Cystic lymphangiomas are focal collections of slow-growing benign lymphatic tissue. They are caused by altered embryonic development, or are secondary to chronic obstruction after surgery, infection or exposure to radiation.1,2 The most common sites are the head, neck (75%), or axilla (20%), although they may also occur in the chest (1%).2,3 They can remain asymptomatic for long periods, although compression of adjacent structures may produce chronic cough, dyspnea, dysphagia, or even pneumothorax and pleural effusion.2,4

We report the case of an 80-year-old woman with a history of arterial hypertension, hypothyroidism, paroxysmal atrial fibrillation, for which she was receiving anticoagulants, breast cancer in 2006, treated with resection and adjuvant radiotherapy with no subsequent recurrence, and pulmonary embolism in 2009.

The patient was admitted to the respiratory medicine department for a 2-year history of repeated respiratory infections, with wheezing on inhalation, predominantly in the left hemithorax. Chest computed tomography (CT) revealed a cystic lesion measuring 10×9cm causing partial atelectasis of the left upper lobe and compression of the mediastinal structures (Fig. 1). This lesion was biopsied using a CT-guided technique and the cytological study reported cystic lymphangioma.

Fig. 1.

Chest computed tomography, showing cystic lymphangioma in the anterior mediastinum before surgery.

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The patient was evaluated in collaboration with the thoracic surgery department, and surgery was proposed. The lymphangioma was resected via anterior thoracotomy. The immediate post-operative period was complicated by respiratory failure with rales and accumulation of secretions that required reintubation and transfer to the intensive care unit. The follow-up X-ray also revealed elevation of the left hemidiaphragm, indicative of post-surgical phrenic nerve palsy. After extubation breathing support continued with non-invasive mechanical ventilation.

In the following days the patient presented several episodes of partial and total atelectasis of the left lung, requiring repeated fiberoptic bronchoscopic procedures and placement of an endobronchial stent. Finally, due to prolonged weaning, a surgical tracheostomy was performed and the endobronchial stent was removed. The patient was stabilized and ventilatory support and oxygen requirements could be gradually reduced. The patient made good progress during her hospital stay, and some days later daytime respiratory cannulas could be withdrawn, although nocturnal non-invasive support continued.

Intrathoracic cystic lymphangiomas are rare benign tumors that generally occur in children and young adults.2,4 Imaging tests are required for diagnosis (CT or magnetic resonance) and histological confirmation can be obtained with CT-guided biopsy or mediastinoscopy.2–4 The most effective treatment is resection, although incomplete resection can be associated with a risk of recurrence.2,3 Other therapeutic options include aspiration and drainage of the cysts for emergency decompression, injection of sclerosing agents, such as bleomycin or OK-432, or low-dose radiation therapy.2

Our case is of particular interest due to both the low incidence of this entity in elderly people and the intrathoracic site. Although surgical treatment is the most widely accepted option, due to its good prognosis and lower risk of recurrence, the procedure can involve considerable morbidity. Such was the case in our patient, who presented multiple complications after surgery.