Images In Medicine: Infected Giant Bulla, An Unusual Presentation Of Lynphangioleiomyomatosis
Abstract
Lynphangioleiomyomatosis (LAM) is a rare disease characterized by progressive cystic destructionof lungs. Giant bulla is rarely found in patient with LAM. We report an unusual presentation ofLAM with infected giant bulla.
A 40-year-old female non-smoker presented to the ED with non productive cough and fever of 4 days duration. There was no associated chest pain, hemoptysis or dyspnea.
She had no significant medical history.
Her vital signs were as follows: blood pressure 110/60 mmHg, pulse 100 beats/min, respiratory rate 22 breaths/min and temperature 37,5°C. Oxygen saturation was 92% in room air.
There were decreased breath sounds particularly on the right side. The rest of the examination was unremarkable.
Laboratory analysis yielded leucocytosis (13.500 cells/ul) and increased PCR (250 mg/dl). Arterial blood analysis revealed that her PaO2 was 55 mmHg and pCO2 32 mmHg.
Chest radiography showed bilateral hyperinflation and a large hypertrasparent image with two fluid levels in the upper lobe of the right hemithorax.
A HRCT revealed multiple round thin walled cysts uniformly distributed throughout the lungs with minimal normal parenchyma and fluid containing giant cyst (size 15 x 10 cm), occupying the superior half of the right hemithorax. Pneumothorax and pleural effusions were not observed (Fig.1).
A lung biopsy was performed and the pathologic analysis enabled us to confirm the diagnosis of lynphangioleiomyomatosis (LAM).
Figure 1: transaxial section of the chest HRCT showing numerous thin-walled cysts of varyng size and fluid-filled giant bulla in the right lung.
LAM is a rare multisystem disorder that occurs predominantly in women of reproductive age and typically presents with progressive dyspnea or pneumothorax.¹∙²
The peculiar presentation of our patients was a fever with radiologic evidence of infection in a large bulla within advanced diffuse cystic lung disease.
To our knowledge, infected giant bulla, common complication of pulmonary emphysema³, has not previously been reported in association of LAM particularly at clinical presentation.
