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Images In Medicine: Infected Giant Bulla, An Unusual Presentation Of Lynphangioleiomyomatosis
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Cristiano Padalino MD
Emergency department
University Hospital “Santa Maria della Misericordia”
Concetta Maria Sabella M.D.
Radiology department
University Hospital “Santa Maria della Misericordia”
Francesco Toso M.D.
Radiology department
University Hospital “Santa Maria della Misericordia”
Emilio Lugatti M.D.
Pneumology department
University Hospital “Santa Maria della Misericordia”
Ciro Paolillo M.D.
Emergency department
University Hospital “Santa Maria della Misericordia”
Rodolfo Sbrojavacca M.D.
Emergency department
University Hospital “Santa Maria della Misericordia”
Citation: C. Padalino, C. Sabella , F. Toso, E. Lugatti, C. Paolillo & R. Sbrojavacca : Images In Medicine: Infected Giant Bulla, An Unusual Presentation Of Lynphangioleiomyomatosis. The Internet Journal of Pulmonary Medicine. 2009 Volume 11 Number 1
Keywords: diagnosis | CT/MRI | pneumonia/infections
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.
References
1. Shan C. Chu, Koji Horiba, Jiro Usuky, et al. Comprehensive evaluation of 35 patients with lynphangioleiomyomatosis. Chest 1999; 115:1041-1052 (s)
2. Niku S, Stark P, Levin L, and Friedman PJ Lynphangioleiomyomatosis: Clinical, Pathologic and Radiologic Manifestations J Thorac Imaging 2005; 20:98-102 (s)
3. Chandra D, Soubra SH, Musher DM. A 57-year-old man with a fluid containing lung cavity. Chest 2006; 130: 1942-1946 (s)
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