Congenital Thoracic Malformations

Moshe Ashkenazi 1 Ran Kremer 2 Alon Ben Nun 3 Ifat Sarouk 1 Saray Sity 1 Moran Lavie 1 Adi Dagan 1 Bat-El Bar-Aluma 1 Ori Efrati 1
1Pediatric Respiratory Diseases, Edmond and Lily Safra Children’s Hospital, Sheba medical center
2Thoracic surgery, Rambam medical center
3Thoracic surgery, Sheba medical center

Introduction: Congenital Thoracic Malformations (CTM) include congenital cystic adenomatoid malformation (CCAM), Sequestration and cysts. The incidence of CTM ranges between 1-5:10,000 and 1:35,000 newborns. They pose a risk for recurrent respiratory infection, respiratory distress, pneumothorax and potential malignancy. Surgery is indicated for symptomatic infants. For asymptomatic patients, treatment is controversial.

Objective: To investigate the epidemiology, diagnosis, symptoms, management and outcome of congenital lung cysts.

Methods: A retrospective patient`s file study to investigate the epidemiology and treatment of those lesions, through a search in the digital archive of Sheba Medical Center and Rambam Medical Center.

Results: We reviewed 44 cases over a period of 10 years, 28 of them had CCAM, 9 had sequestration, 6 had overlap lesion, 1 had bronchogenic cyst.

31 cases were diagnosed antenatal, 5 of them were diagnosed postnatally in the first months of life.

Over the pregnancy period, 6 fetuses had intrauterine intervention.

15 of the cases were symptomatic, all of them underwent surgery. the risk of being symptomatic is higher in Sequestration over CCAM (RR=2.67, P=0.0085). Males have a trend to increased risk of sequestration (RR=6.09, P=0.07).

Median age for surgery was 11 month for symptomatic cases, 10 month for asymptomatic cases.

Postoperative complication included pneumothorax (11.4%), pleural effusion (n=3), fever (n=2). 15 Patients (34%) diagnosed with HRAD with recurrent wheezing, abnormal obstructive spirometry and a good response to Bronchodilators.

Conclusions: Operation should be considered promptly in Sequestration. Those patients should be in a follow up of pediatric pulmonologist also due to higher rate of HRAD. Further study is needed to determine the exact timing for surgery in the asymptomatic cases.









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