OBJECTIVE: To drive pediatrician's attention to a syndrome wwich has as its typical clinical presentation a thoracic cage abnormality which evolves to respiratory distress and death during the first two years of life. METHODS: We describe three cases of patients with clinical and radiographic findings characteristic of asphyxiating thoracic dystrophy (Jeune's Syndrome): short and narrow thoracic cage and abnormal development of the ribs. A brief review of the literature on the subject is also included. RESULTS: The three patients are older then two years of age. They did not present life threatening pulmonary complication nor respiratory distress, in contrast to most of the cases reported in the literature. CONCLUSIONS: Even though the number of occurrences of this syndrome is quite low, it must be consider when conducting a differential diagnostic related to thoracic cage abnormalities. It is important to emphasize the degree of variability of the pulmonary lesions and the progress of the disease. However, most articles on the subject point to a grim outcome.
OBJECTIVE: To drive pediatrician's attention to a syndrome wwich has as its typical clinical presentation a thoracic cage abnormality which evolves to respiratory distress and death during the first two years of life. METHODS: We describe three cases of patients with clinical and radiographic findings characteristic of asphyxiating thoracic dystrophy (Jeune's Syndrome): short and narrow thoracic cage and abnormal development of the ribs. A brief review of the literature on the subject is also included. RESULTS: The three patients are older then two years of age. They did not present life threatening pulmonary complication nor respiratory distress, in contrast to most of the cases reported in the literature. CONCLUSIONS: Even though the number of occurrences of this syndrome is quite low, it must be consider when conducting a differential diagnostic related to thoracic cage abnormalities. It is important to emphasize the degree of variability of the pulmonary lesions and the progress of the disease. However, most articles on the subject point to a grim outcome.
Authors: Ingrid G Azevedo; Norrara S O Holanda; Nivia M R Arrais; Raweny T G Santos; Ana G F Araujo; Silvana A Pereira Journal: BMC Pediatr Date: 2019-09-26 Impact factor: 2.125