INTRODUCTION Congenital lung malformations are rare lesions that are most commonly diagnosed antenatally. Management of such lesions, particularly those that are asymptomatic, remains controversial. We undertook a survey to ascertain current practice of surgeons in the UK and Ireland. METHODS All consultant members of the British Association of Paediatric Surgeons were asked to complete a sur...

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...

Pulmonary arteriovenous fistulas (PAVFs) are rare vascular malformations (PAVMs) of the lung that could lead to severe hypoxiemia due to right-to-left intrapulmonary shunts. They may occur as isolated entities or associated with Osler-Rendu-Weber syndrome or hereditary haemorrhagic telangiectasia (HHT). We report a case of a 70 years old woman with Rendu-Osler-Weber disease and a large arteriov...

In this article, the most important lower respiratory tract malformations are briefly reviewed, with special focus on those factors that may have some impact on the long-term respiratory outcome of specific lesions, like the amount of lung tissue resected, compensatory lung growth, lung hypoplasia, intensive care and mechanical ventilation.

Congenital parenchymal lung malformations have an estimated incidence at 1:25,000-1:35,000 births. We present a case of this rare congenital abnormality in a 38 year-old male, review the current literature with discussion of proposed causes, malignant potential, and management strategies. A 38-year-old white male presented with a 4-day history of chronic stable hemoptysis. Social history was n...

Authors observed five cases of lung arteriovenous malformations in children and young adults. Clinical data and morphological pictures of these lesions were presented.

Esophageal atresia with/without tracheo-esophageal fistula is a relatively common malformation, occurring in around 1 in 3500 births. In around half of cases, additional malformations are present, forming either a syndrome of known genetic aetiology, or a recognised association, of which the VACTERL association (Vertebral anomalies, Anal atresia, Cardiac malformations, Tracheo-Esophageal fistul...

The earliest signs of lung development occur around four weeks after conception with the appearance of a bud (the tracheal diverticulum) from the primitive foregut. Lung development can be described in four stages, embryonic, glandular, canalicular and sacular (TABLE 1). Towards term more subtle changes in the structure of the terminal airspaces, including elastin deposition and septation, whic...

Objective: To determine the main congenital lung malformations treated and the principal diagnostic methods employed, as well as the indications for surgical treatment and the results obtained, at a referral facility for pediatric thoracic surgery. Methods: We reviewed the medical charts of 52 patients anatomopathologically diagnosed with congenital lung malformations and who had been submitted...

OBJECTIVE To determine the main congenital lung malformations treated and the principal diagnostic methods employed, as well as the indications for surgical treatment and the results obtained, at a referral facility for pediatric thoracic surgery. METHODS We reviewed the medical charts of 52 patients anatomopathologically diagnosed with congenital lung malformations and who had been submitted...