Pudendal nerve block for circumcision of pediatric patient with Pierre Robin Sequence: case report

Abstract Pierre Robin Sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, and cleft palate that presents with airway obstruction and developmental delay with or without other congenital anomalies. These patients' anesthesia management is challenging because of difficult ventilation and intubation. Regional anesthesia methods should be considered for these patients on a case-by-case basis. This report presents primary use of regional anesthesia for circumcision of a 9-year-old boy with PRS.

Management and Outcome of a Neurologically Intact Infant with Pierre Robin Sequence and Dandy-Walker Variant Diagnosed with Large Hemispheric Brain Abscess ­ Case Report

Pierre Robin sequence (PRS) is a condition consisting of three essential components: micrognathia or retrognathia, cleft palate, and glossoptosis. It can be part of multiple congenital anomalies. We present the case and outcome of a 3-month-old clinically stable patient who has PRS with Dandy-Walker variant ­ which is a rare presentation in the literature ­ with a large right hemispheric brain abscess, treated with multiple minimally-invasive surgical drainage procedures with adjuvant antibiotics.

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.

Genetic analysis of a case with Pierre-Robin sequence due to partial 1q trisomy and partial 4q monosomy / 中华医学遗传学杂志

OBJECTIVE@#To explore the genetic basis for a neonate with Pierre-Robin sequence.@*METHODS@#The child was subjected to chromosomal karyotyping, single nucleotide polymorphism array (SNP-array)-based comparative genomic hybridization and fluorescence in situ hybridization (FISH) analysis.@*RESULTS@#The child has featured microgthnia, glossoptosis, upper airway obstruction, mandible dehiscence and short neck. He was found to have a karyotype of 46,XY,der(4)add(4)(q34). Her mother's karyotype was determined as 46,XX,t(1;4)(q43;q34), while his father was 46,XY. SNP-array analysis suggested the child to be arr [hg19] 1q42.2q44 (232 527 958-249 202 755)× 3; 4q34.3q35.2 (168 236 901-190 880 409)× 1. The result of SNP-array for both parents was normal. FISH analysis confirmed that his mother has carried a balanced t(1;4)(q42;34) translocation. The aberrant chromosome 4 in the child has derived from his mother's translocation, which gave rise to partial 1q trisomy and 4q monosomy.@*CONCLUSION@#The 1q42.2q44 duplication and 4q34.3q35.2 deletion of the child probably underlay his abnormal phenotype of Pierre-Robin sequence.

Manejo de secuencia de Pierre Robin con distracción externa mandibular y palatoplastia de incisiones mínimas / Managment of Pierre Robin Sequence with external mandibular distraction and palatoplasty of minimal incisions

Antecedentes. La secuencia de Pierre Robin es una patología poco frecuente y entendida en la literatura latinoamericana . La incidencia en el mundo es variable y en Latinoamerica no existen cifras exactas. La etiopatogenia es poco clara y aminorar y manejar las complicaciones respiratorias constituyen un objetivo primordial en el tratamiento de estos pacientes. El objetivo de este artículo es presentar el manejo del Pierre Robin en nuestro Servicio desde un punto de vista íntegro presentando un caso ejemplo. Presentación de caso. Masculino de 9 meses con diagnóstico de Pierre Robin, quien fue tratado con distractores mandibulares y retiro de los mismos tres meses después, con una excelente evolución posoperatoria. Posterior a esto, se realizó una palatoplastia sin complicaciones, con excelentes resultados respiratorios a los 6 meses de seguimiento. Conclusiones. El manejo del síndrome de Pierre Robin es un desafío para el cirujano plástico, con muchas opciones quirúrgicas disponibles, pero la distracción mandibular es actualmente la técnica que tiene mejores resultados confirmados para los parámetros respiratorios a largo plazo.

Background. Pierre Robin's sequence is a rare pathology. The incidence in the world is variable and in Latin America is unknown. The etiopathogenesis is unclear and respiratory complications are a challenge for surgical treatment. The objective of this article is to present the management of Pierre Robin from an integrative point of view through a case presentation. Case presentation. 9 month old male with Pierre Robin diagnosis who was treated with mandibular distractors with subsequent removal 3 months later with excellent postoperative evolution. Later on, a la palatoplasty was performed with no complications, with good respiratory injuries function at 6 month follow up. Conclusions. The management of Pierre Robin syndrome is a challenge for the plastic surgeon, with many surgical options available, with mandíbular distraction currently being the technique of choice because of its confi rmed favorable long term respiratory results

Pierre Robin sequence: airway management via mandibular distraction: case report / Secuencia de Pierre Robin: manejo de vía aérea mediante distracción ósea mandibular: reporte de caso

Pierre Robin sequence is a set of congenital conditions characterized by the presence of micrognathia, glossoptosis, cleft palate and obstruction of the airways. The latter can lead to many other complications such as respiratory difficulties, apnea, weight loss and even death. Currently, mandibular distraction, or the lengthening of the mandibular bone, is the most common surgical procedure used to correct a retracted tongue and the airway obstruction it produces in patients with mandibular hypoplasia. The present paper reports the case of a 26-day-old male patient, who presented obstruction on the upper respiratory tract, mandibular retromicrognathia, and retracted tongue and cleft palate, all conditions characteristic to Pierre Robin sequence. The patient also had a medical record of orotracheal intubation due to respiratory difficulties. The subject underwent mandibular distraction surgery with a horizontal vector, which resulted in a satisfactorily cleared airway.

La secuencia de Pierre Robin es una afección congénita caracterizada por la presencia de micrognatia, glosoptosis, paladar hendido y obstrucción de la vía aérea, siendo ésta una de las principales características de la cual derivan varias complicaciones, entre ellas, dificultad respiratoria, apnea, pérdida de peso y hasta la muerte. En la actualidad la distracción ósea mandibular es la técnica quirúrgica de elección, que tiene como finalidad el alargamiento mandibular corrigiendo la posición posterior de la lengua, con la consecuente desobstrucción de la vía aérea en pacientes con hipoplasia mandibular. Se reporta caso clínico de paciente masculino con 26 días de nacido, que presentó obstrucción de la vía aérea superior, retromicrognatismo mandibular, retracción de la lengua y hendidura palatina, relacionado con la secuencia de Pierre Robin, con antecedentes de intubación orotraqueal por dificultad respiratoria, al cual se le realizó distracción ósea mandibular con vector horizontal, destacando resultados satisfactorios en la resolución de la obstrucción de la vía aérea.

"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.

Síndrome de Pierre Robin associada à Síndrome de Duane familiar do tipo III / Pierre Robin Syndrome associated with type III familial Duane Retraction Syndrome

Resumo A síndrome de Pierre Robin (PRS) consiste em uma tríade de anomalias caracterizada por micrognatia, glossoptose e fissura de palato, comumente associada com outras síndromes e ocasionalmente com alterações oculares. Na Síndrome de Duane (DRS), há uma falha na inervação do reto lateral pelo VI nervo, com inervação anômala do reto lateral por fibras do III nervo. Ainda que a PRS já tenha sido associada com mais de 50 outras síndromes, não existe na literatura relato de casos de associação com a DRS familiar. Dessa forma, esse trabalho tem por objetivo relatar um caso dessa associação em um paciente de 29 anos com recorrência das síndromes na família.

Abstract The Pierre Robin Syndrome (PRS) consists of a triad of anomalies characterized by micrognathia, glossoptosis and fissure of the palate, usually associated with other syndromes e occasionally associated with ocular variations. In Duane Retraction Syndrome (DRS), there is a failure in the lateral rectus innervation by the VI cranial nerve, with anomalous innervation of the lateral rectus by fibers of the III nerve. Even though PRS has already been associated with more than 50 other syndromes, there is not any report in literature of association with familial DRS. Thus, this work aims to report a case of this association in a 29 years old patient with recurrence of the syndromes in the family.

Pierre Robin sequence with severe scoliosis in an adult: A case report of clinical and radiological features

Pierre Robin sequence (PRS) is characterized by the triad of micrognathia, glossoptosis, and airway obstruction. PRS does not have a single pathogenesis, but rather is associated with multiple syndromes. This report presents the case of a 35-year-old woman with PRS and scoliosis. Among the syndromes related to PRS, cerebro-costo-mandibular syndrome (CCMS), which is characterized by posterior rib gap defects and vertebral anomalies, was suspected in this patient. However, no posterior rib gap defect was detected on radiological examinations. Although over 80 cases of CCMS have been reported to date, few cases of PRS with scoliosis alone have been reported. Therefore, this report demonstrated the clinical, radiological, and cephalometric characteristics of an adult patient with PRS and scoliosis, but without rib anomalies.

Three Cases of Pierre Robin Sequence with Upper Airway Obstruction Relieved by Nasopharyngeal Airway Insertion

Pierre Robin sequence (PRS), also called Robin sequence, is a congenital anomaly characterized by a triad of micrognathia, glossoptosis, and upper airway obstruction. Infants with PRS can present with varying degrees of respiratory difficulty secondary to upper airway obstruction. There has been no consensus for the treatment of upper airway obstruction in infants with PRS, but recent studies recommend attempting non-surgical interventions before surgical treatment. In this case report, we present 3 cases of infants diagnosed with PRS who showed persistent respiratory difficulties after birth. Before considering surgical intervention, insertion of a nasopharyngeal airway was attempted in these infants. Following this procedure, symptoms of upper airway obstruction were relieved, and all infants were discharged without surgical interventions; the nasopharyngeal airway was removed 1 to 2 months later. To date, no infant has shown signs of upper airway obstruction. Nasopharyngeal airway insertion is a highly effective and less invasive treatment option for infants with PRS. However, it is not widely known and used in Korea. Nasopharyngeal airway insertion can be preferentially considered before surgical intervention for upper airway obstruction in such infants.

Male individuals with Robin Sequence: emerging significant association with ABO and RhD blood group phenotypes

ABSTRACT Background: This study investigated the association of Robin Sequence with ABO and RhD blood group phenotypes. Methods: A retrospective cross-sectional study was performed of a cohort of Robin Sequence patients of the Hospital de Reabilitação de Anomalias Craniofaciais - Universidade de São Paulo (USP), Brazil. The study group was composed of 339 individuals of both genders with Robin Sequence referred for specific treatment. A control group was composed of 1780 individuals without syndromes. The groups were compared using the Pearson' chi-square test (χ 2) with statistical significance being defined for an alpha error of 5% (p-value < 0.05). Results: A comparison of gender found a significant difference for the AB phenotype between groups (p-value = 0.007). Comparing blood type by gender there was no significant difference within the same group (p-value = 0.117 and 0.388 respectively, for Robin Sequence and the control group). When comparing the AB blood type between groups, there was no difference for females (p-value = 0.577), but there was a significant difference for males (p-value = 0.0029). Conclusions: This study showed that the population with Robin Sequence had different patterns related to gender concerning the phenotypic distribution of ABO and RhD blood group phenotypes. Robin Sequence is more common among females. The AB phenotype was significantly higher in males with Robin Sequence than in males of the Control Group. The prevalence of the RhD-negative phenotype is higher in individuals with Robin Sequence. This result suggests a possible association of ABO and RhD phenotypes with Robin Sequence that should be better investigated by molecular studies, as it deserves greater attention.

Sequência de robin isolada: diagnósticos de Enfermagem / Secuencia de robin aislada: diagnósticos de Enfermería / Isolated robin sequence: Nursing diagnoses

RESUMO Objetivo: identificar os diagnósticos de enfermagem em lactentes com Sequência de Robin isolada. Método: estudo descritivo desenvolvido em um hospital especializado em anomalias craniofaciais e síndromes relacionadas, no período de novembro de 2015 a março de 2016. Participaram 20 lactentes com Sequência de Robin isolada. Para a coleta de dados utilizou-se o histórico de enfermagem. Os diagnósticos de enfermagem foram elencados segundo a Nanda-Internacional. Para confecção dos resultados utilizou-se a análise estatística descritiva. Resultados: foram identificados oito diagnósticos, sendo cinco com foco no problema e três de risco, incluindo: risco de aspiração (n=20, 100%); risco de infecção (n=20, 100%); amamentação ineficaz (n=19, 95%); nutrição desequilibrada: menor do que as necessidades corporais (n=19, 95%); padrão ineficaz de alimentação do lactente (n=19, 95%); risco de integridade da pele prejudicada (n=18, 90%); desobstrução ineficaz das vias aéreas (n=11, 55%) e ventilação espontânea prejudicada (n=11, 55%). Conclusão: lactentes com Sequência de Robin isolada apresentaram diagnósticos de enfermagem que se relacionaram a problemas respiratórios, alimentares, de segurança e conforto e favoreceram o planejamento e implementação dos cuidados de enfermagem ao traçarem um perfil assistencial.

RESUMEN Objetivo identificar los diagnósticos de enfermería en lactantes con Secuencia de Robin aislada. Método estudio descriptivo desarrollado en un hospital especializado en anomalías craneofaciales y síndromes relacionados, en el período de noviembre de 2015 a marzo de 2016. Participaron 20 lactantes con Secuencia de Robin aislada. Para la recolección de datos se utilizó el histórico de enfermería. Los diagnósticos de enfermería fueron enumerados según la Nanda-Internacional. Para la confección de los resultados se utilizó el análisis estadístico descriptivo. Resultados fueron identificados ocho diagnósticos, siendo cinco con foco en el problema y tres de riesgo, incluyendo: riesgo de aspiración (n=20, 100%); riesgo de infección (n=20, 100%); lactancia ineficaz (n=19, 95%); nutrición desequilibrada menor que las necesidades corporales (n=19, 95%); el patrón ineficaz de alimentación del lactante (n=19, 95%); riesgo de perjuicio a la integridad de la piel (n=18, 90%); desobstrucción ineficaz de las vías aéreas (n= 1, 55%) y ventilación espontánea perjudicada (n=11, 55%). Conclusión lactantes con Secuencia de Robin aislada presentaron diagnósticos de enfermería que se relacionaron con problemas respiratorios, alimentarios, de seguridad y confort y favorecieron la planificación e implementación de los cuidados de enfermería al trazar un perfil asistencial.

ABSTRACT Objective: to identify nursing diagnoses in infants with isolated Robin Sequence. Method: a descriptive study developed in a hospital specialized in craniofacial anomalies and related syndromes, from November 2015 to March 2016. Twenty infants with isolated Robin Sequence participated in the study. For the data collection, the nursing history was used. The nursing diagnoses were listed according to Nanda-International. To compose the results, a descriptive statistical analysis was used. Results: eight diagnoses were identified, five of which focused on the problem and three on risk factors, including: risk of aspiration (n=20, 100%); risk of infection (n=20, 100%); ineffective breastfeeding (n=19, 95%); unbalanced nutrition lower than body needs (n=19, 95%); ineffective infant feeding pattern (n=19, 95%); risk of damage to skin integrity (n=18, 90%); ineffective airway clearance (n=11, 55%) and impaired spontaneous ventilation (n=11, 55%). Conclusion: infants with isolated Robin Sequence presented nursing diagnoses that were related to respiratory, feeding, safety and comfort problems and favored the planning and implementation of nursing care when tracing a care profile.

Applicability of Orem: training of caregiver of infant with Robin Sequence / Aplicabilidad de Orem: capacitación del cuidador del lactante con Secuencia de Robin / Aplicabilidade de Orem: capacitação do cuidador do lactente com Sequência de Robin

ABSTRACT Objective: to report the nurses' experience in relation to the training of caregivers of infants with Isolated Robin Sequence (IRS) for maintaining care after hospital discharge from the perspective of Self-Care Theoretical Framework. Method: the following categories were considered in this experience report: self-care action, self-care capacity, therapeutic self-care demand, self-care deficit, and nursing system. The nursing system was wholly compensatory and supportive-educative. Results: caregivers' training by nurses results in the acquisition of technical skills and specific knowledge related to the infant's positioning in elevated ventral decubitus, nasopharyngeal intubation, feeding-facilitating techniques and care with the feeding tube. Final considerations: the continuity of home care is guaranteed from caregivers' training for the therapeutic demand.

RESUMEN Objetivo: relatar la experiencia de los enfermeros en relación a la capacitación de los cuidadores de lactantes con Secuencia de Robin Aislada (SRA) para mantener los cuidados después del alta hospitalaria en la perspectiva del Referencial Teórico del Autocuidado. Método: las siguientes categorías se consideraron en este relato de experiencia: acción de autocuidado, capacidad de autocuidado, demanda terapéutica de autocuidado, déficit de autocuidado y sistema de enfermería. El sistema de enfermería fue del tipo totalmente compensador y por medio de apoyo-educación. Resultados: la capacitación de los cuidadores por el enfermero resulta en la adquisición de habilidades técnicas y conocimientos específicos relacionados al posicionamiento del lactante en decúbito ventral y elevado, intubación nasofaríngea, técnicas facilitadoras de la alimentación y cuidados con la sonda para alimentación. Consideraciones finales: la continuidad de los cuidados domiciliarios está garantizada a partir de la capacitación del cuidador para la demanda terapéutica.

RESUMO Objetivo: relatar a experiência dos enfermeiros em relação à capacitação dos cuidadores de lactentes com Sequência de Robin isolada (SRI) para manutenção dos cuidados após alta hospitalar na perspectiva do Referencial Teórico do Autocuidado. Método: as seguintes categorias foram consideradas neste relato de experiência: ação de autocuidado, capacidade de autocuidado, demanda terapêutica de autocuidado, déficit de autocuidado e sistema de enfermagem. O sistema de enfermagem foi do tipo totalmente compensatório e por meio de apoio educativo. Resultados: a capacitação do cuidador pelo enfermeiro resulta na aquisição de habilidades técnicas e conhecimentos específicos referentes ao posicionamento do lactente em decúbito ventral e elevado, intubação nasofaríngea, técnicas facilitadoras da alimentação e cuidados com a sonda alimentadora. Considerações finais: a continuidade dos cuidados domiciliares é garantida a partir da capacitação do cuidador para a demanda terapêutica.

Dysphasia due to Oral Anomaly / 대한연하장애학회지

Dysphasia related to oral anomaly is a common situation in oral and maxillofacial surgery. The etiology of oral anomalies causing dysphasia can be divided into congenital and acquired disease. Congenital diseases include teratoma or benign tumors and congenital defects such as cleft lip and palate. Benign tumors include cystic hygroma in the neck and hemangioma in the tongue. Certain syndromes with macroglossia and micrognathia are also related to difficulty in swallowing. The three common syndromes are Pierre-Robin syndrome, Beckwith-Widermann syndrome and ectodermal dysplasia. Taken together, these congenital diseases require a multi-discipline approach to obtain optimal results. Representative disease of acquired dysphasia is the oral cavity cancer. Cancer ablation results in tissue defect and decreased motor function. Free flap reconstruction is the choice of treatment following oral cavity caner operation; however, dysphasia after cancer operation is inevitable. In this review article, the full scopes of oral anomaly associated with dysphasia were classified and treatment was suggested.

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.

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.

Intensive treatment of speech disorders in robin sequence: a case report

ABSTRACT Purpose To describe the speech of a patient with Pierre Robin Sequence (PRS) and severe speech disorders before and after participating in an Intensive Speech Therapy Program (ISTP). Methods The ISTP consisted of two daily sessions of therapy over a 36-week period, resulting in a total of 360 therapy sessions. The sessions included the phases of establishment, generalization, and maintenance. A combination of strategies, such as modified contrast therapy and speech sound perception training, were used to elicit adequate place of articulation. The ISTP addressed correction of place of production of oral consonants and maximization of movement of the pharyngeal walls with a speech bulb reduction program. Therapy targets were addressed at the phonetic level with a gradual increase in the complexity of the productions hierarchically (e.g., syllables, words, phrases, conversation) while simultaneously addressing the velopharyngeal hypodynamism with speech bulb reductions. Results Re-evaluation after the ISTP revealed normal speech resonance and articulation with the speech bulb. Nasoendoscopic assessment indicated consistent velopharyngeal closure for all oral sounds with the speech bulb in place. Conclusion Intensive speech therapy, combined with the use of the speech bulb, yielded positive outcomes in the rehabilitation of a clinical case with severe speech disorders associated with velopharyngeal dysfunction in Pierre Robin Sequence.

Management of a Dentigerous Cyst in a Child with Robin Sequence

This is a brief clinical report describing an 18-month-old female with Robin sequence found to have an incidental mandibular cystic lesion on a head computed tomography scan in the preoperative workup before performing mandibular distraction. She underwent enucleation of the tumor, which was found to be a dentigerous cyst. One year following cyst enucleation, mandibular distraction was performed in order to alleviate her tongue-based obstruction. This case demonstrates the ability of the mandibular bone to successfully regenerate after undergoing cyst enucleation.

Management of a Dentigerous Cyst in a Child with Robin Sequence

This is a brief clinical report describing an 18-month-old female with Robin sequence found to have an incidental mandibular cystic lesion on a head computed tomography scan in the preoperative workup before performing mandibular distraction. She underwent enucleation of the tumor, which was found to be a dentigerous cyst. One year following cyst enucleation, mandibular distraction was performed in order to alleviate her tongue-based obstruction. This case demonstrates the ability of the mandibular bone to successfully regenerate after undergoing cyst enucleation.

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)