IT IS generally agreed that the Marfan syndrome is a heritable disorder of connective tissue characterized by widespread malformations affecting the musculoskeletal, cardiovascular, and ocular systems. It was first described by Marfan in 1896 under the name of dolichostenomelie.' In 1902 Archard presented a case and called the syndrome arachnodactyly.2 Thus, the musculoskeletal malformations we...

Congenital lung abnormalities are relatively rare but very dangerous for the fetus and neonate. This article describes the most common pulmonary complications observed during pregnancy such as congenital cystic adenomatoid malformation, diaphragmatic hernia, bronchopulmonary sequestration, hydrothorax, chylothorax, lung agenesia, pulmonary hypoplasia. The diagnostic methods and available therap...

Numerous diverse entities produce cystic lung changes in neonates and young children. This review provides an evidence-based, age-appropriate, differential diagnostic framework to use when confronted with pulmonary cystic changes. The categories of diseases that have been discussed include congenital cystic bronchopulmonary malformations, neoplastic conditions, infections, collagen or soft tiss...

Malformations of the lower limbs are rare and heterogeneous anomalies. Some congenital anomalies involving face, gastrointestinal system, skeletal system, urogenital system, heart, lung and diaphragma associated with lower limb malformations have been described in the literature. Here, we report a case of left proximal femoral focal deficiency (PFFD) together with fibular aplasia associated wit...

Herein, we reported the case of a 32-year-old male patient presented with intermittent attacks of mild right chest pain and dyspnea for 2 years. On contrast-enhanced computed tomography (CT) of the chest, a non-enhancing subcarinal cystic lesion about 6×6.5 cm was detected in the posterior mediastinum, consistent with the features of bronchogenic cyst. The lesion showed small calcific focus cha...

failure of development of the primitive lung bud leads to an extremely rare congenital anomaly with a prevalence of 34 per 10 lac live births termed pulmonary aplasia. in half of such cases, associated congenital malformations of the cardiovascular, skeletal, gastrointestinal, or genitourinary systems are present. the mayer-rokitansky-küster-hauser (mrkh) syndrome is defined as the congenital a...

We developed a rat model of pulmonary arteriovenous malformations after cavopulmonary anastomosis. We sought to determine whether this model reproduces the angiographic and histologic features seen in the human condition. Eight Sprague-Dawley rats underwent a right superior cavopulmonary anastomosis with the use of microsurgical techniques. Between 2 and 13 mo, pulmonary angiography was perform...