Secuencia malformativa de Pierre Robin / Pierre Robin's malformative sequence

RESUMEN Introducción: La secuencia malformativa de Pierre Robin se caracteriza clínicamente por micrognatia, glosoptosis, obstrucción de la vía aérea superior y con frecuencia fisura palatina, donde se pone en riesgo la vida del neonato. Objetivo: Presentar un caso de un recién nacido con diagnóstico de la secuencia de Pierre Robin atendido en el Hospital Universitario Gineco-Obstétrico Mariana Grajales de la ciudad de Santa Clara, Cuba. Caso clínico: Paciente femenino de piel blanca a término y bajo peso, se constató en la exploración física neonatal marcado micrognatismo y retrogmatismo, al examen bucal se observó glosoptosois y fisura del paladar, patrón dismórfico propio de la secuencia malformativa de Pierre Robin. Se indicó tratamiento conservador mediante el reposo en decúbito prono y alimentación enteral por biberón en posición semisentada, colocación de obturador palatino y seguimiento en consulta por las especialidades involucradas. Con una evolución satisfactoria fue egresado del centro hospitalario. Conclusiones: Con un tratamiento conservador y la participación interdisciplinaria de distintas especialidades médicas y estomatológicas, se logró un resultado satisfactorio en el neonato.

ABSTRACT Introduction: The Pierre Robin's malformative sequence is clinically characterized by micrognathia, glossoptosis, upper airway obstruction and frequently cleft palate, which can be life-threatening. Objective: To present a case of a newborn diagnosed with the Pierre Robin's malformative sequence attended at the Mariana Grajales Gyneco-Obstetric University Hospital of the Villa Clara province. Case report: A white female patient, term and low weight was attended. A marked micrognathism and retrogmatism is observed in neonatal physical examination, oral examination showed glossoptosis, and palatine fissure, dysmorphic pattern typical of Pierre Robin's malformative sequence. The neonate was evaluated by different specialties. Conservative treatment was indicated through the prone position and enteral bottle feeding in a semi-seated position, placement of palatal plate and follow-up by consultation of the specialities involved. The patient left the hospital with a satisfactory evolution. Conclusions: With conservative treatment and interdisciplinary participation of different medical and dentistry specialties a satisfactory result is achieved in the neonate.

Manejo de vía aérea difícil en secuencia de Pierre Robin. Reporte de un caso / Difficult airway management in Pierre Robin Sequence. A case report

La secuencia de Pierre Robin (SPR) es una enfermedad rara, caracterizada por una tríada de malformaciones orofaciales como retrognatia, glosoptosis y fisura velopalatina media (paladar hendido); las cuales, generalmente provocan obstrucción de la vía aérea (OVA). La corrección de los defectos palatinos requiere en ocasiones varias intervenciones quirúrgicas, por esta razón el cuidado anestésico y abordaje de la vía aérea en forma adecuada, evita complica-ciones asociadas a esta enfermedad. Se presenta el caso de un paciente de 1 año y 20 días de edad, con antecedentes de vía aérea difícil, intentos fallidos de intubaciones preliminares e intervenido quirúrgicamente para corrección de paladar hendido, luego de una técnica combinada de intubación nasal con fibrobroncoscopio, desplazamiento de glosoptosis con ayuda de pala número 2 de videolaringoscopio (Glidescope®); manejo transoperatorio anestésico y proceso de extubación con excelentes resultados. Destacamos la importancia de realizar una adecuada planificación multidisciplinaria prequirúrgica con valoración exhaustiva de la vía aérea (VA) por laringoscopía directa o fibrobroncospia flexible para conocer el sitio exacto de la obstrucción y evitar desen-laces adversos.

Pierre Robin Sequence (PRS) is a rare disease characterized by a triad of orofacial malformations such as retrognathia, glossoptosis and velopalatine fissure (cleft palate). The malformations in PRS can lead into airway obstruction. The correction of the palatal defects sometimes requires several surgical interven-tions. Proper anesthetic care and a carefully planned approach to the airway can avoid complications associated with this disease. We present the case of a 1 year and 20 days old patient, with history of difficult airway, unsuccessful prior attempts of intubation, who underwent surgery to correct a cleft palate. We used a combined approach that included nasal intubation technique with a fiberoptic bronchoscope aided with a number 2 video laryngoscope blade (Gli-descope®) for displacement of the glossoptosys. Details of the intraoperative anesthetic management and subsequent successful extubation are provided. We highlight the importance of developing an adequate preoperative multidisciplinary plan of action, after a careful and detailed evaluation of the airway with direct laryngoscopy or fiberoptic bronchoscopy in order to identify the exact location of the obstruction and avoid adverse outcomes.

"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.

Secuencia malformativa de Pierre Robin: informe de un caso y revisión de la literatura / Pierre Robin sequence: case report and literature review

Durante la infancia es muy frecuente encontrar alteraciones del desarrollo,las cuales derivan de una defi ciente formación de las estructurasanatómicas durante la embriogénesis. Puede encontrarse un sinnúmerode alteraciones del desarrollo que afectan la región bucal y maxilofacial.La gran mayoría de estas alteraciones han sido catalogadas como síndromes de orden genético; sin embargo, no todas pueden describirse como tales, pues existen anomalías del desarrollo que aparecen como consecuencia de una deficiente embriogénesis de la región facial, provocando alteraciones anatómicas y funcionales, pero que se apartan de componentes genéticos y cromosómicos específi cos. La secuencia malformativa de Pierre Robin es una de ellas, ya que esta condición es producida por una afección inicial, de la cual derivarán otras afeccionesadicionales a nivel del paladar y de la mandíbula que ocasionarán en elpaciente dificultad para la alimentación y respiración. Debido a que las alteraciones de esta condición afectan directamente la cavidad bucal,es crucial que el odontólogo se encuentre familiarizado con esta anomalía. El objetivo del presente artículo es describir las característicasque configuran esta entidad nosológica mediante la exposición de un caso clínico y revisión de la literatura.

During childhood, it is frequent to find development disorders whichare linked to the weak formation of anatomic structures duringembryogenesis. It is possible to find a plethora of developmentdisorders that aff ect the oral and maxillofacial region. The majorityof these disorders has been classifi ed as genetic malformations butnot all can be described as such. That is because some developmentdisorders appear as a result of a defi cient embryogenesis of the face,producing thus anatomic and functional malformations but that standapart from genetic and chromosomic specifi c components. The Pierre Robin sequence is one of them, given that this condition is producedby an initial disorder, followed by other disorders in the palate andjaw; provoking alimentary and breathing disabilities in the patient.Due to these disorders and their impact on the mouth, it is crucial thatdentists be familiarized with such anomalies. The aim of this article isto describe the key characteristics that defi ne this disease through thepresentation of a clinical case and a literature review.

Sequência de Pierre Robin: aplicação da técnica de distração osteogênica em pacientes como abordagem terapêutica / Pierre Robin sequence: application of the osteogenic distraction technique in patients as a therapeutic approach

Introdução: Pierre Robin, em 1923, descreveu a sequência das malformações e as correlacionou com os sinais clínicos de insuficiência respiratória, o que trouxe a constatação da necessidade de tratamento, muitas vezes urgente. A conduta terapêutica deve ser individualizada para cada caso e respeitar o quadro apresentado pelo paciente. Objetivo: O presente estudo consiste em uma revisão sobre a Sequência de Pierre Robin e sua abordagem terapêutica, através de distração osteogênica da mandíbula, com a finalidade de demonstrar a efetividade do procedimento. Método: Estudo descritivo de abordagem qualitativa tipo relato de caso. Resultados e conclusão: A aplicação da técnica possibilitou a correção das anormalidades craniofaciais, o que minimizou os prejuízos causados pela síndrome (AU)

Introduction: In 1923, Pierre Robin described and sequenced the malformations and correlated them with the clinical signs of respiratory failure, which eventually confirmed the need for treatment, often urgent. The therapeutic approach should be individualized for each case and must respect the patient's clinical picture. Aim: The present study consists of a review on the Pierre Robin sequence and its therapeutic approach through osteogenic distraction of the mandible in order to demonstrate the effectiveness of the procedure. Method: A descriptive study with a qualitative approach, case report type. Results and conclusion: The application of the technique made possible the correction of craniofacial abnormalities, which minimized the damage caused by the syndrome (AU)

Pierre Robin syndrome: a case report.

Pierre Robin syndrome is characterized by micrognathia, glossoptosis and palatal malformation. We report a case of a 6 day neonate who presented with complaints of feeding and respiratory difficulty and was later diagnosed as case of Pierre Robin syndrome.

The review of Pierre Robin sequence / 国际儿科学杂志

The Pierre Robin sequence (PRS)is a congenital abnormality which is not only causally heterogeneous but also with a group of physical findings, characterized by the presence of the combination of underdeveloped(micrognathia) or retropositioned(retrognathia) mandible,glossoptosis that leads to respiratory tract obstruction with or without cleft palate. PRS is clinically defined by obstruction of the upper airways, feeding difficulties, inspiration pneumonia and some accompany with multiple -anomalies that are more frequent and more severe during the immediate postnatal and neonatal period. Diagnosis of PRS may be made immediately when a neonate presents in respiratory distress with micrognathia. Further examination may show the posterior placement of the tongue and possibly a cleft palate. Now that the strong association of PRS with many multiple - anomaly syndromes is recognized, treatment must be individualized to optimize the outcome. Most PRS children have failure to thrive,and long -term outcomes vary greatly,depending on whether the PRS is an isolated occurrence or part of an associated syndrome and on whether the infant experienced any obstructive-hypoxic episodes.