate artery

El origen anómalo de la arteria innominada es una de las causas de síndrome de dificultad respiratoria asociado a "crisis de apnea refleja" en niños. Comunicamos dos pacientes (de 2 y 11 meses de edad) en los cuales fue realizada exitosamente la arteriopexia de la arteria innominada. Ninguno de los dos ha repetido "crisis de apnea refleja" y se encuentran libres los síntomas. Los episodios de "apnea refleja" constituyen una indicación absoluta para el tratamiento quirúrgico (AU)

Síndrome de compresión traqueal y apnea refleja causado por arteria innominada anómala (AIA) / Tracheal compression and reflex apnea syndrome caused by anomalus innominate artery

El origen anómalo de la arteria innominada es una de las causas de síndrome de dificultad respiratoria asociado a "crisis de apnea refleja" en niños. Comunicamos dos pacientes (de 2 y 11 meses de edad) en los cuales fue realizada exitosamente la arteriopexia de la arteria innominada. Ninguno de los dos ha repetido "crisis de apnea refleja" y se encuentran libres los síntomas. Los episodios de "apnea refleja" constituyen una indicación absoluta para el tratamiento quirúrgico