Congenital cystic adenomatoid malformations (CCAMs) are rarely diagnosed in adulthood. The present case study reports a case of a CCAM presenting as a cerebral air embolus during an international flight.In the present case, supportive therapy resulted in a full recovery. The patient later underwent elective excision of the pulmonary malformation.Since the overall mortality of in-flight cerebral air embolisation is high, patients with such asymptomatic cysts should be considered for elective surgery.KEYWORDS: Congenital lung disease, stroke, thoracic surgery T he term congenital cystic adenomatoid malformation (CCAM) of the lung encompasses a spectrum of conditions from disordered airway development (types 0, 1 and 2) to abnormalities in the development of alveolar parenchyma (type 4; table 1) [1]. Symptoms of the latter type commonly include respiratory failure or infection. The present case study describes a type-4 CCAM presenting as a cerebral air embolus during an international flight. CASE REPORTA 17-yr-old male nonsmoker collapsed suddenly 15 min after take-off during a flight from London, UK, to Salzburg, Austria. He had flown uneventfully on four previous occasions. He initially complained of generalised ''pins and needles'' followed by loss of consciousness and urinary incontinence without evidence of seizure. He regained consciousness shortly prior to landing. On admission to hospital, he had a left hemiparesis and bulbar palsy. Computed tomography (CT) and magnetic resonance imaging of the brain showed no abnormalities. An EEG was performed and found to be normal. Laboratory investigations were normal, with the exception of a mildly raised troponin T (0.07 ng?mL -1 ). All neurological signs and symptoms resolved within 72 h and hyperbaric oxygen therapy was not instituted. A chest radiograph ( fig. 1) showed a large thin-walled cyst in the left upper lobe. CT of the chest ( fig. 2) revealed a 9-cm thin-walled cystic airspace in the left upper lobe with peripherally displaced vessels. A further smaller cystic lesion was noted in the posterobasal segment of the left lower lobe. Transthoracic echocardiography was normal. Aspirin therapy was commenced and he returned to the UK by ground transportation 10 days after presentation. At his local hospital he underwent a normal transoesophageal echocardiogram effectively ruling out paradoxical embolisation via a patent foramen ovale. A ventilation scan showed absence of ventilation in the left upper zone lesion. Spirometry recorded a forced expiratory volume in one second of 4.35 L (98% predicted) and a forced vital capacity of 4.82 L (92% pred), with a residual volume of 1.89 L (114% pred). He was referred for surgical treatment.The patient remained well and underwent elective left thoracotomy, atypical excision of the left apical segment of the upper lobe and wedge resection of the lower lobe cyst. Histological examination of both lesions showed features characteristic of a type-4 CCAM, with disordered cystic dilated airspaces lined by type-2 pneumoc...
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