Abstract
Intrathoracic kidney is a very rare congenital anomaly with only about 50 cases reported in the world literature. Incidence of intrathoracic kidney with Bochdalek hernias was reported to be less than 0.25 percent. The relationship between them remains uncertain. We report a case in which the patient had a normal chest x-ray at birth, but at the age of 5 months an isolated left sided intrathoracic kidney with incomplete duplication was discovered. This was followed by ipsilateral diaphragmatic hernia at the age of nine months. Other coexisting malformations included hydrocephalus and an imperforate anus with perineal fistula. A primary repair of the diaphragmatic defect and a minimal posterior sagittal anorectoplasty were carried out at the age of nine months. Since then, the patient has had repeated admissions due to viral infection. Eventually she succumbed to pneumonia at the age of one year. This case raised the question "Does the ascent of metanephron continue postnatally and affect development of the diaphragm?"
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