Congenital cystic adenomatoid malformation: is there a difference between the antenatally and postnatally diagnosed cases?

BACKGROUND: The majority of congenital cystic adenomatoid malformation (CCAM) lesions are diagnosed antenatally. A few cases however may not be recognised antenatally and present in infancy or later childhood with chest symptoms, including chest infection. OBJECTIVE: To review the clinical and radiological spectrum of CCAM, comparing the antenatally with the postnatally diagnosed cases. MATERIALS AND METHODS: Fifteen cases of antenatally and/or postnatally diagnosed and histopathologically proven CCAM were retrospectively identified over a period of 4 years. Clinical notes, chest radiograph and chest CT were reviewed in all cases. RESULTS: Nine patients were diagnosed antenatally and six postnatally. All antenatally diagnosed patients were asymptomatic at birth, six remained asymptomatic until they had elective surgery and the remaining three developed symptoms before the age of 2 years. In the postnatally diagnosed group, one patient was symptomatic at birth and one patient presented at 16 years; the remaining four presented before the age of 2 years. Depending on the type of lesion, we recognised five radiographic patterns of CCAM. CCAM lesions were classified as CT Stocker type I in seven cases, type II in seven cases and type III in one case. CONCLUSIONS: No significant difference was found between the two groups. Recognition of these lesions antenatally would benefit patients by avoiding delay in making the diagnosis, which can lead to serious complications. CT was successful in accurately diagnosing and grading CCAM lesions.

Atypical chronic lung disease in preterm infants.

An atypical pattern of chronic lung disease (CLD) has been described in preterm infants and a potential association with intrauterine inflammation has been proposed. We aimed to describe patterns of CLD, to determine the incidence of atypical CLD, and to compare the distribution of various perinatal factors in infants with classic and atypical CLD. Information about demographics, respiratory status and various perinatal variables was collected for all neonatal admissions <1250 g. CLD was defined as oxygen dependency at 28 days of age. Ninety (51%) survivors at 28 days of age developed CLD; of these 37 (41%) were classified as atypical CLD. Factors significantly and independently associated with development of atypical CLD included being inborn, receiving natural surfactant, fewer days of mechanical ventilation within the first 28 days of life and higher birthweight. Chorioamnionitis, postnatal infection and symptomatic PDA were not found to be significantly associated with atypical CLD. Atypical CLD is a common pattern of prolonged oxygen dependency in preterm survivors and is a feature of larger, more mature babies. Our findings do not support the hypothesis that exposure to intrauterine inflammation is an important aetiological factor in the development of atypical CLD.

Left pulmonary artery sling presenting as unilateral echogenic lung on 20-week detailed antenatal ultrasound examination.

BACKGROUND: We report the first case of left pulmonary artery sling (LPAS) presenting as unilateral echogenic lung at a routine 20-week antenatal US examination. The infant had minimal symptoms at delivery, but developed an oxygen requirement and respiratory distress from day 10 of postnatal life. Antenatal US and antenatal MRI indicated an intrathoracic anomaly, so permitting definitive investigations in the neonatal period using CT, MRI and helical-CT-acquired virtual bronchoscopy. The infant successfully underwent corrective surgery. REASON TO REPORT: Antenatal ultrasound detected an anomaly permitting definitive diagnostic investigations to be performed shortly after birth. Investigations were completed before significant signs and symptoms developed. WHAT WAS UNIQUE: This is the first report of antenatally detected unilateral echogenic lung leading to the diagnosis of pulmonary artery sling. RAMIFICATIONS OF THIS REPORT: This report provides further evidence that improvements in antenatal ultrasound and MRI permit earlier diagnosis and improve patient management.