ABSTRACT

Jeune syndrome or asphyxiating thoracic dystrophy characterized by narrow rib cage and respiratory distress, accompanied by a multi-organ anomaly is a rare disease. Patients usually die due to complications of respiratory distress and in their first year. 25-year-old man was admitted to our clinic with the diagnosis of Jeune syndrome. We detected radiologically that patient's rib cage was small and narrow and transverse and sagittal diameter of the rib cage was short . It has been reported in a small number of adult patients with Jeune syndrome. The respiratory distress determines the prognosis of patients. The respiratory problems decrease with age. Over time, kidney and liver damage may occur. For this reason, intensive respiratory support for these patients from birth and the follow-up of liver and renal functions are important.