A 5 year old boy attended the emergency department with a tension pneumothorax secondary to congenital cystic adenomatous malformation of the lung. Management problems are discussed and congenital cystic adenomatous malformation reviewed.
Statistics from Altmetric.com
A 5 year old boy presented to the emergency department with central chest pain and dyspnoea, which had developed during the morning before admission. He complained of two recent minor falls from which he had sustained no apparent injuries. He had no significant immediate past medical history and had a normal exercise tolerance. The patient had been born at term and had no neonatal problems. As an infant he had had a cough that had been treated as asthma with inhalers and that settled spontaneously by the age of 1 year. There was no relevant family history.
On examination the respiratory rate was increased, the trachea was deviated to the left, and there was decreased air entry on the right. Other than an increased respiratory rate the child displayed no other signs of cardiovascular compromise. An urgent chest radiograph was performed (fig 1), which showed a right sided pneumothorax with mediastinal shift. A chest drain was inserted under sedation. On insertion air was heard to escape from the chest and once secured the drain was swinging satisfactorily. The procedure relieved the child’s symptoms. On repeat radiography while the mediastinum was now central a large air collection appeared to remain on the right hand side (fig 2). On closer examination of the radiograph thin walled bullae were identified filling the right chest cavity.
The child was referred to the Regional Children’s Cardiothoracic Centre. Computed tomography (fig 3) showed the right hemithorax to be slightly larger than the left suggesting a longstanding abnormality. There was a small amount of mediastinal air anterioraly but no significant residual pneumothorax. The left lung appeared normal. A complex cystic entity replaced virtually all of the right lung with the appearance of a congenital cystic adenomatous malformation (CCAM). Two days later he underwent a right pneumonectomy. He made an uneventful recovery and was discharged four days later. A histological report confirmed the diagnosis of CCAM.
CCAM is rare and usually presents before the age of 3 years.1,2 The exact incidence is now known. It is more common in boys than girls, and it is usually unilateral. Patients can present with life threatening respiratory distress or a history of recurrent chest infections.1–3 Some cases are asymptomatic and are discovered as an incidental finding on radiography. CCAM is best diagnosed with computed tomography and is treated with lobectomy.1,3,4,6 This may have to be done as an emergency depending on clinical state, and some cases have been operated on prenatally. Treatment can be postponed if the patient is asymptomatic and the cyst is resolving.2 Long term outcome is very good, affected children leading normal lives with only slight decrease in lung volume.1,5,6 Congenital emphysema can present in a similar way and should be considered in the differential diagnosis.1,2,3,4,6
This case presented an acute management problem to the staff dealing with it. The child had the classic respiratory signs and symptoms of a tension pneumothorax, but was cardiovascularly stable. There was no obvious cause for the child to have this condition and spontaneous tension pneumothorax is extremely rare in children. Rather than following standard treatment guidelines for management of a tension pneumothorax staff opted for a “look before you leap” approach and performed a chest radiograph before performing thoracocentesis or chest drain insertion.
Once the diagnosis of tension pneumothorax had been made a decision was made to insert a chest drain as a single procedure rather than to perform a needle decompression followed by chest drainage. This decision was made to cut down the number of invasive procedures performed, and in the knowledge that the child remained cardiovascularly stable. In retrospect, it was felt that the best treatment option had been taken as, in the presence of large air filled cysts, the insertion of a chest drain using blunt dissection and an open technique run less chance of puncturing a cyst than inserting a needle into the chest.
Dr Daranee Boon wrote the first draft of the paper and performed the literature search, and collated the illustrations. Dr Peter Rushton was involved in the initial management of the clinical case and identified the case as being worthy of a case report. Dr Thomas Llewellyn re-wrote the first draft of the paper and is guarantor of the paper.
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.