1,721,990 research outputs found
Rigid Bronchoscopy Through Tracheostome in a Case of Pierre Robin Syndrome - a Case Report
No full textA 3-year-old girl with Pierre Robin Syndrome, having complaints of respiratory distress in supine position and disturbance during sleep due to airway obstruction, was scheduled for adenoiodectomy. Gas induction without muscle relaxation was done and intubation was successful on fourth attempt. Five teeth were avulsed & one tooth was lodged in right main bronchus
Pierre Robin: algoritmo de manejo basado en la evidencia
Get PDFEl siguiente trabajo está basado en una extensa revisión bibliográfica e incluye la información más relevante sobre el tratamiento de la secuencia de Pierre Robin y el síndrome de mandíbula pequeña. Esta revision ofrece una guía de manejo basado en la evidencia con el objetivo de disminuir la morbimortalidad, mediante la unificación de los criterios diagnósticos y de tratamiento que permitan una atención óptima y oportunaAbstract. The following work is based on an extensive literature review and includes the most relevant information about Pierre Robin sequence and small jaw syndrome. This review offers a guide evidence based management with the goal of reducing morbidity and mortality, by unifying diagnostic criteria and treatment ensuring optimum and timely care.Otr
Cervico-thoracic kyphosis in a girl with Pierre Robin sequence
No full textCongenital cervico-thoracic kyphosis has been encountered in a girl with Pierre Robin sequence. The constellation of the spine malformation complex such as incomplete development of the vertebral bodies associated with defective ossification of the cervico-thoracic pedicles causing effectively the development of complete spinal cord injury at the kyphotic level of C7/T1 were present. Congenital kyphosis secondary to vertebral body hypoplasia has not been reported in connection with Pierre Robin sequence
SÍNDROME PIERRE ROBIN
No full textIntrodução: A Síndrome de Pierre Robin, também conhecida como Sequência de Pierre Robin (SPR) é considerada uma doença rara, caracterizada por uma tríade de anomalias, micrognatia (queixo pequeno), glossoptose (queda da língua para trás) que causa a obstrução de vias pulmonares e fissura de palato (céu da boca aberto). A Síndrome pode ser constatada desde o nascimento através de exames físico. Objetivo: identificar a assistência prestada pelos pais de crianças com a Síndrome de Pierre Robin no extremo oeste de Santa Catarina. Método: Trata-se de uma nota prévia, de um estudo a ser realizado no Oeste catarinense – SC. Os sujeitos de pesquisa serão os pais de crianças com SPR. A coleta de dados ocorrerá para fins de elaboração do trabalho de conclusão de curso em 2017, após receber a aprovação no Comitê de Ética em Pesquisa da UNOESC. Para a coleta de dados será utilizada as dependências domicílio/residência dos sujeitos pesquisados. Resultados esperados: busca-se a partir deste estudo identificar as dificuldades encontradas pelos pais, ao buscar recursos para diagnóstico, tratamento e os cuidados necessários com as crianças de SPR.Palavras-chave - Síndrome de Pierre Robin. Sequência de Pierre Robin. Enfermagem
Management of Feeding Problems in Infants with Cleft Palate and Review on Pierre-Robin Syndrome
Get PDFPierre Robin syndrome is a congenital disorder with triad of features micrognathia, glossoptosis which result in airway obstruction and feeding difficulties and cleft palate. Cleft palate is the most common congenital anomalies of craniofacial region seen in Pierre-Robin syndrome. Infants with cleft palate have to face various problems. Difficulty in feeding is the most common problem faced by cleft palate neonates that made them difficult to maintain adequate nutrition, result in failure to thrive. There are many methods given in literature to overcome this problem including special type of nursing bottles. In this case report, fabrication of feeding plate to obturate the defect in palate and its use in managing feeding problem is described. Keywords: Cleft palate, Feeding plate, PierreRobin Syndrom
Debunking the Myth: Should Pierre Robin be Credited for Defining the Pierre Robin Sequence?
No full textInternational audienceThe Pierre Robin Sequence is a rare craniofacial malformation that invariably combines retrognathism, glossoptosis, and upper airway obstruction, with a prevalence ranging from 1 in 8000 to 1 in 14,000 newborns. Although formally described by the French physician Pierre Robin in 1923, older and even ancient as far back as the Babylonian times, descriptions have been identified. Dr Pierre Robin s perspicacity lay in putting up a treatment for the patients presenting those symptoms. That is why his successors honored his work by naming this disease after him
Pierre Robin syndrome: a case report
Get PDFPierre 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
Pierre Robin sequence: two case reports
No full textPierre Robin sequence was first described as a syndrome;
however, it is currently recognized as a sequence that includes mi crognathia, glossoptosis, and paroxysmal respiratory obstructions.
Airway long-term treatment is necessary to avoid serious compli cations of this condition. Mandibular distraction osteogenesis (MDO)
is an effective treatment for tongue-based airway obstruction in
children with severe Pierre Robin sequence. The authors de scribed 2 cases of new-born children diagnosed with severe Pierre
Robin sequence, treated according to a fixed protocol, that is, fol lowing diagnosis, the children were taken to the surgical center
for tracheofibroscopy and tracheostomy in cases of tongue im pingement. Following general anesthesia, the patients were submitted
to MDO using a 20 mm pediatric distractor, bilaterally. After 1 mm
of distraction daily and adequate bone healing, distractors were removed and the airway evaluated. The patients showed an im provement of the airway, allowing tracheostomy removal. In
conclusion, mandibular distraction is an effective procedure for
Pierre Robin sequence
Management of infants with Pierre Robin sequence
No full textPierre Robin sequence is a congenital disorder classically characterized by retrognathia, glossoptosis and upper airway obstruction with or without cleft palate. This condition affects neonates and can cause serious respiratory and feeding difficulties requiring prompt intervention. Currently there are no standardized management algorithms for neonates with Pierre Robin sequence and management of the condition remains a challenge. Assuring adequate breathing and feeding should always be the first point of concern. Early diagnosis, sequential planning of treatment, adequate monitoring and multidisciplinary approach are essential for infants referred with Pierre Robin sequence. We discuss here the full scope of the disease and the various management options
Familial Occurrence of Pierre Robin Anomalad
Get PDFWe have presented a family in which three children (1 male and 2 females) who had two different fathers were affected with the Pierre Robin anomalad. The mother exhibits some of the characteristic stigmata of this syndrome which strongly suggests that an autosomal dominant from of the Pierre Robin anomalad exists with variable expressivity
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