Congenital pulmonary<unk>  (PL) is a rare developmental disorder involving         the lung, and characterized by pulmonary subpleural, interlobar, perivascular and         peribronchial lymphatic dilatation. The prevalence is unknown. PL presents at birth         with severe respiratory distress, tachypnea and cyanosis, with a very high mortality         rate at or within a few hours of birth. Most reported cases are sporadic and the etiology         is not completely understood. It has been suggested that PL lymphatic channels of         the fetal lung do not undergo the normal regression process at 20 weeks of gestation.         Secondary PL may be caused by a cardiac lesion. The diagnostic approach includes complete         family and obstetric history, conventional radiologic studies, ultrasound and magnetic         resonance studies, lymphoscintigraphy, lung functionality tests, lung biopsy, bronchoscopy,         and pleural effusion examination. During the prenatal period, all causes leading to         hydrops fetalis should be considered in the diagnosis of PL. Fetal ultrasound evaluation         plays a key role in the antenatal diagnosis of PL. At birth, mechanical ventilation         and pleural drainage are nearly always necessary to obtain a favorable outcome of         respiratory distress. Home supplemental oxygen therapy and symptomatic treatment of         recurrent cough and wheeze are often necessary during childhood, sometimes associated         with prolonged pleural drainage. Recent advances in intensive neonatal care have changed         the previously nearly fatal outcome of PL at birth. Patients affected by PL who survive         infancy, present medical problems which are characteristic of chronic lung disease.
Pulmonary cystic lymphangiectasis
Aortopulmonary fistula
Bronchopleural fistula
Tracheal diverticulum
Bronchial atresia
Pulmonary arteriovenous fistula
Bronchogenic cyst
Mediastinal lipomatosis