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Master's Dissertation
DOI
10.11606/D.61.2011.tde-21062011-154627
Document
Author
Full name
Isabel Cristina Drago Marquezini Salmen
E-mail
Institute/School/College
Knowledge Area
Date of Defense
Published
Bauru, 2011
Supervisor
Committee
Marques, Ilza Lazarini (President)
Borgo, Hilton Coimbra
Trindade, Amélia Arcângela Teixeira
Title in Portuguese
Sequência de Robin: estudo retrospectivo dos lactentes internados no HRAC-USP
Keywords in Portuguese
Fissura palatina
obstrução das vias respiratórias
síndrome de Pierre Robin
transtornos de deglutição
Abstract in Portuguese
Objetivos: descrever as características dos lactentes com Sequência de Robin (SR) atendidos no Hospital de Reabilitação de Anomalias Craniofaciais da Universidade de São Paulo (HRAC-USP) e identificar as variáveis associadas a um protocolo terapêutico: documentar as síndromes associadas; verificar o tipo de obstrução respiratória, os sintomas clínicos e intervenções terapêuticas; verificar a ocorrência de disfagia, complicações, comorbidades e óbitos. Material e Métodos: Foram avaliados retrospectivamente 223 lactentes com SR, menores de um ano de idade, internados no HRAC-USP no período de julho de 2003 a junho de 2008. Resultados: 52% dos lactentes eram do sexo masculino, 121 (54%) apresentavam provável Sequência de Robin isolada (SRI) e102 (46%) apresentavam Sequência de Robin associada à síndrome ou associada a outras anomalias (SRS). As síndromes mais freqüentes foram síndrome de Stickler e síndrome de Moebius. 45% dos lactentes internaram antes de um mês de idade e o tempo médio de hospitalização foi de 20 dias. O tipo de obstrução respiratória mais freqüente, diagnosticado pela nasofaringoscopia, foi tipo 1, presente em 68% dos casos. A maioria dos lactentes (81%) foi tratada conservadoramente e a intubação nasofaríngea foi o tratamento mais utilizado (48%). A traqueostomia foi realizada em 19% dos lactentes e destes a maioria era do grupo SRS. A quase totalidade dos lactentes apresentava disfagia, a qual foi mais grave nos que apresentavam obstrução tipo 3 e 4, nos submetidos à traqueostomia e nos do grupo SRS. A gastrostomia foi realizada em 25% dos lactentes e a doença de refluxo gastroesofágico ocorreu em 54% do total de lactentes estudados. A complicação mais freqüente foi pneumonia e a mortalidade foi 5,38%, sendo que todos os pacientes que evoluíram para óbito eram sindrômicos. Conclusões: A maioria dos lactentes com SR pode ser tratada conservadoramente e a intubação nasofaríngea foi o método mais empregado. As dificuldades alimentares foram universais e relacionadas ao grau de obstrução respiratória.
Title in English
Robin sequence: retrospective review of infants hospitalized at HRAC - USP
Keywords in English
Airway obstruction
cleft palate
Pierre Robin syndrome
swallowing disorders
Abstract in English
Objectives: to describe the characteristics of infants with Robin Sequence (SR) treated at the Hospital for Rehabilitation of Craniofacial Anomalies - University of São Paulo (HRAC-USP) and identify the variables associated with a therapeutic protocol; record the associated syndromes; verify the type of respiratory obstruction, clinical symptoms and therapeutic interventions; and to verify the occurrence of dysphagia, complications, comorbities and death. Material and Methods: A total of 223 infants with SR were retrospectively evaluated, all younger than one month of age, hospitalized at HRAC-USP in the period July 2003 to June 2008. Results: 52% of the infants were males, 121 (54%) presented probable Isolated Robin Sequence (SRI) and 102 (46%) exhibited Robin Sequence with associated syndrome or anomalies (SRS). The most frequent syndromes were Stickler syndrome and Moebius syndrome. Among the infants, 45% were hospitalized before one month of age and the mean time of hospitalization was 20 days. The most frequent respiratory obstruction diagnosed through nasopharyngoscopy was type 1, present in 68% of cases. Most of the infants were treated conventionally and nasopharyngeal intubation was the most used procedure (48%). Tracheostomy was performed in only 19% of infants, most of whom were syndromic. Nearly all infants presented dysphagia, which was more severe in infants with obstruction type 3 and type 4, submitted to tracheostomy, and in the SRS group. Gastrostomy was performed in 25% of infants and gastroesophageal reflux occurred in 54% of SR infants. The most frequent complication was pneumonia and the mortality rate was 5.38%; all cases of death occurred among syndromic children. Conclusions: Most of the SR infants were treated conventionally and nasopharyngeal intubation was the most used procedure. Feeding difficulties were universal and related to the degree of respiratory obstruction.
 
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Publishing Date
2011-06-21
 
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