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Unilateral Pulmonary Agenesis and Gastric Duplication Cyst: A Rare Association

DOI: 10.1155/2013/608706

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Abstract:

Lung agenesis and gastric duplication cysts are both rare congenital anomalies. Gastric duplication cysts can present with nausea, vomiting, hematemesis, or vague abdominal pain. Unilateral pulmonary agenesis can present with respiratory distress which usually occurs due to retention of bronchial secretions and inflammations. We report the unique case of right pulmonary agenesis associated with gastric duplication cyst. 1. Introduction Lung agenesis is rare congenital anomaly, is often associated with acute respiratory distress, and has a high mortality rate [1]. Pulmonary agenesis implies the absence of a lung and its supporting vasculature, whereas the main bronchi may be either absent or hypoplastic [2]. Fifty percent born with pulmonary aplasia are stillborn or die within the first five years of??life. In unilateral lung agenesis, the trachea continues directly into the main bronchus of the normally developed lung, and respiratory distress usually occurs due to retention of bronchial secretions and inflammations [1]. Duplication of the alimentary tract is a relatively rare congenital anomaly. Duplication cysts of the stomach represent four per cent of all alimentary tract duplications [3]. When symptomatic, gastric duplication cysts can present with nausea, vomiting, hematemesis, or vague abdominal pain [4]. To our knowledge, we report the first case of a male newborn with right pulmonary agenesis associated with gastric duplication cyst. 2. Case Report Our patient was a male newborn, 4300?g, born at term by an uncomplicated vaginal delivery. The first- and five-minute Apgar score was 9/9. The mother had no significant past or obstetric history. On second day of life, the newborn became cyanotic with a respiratory rate up to 80/minute with chest retractions. There was absence of breath sounds in the right side of the chest. The heart sounds seemed loudest in the right chest. Her hematologic and other blood parameters were all normal. Posterior-anterior chest X-ray showed opaque right hemithorax with cardiac displacement and left lung hyperinflation (Figure 1). Contrast enhanced CT-scan thorax was done which showed absence of right bronchus and pulmonary parenchyma with normal hyperinflated left lung (Figure 2). Figure 1: Opaque right hemithorax and left lung hyperinflation. Figure 2: Absence of right main bronchus and right lung on CT scan. Echocardiogram showed ventricular septal defect and heart positioning in right hemithorax. Large cyst in upper abdomen measuring 6 × 2 centimeters was identified with abdominal ultrasound. Contrast enhanced

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