chest clinical cases A Case of Recurrent PneumothoracesSubmitted byJ. Shaun Smith, DOFellowPulmonary and Critical Care MedicineThe Ohio State UniversityColumbus, OhioJames N. Allen, MDProfessor of MedicineThe Ohio State UniversityColumbus, Ohio
History• A 41-year-old female presents to pulmonary outpatient clinic with a history of right-sided pneumothorax, which had resolved.• She gives a history of several spontaneous pneumothoraces on her right side over the past 6 years.• She provides a past medical history of endometriosis and is being treated symptomatically with occasional over-the-counter medications.• She also has a history of right-sided pleural procedure after the second pneumothorax.• However, despite this surgical intervention, she continues to have recurrence of the pneumothorax.• Of note each pneumothorax resolved without intervention or complication.• Pulmonary function testing (PFT) was performed demonstrating a FEV1 of 86% predicted, total lung capacity (TLC) of 112 % predicted and diffusion capacity of 93 % predicted.• There was no significant response to bronchodilator challenge.• Her remaining past medical history is significant for chronic low back pain from an L4-5 disc herniation, now status-post discectomy, chronic knee pain from osteoarthritis, and arthroscopic anterior cruciate ligament surgery.• Her only medications are nonprescription anti-inflammatories and a multivitamin.• She has never used tobacco or recreational drugs.• Family history is unremarkable. Sunday, January 13, 2013
Question 1• What is the most likely diagnosis?• A. Catamenial Pneumothorax• B. Lymphangioleiomyomatosis• C. Congenital bullous disease• D. Marfan’s syndrome Sunday, January 13, 2013
Question 2• Which of the following is NOT associated with thoracic endometriosis?• A. Hemoptysis• B. Neck pain• C. Pulmonary nodules• D. Transudative effusion Sunday, January 13, 2013
discussion• Thoracic endometriosis, which is most likely cause of catamenial pneumothorax, can present in various other forms.• Pneumothorax is the most common presentation (73%) followed by hemothorax (14%), hemoptysis (7%), and lung nodules (6%) (6).• Interestingly, hemothorax correlated most with the presence of either pleural or pelvic endometriosis.• Hemoptysis is caused by endobronchial or parenchymal ectopic endometrial tissue.• During bronchoscopy, the lesions appear as purplish-red patches which bleed easily on contact.• Cytology will show clusters of small cuboidal cells characteristic of endometrial tissue .• Pain is also quite common, and is often due to diaphragmatic endometrial implants.• Discomfort may be referred to the ipsilateral neck, shoulder, chest or arm. Sunday, January 13, 2013
Question 3• What is the proposed mechanism for catamenial pneumothorax?• A. Congenital diaphragmatic fenestrations• B. Acquired diaphragmatic fenestrations from endometrial implants• C. Metastatic spread of endometrial tissue• D. Release of dinoprost tromethamine during menstruation• E. All of the above Sunday, January 13, 2013
discussion• The mechanism for catamenial pneumothorax is unclear.• Four possible mechanisms have been proposed.• The first potential mechanism is congenital diaphragmatic defects providing an opening between the peritoneal cavity and the atmosphere.• When the cervical mucus plug is absent, air is allowed to migrate upward through the peritoneal cavity into the pleural space.• The second mechanism involves necrotic endometrial implants on the diaphragmatic surface creating a perforation in the diaphragm.• Thirdly, catamenial pneumothorax could also be caused by metastatic spread of endometrial implants through the pelvic veins reaching systemic circulation.• This last proposed mechanism involves implants disrupting the pleural surface during menses.• The prostaglandin dinoprost tromethamine, which is present in the plasma of some women during menses, constricts both bronchioles and vasculature, which may result in pneumothorax when alveolar tissue is damaged and associated bronchospasm prohibits expiration . Sunday, January 13, 2013
Question 4• What is the preferred treatment of catamenial pneumothorax?• A. Oral Contraceptives• B. Pleurodesis• C. Repair of diaphragmatic defects• D. Combined surgical intervention and hormonal manipulation Sunday, January 13, 2013
discussion• There are several therapeutic options to treat catamenial pneumothorax.• Nonsurgical treatment includes hormonal suppression with medications, such as leuprolide, a gonadotropin-releasing hormone, and standard oral contraceptives .• Surgical intervention includes bilateral salpingo-oophorectomy, closure of diaphragmatic defects and pleurodesis.• Polyglactin mesh has been used to cover the diaphragm as well in an effort to occlude occult fenestrations. This also promotes adhesion of the lung to the diaphragm .• Overall, a combined surgical approach followed by hormonal manipulation has been most successful in preventing recurrences, with a 50% success rate .• Video-assisted thoracoscopy is the preferred method for surgical assessment and treatment (9).• The pleural and diaphragmatic surfaces can be inspected at which time superficial endometrial implants are vaporized using combination of hydrodissection and carbon dioxide laser.• Deeper implants, however, require sharp dissection.• With video-assisted thoracoscopy diaphragmatic defects can be resected and closed with an endoscopic stapler device.• Standard thoracotomy may be employed when video-assisted thoracoscopy in not adequate or prior surgical intervention has failed.• Talc pleurodesis and pleurectomy should only be considered in cases of treatment failure . Sunday, January 13, 2013
HRCT-2• Find nodules along the inter-lobar fissure in the right lung.• Find nodules at the peripheral pleura.• Find centrilobular nodules.• Find a nodule at the end of a vessel in the in the left lung
• The lesions in this slice of lung correspond to those shown above.• Compare the size of the nodules in the lower lung to that in the upper lung.• Are they larger or smaller?• SMALLER• Can you give a reason for your answer?
• Compare the amount of cytoplasm in the rounded collection of cells indicated by the thin arrows to the amount in the surrounding cells.• Which cells have more cytoplasm?• What types of cells are in the rounded nodule?• What type of cell is indicated by the thick arrow?• What is the histologic diagnosis?
• Which cells have more cytoplasm?• The cells delimited by arrows• What types of cells are in the rounded nodule?• Mostly epithelioid histiocytes (with prominent cytoplasm), some lymphocytes• What type of cell is indicated by the thick arrow?• A multinucleated giant cell• What is the histologic diagnosis?• Ill-defined, non-necrotizing granuloma
Differential diagnosis of random nodules on HRCT:• miliary tuberculous.• Fungal.• viral infections.• hematogenous metastasis (particularly from thyroid, kidney, and breast).