[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.

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

Las malformaciones craneofaciales (secuencia de Pierre Robin, síndrome de Threacher Collins, síndrome de Nager, etc.) con frecuencia van asociadas a hipoplasia mandibular severa, que puede causar obstrucción de la vía aérea superior por retroposición de la base de la lengua dentro del espacio faríngeo posterior. La mayoría de los pacientes responden al tratamiento postural; ende cúbito prono puede ser necesario monitorizar la saturación de oxígeno ,insertar un tubo nasofaríngeo e incluso endotraqueal. En casos más severos con pausas prolongadas y frecuentes de apnea la traqueostomía puede ser necesaria, pero se asocia a una alta morbilidad y ocasional mente mortalidad. En los últimos dos años, en la Unidad Multidisciplinar de Labio y Fisura Palatina del Hospital Virgen de las Nieves, se ha tratado a 4 niños con apnea obstructiva grave secundaria a hipoplasia mandibular severa mediante distracción mandibular osteogénica, mostrándose este procedimiento eficaz en la resolución del problema. Ha evitado la traqueostomía, se ha elongado la mandíbula en el plazo de 2-3 semanas, en este tiempo han desaparecido los problemas respiratorios obstructivos y también de la deglución, siendo los resultados estéticos obtenidos excelentes y las complicaciones, por el momento, mínimas (AU)

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 maybe 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, mínimum (AU)

Generación de un sustituto de mucosa oral humana y comprobación de su viabilidad mediante ingeniería tisular / Generation of a substitute for human oral mucosa and verification of its viability by tissue-engineering

La reconstrucción de grandes defectos de mucosa oral a menudo es desafiante, por la escasez de mucosa oral sana para reemplazar tejidos dañados. De esta forma, las técnicas de ingeniería tisular pueden suponer una fuente de tejidos autólogos disponible para trasplantar a estos pacientes. En este trabajo hemos desarrollado un nuevo modelo de mucosa oral artificial generada mediante ingeniería tisular usando un soporte de fibrina-agarosa. Para ello, se han generado cultivos primarios de fibroblastos de la mucosa oral humana y queratinocitos a partir de pequeñas biopsias de mucosa oral normal y aplicándoles tratamiento senzimáticos. Después, se ha determinado la viabilidad de las células cultivadas mediante microanálisis por rayos-X, demostrando que la mayoría de las células de los cultivos primarios estaban vivas y tenían elevados K/Na ratios. Una vez que la viabilidad celular fue determinada, se usaron los fibroblastos y queratinocitos cultivados para desarrollar un constructo de mucosa oral sobre una matriz extracelular de (..) (AU)

Reconstruction of large oral mucosa defects is often challenging, since the shortage of healthy oral mucosa to replace the excised tissues. This way, tissue ingineering techniques may provide a source of autologoustissues available for transplant in these patients. In this work, wehave developed a new model for artificial oral mucosa generated by tissue engineering using a fibrin-agarosa scaffold. For that purpose, we have generated primary cultures of human oral mucosa fibroblasts and keratinocytes from small biopsies of normal mucosa oral using enzymatic treatments. Then, we have determined the viability of cultured cells by electron probe quantitative X-ray microanalysis, and we have demonstrated that most of the cells in the primary cultures were alive and hd high K/Na ratios. Once cell viability was determined, we used cultured fibroblasts and keratinocytes to develop an artificial oral mucosaconstruct by using a fibrin-agarosa extracellular matrix and a sequential culture technique using porous culture inserts. Histological analysis of the artificial tissues showed high similarities with normal oral (..) (AU)

[Generation of a substitute for human oral mucosa and verification of its viability by tissue-engineering]. / Generación de un sustituto de mucosa oral humana y comprobación de su viabilidad mediante ingeniería tisular.

Reconstruction of large oral mucosa defects is often challenging, since the shortage of healthy oral mucosa to replace the excised tissues. This way, tissue ingineering techniques may provide a source of autologous tissues available for transplant in these patients. In this work, we have developed a new model for artificial oral mucosa generated by tissue engineering using a fibrin-agarosa scaffold. For that purpose, we have generated primary cultures of human oral mucosa fibroblasts and keratinocytes from small biopsies of normal mucosa oral using enzymatic treatments. Then, we have determined the viability of cultured cells by electron probe quantitative X-ray microanalysis, and we have demonstrated that most of the cells in the primary cultures were alive and hd high K/Na ratios. Once cell viability was determined, we used cultured fibroblasts and keratinocytes to develop an artificial oral mucosa construct by using a fibrin-agarosa extracellular matrix and a sequential culture technique using porous culture inserts. Histological analysis of the artificial tissues showed high similarities with normal oral mucosa controls. The epithelium of the oral substitutes had several layers, with desmosomes and apical microvilli and microplicae. Both the controls and de oral mucosa substitutes showed high suprabasal expression of cytokeratin 13 and low expression of cytokeratin 10. All these results suggest that our model of oral mucosa using fibrin-agarose scaffolds show several similarities with native human oral mucosa.