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OBJECTIVE: To describe the findings of children with Robin Sequence (RS) who received sensory-motor-oral stimulation combined with early sucking during mandibular distraction osteogenesis (MDO), compared with children who did not receive the intervention. DESIGN: A quasi-experimental study. Setting: A tertiary public hospital. Patients: Children with RS referred to MDO. A historical group from the same population but managed according to the institution's standard protocol (no sucking) served as a control group. Interventions: Sensory-motor-oral stimulation, including sucking, starting 24 h after MDO (intervention group). Main Outcome Measure: Our hypothesis is that sensory-motor-oral stimulation, including sucking during the DOM process, do not negatively affect surgical outcomes. RESULTS: Twenty-nine children were included. Eight (72.7%) of the 11 patients in the intervention group and 13 (72.2%) of the 18 controls had MDO complications, with no significant difference between the groups (p = 1.000). The most common surgical outcome was antibiotic therapy for surgical site infection (76.2%). Six months after MDO, 22 (75.9%) children attained full oral feeding or associated with alternative feeding methods. CONCLUSION: The intervention group did not have higher complication rates, from a surgical point of view, than control group. The protocol adopted by some centers that contraindicates sucking during MDO should be revised to consider the benefits of such stimulation. Keywords: Pierre Robin Syndrome, deglutition, therapeutics, child development.

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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.

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Dysphagia in Robin Sequence can be present in varying degrees, requiring multidisciplinary management and specific swallowing assessment by a specialist. Most studies published to date have evaluated only respiratory outcomes, and the available evidence on the improvement of swallowing is questionable. To conduct a systematic review and meta-analysis of studies evaluating swallowing in children with Robin Sequence before and after airway clearance procedures. The research question was developed based on the PICO strategy. The literature search was performed in electronic databases and gray literature. Studies were selected by 3 independent reviewers. The risk of bias and level of evidence of the studies were assessed. A proportion meta-analysis was performed to calculate the prevalence of dysphagia after airway clearance procedures. The search identified 4938 studies, 5 of which were included. All studies had limitations in terms of design and sample size. The prevalence of dysphagia after airway clearance was obtained by analyzing treatment subgroups: mandibular distraction osteogenesis, mandibular distraction osteogenesis + tracheostomy tube, and nasopharyngeal tube. Clinical and/or instrumental assessment was assessed by a swallowing specialist. The meta-analysis was precluded by the limitations of the studies, especially regarding sample size, which affected the accuracy of the findings. Dysphagia remained unresolved in 55% of children (95% CI 1-99%). The methodological quality of the studies indicated a high risk of bias and very low level of evidence. It was not possible to confirm that airway clearance techniques used in Robin Sequence improve dysphagia.

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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.

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INTRODUCTION: Obstructive sleep apnea is highly prevalent in non-syndromic Pierre Robin sequence patients. Studies have found a probable relationship between obstructive sleep apnea and nasal obstruction and between obstructive sleep apnea and enuresis. Assessment of the relationship between these variables in non-syndromic Pierre Robin sequence patients is scarce. OBJECTIVE: The present study aims to evaluate the relationship between symptoms of obstructive sleep apnea, nasal obstruction and enuresis, determining the prevalence of symptoms suggestive of these conditions, in schoolchildren with non-syndromic Pierre Robin sequence, and describe the prevalence of excessive daytime sleepiness habitual snoring and voiding dysfunction symptoms associated with enuresis. METHODS: This was a prospective analytical cross-sectional study developed at a reference center. Anthropometric measurements and a structured clinical interview were carried out in a sample of 48 patients. The instruments "sleep disorders scale in children" "nasal congestion index questionnaire" (CQ-5), and the "voiding dysfunction symptom score questionnaire" were used. Statistical analysis was performed for p < 0.05. RESULTS: Positive "sleep disorders scale in children" scores for obstructive sleep apnea and CQ-5 for nasal obstruction were observed in 38.78% and 16.33%, respectively. Enuresis was reported in 16.33% of children, being characterized as primary in 71.43% and polysymptomatic in 55.55%; according to the "voiding dysfunction symptom score questionnaire". There was a significant relationship between nasal obstruction and obstructive sleep apnea symptoms (p < 0.05), but no significance was found between obstructive sleep apnea symptoms and enuresis, and between nasal obstruction and enuresis. The prevalence of excessive daytime sleepiness was 12.24% and of habitual snoring, 48.98%. A family history of enuresis, younger age in years and a positive "voiding dysfunction symptom score questionnaire" score were associated with a higher prevalence of enuresis (p < 0.05). CONCLUSION: Children with non-syndromic Pierre Robin sequence are at high risk for obstructive sleep apnea symptoms and habitual snoring, with a correlation being observed between nasal obstruction and obstructive sleep apnea symptoms. In addition, the study showed that non-syndromic Pierre Robin sequence, obstructive sleep apnea and nasal obstruction symptoms were not risk factors for enuresis in these patients.

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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.

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Abstract Introduction Obstructive sleep apnea is highly prevalent in non-syndromic Pierre Robin sequence patients. Studies have found a probable relationship between obstructive sleep apnea and nasal obstruction and between obstructive sleep apnea and enuresis. Assessment of the relationship between these variables in non-syndromic Pierre Robin sequence patients is scarce. Objective The present study aims to evaluate the relationship between symptoms of obstructive sleep apnea, nasal obstruction and enuresis, determining the prevalence of symptoms suggestive of these conditions, in schoolchildren with non-syndromic Pierre Robin sequence, and describe the prevalence of excessive daytime sleepiness habitual snoring and voiding dysfunction symptoms associated with enuresis. Methods This was a prospective analytical cross-sectional study developed at a reference center. Anthropometric measurements and a structured clinical interview were carried out in a sample of 48 patients. The instruments "sleep disorders scale in children" "nasal congestion index questionnaire" (CQ-5), and the "voiding dysfunction symptom score questionnaire" were used. Statistical analysis was performed for p< 0.05. Results Positive "sleep disorders scale in children" scores for obstructive sleep apnea and CQ-5 for nasal obstruction were observed in 38.78% and 16.33%, respectively. Enuresis was reported in 16.33% of children, being characterized as primary in 71.43% and polysymptomatic in 55.55%; according to the "voiding dysfunction symptom score questionnaire". There was a significant relationship between nasal obstruction and obstructive sleep apnea symptoms (p< 0.05), but no significance was found between obstructive sleep apnea symptoms and enuresis, and between nasal obstruction and enuresis. The prevalence of excessive daytime sleepiness was 12.24% and of habitual snoring, 48.98%. A family history of enuresis, younger age in years and a positive "voiding dysfunction symptom score questionnaire" score were associated with a higher prevalence of enuresis (p< 0.05). Conclusion Children with non-syndromic Pierre Robin sequence are at high risk for obstructive sleep apnea symptoms and habitual snoring, with a correlation being observed between nasal obstruction and obstructive sleep apnea symptoms. In addition, the study showed that non-syndromic Pierre Robin sequence, obstructive sleep apnea and nasal obstruction symptoms were not risk factors for enuresis in these patients.

Resumo Introdução A apneia obstrutiva do sono apresenta elevada prevalência na população com sequência de Pierre Robin não sindrômica. Estudos constataram provável relação entre apneia obstrutiva do sono e obstrução nasal e entre apneia obstrutiva do sono e enurese, é escassa a avaliação da relação entre essas variáveis na sequência de Pierre Robin não sindrômica. Objetivo Avaliar a relação entre sintomas de apneia obstrutiva do sono, obstrução nasal e enurese, determinar a prevalência de sintomas sugestivos dessas condições, em crianças em idade escolar com sequência de Pierre Robin não sindrômica, assim como descrever a prevalência de sonolência excessiva diurna, ronco habitual e sintomas de disfunção miccional associados à enurese. Método Estudo prospectivo transversal analítico desenvolvido em centro de referência. Fez‐se aferição antropométrica e entrevista clínica estruturada em amostra de 48 pacientes. Os instrumentos usados foram: "escala de distúrbios do sono em crianças", questionário "índice de congestão nasal" (CQ‐5) e questionário "sistema de escore de disfunção miccional". A análise estatística foi feita para p < 0,05. Resultados Escores positivos da "escala de distúrbios do sono em crianças" para apneia obstrutiva do sono e do CQ‐5 para obstrução nasal foram observados em 38,78% e 16,33%; respectivamente. Enurese foi relatada em 16,33% das crianças e foi caracterizada como primária em 71,43% e polissintomática em 55,55%; conforme "sistema de escore de disfunção miccional". Observou‐se relação significante entre obstrução nasal e sintomas de apneia obstrutiva do sono (p < 0,05), porém não significante entre sintomas de apneia obstrutiva do sono e enurese, e obstrução nasal e enurese. A prevalência de sonolência excessiva diurna foi de 12,24% e de ronco habitual, de 48,98%. História familiar de enurese, menor idade em anos e escore positivo na "sistema de escore de disfunção miccional" foram associados a maior prevalência de enurese (p < 0,05). Conclusão Crianças com sequência de Pierre Robin não sindrômica estão sob alto risco para sintomas de apneia obstrutiva do sono e ronco habitual, foi observada correlação entre obstrução nasal e sintomas de apneia obstrutiva do sono. Demonstrou‐se, também, que sequência de Pierre Robin não sindrômica, sintomas de apneia obstrutiva do sono e obstrução nasal não constituíram fator de risco para enurese na população estudada.

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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.

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RESUMEN El Síndrome de Ohtahara es una encefalopatía epiléptica de inicio temprano en la vida, caracterizada por convulsiones intratables, trazado de ondassupresión, tanto en etapa de sueño como vigilia en electroencefalograma, y asociado a retraso en el desarrollo motor y cognitivo. Se presenta el caso de un niño, derivado a los quince días de vida al Hospital Nacional, Paraguay, para descartar cardiopatía congénita, debido a la dependencia de oxígeno. Al ingreso se constata aspecto facial coincidente con el síndrome de Pierre Robin, estridor laríngeo, hipotonía cervical y micropene. Internado se descarta cardiopatía congénita con ecocardiografía. Se asume dificultad respiratoria y necesidad de oxígeno secundarios a obstrucción respiratoria por glosoptosis. Ecografía transfontanelar revela hemorragia intraventricular grado II, imagen quística en fosa posterior, megacisterna versus quiste neuroepitelial. A los 47 días presenta convulsiones tónico-clónicas en miembros y posición de opistótonos, es medicado con AcidoValproico, luego Vigabatrina, sin respuesta. Trazado electroencefalográfico muestra patrón de descargas-supresión. Desarrolla infección asociada a cuidados de la salud con foco respiratorio, presenta mal manejo de secreciones y evolución tórpida, falleciendo a los 110 días. El caso se destaca por presentarse síndrome epiléptico en un paciente con fenotipo correspondiente al síndrome de Pierre Robin; pero que además tiene micropene. La presencia de las tres características citadas no pudo ser englobada dentro de un síndrome descripto con anterioridad. En recién nacidos con dismorfia y síndrome convulsivo es importante descartar la presencia de alteración estructural en el sistema nervioso como responsable del síndrome.

ABSTRACT Ohtahara syndrome is an epileptic encephalopathy that starts early in life, characterized by intractable seizures, wave-suppression tracing, both in the sleep and a waking phases of the electroencephalogram, and associated with delayed motor and cognitive development. We present the case of a child, to the fifteen days of life referred to the Hospital Nacional, Paraguay, with is the days of like to rule out congenital heart disease, due to oxygen dependence. On admission, the facial appearance was coincident with Pierre Robin syndrome, laryngeal stridor, cervical hypotonia and micropenis was observed. Once admitted congenital heart disease was excluded by echocardiography. Respiratory failure and need for oxygen secondary to respiratory obstruction due to glossoptosis are assumed. Transfontanelar ultrasound reveals grade II intraventricular hemorrhage, cystic image in the posterior fossa, megacisternal versus neuroepithelial cyst. At 47 days, he presented tonic-clonic seizures in the limbs opisthotonos and position of, he was treated with valproic acid, then Vigabatrina, with no response. Electroencephalographic trace shows discharge-suppression pattern. Develops infection associated with health care from respiratory origen, presents poor management of secretions and torpid evolution, dying at 110 days. The case is characterized by epileptic syndrome in a patient with a phenotype corresponding to Pierre Robin syndrome; but also has micropene. The presence of the three mentioned fatures could not be included in a previously described syndrome. In newborns with dysmorphia and seizures it is important to rule out the presence of structural alteration in the nervous system as responsible for the syndrome.

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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.

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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.

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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.

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OBJECTIVES: To evaluate the association of polysomnographic parameters with clinical symptom severity in Robin sequence (RS) patients. METHODS: All patients diagnosed as presenting with RS at Hospital de Clínicas de Porto Alegre from October 2012 to June 2016 were enrolled. They were classified as isolated RS, RS-plus, and syndromic RS. Polysomnography (PSG) was performed, except for those patients in need of respiratory support. Symptom severity was evaluated as defined by the Cole et al. CLASSIFICATION: Ordinal OR (for the chance of increase in one grade on the clinical severity scale) and R2 (determination coefficient from ordinal logistic regression) were computed from data analysis. RESULTS: A total of 80 participants were enrolled in the study. Fifty-five of these were able to undergo polysomnography. Worsening of the studied PSG parameters was associated with increase in clinical severity grading, as follows: desaturation index (OR 1.27; 95% CI; 1.07-1.51; R2 = 19.8%; p = 0.006); apnea/hypopnea Index (OR 1.13; 95% CI; 1.01-1.26; R2 = 12.5%; p = 0.02); sleep mean oxygen saturation (OR 0.16; 95% CI; 0.05-0.52; R2 = 22.6%; p = 0.002); oxygen saturation nadir (OR 0.73; 95% CI; 0.56-0.96; R2 = 10.0%; p = 0.02); percentage of time with oxygen saturation <90% (OR 9.49; 95% CI; 1.63-55.31, R2 = 37.6%; p = 0.012); and percentage of time presenting with obstruction (OR 2.5; 95% CI; 1.31-4.76; R2 = 25.1%; p = 0.006). CONCLUSIONS: Polysomnography parameters were associated with severity of clinical manifestations in patients with RS. Oxyhemoglobin saturation-based parameters had surprisingly significant R2 values. Therefore, those parameters, which have traditionally been undervalued in other clinical settings, should also be assessed in the polysomnographic evaluation of RS patients.

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OBJECTIVES/HYPOTHESIS: To assess the performance of endoscopic grading systems of glossoptosis in identifying severe clinical manifestations in children with Robin sequence (RS). STUDY DESIGN: Nested cohort cross-sectional study. METHODS: All RS patients diagnosed at Hospital Clinics of Porto Alegre from October 2012 to June 2016 were enrolled in this cohort. Patients underwent sleep endoscopy and were classified according to Yellon (Y) and de Sousa et al. (S) scales. Symptom severity evaluation was performed as defined by Cole et al. The outcome of interest was Cole's clinical classification grade 3. RESULTS: Eighty patients were eligible for analysis. Sensitivity (Y: 56.2%, S: 28.1%, P < .001) and specificity (Y: 85.4%, S: 93.8%, P = .038) in identifying severe clinical symptoms patients (i.e., Cole grade 3) were statistically different between Y and S classifications. A low but significant overall correlation was observed for both Y (rho = 0.372, P < .001) and S (rho = 0.439, P < .001) classifications when compared with Cole classification. Diagnostic odds ratio (DOR) for Y (DOR: 7.53, 95% confidence interval [CI]: 4.15-10.90) and S (DOR: 5.87, 95% CI: 1.86-9.87) were equivalent (P = .92). Also, receiver operating characteristic curves area under the curve were not significantly different between them. The positive likelihood ratio was 3.86 (95% CI: 1.82-8.16) and 4.50 (95% CI: 1.32-15.36) for Y and S, respectively. CONCLUSIONS: Y and S grading systems showed a low sensitivity and moderate to high specificity in detecting patients with severe clinical manifestations. Correlation between Y/S and Cole et al. grading were also considered low. Development of a more discriminative anatomic grading system is still needed for this specific disorder. LEVEL OF EVIDENCE: 2b. Laryngoscope, 128:502-508, 2018.

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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.

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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.

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The study assessed the neurodevelopment of children with isolated Robin sequence (IRS) and evaluated if children treated exclusively with nasopharyngeal intubation (NPI) present delay in neurological development. The prospective and cross-sectional study was conducted at the Hospital for Rehabilitation of Craniofacial Anomalies, Brazil. Children with IRS were divided into two groups according to the type of treatment in early infancy: 38 were treated with NPI (more severe cases) and 24 with postural treatment (less severe cases). Regarding interventions, children were assessed at 2 to 6 years of age using the Denver II Developmental Screening Test (Denver II) and Neurological Evolutionary Examination (NEE). According to Denver II, 73.7% in the NPI group and 79.2% in the postural group presented normal development. This result was similar to the results of different studies in the literature with typical population. Considering all areas of development, there were no significant differences in Denver II between the NPI and postural groups (P = .854). In the NPI group, 89.5% of children and 87.5% in the postural group presented normal development in NEE. Language was the most affected area, as 18.4% and 20.8% of children in NPI and postural group, respectively, presented risk for delay in the Denver II. The increased risk for delay in language area was probably due to anatomical conditions of the muscles involved in speech, and to hearing oscillations, as 47.4% in NPI group and 58.3% in postural group underwent myringotomy. IRS treated with NPI had neurological development similar to those in less severe cases. Children treated exclusively with NPI did not present delay in neurological development.

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Abstract OBJECTIVES To assess the impact of promoting self-care in nursing workload and associate it to the variables: age, gender, socioeconomic status, education, marital status and number of children of caregivers. METHODS Prospective study with 31 children and their caregivers. Participants were assessed at two moments, 1st and 2nd hospitalization, the nursing workload was measured by the Nursing Activities Score (NAS). RESULTS The mean NAS in the 1st hospitalization was 60.9% and in the 2nd hospitalization was 41.6%, that is, 14.6 and 9.9 hours of nursing, respectively. The nursing workload on the first day of hospitalization was higher compared to the last day, both for the 1st (p<0.001) and for the 2nd hospitalization (p<0.001), and higher in the first (p<0.001) and in the last day (p=0.025) in the 1st hospitalization. Comparing the 1st hospitalization to the 2nd hospitalization, the first was higher (p<0.001), and NAS items related to the training of self-care was influenced (p<0.001). CONCLUSION The nursing workload associated to self-care promotion corresponded to 14.6 hours and was higher than determined by the existing legislation.

Resumen OBJETIVOS Verificar el impacto de la promoción del autocuidado en la carga laboral de enfermería y asociarla con las variables: edad, género, clasificación socioeconómica, escolaridad, estado civil y número de hijos de los cuidadores. MÉTODO Estudio prospectivo, en el que participaron 31 niños y sus respectivos cuidadores. Los participantes fueron evaluados en dos momentos, 1ª y 2ª hospitalización, en cuanto a la carga laboral de enfermería medida por medio del Nursing Activities Score (NAS). RESULTADOS El promedio NAS en la 1ª hospitalización fue del 60,9% y, en la 2ª, fue del 41,6%, es decir, 14,6 y 9,9 horas de enfermería, respectivamente. La carga de trabajo de enfermería el primer día de hospitalización fue mayor cuando comparada con el último día, tanto en la 1ª (p<;0,001) como en la 2ª hospitalización (p<;0,001), y mayor el primero (p<;0,001) y último día (p=0,025) en la 1ª hospitalización. En la 1ª hospitalización fue aún mayor cuando comparada con la 2ª hospitalización (p<;0,001), y los ítems NAS referentes a la capacitación del autocuidado la influenciaron (p<;0,001). CONCLUSIÓN La carga laboral de enfermería referente a la promoción del autocuidado correspondió a 14,6 horas y fue superior a lo determinado por la legislación existente.

Resumo OBJETIVOS Verificar o impacto da promoção do autocuidado na carga de trabalho de enfermagem e associá-la às variáveis: idade, gênero, classificação socioeconômica, escolaridade, estado civil e número de filhos dos cuidadores. MÉTODO Estudo prospectivo, onde participaram 31 crianças e seus respectivos cuidadores. Os participantes foram avaliados em dois momentos, 1ª e 2ª internação, quanto à carga de trabalho de enfermagem mensurada por meio do Nursing Activities Score (NAS). RESULTADOS A média NAS na 1ª internação foi de 60,9%, e na 2ª internação foi de 41,6%, ou seja, 14,6 e 9,9 horas de enfermagem, respectivamente. A carga de trabalho de enfermagem no primeiro dia de internação foi maior quando comparada ao último dia, tanto na 1ª (p<0,001) como na 2ª internação (p<0,001), e maior no primeiro (p<0,001) e último dia (p=0,025) na 1ª internação. Ainda, na 1ª internação, foi maior quando comparada à 2ª internação (p<0,001), e os itens NAS referentes à capacitação do autocuidado a influenciaram (p<0,001). CONCLUSÃO A carga de trabalho de enfermagem referente à promoção do autocuidado correspondeu a 14,6 horas e foi superior ao determinado pela legislação existente.

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ABSTRACTPierre Robin sequence is a neonatal disorder characterized by micrognathism, glossoptosis and cleft palate. We reported an autopsy case of a child whose malformations of the oropharynx were identified only at birth. The child was extremely preterm with severe neonatal depression and poor recovery, and the orofacial alterations prevented the correct treatment. There was facial disorder characterized by micrognathia associated with cleft palate and posterior displacement of the tongue, compressing the vallecula, structurally compatible with glossoptosis. This autopsy surpassed the scientific and epidemiological relevance, allowing the family genetic counseling and close monitoring of a subsequent pregnancy.

RESUMOSequência de Pierre Robin é uma doença neonatal caracterizada por micrognatismo, glossoptose e fenda palatina. Relatamos um caso de autópsia de uma criança cujas malformações da orofaringe somente foram identificadas ao nascimento. A criança era pré-termo extremo com depressão neonatal grave e má recuperação, e as malformações orofaciais impediram o correto tratamento. Havia alteração facial caracterizada por micrognatia associada a fenda palatina e posteriorização da língua, comprimindo a valécula, estruturalmente compatível com glossoptose. A autópsia extrapola as relevâncias científica e epidemiológica, permitindo o aconselhamento genético familiar e o monitoramento adequado da gestação subsequente.

RESUMO

A fissura de palato, em associação à Sequência de Pierre Robin, pode favorecer o desenvolvimento de produções atípicas (compensatórias), na fala da criança, como é o caso da oclusiva glotal (golpe de glote) comumente observada em substituição aos sons oclusivos (vozeados ou não). No presente estudo, foi realizada a análise dos parâmetros fonético-acústicos da oclusiva glotal produzidas em /k/ e /g/ por uma criança do gênero feminino, com 5 anos, que apresentava fissura de palato reparada, associada à Sequência de Pierre Robin. Para isso, foram selecionadas seis palavras em que a oclusiva velar encontrava-se na posição inicial da palavra e combinada com as vogais /a/, /i/ e /u/ na posição acentuada. Foi ainda realizado julgamento perceptivo-auditivo por três fonoaudiólogos, que apresentou concordância quanto à presença da oclusiva glotal de 100% para ambas as relações (intra e inter-juízes). Na inspeção dos dados via espectrograma foi observada variabilidade dos parâmetros espectrais (burst e transição formântica) e essas variações também puderam ser computadas considerando as vogais separadamente. A análise estatística revelou diferença estatisticamente significante entre as duas consoantes velares (/k/ e /g/) nos parâmetros espectral (burst), temporal (VOT e duração relativa da oclusiva na palavra) e os relativos às características acústicas das vogais adjacentes às oclusivas (período estacionário de F3). Por fim, as características acústicas da oclusiva glotal sugeriram que a criança pode ter utilizado de estratégias para marcar contrastes fônicos na língua, ainda que os mesmos não tenham magnitude suficiente para serem resgatados auditivamente pelo ouvinte.

The cleft palate, in association with Pierre Robin Sequence, may foster the development of atypical (compensatory) productions on the child’s speech such as the use of glottal stop as a substitution for occlusive sounds (voiced or voiceless). Acoustic parameters of glottal stop for /k/ and /g/ produced by a 5 year-old girl with cleft palate repaired in association with Pierre Robin Sequence were analyzed. For this study, we used six words consisting of velar stops in the initial word position combining the vowels /a/, /i/ e /u/ in the stressed position were selected. There was total agreement (100%) as for the presence of the glottal stop for both intra and inter-judges. Inspection of the data via spectrogram showed variability of spectral parameters (burst and formant transition), and variations could also be computed, separately considering the vowels. Statistical analysis revealed a statistical difference between the two velar consonants (/k/ and /g/) in spectral (burst), temporal (VOT and duration of occlusion on the word) parameters and those relating to the acoustic features of vowels adjacent to stops (stationary period of F3. The acoustic features of glottal stop suggest that the child may have used strategies to set out phonic contrasts in the language, but these strategies did not have enough magnitude to be perceived by the listener.