Prenatal Diagnosis followed by Minimal Surgical Resection: The New Paradigm for the Treatment of Congenital Pulmonary Malformations
The diagnosis, clinical presentation and operation for congenital pulmonary malformations (CPM) was studied in the era of prenatal detection and minimally invasive surgery.
A retrospective review of patients operated for CPM between 1990 to 2003 at a children’s hospital.
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.
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%.
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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