Complications related to Pneumothorax and Chylous Fluid Accumulation

Complications related to Pneumothorax and Chylous Fluid Accumulation Jay H. Ryu Division of Pulmonary and Critical Care Medicine Mayo Clinic, Rochester, Minnesota, USA

Outline • Chest anatomy and imaging • Pneumothorax • General aspects • In LAM • Chylothorax • General aspects • In LAM

Clinical Features of LAM Presentation, % During course, % Breathlessness 50 -60 80 Chest pain 10 -30 30 -40 Pneumothorax 30 -50 70 -80 Chylothorax 0 -10 20 -30 Renal AML 30 -40 30 -60

Coronal Sagittal Transverse

Pleura and Pleural Space

LAM - Chylothorax

Pneumothorax • Definition: air within the pleural cavity • Source of air: • Lung - most common • Outside air - due to trauma • Hollow visceral organ, e. g. , esophagus

LAM - Pneumothorax 32 F LAM

HRCT Normal LAM Emphysema

32 F LAM

Pneumothorax Classification 1. Spontaneous • Primary (no identifiable lung disease) • Secondary (underlying lung disease, e. g. , LAM) 2. Traumatic - penetrating or blunt trauma 3. Iatrogenic – thoracentesis, transthoracic needle lung biopsy, etc.

Pneumothorax Presenting Features • Symptoms (varies with size of pneumothorax) • Breathlessness • Chest pain, pleuritic • Signs (varies with size of pneumothorax) • Tachypnea, tachycardia – if large • pneumothorax Decreased respiratory movement and hyperresonance to percussion on the affected side

Pneumothorax Diagnosis • Chest radiography or CT demonstrating air in the pleural cavity

Pneumothorax in LAM • Highest rate of pneumothorax (60 -80%) of all lung diseases. • Most episodes occur at rest or with usual activities. • Pregnancy, air travel issues. • Recurrence rate (60 -70%)

Pneumothorax in LAM Management • Goals • Remove air from the pleural cavity and re • expand lung, if needed Prevent recurrence • Options – choice depends on size, clinical effects, continuing air leak, available resources. • Observation oxygen • Aspiration with catheter • Chest tube drainage • Surgical maneuvers – air leak closure, etc. • Pleurodesis – via chest tube or surgery

Chest Tube

Video-Assisted Thoracoscopic Surgery (VATS)

VATS - Mechanical Pleurodesis

Thoracotomy

Pleurectomy

Frequency of Spontaneous Pneumothorax and Recurrence Rates Disease Frequency, % Recurrence rate, % No lung disease <1 30 COPD <1 40 -50 Tuberculosis 1 -3 Not known AIDS 2 -6 10 -65 Cystic fibrosis 3 -9 50 -80 PLCH 4 -17 60 25 75 60 -80 60 -70 Birt-Hogg-Dubé LAM

COPD-Emphysema 65 F Smoker

Pulmonary Langerhans’ Cell Histiocytosis 39 F smoker

Birt-Hogg-Dubé Syndrome 58 M

Pleurodesis • Definition: obliteration of the pleural cavity by inducing adherence of the visceral and parietal pleural layers • Via chest tube • Chemical pleurodesis (e. g. , talc) • Surgical • Video-assisted thoracoscopic surgery • (VATS) or thoracotomy Chemical (e. g. , talc), mechanical (abrasion), pleurectomy

Pneumothorax in LAM Summary • Pneumothorax is a common complication of LAM and has a high recurrence rate. • Pleurodesis (surgical or nonsurgical) is a procedure to achieve obliteration of the pleural cavity. • Pleurodesis is generally recommended for LAM patients to reduce the high risk of recurrence.

Chylothorax • Definition: presence of chyle (milky body fluid consisting of lymph and emulsified fats) in the pleural cavity • Source: • Chyle leak from thoracic duct or its branches • Flow of chyle in the abdomen through diaphragm

Chylothorax Thoracic Duct Anatomy • “Normal” anatomy of the thoracic duct is present in only 50% of patients • The most common variation is a double system (39%) • Originates in the abdomen as the cisterna chyli, located anterior to vertebral bodies T 12 - L 2

Chylothorax Causes • Traumatic • Surgical • Nonsurgical • Nontraumatic • Malignancies (lymphoma, etc. ) • Nonmalignant § Congenital or acquired lymphatic dis. § Chylous ascites § Various medical diseases. , including LAM

Chylothorax Presenting Features • Symptoms (varies with size of chylothorax) • Breathlessness • Chest discomfort • Signs (varies with size of chylothorax) • Tachypnea, tachycardia – if large amount • • of fluid Decreased breath sounds on affected side Dullness to percussion

LAM - Chylothorax 30 F LAM

LAM - Chylothorax

Chylothorax Diagnosis • Thoracentesis demonstrating the presence of chyle in the pleural cavity.

Thoracentesis

Chylothorax

Chylothorax in LAM • Identified at presentation in 0 -10% of LAM patients. • Occurs in 20 -30% over the clinical course. • From obstruction/leak of thoracic duct or tributaries, or transdiaphragmatic flow of ascites. • Sometimes associated with chylous ascites, chyloptysis, chyluria.

Chylothorax in LAM Management Tailor to size of effusion, effects, available expertise & resources: • Observation • Dietary - low-fat diet or total parenteral nutrition • Pharmacologic – sirolimus, octreotide (somatostatin) • Drainage - thoracentesis or chest tube • Pleurodesis - chemical/talc, mechanical, pleurectomy • Thoracic duct ligation • Percutaneous thoracic duct embolization • Pleuroperitoneal shunt

Chylothorax in LAM - Sirolimus (Taveira-Da. Silva et al. Ann Intern Med 2011)

Chylothorax in LAM - Sirolimus (Moua et al. Am J Respir Crit Care Med 2012)

Thoracic Duct Ligation

Percutaneous Embolization of Thoracic Duct

Pleuroperitoneal Shunt

Chylothorax in LAM Summary • Chylothorax is accumulation of chyle in the pleural space. • Optimal management depends on size of effusion, effects, available expertise & resources.

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