Assuntos
Sons Respiratórios/etiologia , Cistos/complicações , Endoscopia , Hemangioma/complicações , Humanos , Lactente , Intubação/efeitos adversos , Doenças da Laringe/complicações , Laringe/anormalidades , Sons Respiratórios/congênito , Traqueia/anormalidades , Doenças da Traqueia/complicações , Estenose Traqueal/complicações , Paralisia das Pregas Vocais/complicaçõesAssuntos
Sons Respiratórios/congênito , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Sons Respiratórios/etiologiaRESUMO
Most laryngeal anomalies are supraglottic and laryngomalacia is the most common. Cysts, bifid epiglottis and absence of the epiglottis are uncommon. An 18-year-old Caucasian man had long-standing stridor caused by anomalous supraglottic structures: a small floppy epiglottis, enlarged accessory cartilages and redundant aryepiglottic folds. These structures were excised and the airway was improved. The ventral portions of the fourth arches become the aryepiglottic folds and lateral segments of the epiglottis. A disturbance in this portion of the fourth arch may explain the anomaly. The cartilaginous contributions to the epiglottis were possibly isolated as accessory cartilages. Epiglottic anomalies may be associated with other anomalies, especially the digits of the hand. This patient had a short lingual frenulum and mild macroglossia.
Assuntos
Epiglote/anormalidades , Cartilagens Laríngeas/anormalidades , Doenças da Laringe/congênito , Sons Respiratórios/congênito , Adolescente , Cartilagem Aritenoide/anormalidades , Cartilagem Aritenoide/patologia , Epiglote/patologia , Humanos , Cartilagens Laríngeas/patologia , Doenças da Laringe/etiologia , Masculino , Sons Respiratórios/etiologiaRESUMO
Two maternal cousins are described with the connatal form of Pelizaeus-Merzbacher Disease (PMD) and congenital stridor. Study of brain biopsy material confirms the diagnosis of PMD. The neuropathological findings are suggestive for the transitional form of this disease. Quantitative morphology gives support to the hypothesis that PMD is a disturbance in maturation of neurons and in myelin formation rather than an active degenerative process. The hereditary transmission is most consistent with a sex-linked recessive pattern. Different X-linked signs seem combined in the presented cases.
Assuntos
Esclerose Cerebral Difusa de Schilder/genética , Sons Respiratórios/congênito , Biópsia , Encéfalo/patologia , Pré-Escolar , Esclerose Cerebral Difusa de Schilder/congênito , Esclerose Cerebral Difusa de Schilder/patologia , Humanos , Lactente , Masculino , LinhagemRESUMO
Congenital stridor in children is a major sign of airway obstruction which occasionally may be alarming. The relevant embryology is discussed and the developmental, acquired or combined causes of airway obstruction producing congenital stridor are described. The need for careful assessment of chronic stridor in childhood is emphasized and the pros and cons of direct laryngoscopy under general anaesthesia in assessment are mentioned.