Adulut Congenital Lboar Emphysema:a Case Report And Literature Review

Objectives:To investigate the clinical features of adults congenital lobar emphysema (CLE) and to improve the recognition of the diease.Methods:The clinical data of a case of adult CLE patient diagnosed in the First Affiliated Hospital of Guangxi Medical University was retropectively analyzed, and the relevant literatures from Pubmed and other databases were reviewed.Results:1. A 17 year and 10 month-old female presented recurrent chest discomfort and exertional breathlessness since 1 year with a history of hyperthyroidis. CT scan of thorax showed hyperlucent and hyperexpanded of left upper lobe with stenosis of left main bronchus. Pulmonary function tests showed mixed ventilation disturbances and fibreoptic bronchoscopy revealed the stenosis of the left main brochus. The patient was treated conservatively and the symptoms improved significantly.2. The results of literature review:more than 500 cases of CLE were reported from 1950 with a toal of 364 cases having detailed information. Among them,15 were adult cases, including 9 males and 6 females. The average is 22±6 year old. The most common clinical manifestations were dyspnea (11/15), chest pain (6/15), cough (6/15) and asymptomatic (3/15). All the cases underwent X ray or CT scan showing the hyperlucent and hyperexpanded of the affected lobe (15/15), collapsed of the surrounding lung tissue (13/15) and the controlateral mediastinum shifting (12/15). The pulmonary function test of revealed a predominantly change of obstructive ventilation dysfunction (5/7). Fibreoptic bronchoscopy showed bronchial stenosis (2/8) or bronchial atresia (1/8) and no abonormal findings (4/8). The histological examination showed distended alveolar air spaces with minimal destruction of lung tissue (7/7) and hypopalstic cartilage (6/7).2 patients were misdiagnosed as pneumothorax.8 patients underwent lobectomy and the remaining 7 patients recevied conservately treatment. None of them died and the symptoms were not deteriorate as follow up.Conclusions:1. Adults CLE is a rare disease with mild symptoms, mainly presented as dyspnea and chest pain; 2. Radiolgraphic features include the hyperinflation of the affected lobe. Obstructive ventilation dysfunction were the most common changes of pulmonay function test. The fibreoptic bronchoscopy may find the abormal bronchus and the pathology is according with pulmonary emphysema, and misdiagnosis of pneumothorax is not rare; 3. The diagnosis should be made acorroding to its symptoms, emphysema signs, typical imaging findings and exclude secondery factors; 4. The treatment could be surgical or conservatively with a good prognosis.