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Prenatal Diagnosis followed by Minimal Surgical Resection: The New Paradigm for the Treatment of Congenital Pulmonary Malformations

Robert J Touloukian1,2,2, John Seashore1
1Yale University School of Medicine, New Haven, CT;2

Objective:
The diagnosis, clinical presentation and operation for congenital pulmonary malformations (CPM) was studied in the era of prenatal detection and minimally invasive surgery.
Design/Setting:
A retrospective review of patients operated for CPM between 1990 to 2003 at a children’s hospital.
Patients:
Forty-seven patients with a pre-operative diagnosis of CPM.
Main Outcome Measures:
Type of CPM, age , lobe , size, symptoms, age at operation, procedure and outcome.
Results:
Prenatal diagnosis in 16 of 22 with congenital cystic adenomatoid malformation (CCAM), 11 of 16 extralobar sequestration (ELS) and intralobar(ILS)patients and 1 of 9 with lobar emphysema (CLE). Lower lobe involved in 15 of 22 CCAM; 8 of 8 ILS. Operation for CCAM with prenatal diagnosis ranged 1 to 240 days old (M=106) for asymptomatic patients (N=10) versus 1-5 days (M=2.3) for symptomatic patients (N=6). CCAM size in asymptomatic patients initially decreased but persisted in 8 of 10. Mean age at operation for 5 out of 8 ILS patients with prenatal diagnosis was 40 days versus 5 to 11 years (for 3 of 8) with recurrent pneumonia. Mean age at operation for CLE was 39 days (4 to 109) including 4 of 9 asymptomatic at birth. Lobectomy was performed in 17 of 22 CCAM, 4 of 8 ILS, and 9 of 9 CLE with the remaining asymptomatic patients with prenatal diagnosis having open segmentectomy or thoracoscopy. Overall survival was 98%.
Conclusions:
Prenatal diagnosis resulted in earlier detection and increased usage of open segmentectomy or video-assisted thoracoscopic surgery in asymptomatic patients with CPM.

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