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.

A case of BMP2 gene variation-caused short stature, facial dysmorphism and skeletal anomalies with or without cardiac anomaly syndrome / 中华围产医学杂志

This article reported the genetic analysis of a case diagnosed with fetal micrognathia and cleft palate by mid-trimester ultrasound in two consecutive pregnancies. In the first pregnancy, the pregnant woman delivered a full-term boy transvaginally, who died two weeks after birth and was diagnosed with Pierre Robin sequence (PRS). Chromosome karyotype and genomic copy number variation. In the second pregnancy, the woman underwent amniocentesis due to suspected PRS presenting by fetal cleft palate, micrognathism, and additional ultrasound anomalies. No abnormalities were detected in fetal karyotype or genomic copy number variation. Whole-exome sequencing, bioinformatics analysis, and Sanger sequencing suggested that both the fetus and the firstborn boy inherited a possible pathogenic variant of c.79delG p.E27Sfs*24 in the BMP2 gene from the mother. The pregnancy was terminated after the genetic consultation. Fetal phenotypes in the two fetuses were similar, indicating that short stature, facial dysmorphism, and skeletal anomalies with or without cardiac anomaly in the pedigree were caused by the heterozygous variant of c.79delG p.E27Sfs*24 in the BMP2 gene.

Respiratory sleep disorders, nasal obstruction and enuresis in children with non-syndromic Pierre Robin sequence / Desordens respiratórias do sono, obstrução nasal e enurese em crianças com sequência de Pierre Robin não sindrômica

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.

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

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

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

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.

Síndrome de Ohtahara: presentación de un caso asociado a Síndrome de Pierre Robin / Ohtahara syndrome: a case presentation associated with Pierre Robin syndrome

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.

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.

Efficacy of nasotracheal intubation for airway management in neonates with Pierre Robin sequence undergoing surgery with general anesthesia / 中华麻醉学杂志

Objective To evaluate the efficacy of nasotracheal intubation for airway management in neonates with Pierre Robin sequence undergoing surgery with general anesthesia. Methods Sixty full-term neonates of both sexes with Pierre Robin Sequence, aged 2-28 days, weighing 2. 4-3. 8 kg, scheduled for elective mandibular distraction osteogenesis, were divided into 2 groups ( n=30 each) using a random num-ber table method: nasotracheal intubation group (group N) and orotracheal intubation group (group O). Combined intravenous-inhalational anesthesia was used during surgery. The success rate of intubation, intu-bation time, heart rate, mean arterial pressure and SpO2 during intubation, and occurrence of complica-tions during intubation were recorded, and the time of extubation and occurrence of complications after extu-bation were also recorded. Results Compared with group O, the intubation time was significantly short-ened, the first-time intubation success rate was increased, the heart rate and mean arterial pressure were decreased at 2 min after intubation, the lowest SpO2 was increased during intubation, and the incidence of pharyngeal soft tissue injury during intubation and hoarseness after extubation was decreased in group N ( P<0. 05) . Conclusion Nasotracheal intubation can be used for airway management in neonates with Pierre Robin sequence undergoing surgery under general anesthesia, and the efficacy is superior to that of orotra-cheal intubation.

Analysis of anatomical characteristics of upper airway in Pierre Robin sequence pediatric patients with difficult laryngoscopy: computed tomography-based three-dimensional reconstruction / 中华麻醉学杂志

Objective To analyze the anatomical characteristics of the upper airway in Pierre Robin sequence pediatric patients with difficult laryngoscopy using the computed tomography-based three-dimensional reconstruction.Methods Fifty pediatric patients of both sexes with Pierre Robin sequence,aged 10-101 days,weighing 2.0-6.3 kg,of American Society of Anesthesiologists physical status Ⅲ,scheduled for elective mandibular distraction osteogenesis under general anesthesia,were enrolled in this study.Cone beam CT scan was performed to obtain upper airway anatomy information during the natural sleep before operation.Images were imported into medical engineering software MIMICS 17.0 to reconstruct the three-dimensional images of the oral and maxillofacial bones and airways.The related anatomical parameters were measured,including the distance between the alveolar ridge of the upper central incisor and root of the epiglottis (D1),distance between the root of the epiglottis and midpoint of glottis (D2),distance between the bilateral lower edge of the mandible and midpoint of glottis (D3),distance between the alveolar ridge of the lower central incisor and the lower edge of the mandible (D4),length of the mandibular ramus (D5),length of the mandible body (D6),and length of the total mandible (D7),angle between lines D1 and D2 (angle 1),the angle between line D2 and the alveolar ridge of the upper central incisor to the midpoint of glottis (angle 2),the angle between lines D3 and D4 (angle 3),the angle of the point of the upper central incisor alveolar ridge to the trailing edge of the hard palate and then to the root of epiglottis (angle 4),the angle of bilateral mandible (angle 5),the angle of the point of gnathion to the two gonions (angle 6),the airway cross-sectional area at the tip of epiglottis,volume of oral cavity,volume of velopharyngeal cavity,and volume of glossopharyngeal cavity.Fiberoptic bronchoscope-guided endotracheal intubation was performed under topical anesthesia with lidocaine.Propofol,sufentanil and cis-atracurium were intravenously injected to induce anesthesia after successful intubation,and then the pediatric patients were sent to the operating room.Anesthesia was maintained by inhalation of sevoflurane.The exposure of glottis was observed with a laryngoscope.Pediatric patients were divided into difficult laryngoscopy group (group A) and non-difficult laryngoscopy group (group B) according to whether they presented with difficult laryngoscopy (Cormack-Lehane classification Ⅲ or Ⅳ).Results Compared with group B,the airway cross-sectional area at the tip of epiglottis and in the volume of velopharyngeal cavity were decreased (P＜0.05),and no significant change was found in D1,D2,D3,D4,D5,D6,D7,angle 1,angle 2,angle 3,angle 4,angle 5,angle 6,volume of oral cavity or volume of glossopharyngeal cavity in group A (P＞0.05).Conclusion The three-dimensional CT images of the upper airway show characteristic changes in Pierre Robin sequence pediatric patients with difficult laryngoscopy,and the main manifestations are the decrease in the airway section area and in the volume of the palatopharyngeal cavity at the tip of the epiglottis.

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.

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.

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.

Perioperative monitoring characteristics of infants with severe Pierre Robin syndrome / 中国小儿急救医学

Objective To study perioperative monitoring characteristics of severe Pierre Robin syn-drome in children. Methods The clinical data of 48 infants with Pierre Robin syndrome admitted to the de-partment of surgical intensive care unit from March 2013 to January 2015 were retrospectively reviewed. Results All 48 cases were cured and discharged without death. The average duration of mechanical ventila-tion after the operation was(2. 76 ± 3. 58)days. There were significant differences in blood gas analysis before and after treatment in surgical intensive care unit(pH:7. 33 ± 0. 07 vs. 7. 44 ± 0. 08,PaCO2:(49. 12 ± 15.63)mmHg vs.(36.19 ±8.13) mmHg,PaO2:(74.28 ±17.09) vs. (114.00 ±53.55) mmHg)(P ＜0. 05,respectively). The levels of prealbumin and retinol binding protein increased significantly after treatment [prealbumin:(0. 10 ± 0. 04) g/L vs. (0. 14 ± 0. 05) g/L,retinol binding protein:(19. 85 ± 9. 76) mg/L vs. (25.15 ±8.72)m g/L](P＜0.05,respectively). Conclusion Reasonable mechanical ventilation combined with available nourishments support could help to improve the prognosis of severe Pierre Robin syndrome.

Efficacy of airway topical anesthesia with combination of superior laryngeal nerve block and thyro-cricoid membrane puncture for tracheal intubation in pediatric patients with Pierre Robin Sequence / 中华麻醉学杂志

Objective To evaluate the efficacy of airway topical anesthesia with combination of su-perior laryngeal nerve block ( SLNB) and thyrocricoid membrane puncture for tracheal intubation in the ped-iatric patients with Pierre Robin Sequence. Methods Thirty-two American Society of Anesthesiologist physical statusⅠorⅡpediatric patients with Pierre Robin Sequence, aged 5-12 months, weighing 5-13 kg, scheduled for elective cleft palate repair under general anesthesia, were divided into 2 groups ( n=16 each) using a random number table method: control group ( group C) and airway topical anesthesia with SLNB-thyrocricoid membrane puncture group ( group ST) . After anesthesia was induced by inhaling sevoflu-rane by mask on admission to the operating room, 2％ lidocaine 0. 5 ml was injected around the bilateral su-perior laryngeal nerve under ultrasound guidance, and then 2％ lidocaine 1 ml was injected via the thyrocri-coid membrane in group ST, and the root of tongue, pharynx and larynx were sprayed with 2％ lidocaine by using a laryngotracheal mucosal atomization device in group C. The pediatric patients were tracheally intuba-ted guided by a video laryngoscope 3 min later. The development of cardiovascular responses, vocal cord activity and body movement was recorded during intubation. The intubation time, success rate of intubationat first attempt and patient′s tolerance to tube were recorded. The occurrence of postoperative hoarseness was also recorded. Results Compared with group C, the incidence of cardiovascular responses, vocal cord activity and body movement was significantly decreased, the intubation time was shortened, the suc-cess rate of intubation at first attempt was increased, and the patient′s tolerance to tube score was decreased in group ST ( P<0. 05) . Conclusion Airway topical anesthesia with combination of SLNB and thyrocricoid membrane puncture can provide better intubation conditions when used for the pediatric patients with Pierre Robin Sequence.

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.

Impact of promoting self-care in nursing workload / Impacto de la promoción del autocuidado en la carga laboral de enfermería / Impacto da promoção do autocuidado na carga de trabalho de enfermagem

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.

Tongue-lip adhesion in Pierre Robin sequence

Patients with Pierre Robin sequence exhibit varying degrees of airway obstruction and feeding difficulty. In some patients, airway obstruction may be profound, warranting surgical intervention to maintain a patent airway. The purpose of this article is to highlight the advantages of the tongue-lip adhesion procedure for the management of airway obstruction in such patients compared to the currently available options.

Clinical Factors Associated with the Non-Operative Airway Management of Patients with Robin Sequence

BACKGROUND: The indications for surgical airway management in patients with Robin sequence (RS) and severe airway obstruction have not been well defined. While certain patients with RS clearly require surgical airway intervention and other patients just as clearly can be managed with conservative measures alone, a significant proportion of patients with RS present with a more confusing and ambiguous clinical course. The purpose of this study was to describe the clinical features and objective findings of patients with RS whose airways were successfully managed without surgical intervention. METHODS: The authors retrospectively reviewed the medical charts of infants with RS evaluated for potential surgical airway management between 1994 and 2014. Patients who were successfully managed without surgical intervention were included. Patient demographics, nutritional and respiratory status, laboratory values, and polysomnography (PSG) findings were recorded. RESULTS: Thirty-two infants met the inclusion criteria. The average hospital stay was 16.8 days (range, 5–70 days). Oxygen desaturation (<70% by pulse oximetry) occurred in the majority of patients and was managed with temporary oxygen supplementation by nasal cannula (59%) or endotracheal intubation (31%). Seventy-five percent of patients required a temporary nasogastric tube for nutritional support, and a gastrostomy tube placed was placed in 9%. All patients continued to gain weight following the implementation of these conservative measures. PSG data (n=26) demonstrated mild to moderate obstruction, a mean apneahypopnea index (AHI) of 19.2±5.3 events/hour, and an oxygen saturation level <90% during only 4% of the total sleep time. CONCLUSIONS: Nonsurgical airway management was successful in patients who demonstrated consistent weight gain and mild to moderate obstruction on PSG, with a mean AHI of <20 events/hour.

Pierre Robin syndrome: a case report.

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