Outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung: 10 Years' experience 1991-2001

University of Oxford, Oxford, England, United Kingdom
Archives of Disease in Childhood - Fetal and Neonatal Edition (Impact Factor: 3.12). 02/2006; 91(1):F26-8. DOI: 10.1136/adc.2004.068866
Source: PubMed


To determine the outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung (CCAM) over a 10 year period.
This is a retrospective study of all babies diagnosed antenatally in the Prenatal Diagnosis Unit and delivered in Oxford between 1991 and 2001. Data were obtained from the Oxford Congenital Anomaly Register, theatre records, and histopathology reports.
Twenty eight cases of CCAM were diagnosed antenatally. Five pregnancies were terminated. Data are available on all 23 of the pregnancies that continued and resulted in two neonatal deaths and 21 surviving babies. Eleven of the 23 cases (48%) showed some regression of the lesion antenatally, and four of these cases appeared to resolve completely on prenatal ultrasound. Three of the 23 babies (13%) were symptomatic in the early neonatal period, and three developed symptoms shortly afterwards. Seventeen of the 23 babies (74%) were asymptomatic, of whom 12 had abnormalities on chest radiograph or computed tomography scan and had elective surgery. Two babies (8%) had completely normal postnatal imaging, and three had abnormalities which resolved in the first year of life. Seventeen of the 23 babies (74%) had surgery. Histology at surgery was heterogeneous. Of the 23 live births, all 21 survivors (91%) are well at follow up or have been discharged.
All babies diagnosed antenatally with CCAM require postnatal imaging with computed tomography irrespective of signs of antenatal resolution. In asymptomatic infants, the recommendations are close follow up and elective surgery for persistent lesions within the first year of life. Histology at surgery was heterogeneous, and this should be considered when counselling parents.

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    ABSTRACT: Objective: To report a case of a newborn with congenital cystic adenomatoid malformation (CCAM), and to revise the literature focusing on the current methods of diagnosis and treatment for this rare cause of acute respiratory distress in the neonatal period. Case description: A full-term newborn presented respiratory distress in the fi rst day of life. The respiratory distress got worse on the following day, and mechanical ventilation was needed. The diagnosis of CCAM was made by radiograph and computed tomography of the chest. The patient underwent excision of the middle lobe and the histological analysis confi rmed the diagnosis of CCAM type I. The postoperative period was uneventful and mechanical ventilation was discontinued on the fourth day. Comments: CCAM is an anomaly of the lung develop- ment that results in the overgrowth of the terminal respi- ratory structures. Patients may present signs of respiratory distress in the neonatal period and the diagnosis can be made by radiography and/or computed tomography of the chest. Lesions are classifi ed in three types according to the size and the histological type of the cysts. The recommen- ded treatment is a full lobotomy of the lesion in order to avoid the risk of recurrent lung infection and carcinoma. The early excision of the affected lobe allows the residual lung to reach its normal volume and the lung function tests are normal in the majority of the patients.
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