ABSTRACT
El síndrome de Jarcho-Levin es un epónimo usado para describir un espectro de displasias esqueléticas de tórax pequeño con el compromiso variable de vertebras y costillas. Inicialmente considerado letal, actualmente es compatible con la vida en sus presentaciones más leves. Las alteraciones óseas que conllevan un patrón respiratorio restrictivo, las infecciones respiratorias de repetición y el fenotipo particular pueden dificultar el manejo anestésico perioperatorio. Es de especial interés la adecuada valoración de la vía aérea por presentar predictores de vía aérea difícil, así como la prevención, el diagnóstico precoz y el tratamiento adecuado de la insuficiencia respiratoria. Presentamos el caso de un paciente con síndrome de Jarcho-Levin intervenido de distracción vertebral, con sus implicaciones más destacables en el manejo anestésico.(AU)
Jarcho-Levin syndrome is an eponym used to describe a spectrum of small thoracic skeletal dysplasias with variable involvement of vertebrae and ribs. Initially considered lethal, it is currently compatible with life in its mildest forms. Bone alterations that lead to a restrictive respiratory pattern, recurrent respiratory infections and particular phenotype can make perioperative anesthetic management difficult. The proper assessment of the airway is of special interest because it presents predictors of a difficult airway, as well as the prevention, early diagnosis and adequate treatment of respiratory failure. We present the case of a patient with Jarcho-Levin syndrome who underwent vertebral distraction surgery, with its most notable implications in anesthetic management.(AU)
Subject(s)
Humans , Female , Child , Respiratory Insufficiency , Thorax/abnormalities , Radiography, Thoracic , Congenital Abnormalities , Anesthesia/methods , Perioperative Period , Anesthesiology , Inpatients , Physical ExaminationABSTRACT
Jarcho-Levin syndrome is an eponym used to describe a spectrum of small thoracic skeletal dysplasias with variable involvement of vertebrae and ribs. Initially considered lethal, it is currently compatible with life in its mildest forms. Bone alterations that lead to a restrictive respiratory pattern, recurrent respiratory infections and particular phenotype, can make perioperative anesthetic management difficult. The proper assessment of the airway is of special interest because it presents predictors of a difficult airway, as well as the prevention, early diagnosis and adequate treatment of respiratory failure. We present the case of a patient with Jarcho-Levin Syndrome who underwent vertebral distraction surgery, with its most notable implications in anesthetic management.
Subject(s)
Abnormalities, Multiple , Anesthetics , Hernia, Diaphragmatic , Humans , Hernia, Diaphragmatic/surgery , Abnormalities, Multiple/diagnosis , Abnormalities, Multiple/surgery , SpineABSTRACT
ABSTRACTJarcho Levin syndrome is a rare disorder. There are various vertebral and costal anomalies. Severe deformities and abnormal fusion of ribs and vertebrae cause respiratory insufficiency and pneumonia. We present anaesthesia in a patient with Jarcho Levin syndrome for vesicoureteral reflux.
RESUMOA síndrome de Jarcho-Levin é um distúrbio raro que apresenta várias anomalias vertebrais e costais. Deformidades graves e fusões anormais das costelas e vértebras causam insuficiência respiratória e pneumonia. Apresentamos um caso de anestesia em paciente com síndrome de Jarcho-Levin para refluxo vesicuretral.
Subject(s)
Humans , Female , Infant , Hernia, Diaphragmatic/complications , Anesthesia/methods , Vesico-Ureteral Reflux/surgery , Abnormalities, Multiple , Laryngeal MasksABSTRACT
Jarcho Levin syndrome is a rare disorder. There are various vertebral and costal anomalies. Severe deformities and abnormal fusion of ribs and vertebrae cause respiratory insufficiency and pneumonia. We present anaesthesia in a patient with Jarcho Levin syndrome for vesicoureteral reflux.
ABSTRACT
Jarcho Levin syndrome is a rare disorder. There are various vertebral and costal anomalies. Severe deformities and abnormal fusion of ribs and vertebrae cause respiratory insufficiency and pneumonia. We present anaesthesia in a patient with Jarcho Levin syndrome for vesicoureteral reflux.