A 12-year-old male child was referred to our hospital with a 6-year history of repeated attacks of chest infection. He received several courses of antibiotics and was also investigated for pulmonary tuberculosis because of persistent lung infiltrates on his chest x-ray. All investigations for tuberculosis were negative, but despite this he was treated empirically with antituberculous drugs for 6 months without improvement. At our hospital, he had a CT scan of the chest, which showed multiple cysts of variable sizes affecting the right upper lobe of the lung (Figure 1) as well as a cystic swelling in the posterior mediastinum. He underwent a diagnostic right thoracotomy. The right upper lobe was found to be firm in consistency and contained adhesions to the inner chest wall. These adhesions were released and there were multiple cysts in the right upper lobe of variable sizes (Figure 2). There was no consent for a lobectomy, so it was decided to take a large biopsy, including one of these cysts. Exploration of the posterior mediastinum revealed a cyst of about 3X2 centimeter in size overlaying the esophagus. The cyst was dissected using both sharp and blunt dissection. It was found to be adherent to the esophageal wall. The cyst was separated from the esophageal wall and excised totally. Postoperatively, the patient did well and was discharged home on the tenth postoperative day. Histopathology of the lung biopsy showed large areas of fibrosis with cystic formations of varying sizes filled with mucus and macrophages, lined by cuboidal or columnar epithelium and rarely metaplastic squamous epithelium. There was chronic inflammatory cell infiltrate in some areas with formation of lymphoid follicles, but no epithelioid or giant cell granulomas. The esophageal cyst showed a wall composed of smooth muscles with the presence of ganglion cells and lined by ciliated columnar epithelium, but no cartilage. These features are consistent with CCAM in association with an EDC. The patient was followed up in the outpatient clinic and the condition was explained to the father who agreed to a lobectomy. The patient subsequently underwent right upper lobectomy.
CT-scan of the chest showing multiple cysts of variable sizes in the right upper lobe.