Congenital Cystic Adenomatoid Malformation of the Left Upper and Lower Lobes in a Neonate

Abstract

Congenital cystic adenomatoid malformation of the lung (CCAM) is a relatively rare pulmonary anomaly. Usually lobectomy alone is satisfactory because most of the lesions involve only one lobe. Although pneumonectomy is performed when the malformation affects all lobes of one lung, it sometimes induces respiratoy insufficiency secondary to tracheal kinking from a marked mediastinal shift. In this paper, a successful surgical treatment in a neonate with CCAM of the left upper and lower lobes is reported. A 3-day-old girl was admitted to our hospital with a symptom of severe respiratory distress. A chest roentgenogram revealed the left pleural space filled with a mass containing innumerable small cysts. The mediastinum shifted to the right chest. The pre-operative diagnosis was CCAM of the left lower lobe. Left thoracotomy was performed at the age of 4 days. A microcystic CCAM involved both the lower lobe and the segments (S_1 + 2a, S_4, S_5) of the upper lobe. Lower lobectomy and segmentectomy of the upper lobe resulted in immediate improvement of respiratory condition. Her respiratory condition grew serious on the 5th postoperative day because of the marked mediastinal shift to the left. An injection of 50ml air into the left pleural cavity promptly improved her condition. She was discharged on the 18th postoperative day. Gross and microscopic findings confirmed the diagnosis of CCAM. A follow-up roentgenogram of the chest demonstrated a midline mediastinum and a well-expanded residual lung tissue of the left upper lobe.