This is a 22 year old female with no prior respiratory complaints. She devloped a bronchitis in February and May, 2008 and had a CXR that suggested bullous disease in the RUL. PFT’s showed normal spirometry and the diffusion capacity was 89% predicted. She had no respiratory illness as an infant.
Her CT scan showed the following
Which of the following would you recommend?
1) Leave the bulla alone, no Rx recommended, inform to go to ED with SOB or pain
2) Recommend Scuba diving lessons to utilize hyperbaric conditions to shrink the bulla
3) Refer for an experimental endobronchial valve to deflate the bulla
4) Refer to thoracic surgery for surgical bullectomy
5) Insert a Heimlich valve to permit deflation of the bulla through chest wall
I include a reference regarding 1 way valves:valve
August 7, 2008 at 1:40 pm |
I think one can make an arguement for definitive surgical management in the near future based on what seems to be fairly optimal surgical candidacy, the potential for worseing lung function and complications of bullae like infection and development of pneumothorax under tension. One article makes a reference to a bulla occupying 50% of the hemithorax being an indication for surgical intervention, but most of what’s out there in the literature seems to be case reports after a preliminary check on OVID. It seems like there are good results from bullectomy in those with reduced FEV1 and more symptoms, but this is by no means solid evidence. An alternative would be to follow her imaging, symptoms, and PFT’s for any signs of progression, provide extensive counseling about signs and symptoms of pneumothorax, and pulmonary infection, and keep definitive surgical management in your back pocket as a trump card. Not having met her, it’s hard to say which is right for her.
I would also check an alpha-1 antitrypsin level.
September 29, 2008 at 9:55 pm |
The upper lobe location and the unilateral process makes alpha 1 unlikely. This is likely congential lober emphyema or within the realm of giant bullae. Surgical resection is usually needed, with avoidance of high altitudes or scuba diving becasue of the changes in Pb. I would obatain a perfusion scan to see if there is any functional loss of perfusion and look for hypoxemia nocturnally; abeit at her yound age likely not. If pulmonary symptoms and decrease FVC then refer should be made for resection. Remeber surgery has a high rate of BP fistulas and should be done at a hightly trained center.