Cystic Adenomatoid Malformation vs. Congenital Diaphragmatic Hernia
Left picture: The clinical, radiological and histological diagnosis is a cystic adenomatoid malformation of the right upper and middle lobe of the lung, which may be a combined anomaly of esophageal atresia; the anomaly became only apparent due to a gradual filling of the cysts on follow-up in the neonatal period, and the clinical presentation was initially attributed to the operated esophageal atresia. Right picture: Although the herniation of the heart to the contralateral side and the annular shadows of different sizes may suggest a cystic adenomatoid malformation, in fact a congenital diaphragmatic hernia of the left side is present. Although in cystic adenomatoid malformation an earlier clinical manifestation is known than in the other cystic lung malformations (lung cyst and lobar emphysema), a clinical presentation does not happen as rapidly as in congenital diaphragmatic hernia; the latter exhibits usually an RDS immediately after birth or within 24 hours. Also, the radiological picture develops only gradually, and the extent and the shape of the cyst formation (considerable difference in sizes) may be better recognizable in CT than in a chest x-ray.
Left picture: 1-day-old newborn with RDS and foaming saliva at the mouth. Diagnosis of an esophageal atresia (type 3b according to Vogt); closure of the esophageotracheal fistula and primary anastomosis of the esophagus. Postoperatively recurrent pneumothoraces and in spite of drainage signs of an increasing respiratory distress syndrome. Right picture: Neonate with progressive RDS in the first 24 hours after birth. Left picture: Chest x-ray prior to surgery of the esophageal atresia (first picture from the top): Increased pattern of lung structure on the right side and air in the gastrointestinal tract. Left picture: Chest x-ray on the third postoperative day (second picture from the top): Striated opacity and herniation of the right lung to the left hemithorax. Left picture: Lateral chest x-ray with peroral contrast administration on the eleventh postoperative day (third picture from the top): Neither esophageal stenosis at the site of the former anastomosis, nor leakage of contrast; striated opacities and multiple suggested annular shadows of the lung of different sizes. Left picture: CT 4.5 weeks postoperatively (fourth picture from the top): Multiple annular shadows of different sizes of the right upper and middle lobe (1) and an encapsulated residual pneumothorax (2) are visible. Right picture: The chest x-ray which was performed on the first day after birth, combines all findings which are present in the contralateral patient on the third and eleventh postoperative day, as well as in the CT 4.5 weeks after surgery; namely, a displacement of the mediastinum and of the heart to the contralateral side (in this case to the rigth side) and multiple annular shadows of different sizes in the totally involved hemithorax. Left picture: Chest x-ray at the age of 3 months 1.5 months following resection of the upper and middle lobe on the right side (fifth picture from the top): Nearly normal structure of the residual lung, and clinically alleviation of all respiratory signs.