A 39 yo Man with Atypical Chest Pain General Internal Medicine Primary Care Conference K. Mae Hla, M.D. October 11, 2006

The Case 39 y.o. healthy epidemiologist with no personal or family history of cardiac or pulmonary disease Training for marathon: running 30 mins on treadmill, lifting weights and biking 40 mins. Developed sharp right anterior chest pain while walking home from work

I need to see “Dr. Hla” History of pain in the upper pectoralis muscle past few days after a hard workout Denies trauma to the chest, travel hx, SOB, cough, fever, leg pain or swelling, syncope Non-smoker PMH: labile hypertension with mild postural dizziness

Exam- in the office tall male, mild distress, not dyspneic no hyper resonance, normal breath sounds, no subcutaneous emphysema no murmurs or rubs, no intra-scapular murmurs, BPs in both arms were normal and equal point tenderness right pectoralis muscle

Clinical Course--at home Did not follow up with primary MD as advised Continued to have pleuritic pain Stopped all weight lifting and minimized exertion Switched to stationary biking due to notable reduced exercise capacity

Clinical Course--in the ER 4 days later, syncopal episode in bathroom Was seen in the ER and worked up for possible cardiac syncope The admitting resident was surprised when he looked up one of the routine tests obtained

“This was the reason for my muscle pain”

In retrospect… He realized he had ceased all lifting and exercise on the weekend prior to admission because of shortness of breath

Clinical Course--in the hospital A chest tube was inserted in the ER Patient was admitted for observation and syncope workup A TTE and a Holter Monitor showed no abnormalities A cardiology consult recommended no further syncope workup The pneumothorax was felt to be the cause of the syncope

“Another Clinical Question”! How safe is it to fly to the west coast to present the “invited” talk at the meeting 10 days after my discharge?

Objectives Describe types and incidence of spontaneous pneumothorax Review pathogenesis, clinical presentation Evaluate evidence for recurrence risk and treatment options Discuss guidelines on air travel safety in patients with recent pneumothorax

Spontaneous Pneumothorax Definition: Air in pleural space, between the lung and the chest wall First coined as “pneumothorax simple” by Itard in 1803 No antecedent traumatic or iatrogenic cause

Types of Spontaneous Pneumothorax Primary spontaneous pneumothorax   in healthy persons with no apparent underlying lung abnormalities or underlying conditions Secondary spontaneous pneumothorax   Clinically apparent underlying lung disease

Global Incidence, Morbidity & Mortality (U.K.) GenderIncidence100,000/yrPSPMorbidity100,000/yrPSP+SSPMortality Per million PSP+SSP Women Men

U.S. Incidence Olmsted County ( ) 318 with diagnosis of pneumothorax   Age-adjusted annual incidence of primary pneumothorax:   7.4 per 100,000 men   1.2 per 100,000 women   Annual incidence of secondary pneumothorax   6.3 per 100,000 men   2.0 per 100,000 women

Pathogenesis of PSP 90% of cases at thoracoscopy or thoracotomy 80% of cases on CT showed subpleural blebs or bullae Etiology of bullous changes in healthy:   airway inflammation from smoking   lifetime risk in smoking men 12% vs. 0.1% non-smokers   tall stature-subpleural blebs in apex

Effect of smoking on PSP recurrence risk Bense et. al. Chest 1987; 92:1009 No. of cigarettes/day Relative Risk (men) (women) >

Pathogenesis of PSP (cont’d) Other causes Marfan’s syndrome Homocystinuria Catamenial pneumothorax in thoracic endometriosis Familial spontaneous pneumothorax: autosomal dorminant, recessive, polygenic and X-linked recessive inheritance

Genetic mutation and PSP Gene for Familial cancer syndrome- chromosome17p11.2 Birt-Hogg-Dube syndrome: benign skin tumors and renal cancer: high PSP incidence-23% in one study Other mutations of FLCN-bullous lung disease and spontaneous pneumothorax only Autosomal dorminant inheritance of bullous lung disease with 100% penetrance in a Finnish family

Clinical Presentation Primary spontaneous pneumothorax Usually occurs at rest Peak age is early 20s; rare after 40 Sudden onset of dyspnea and pleuritic chest pain Severity of sx related to size of pneumothorax Secondary spontaneous pneumothorax More severe sx for same size of pneumothorax

Physical Findings Decreased chest excursions Decreased breath sounds Hyperresonant percussion Subcutaneous emphysema Pleural line on chest radiograph

Risk of Recurrence Range 25 - >50%; 54% within first 4 years in one study Risk factors for recurrence in PSP smoking tall stature female gender low body weight Risk factors for recurrence in SSP age over 60 years pulmonary fibrosis emphysema

Cumulative freedom from recurrence Lippert H et al. Eur Respir J 1991;4:324-31

Types of Treatment Options 1. 1.Observation and supplemental oxygen 2. 2.Aspiration of the pleural space 3. 3.Tube thoracostomy 4. 4.Tube thoracostomy with pleurodesis 5. 5.Thoracoscopy 6. 6.Open thoracotomy British Thoracic Society (2003) and American College of Chest Physicians guidelines (2001)

Choice of Treatment Options Size of pneumothorax Symptoms Clinical stability: %, can speak whole sentences in between breaths Recurrence risk, underlying conditions Patient occupation

Size of Pneumothorax Small < 2cm between lung margin and chest wall (BTS) < 3 cm apex-to-cupola distance (ACCP) < 15% of the hemithorax (UpToDate) Large > 2 cm between lung margin and chest wall (BTS) > 3cm apex-to-cupola distance (ACCP) > 15% of hemithorax (UpToDate)

Treatment Recommendations based on Size Small < 15% SP and stable patients: observation and supplemental oxygen to facilitate absorption of pleural air Large >15% Initial simple aspiration of the pleural space Tube thoracostomy if persistent airleak and no lung reexpansion

Manual Aspiration vs. Thoracostomy Meta-analysis of 3 randomized controlled trials (194 patients) Similar Clinical outcomes at 1 week and 1 year Manual aspiration group--shorter hospital stay A randomized controlled trial (137 patients) Similar rates of immediate success (62% vs. 68% in 1 day and 89% vs. 88% in 1 week) Aspiration associated with shorter hospital stay (1.8 vs. 4 days)

Tube Thoracostomy Indications PSP that fails aspiration treatment Recurrent spontaneous pneumothorax Presents with hemopneumothorax Small chest tube in most (5.5 or 7.0 French) Clamp chest tube when no bubbles emanate from a patent tube in 12 hours Remove after 24 hours if no clinical or x-ray evidence of recurrence

Pleurodesis Procedures Chemical pleurodesis Intrapleural instillation of sclerosing agents Tetracycline, doxycycline, talc, premedicate with midazolam and an opiate Open or surgical pleurodesis thoracoscopy vs. a limited or full thoracotomy Indications: lung remains unexpanded after 3 days of chest tube, bronchopleural fistula, recurrence after chemical pleurodesis, bullae resection, patient occupation

Postgrad Med 2005;118(6) (online article)

Back to the Future… Is he safe to fly? Safety of air travel following pneumothorax- a subject of debate Concerns during air travel (speculative): ? expansion of trapped air at high altitude ? pressure change at ascent and descent ? change in barometric pressure may open a sealed air leak lack of appropriate medical care

BTS Air Travel Safety Review and Recommendations Airlines recommend 6 week-waiting period-arbitrary Evidence for timing of maximum risk of a recurrence (review 37 papers) Data on recurrence: variable, no data on timing Recurrence risk >54-72% in first 1-2 yrs, higher in smokers, COPD Surgical/pleurodesis intervention makes risk negligible Recurrence while flying more likely to suffer serious consequences especially in patients with pre- existing lung disease BTS recommendations. Air Travel Working Party-Thorax 2002;57; (Page 298)

Safety in flying--what’s the evidence? A small prospective study (n=12) of pts with traumatic pneumothorax in a Level I Trauma Center, Orlando, Fl, 14 day waiting period after resolution in adults 10/12 patients flew >14 days (mean 17.5 days) remained asymptomatic 2/12 flew in < 14 days: 1 developed sx suggestive of pneumothorax in flight Cheatham, et al. The Am Surgeon 1999;65:

What did the patient do? Like a true epidemiologist, decided the data on waiting period and increased recurrence risk during air travel was not supported by good evidence Decided to fly 12 days after his re-expansion Completed an uneventful trip!!!

Bibliography BTS guidelines for the management of spontaneous pneumothorax. Henry M. et al. Thorax 2003;58 (Suppl II):ii39-ii Management of spontaneous pneumothorax An AACP delphi concensus statement. Baumann MH, et al. Chest 2001;119: Air travel following traumatic pneumothorax: when is it safe? Cheatham ML, Safcsak K. Am Surg 1999;65(12): Managing passengers with respiratory disease planning air travel BTS recommendations. Air Travel Working Party. Coker RK, et al. Thorax 2002;57: pp The Intricacies of Pneumothorax Management depends on accurate classification. Dincer HE, Lipchik RJ. Postgrad Med 2005;118(6) (online article) Primary spontaneous pneumothorax in adults. Light RW UpToDate