The common congenital lung malformations have traditionally been divided into separate pathological entities such as pulmonary airway malformations, cystic adenomatoid malformations, congenital lobar emphysema, intra or extra pulmonary sequestrations, bronchogenic and foregut cysts. Some may produce respiratory compromise immediately after birth or even in utero. The management of such life thr...

Horseshoe lung (HL) is often associated with cardiovascular malformations such as scimitar syndrome and unilateral lung hypoplasia. In patients with HL, the hypoplastic lung is almost always located on the right side. Cases of HL with a hypoplastic left lung are extremely rare. In this paper, we describe a case of a one-day-old boy with HL involving left lung hypoplasia and perform a systematic...

Isolated Pierre Robin sequence (PRS) is characterized by underdeveloped jaw, backward displacement of the tongue and upper airway obstruction with or without cleft palate be part a known syndrome as VACTREL CHARGE, associated other malformations congenital heart disease, lung malformations, musculoskeletal system anomalies, hereditary progressive arthro-ophthalmopathy. We report neonate PRS who...

Lung arteriovenous (AV) shunts or malformations cause significant morbidity and mortality in several distinct clinical syndromes. For most patients with lung AV shunts, there is still no optimal treatment. The underlying molecular and cellular etiology for lung AV shunts remains elusive, and currently described animal models have insufficiently addressed this problem. Using a tetracycline-repre...

Bronchoscopy is an indispensable tool in the clinical evaluation and management of pediatric airway and lung disease. Common indications for pediatric bronchoscopy are varied and extend over a spectrum of congenital, infectious, structural conditions and diagnostic applications . Indications for flexible bronchoscopy in children include the evaluation of stridor, persistent or recurrent wheezin...

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...

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...