[Pneumothorax in cystic fibrosis: intrapleural instillation of Atabrine].

Intrapleural instillation of quinacrine HC1 (Atabrine) was used to treat spontaneous pneumothorax in 2 young men, aged 26 and 36 years, respectively, with advanced pulmonary disease due to cystic fibrosis. Pneumothorax did not recur until 1 year later in 1 case and 2 years later in the other. This mode of therapy for pneumothorax provides a valuable alternative to surgery for the patient with cystic fibrosis, severe lung disease and marginal pulmonary reserve. Quinacrine sclerosis should be considered for management of pneumothorax in cystic fibrosis because of the high rate of recurrence. However, caution should be exercised since heart-lung transplantation cannot be performed after pleural sclerosis.

Review of smooth muscle tumours of the lower respiratory tract.

A case of leiomyoma of the lung was described by our department 17 years ago. We now report three new cases of smooth muscle tumours of the lower respiratory tract: a leiomyoma of the lung, a leiomyosarcoma of the right main bronchus, and a leiomyoma of the trachea. Whereas in the previous case the tumour was bronchoscopically removed, all the recent tumours were surgically resected. All the patients have done well, including the one with the leiomyosarcoma who underwent surgery 7.5 years ago. One hundred and eighty cases of leiomyoma and leiomyosarcoma of the lung and trachea have been reported to date. Pulmonary leiomyoma most commonly presents as an asymptomatic solitary lung nodule. The endobronchial variety may cause cough, haemoptysis, or shortness of breath. Most of these tumours were dealt with by major pulmonary resection, although a lesser resection would have sufficed in many cases if a histological diagnosis had been established prior to surgery. Pulmonary leiomyosarcoma may occur at any age, and is much more common in females. The majority of the patients have thoracic symptoms. These neoplasms seem resistant to chemotherapy or radiotherapy and should be treated by radical resection whenever possible, which results in a 45% 5-year survival rate. Only seven cases of leiomyosarcoma and 12 cases of leiomyoma of the trachea have been reported. These usually cause respiratory obstruction and/or haemoptysis. The malignant as well as the benign tumour is probably best treated by complete surgical resection.

Localized fibrous mesothelioma of the mediastinum devoid of pleural connections.

Localized fibrous mesothelioma of the pleura is an uncommon neoplasm. Very rarely (there have been three previous cases) it may occur as an isolated mediastinal tumour. Such a tumour was present for at least 3 years in a 47 year old man, causing shortness of breath and superior vena cava syndrome. A mass weighing over 600 g was completely resected through a median sternotomy. The pertinent literature is reviewed.

Ankyloblepharon filiforme adnatum: congenital eyelid-band syndromes.

Three infants had congenital eyelid bands, ankyloblepharon filiforme adnatum (AFA), in association with cleft lip and palate. A review of the literature of all previously reported cases of AFA was performed and a new classification of the various clinical types of AFA is proposed. This new classification should aid in genetic counseling.