Abstract
Thoracoscopic excision of a Type 0 congenital pulmonary airway malformations (CPAM) is rarely described in literature. A 1-day-old female neonate presented with prenatal diagnosis of right lung CPAM. Mother had undergone fetoscopic thoracoamniotic shunt placement which led to radiographic resolution of large CPAM cyst. Soon after delivery, the patient developed multiple premature ventricular contractions (PVCs) that were thought to be secondary to retained shunts. Thoracoscopic excision of CPAM performed.
Highlights
Congenital pulmonary airway malformation (CPAM) is rare congenital anomaly with a prevalence of 1 in 10,000-35,000 pregnancies.[1]
Five different CPAM types have been described in the literature.[2]
Type 0 CPAM, known as acinar dysplasia or dysgenesis, is the most rare and comprises less than 2% of CPAM types and is usually associated with pulmonary hypoplasia and poor prognosis.[3,4]. This is the first report on an atypical CPAM type 0 presenting without pulmonary hypoplasia
Summary
Congenital pulmonary airway malformation (CPAM) is rare congenital anomaly with a prevalence of 1 in 10,000-35,000 pregnancies.[1]. Rest of the pregnancy was uncomplicated with delivery at 39th week of gestation After birth, she developed intermittent premature ventricular contractions (PVCs) that were thought to be secondary to retained shunts. She developed intermittent premature ventricular contractions (PVCs) that were thought to be secondary to retained shunts She was subsequently transferred to our institution for management of CPAM and PVCs. Chest radiography confirmed retained shunts (Fig.). Using a combination of the 3 mm sealer, blunt dissection and sharp scissors, we were able to further skeletonize, which was noted to arise from the trachea (Fig.). The cystic mass was sent to pathology and reported as a type 0 CPAM She was extubated on postoperative day 1 and chest tube was removed day.
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