The airway management and treatment of newborns with micrognathia and laryngomalacia / 临床耳鼻咽喉头颈外科杂志

Objective:To explore the perioperative airway management and treatment of newborns with micrognathia and laryngomalacia. Methods:From January to December 2022, a total of 6 newborns with micrognathia and laryngomalacia were included. Preoperative laryngoscopy revealed concomitant laryngomalacia. These micrognathia were diagnosed as Pierre Robin sequences. All patients had grade Ⅱ or higher symptoms of laryngeal obstruction and required oxygen therapy or non-invasive ventilatory support. All patients underwent simultaneous laryngomalacia surgery and mandibular distraction osteogenesis. The shortened aryepiglottic folds were ablated using a low-temperature plasma radiofrequency during the operation. Tracheal intubation was maintained for 3-5 days postoperatively. Polysomnography（PSG） and airway CT examination were performed before and 3 months after the surgery. Results:Among the 6 patients, 4 required oxygen therapy preoperatively and 2 required non-invasiveventilatory support. The mean age of patients was 40 days at surgery. The inferior alveolar nerve bundle was not damaged during the operation, and there were no signs of mandibular branch injury such as facial asymmetry after the surgery. Laryngomalacia presented as mixed type: type Ⅱ+ type Ⅲ. The maximum mandibular distraction distance was 20 mm, the minimum was 12 mm, and the mean was 16 mm. The posterior airway space increased from a preoperative average of 3.5 mm to a postoperative average of 9.5 mm. The AHI decreased from a mean of 5.65 to 0.85, and the lowest oxygen saturation increased from a mean of 78% to 95%. All patients were successfully extubated after the surgery, and symptoms of laryngeal obstruction such as hypoxia and feeding difficulties disappeared. Conclusion:Newborns with micrognathia and laryngomalacia have multi-planar airway obstruction. Simultaneous laryngomalacia surgery and mandibular distraction osteogenesis are safe and feasible, and can effectively alleviate symptoms of laryngeal obstruction such as hypoxia and feeding difficulties, while significantly improving the appearance of micrognathia.

"Ortoglossopelveplastia" e o algoritmo de sua utilização na sequência de Pierre-Robin / "Orthoglossopelveplasty" and the algorithm for its use in the Pierre Robin sequence

Introdução: Muitos pacientes portadores de sequência de Pierre Robin (micrognatia, glossoptose e obstrução de via aérea) apresentam o músculo genioglosso alterado, encurtado e retrátil, que impede a protração lingual, mantendo a parte anterior da língua verticalizada e seu volume deslocado em direção posterior. Isso pode corroborar para obstrução supraglótica, dificuldade alimentar e inversão das forças de estímulo do crescimento do corpo mandibular. Métodos: Estudo retrospectivo de pacientes com Pierre Robin tratados entre 2012 e 2017 pela equipe, com descrição da "ortoglossopelveplastia", que propõe uma modificação na glossopexia, soltando o genioglosso anômalo da sua inserção, liberando a língua para elevar seu terço anterior e avançar o volume de sua base, sendo auxiliada por ponto de tração da base lingual à sínfise mandibular. Apresentamos um algoritmo de tratamento proposto que prioriza a necessidade desta cirurgia, associada ou não à distração mandibular, de acordo com a gravidade da dificuldade respiratória e/ou alimentar. Resultados: São apresentados 12 casos de obstrução da orofaringe atendidos de 2012 a 2017, discutem-se suas prioridades, a ortoglossopelveplastia e se aplica o algoritmo proposto. Conclusão: A reorganização anatômica da musculatura em uma posição anteriorizada correta proporciona protração e funcionalidade à língua, com desobstrução da via aérea na orofaringe, melhora da função alimentar e do desenvolvimento mandibular, com baixa morbidade cirúrgica e poucas complicações.

Introduction: Several patients with the Pierre Robin sequence (micrognathia, glossoptosis, and airway obstruction) have an altered, shortened, and retractable genioglossus muscle that prevents protraction of the tongue and keeps the anterior part of the tongue vertical and its volume posteriorly displaced. This can contribute to supraglottic obstruction, feeding difficulty, and inversion of the growth stimulation forces of the mandibular body. Methods: A retrospective study of patients with the Pierre Robin sequence treated between 2012 and 2017 with "orthoglossopelveplasty," which includes modification of glossopexy, releasing the anomalous genioglossus of its insertion and releasing the tongue to raise its anterior third and advance the volume of its base using a traction suture of the tongue base to the mandible symphysis. We present a treatment algorithm that prioritizes the need for surgery associated, or not, with mandibular distraction in accordance with respiratory and/or feeding difficulty severity. Results: Twelve cases of oropharyngeal obstruction treated from 2012 to 2017 are presented, their priorities and orthoglossopleoplasty are discussed, and the proposed algorithm is applied. Conclusion: Anatomical reorganization of the musculature in a correct anterior position provides protraction and functionality to the tongue, clears the airway in the oropharynx, and improves the feeding function and mandibular development, with low surgical morbidity rates and few complications.

Fisura palatina en la secuencia de Pierre Robin / Palatal cleft in Pierre Robin sequence

Entre los niños que nacen con falta de fusión de la bóveda palatina, un número determinado corresponde a recién nacidos con síndrome de Pierre Robin. Aquí se abre un abanico de pacientes con un grado variable de complejidades y anomalías. Algunos que portan otros síndromes aún más complejos, que padecen la entidad por hipoplasia mandibular, hasta otros que solo poseen una modesta micrognatia que permitió el desarrollo de la secuencia. Si bien la fi sura del paladar es una arista más dentro de anomalías que pueden ser más complejas, debe corregirse a tiempo y de manera efi caz para permitir una adecuada fonación. En este trabajo se presentan, sobre 126 fi surados tratados durante 10 años en un hospital de atención pediátrica privado en Buenos Aires, 17 casos de síndrome de Pierre Robin, así como sus características, edad, sexo, tratamiento, resultados quirúrgicos y foniátricos, y complicaciones.

Between children born with cleft palate, a number of them are newborn with Pierre Robin Syndrome. These have a variety and diff erent degrees of complexity and abnormalities. Added complex syndromes may be found aff ecting them with hypoplasia and others with a slight micrognathia that allowed the development of the sequence. Cleft palate is only one between other abnormalities, but needs and must be corrected effi ciently on time to allow proper phonation. A number of 126 cleft palate patients treated and followed during 10 years in a private childrens hospital, in Buenos Aires, are included, and between them, 17 have Pierre Robin sequence. Their age, sex, treatment, surgical results and phonetic results are included.

Distracción ósea mandibular con aparatos reabsorbibles: reporte de tres casos / Mandibular distraction osteogenesis with resorbable devices: report of three cases

La micrognatia e hipoplasia unilateral mandibulares son un rasgo característico en algunos síndromes que incluyen defectos faciales, pero también se pueden presentar como secuelas de traumatismo sobre los tejidos duros del complejo maxilofacial. Dichas alteraciones adquiridas o congénitas, ocasionan grandes trastornos estéticosy funcionales. La distracción ósea descrita por Ilizarov como un método de elongación de los huesos largos, aplicado al área maxilofacial en años recientes, ha demostrado ser una técnica eficaz para la corrección de estos defectos. En este artículo se reportan tres casos de corrección de deformidades mandibulares en niños mediante la técnica de distracción ósea utilizando aparatos reabsorbibles, siendouna de sus ventajas el que no se requiere una segunda intervenciónquirúrgica para su retiro. Los pacientes fueron intervenidos en la Unidad Médica de Alta Especialidad (UMAE) No. 71 del Instituto Mexicano del Seguro Social, en donde desde hace 10 años se ha estadoutilizando el método de distracción ósea para manejo de deformidades mandibulares en niños con muy buenos resultados.

Mandibular micrognathia and unilateral mandibular hypoplasia are atypical feature of certain facial deformity syndromes, though can alsoappear as sequelae of maxillofacial hard tissue trauma. Acquired orcongenital alterations of this nature can have a signifi cant aestheticand functional impact. Ilizarov describes distraction osteogenesis as amethod for lengthening long bones, one that in recent years has beenapplied to the maxillofacial region and proved an eff ective technique forcorrecting these defects. This article reports three cases of mandibulardeformity correction in children, carried out using the distraction osteogenesistechnique with resorbable devices. One of the advantagesof using such devices is that the need for a second surgical interventionto remove them is eliminated. All patients were treated at AdvancedSpecialty Medical Unit (UMAE) 71 of the Mexican Institute of SocialSecurity, which, for the last ten years, has been using the distractionosteogenesis method to correct mandibular deformities in children,with optimal results.

Correction of airway obstruction in congenital micrognathia by mandibular distraction osteogenesis

Varying degrees of upper airway obstruction is almost universally present in patients of congenital micrognathia which needs to be corrected as early as possible. This allows appropriate feeding and growth and prevents long-term complications such as pulmonary hypertension and cor pulmonale. We report the case of a tracheostomy-dependent, 4-year-old child with congenital micrognathia who was treated with mandibular distraction osteogenesis. This is the treatment of choice for surgical correction of mandibular hypoplasia and for the challenging airway management in infants. Once a bone length of 2 cm was achieved through distraction osteogenesis, the child was completely relieved of respiratory obstruction and tracheostomy tube was removed through the process of decannulation

Micrognatias: tratamiento por distracción osteogénica gradual (DOG), resultados iniciales / Distraction osteogenesis for elongation in maxillofacial surgery

La distracción osteogénica (DOG) descripta por Ilizarov como método de elongación de los huesos largos, se aplica en cirugía maxilofacial desde los comienzos de la década actual. El objetivo de este trabajo es presentar la experiencia en elongaciones mandibulares realizada en los últimos años. Fueron tratados diez pacientes, de edades comprendidas entre dos y veintidós años con hipoplasias mandibulares congénitas o secuelares de traumatismos. Se hicieron diez elongaciones mandibulares, tres unilaterales y siete bilaterales. La elongación obtenida estuvo entre 12 y 32 mm, el tiempo de distracción entre 20 y 40 días y los distractores fueron retirados entre 30 y 80 días posteriores. Se analizan los fundamentos del método, sus aplicaciones en micrognatias, tácticas, técnicas y resultados inmediatos. Los resultados estéticos y funcionales en el corto plazo fueron muy satisfactorios. Se propone el uso de la DOG, como un medio efectivo, con menos riesgo y complicaciones si se comparan con osteotomías e injertos óseos tradicionales

Micrognatismo Transversal Superior y sus Tecnicas Correctivas / Transversal Superior Micrognathism and its Corrective Techniques

El micrognatismo transverssal superior es una de las mas frecuentes patologias en cavidad oral debido a que son multiples factores etiologicos que la producen ya sean estos de origen esqueletico como es el caso de la hemotrofia facial displacias, mongolismo, paladar hendido, quienes nos van a producir la patologia anteriormente mencionada por una detencion o disminucion del patron de crecimiento que debe cumplir el maxilar superior y en conjunto las estructuras maxilo-faciales, para lograr una correcta armonia entre tejidos duros, blandos y dentales. Tambien encontramos que existen otras causas de micrognatismo pero estas son de origen adquirido, tales como el raquitismo, respiracion bucal, succion digital, deformacion por traumas, etiologias que si son eliminadas a tiempo evitaran complicaciones mayores como la disminucion de la capacidad nasal respiratoria, desviaciones maxilares y dentarias. Para tal fin se ha dividido el presente trabajo en capitulos claramente definidos que explican secuencialmente la patologia. El primero se refiere a anatomia y embriologia de suma importancia puesto que es necesario conocer las estructuras y la formacion de estas para asi determinar las posibles anormalidades que se presenten alli. El segundo capitulo se refiere a los factores causales anteriormente descritos, tambien hablamos de los medios usuales de diagnostico. La cefalometria por ser un medio tan importante para el diagnostico de la patologia fue clasificado como un capitulo aparte hablando alli de los..