Risk factors for first weaning failure following mandibular distraction osteogenesis in pediatric patients with Pierre Robin sequence: a nested case-control study / 中华麻醉学杂志

Objective:To identify the risk factors for the first weaning failure following mandibular distraction osteogenesis in pediatric patients with Pierre Robin sequence (PRS).Methods:Clinical data of pediatric patients with PRS who underwent mandibular distraction osteogenesis from January 2018 to February 2023 were collected, including sex, age, premature birth, birth weight, surgical weight, cleft palate, syndrome type PRS, laryngeal/tracheobronchial malacia, simple congenital heart disease, complex congenital heart disease, preoperative mechanical ventilation, preoperative pulmonary infection, blood albumin concentration, difficulty in tracheal intubation under a visual laryngoscope, surgical duration, postoperative ventilator-associated pneumonia, duration of mechanical ventilation at first weaning, and traction length at first weaning. Children in whom the first postoperative machine withdrawal failed were included in observation group and matched to control cases(control group) in a 1∶4 ratio. The risk factors of which P values were less than 0.05 would enter the logistic regression analysis to stratify the risk factors for postoperative weaning failure. Results:There were significant differences in birth weight, cleft palate, duration of mechanical ventilation and traction length at first weaning, rate of combined cleft palate, preoperative pulmonary infection rate, rate of preoperative mechanical ventilation, and rate of postoperative ventilator-associated pneumonia between the two groups ( P<0.05). Binary logistic stepwise regression analysis showed that the preoperative mechanical ventilation ( OR=18.154, 95% CI 3.971-82.990, P<0.001) and postoperative ventilator-associated pneumonia ( OR=36.942, 95% CI 1.307-1043.985, P=0.034) were independent risk factors for first weaning failure after mandibular distraction osteogenesis, while birth weight gain ( OR=0.225, 95% CI 0.076-0.668, P=0.007) was a protective factor for first weaning failure ( P<0.05). Conclusions:Preoperative mechanical ventilation and postoperative ventilator-associated pneumonia are independent risk factors and birth weight gain is a protective factor for first weaning failure following mandibular distraction osteogenesis in pediatric patients 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.

Perioperative evaluation and management of neonates with Pierre-Robin sequence / 中华实用儿科临床杂志

Objective:To summarize the clinical characteristics of neonates with Pierre-Robin sequence (PRS) who received mandibular distraction osteogenesis (MDO), in order to provide a basis for perioperative evaluation and management.Methods:Data of neonates diagnosed with PRS and receiving MDO in the Department of Neonatology, Guangzhou Women and Children′s Medical Center between December 2016 and December 2019 were collected for a retrospective study.According to the types of respiratory support needed before surgery, they can be categorized into 3 groups: the invasive ventilation group, the noninvasive ventilation group, the oxygen therapy/positioning therapy group.According to the usage of sedative and analgesic drugs after surgery, they were categorized into 2 groups: the sedative-analgesic group(Fentanyl Citrate and Midazolam) and the single sedative group(Midazolam alone).Results:A total of 31 children were admitted, 22 cases of whom received MDO at an age of (22.09±8.42) d, including 11 cases in the invasive ventilation group, 5 cases in the non-invasive ventilation group, and 6 cases in the therapy/positioning therapy group before surgery; there was statistical significance in severe respiratory distress (81.8% vs.60.0% vs.0) and feeding difficulties (100.0% vs.80.0% vs.50.0%) among the 3 groups (all P<0.05), while there was no difference in laryngomalacia rate(63.6% vs.20.0% vs.33.3%), non-gain in weight before surgery(81.8% vs.80.0% vs.66.7%), postoperative ventilation duration[(7.5±3.4) d vs.(7.2±5.3) d vs.(6.9±4.1) d]and hospital stay[(36.3±9.3) d vs.(34.1±0.3) d vs.(33.8±7.5) d] (all P>0.05). Among these 22 patients, there were 9 cases in the sedative-analgesic group, and 13 cases in the single sedative group.Compared with the single sedative group, cases in the sedative-analgesic group had significantly lower extubation rate (0 vs.15.4%) and shorter hospital stay[(32.3±5.2) d vs.(39.8±7.3) d], and the difference were statistically significant (all P<0.05). Besides, there were no differences in postoperative ventilation duration[(7.4 ± 3.9) d vs.(7.6 ± 4.2) d]between both groups ( P>0.05). The postoperative remission rate of airway obstruction was 100%. Conclusions:MDO in children in their neonatal period could achieve favorable efficacy.The duration of postoperative ventilator support and hospital stay in children with severe airway obstruction could not be prolonged compared with children with mild obstruction, and postoperative sedation and analgesic management can reduce the extubation rate.

"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.

Effect of mandibular distraction osteogenesis on quality of life in children with Pierre-Robin sequence / 中华整形外科杂志

Objective@#To explore the impact role of Mandibular Distraction Osteogenesis technique (MDO) on the health quality of life in children with Pierre-Robin Sequence (PRS).@*Methods@#From January 2017 to August 2017, 44 cases of children with PRS admitted in children′s hospital of Nanjing Medical University were recruited. Glasgow Children′s benefit inventory(GCBI), Chinese version was used to study postoperative qualify of life in four aspects: physiology, emotions, learning, and vitality. Clinical data and the quality of life score were collected from the patients, and comparison analysis was performed.@*Results@#GCBI total score of the 44 cases of PRS treated with MDO surgery was 46, indicating that the overall quality of life was improved; Physical dimension score was 72, which was the highest, showing the most positive changes. GCBI total scores for the children with different types of PRS were type Ⅰ62, type Ⅱ43, type Ⅲ18, respectively. Nonparametric test showed that quality of life in typeⅠpatients was improved compared to typeⅢ. The difference was statistically significant (Z = -3.34, P=0.01). For the comparisons between type Ⅰ and Ⅱ and type Ⅱ and type Ⅲ, there was no statistically significant difference (P>0.05).@*Conclusions@#MDO plays a positive role in improving life of quality . Physiological conditions were the aspects that were improved most. The milder the case is, the more the postoperative quality of life is improved.

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.

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.

Labio-glosopexia de Routledge en un paciente con Secuencia Robin. Reporte de un caso / Rutledge’s lip-glossopexy in a patient with Robin sequence. Case report

La secuencia Robin, previamente conocida como síndrome de Pierre Robin, se caracteriza por la presencia de micrognatia o retrognatia, glosoptosis con o sin fisura palatina. Los recién nacidos con este síndrome, pueden presentar obstrucción de la vía aérea, dificultad para la alimentación, retraso del crecimiento e hipoxemia crónica. La mitad de estos pacientes presentan malformaciones asociadas. La incidencia es aproximadamente de 1: 8,500. Las opciones actuales del tratamiento de la obstrucción de la vía aérea en estos pacientes, van desde la posición prona, intubación nasofaríngea, glosopexia con adhesión labial, distracción mandibular hasta la traqueostomía. Se presentó una variante de tratamiento quirúrgico de urgencia realizada a un recién nacido portador de una secuencia Robin con dificultad respiratoria. Se realizó una labio-glosopexia de Routledge. El paciente tuvo una evolución postoperatoria favorable sin dificultades en su ventilación y alimentación.

The Robin sequence, previously known as Pierre Robin syndrome, is charaterized by the presence of micrognathia or retrognathia, glossoptosis with or without cleft palate. The newborns with this syndrome may present airway obstruction, feeding difficulties, growth retardation and chronic hypoxemia. Half of these patients have associated malformations. The incidence is almost 1: 8 500. The current options for the treatment of the airway obstruction in these patients range from prone position, nasofaringeal intubation, glossopexy with lip adhesion, mandibular distraction up to tracheostomy. We presented a variant of emergency surgical treatment carried out in a newborn having a Robin sequence with respiratory difficulty. A Routledge´s lip-glossopexy was made. The patient had a favorable post-surgery evolution in his ventilation and feeding.

A two-stage approach to induction and intubation of two infants with Pierre Robin Sequence using a LMA Classic™ and Air-Q®: two cases report / 대한마취과학회지

The laryngeal mask airway (LMA) Classic™ and Air-Q® are supralaryngeal devices used for airway management in routine and difficult pediatric airways. We describe a novel two-stage technique of insertion of the LMA Classic™ awake prior to induction of anesthesia, to assure oxygenation and ventilation, and after induction removal and placement of the Air-Q® for intubation using the flexible fiberoptic bronchoscope. The LMA Classic's™ pliable design and relatively small size allow it to be easily placed in awake infants. In contrast, the Air-Q® is an excellent device for intubation because of its larger internal diameter and removable 9 mm adapter. Our goal was to reduce unpredictability and potentially increase the safety of induction of anesthesia and intubation in infants with Pierre Robin sequence. By using these devices in a two-stage approach we created a technique for consistent oxygenation, ventilation, and intubation in these infants.

Assessment of speech nasality in children with Robin Sequence / Avaliação da nasalidade de fala em crianças com Sequência de Robin

PURPOSE: To report the outcomes of primary palatoplasty in Robin Sequence (RS); to verify the relationship between modalities of assessment of nasality; to compare nasality between techniques at palatoplasty. METHODS: This study involved the identification of hypernasality in four modalities: live assessment with 4-point scale; live assessment with cul-de-sac test; multiple listeners' ratings of recorded phrase; nasometric assessment. Live ratings of speech nasality and nasalance scores were retrieved from charts, while a recorded phrase was rated by listeners for occurrence of hypernasality. Agreement between the modalities was established as well as association between nasality, nasal turbulence and age at surgery and at assessment. Fisher's exact test was used to compare findings between surgical techniques. RESULTS: Agreement between nasalance, live assessment with 4-point scale, live assessment with cul-de-sac, and multiple listeners' ratings of recorded samples ranged between reasonable (0.32) and perfect (1.00). Percentage occurrence of hypernasality varied largely between assessment modalities. Mean occurrence of hypernasality was lower for the group submitted to Furlow technique (26%) than the group that received von Langenbeck technique (53%). Only findings obtained live were statistically significant (scale: p=0.012; cul-de-sac: p<0.001). Listeners identified nasal turbulence for 22 (32%) samples out of the 69 recordings, and an association was found between hypernasality and nasal turbulence. CONCLUSION: Lower occurrence of hypernasality was identified for patients with RS in Furlow group. Identification of hypernasality varied largely among the four assessment modalities. .

OBJETIVO: Reportar os resultados da palatoplastia primária na Sequência de Robin (SR); verificar a relação entre modalidades de avaliação da nasalidade; comparar nasalidade entre técnicas na palatoplastia. MÉTODOS: Este estudo envolveu a identificação da hipernasalidade em quatro modalidades: avaliação ao vivo com escala de quatro pontos; avaliação ao vivo com teste cul-de-sac; julgamento de gravações por juízes e avaliação nasométrica. Julgamentos ao vivo da nasalidade e escores de nasalância foram obtidos em prontuários, enquanto uma frase gravada foi julgada por juízes para ocorrência de nasalidade. Concordância entre as quatro modalidades foi estabelecida assim como associação entre nasalidade, turbulência nasal e idades na cirurgia e na avaliação. Teste exato de Fisher foi usado para comparar achados entre as técnicas cirúrgicas. RESULTADOS: A concordância entre nasalância, avaliação ao vivo com escala de quatro pontos e com teste cul-de-sac e julgamentos de gravações por juízes variou entre razoável (0,32) e perfeita (1,00). Porcentagem de ocorrência de hipernasalidade variou muito entre as diferentes modalidades. Ocorrência média de hipernsalidade no grupo operado com técnica de Furlow foi menor (26%) do que no grupo que recebeu a técnica de von Langenbeck (53%). Somente os resultados avaliados ao vivo foram estatisticamente significantes (escala: p=0,012; cul-de-sac: p<0,001). Juízes ouviram turbulência nasal em 22 (32%) das 69 gravações e uma associação entre hipernasalidade e turbulência nasal foi encontrada. CONCLUSÃO: Ocorrência de hipernasalidade foi menor para os pacientes com SR que receberam a técnica de Furlow. Identificação da hipernasalidade variou grandemente entre as quatro modalidades de avaliação. .

Sequência de Robin e suas repercussões sobre a microbiota bucal: revisão de literatura / Robin sequence and its repercussions on the oral microbiota: literature review

A cavidade bucal é a principal porta de entrada dos micro-organismos existentes no meio externo para o interior do corpo humano. A sequência de Robin é descrita como uma tríade de anomalias, caracterizada por micrognatia (hipodesenvolvimento mandibular) e glossoptose (deslocamento posterior da língua, que causa obstrução na faringe), com ou sem fissura palatina associada. Diante disso, o objetivo do presente estudo foi realizar uma revisão de literatura enfatizando as características principais e peculiares da microbiota bucal de crianças com sequência de Robin. A dificuldade respiratória, de ingestão de nutrientes, aleitamento materno insuficiente, otites recorrentes, entre outras complicações, ocorrem com frequência em crianças com essa patologia, influenciando a composição da microbiota bucal. A associação da palatoplastia e o uso de antibiótico intensificam as mudanças nessa microbiota. Novas investigações devem ser realizadas, para verificar quais fatores relacionados com a malformação craniofacial, ao tratamento cirúrgico e ao uso de antimicrobianos estariam implicados na alteração da microbiota bucal descrita neste trabalho, bem como suas repercussões clínicas...

Progress in diagnosis and treatment of neonatal upper airway obstruction / 国际儿科学杂志

The most common etiology of neonatal upper airway obstruction is congenital abnormalities,including choanal atresia,Pierre Robin sequence,occupational lesions,laryngomalacia,and so on.Some patients are after birth with dyspnea,severe cases with suffocation,and even death.Due to lack of specific clinical manifestations in early neonatal periode,it is usually misdiagnosed as other respiratory diseases.Therefore,early identification of the etiology,as soon as possible to relieve the obstruction,is particularly critical in the prevention of suffocation for the upper airway obstruction in the neonatal period.

Sequential Management of Pierre Robin Sequence: Case Report

Postural management of airway obstruction secondary to Pierre Robin Sequence

Pierre Robin Sequence commonly presents with upper airway obstruction secondary to craniofacial anomalies and can be managed with several treatment modalities. In mild cases, non-invasive postural management is a good treatment option. Prone position has been established as the posture of choice to reduce potential upper airway obstruction. We report a case of Pierre Robin Sequence with airway obstruction that did not respond to prone position but responded to supine position with slight tilting of the head.

Tongue-Lip Adhesion Using an Alveolar Protector Appliance for Management of Pierre Robin Sequence

PURPOSE: Pierre Robin sequence is a congenital malformation in which micrognathia causes glossoptosis and airway obstruction. If conservative treatment fails, surgical procedures such as tongue-lip adhesion can be performed. However, this procedure remains a subject of debate, with favorable results being countered by reports of complications. To overcome the above limitations, we revised the traditional method of tongue-lip adhesion using an alveolar protector. METHODS: Between 1992 and 2011, a total of eight patients were identified with Pierre Robin sequence and were treated with tongue-lip adhesion. Two of these eight tongue-lip adhesion procedures were performed with an alveolar protector. The operative technique for tongue-lip adhesion was similar to that described in other published reports. The alveolar protector was inserted between the ventral surface of the tip of the tongue and the lower labial sulcus. RESULTS: Tongue-lip adhesion failed in two patients because of wound dehiscence. The primary surgical success rate was 66.7%. In the two tongue-lip adhesion procedures performed with the alveolar protector, we observed no postoperative complications. CONCLUSION: Resistance to traction of the tongue can be encountered with nonunionized symphysis menti, causing loosening of the traction suture through the symphysis menti. This can lead to backward positioning of tongue, resulting in dehiscence of tongue lip adhesion. The alveolar protector is a good adjunct to tongue-lip adhesion because this method avoids postoperative loosening of the traction suture and wound dehiscence. It is a simple and effective auxiliary method that yields functional improvement.

Síndrome de Pierre-Robin y hepatoblastoma: reporte de un caso / Pierre-Robin syndrome and hepatoblastoma: a case report

Introducción. La secuencia de Pierre-Robin (SPR) es una afección congénita caracterizada por micrognatia, glosoptosis y fsura del paladar blando. La prevalencia es 1:8500 recién nacidos (RN) vivos. El hepatoblastoma (HB) es el tumor maligno primario de hígado más frecuente en pediatría. Afecta niños menores de 3 años. Caso clínico. Se trata de un paciente masculino de 2 años de edad, producto de la gesta II, de término, obtenido por cesárea, peso al nacer de 2, 750 g, con diagnóstico de SPR. A los 2 años se detecta masa abdominal dependiente de hígado. Se diagnostica HB, por lo que requiere quimioterapia y cirugía (lobectomía izquierda). A 5 años de seguimiento está vivo sin actividad tumoral. Conclusiones. La asociación de SPR y HB no se encontró reportada previamente en la literatura. Ambas presentan alteraciones en el cromosoma 2. Dado que el cáncer cursa con alteraciones citogenéticas, debemos de establecer asociaciones entre aquellos pacientes que cursan con síndromes genéticos. Esto permite evaluar blancos moleculares útiles y diseñar terapias dirigidas. También permite detectar poblaciones de riesgo de padecer cáncer y crear programas de seguimiento y evaluación que permitan una detección temprana para mejorar la sobrevida de dichos grupos. Se trata del primer caso de asociación entre estas dos patologías reportado en nuestro instituto y en el mundo.

Background. Pierre-Robin Sequence (PRS) is a congenital disease characterized by micrognatia, glosoptosis and U-shaped posterior cleft palate. Its prevalence is 1:8500 newborns. Hepatoblastoma (HB) is the most frequent hepatic malignant tumor in childhood and affects patients <3 years of age. Case report. A 2-year old male with PRS was diagnosed with HB. The boy was the product of the second full term pregnancy. He was born by cesarean with a birth weight of 2750 g. The patient presented with a 1-month history of abdominal mass. Diagnosis of HB was done by biopsy. The patient received chemotherapy with cisplatin, vincristin and 5-fuorouracil as well as left lobectomy. Five years later he is alive without tumor activity. Conclusions. PRS and HB association has not been reported in the literature. Both entities have chromosome 2 alterations. Because cancer is associated with cytogenetic alterations, future considerations must be made to fnd a relationship between cancer patients and other genetic entities. This will be useful for fnding molecular targets. We can also detect the at-risk population in order to create follow-up programs that will allow an early diagnosis with an improved better survival. It is the frst case of PRS and HB reported in either the national or international literature.

COMBINATION THERAPY USING GLOSSOPEXY AND RADIOFREQUENCY THERAPY IN PIERRE ROBIN SEQUENCE

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.