Section 5 Pneumothorax

#### **Chapter 7**

## Pneumothorax: A Concise Review and Surgical Perspective

*Shilpi Karmakar*

#### **Abstract**

Pneumothorax is the collection of air in pleural cavity, which is commonly due to development of a communication between pleural space and alveolar space (or bronchus) or the atmosphere. In this chapter, we will discuss the various aetiologies of pneumothorax, the differences in their pathophysiology and the implications on the management of the disease. The chapter focusses on the surgical aspects in the management, the revolution brought in by video-assisted thoracoscopic surgery (VATS) and the advancement of the field by introduction of uniportal VATS and robotic-assisted thoracic surgery. The principles of management of catamenial pneumothorax are revisited. The chapter also throws light on the nuances of anaesthesia techniques and the latest developments are outlined. Lastly, a section is dedicated to COVID-19 associated pneumothorax and the approach to its management.

**Keywords:** pneumothorax, tube thoracostomy, VATS, pleurodesis, bullectomy, chest X-ray, flap, COVID-19, intercostal tube

#### **1. Introduction**

The term "pneumothorax" was coined by a French physician Itard, in 1803 [1]. Pneumothorax is defined as the presence of air in the pleural space. Even though intrapleural pressures are negative throughout the respiratory cycle, air does not enter the pleural space, as the net movement of gases from capillary blood into pleural space requires pleural pressures to be lower than −54 mmHg, which does not occur in normal circumstances. Hence, for air to be present in pleural space, one of the three events must occur: communication between pleural space and alveolar space (or bronchus), or communication between pleural space and the atmosphere, or presence of gas-producing organism in the pleural space [2].

Clinically, pneumothorax is classified as spontaneous (no obvious precipitating factor present) and non spontaneous (consequence of any thoracic injury). Spontaneous pneumothorax may be primary (no apparent underlying lung disease) or secondary (associated with clinically apparent underlying disease, like chronic obstructive pulmonary disease, cystic fibrosis), or catamenial (associated with menstruation). Pneumothorax can be of varying clinical severity, ranging from a small pneumothorax, which is likely to resolve spontaneously, to those with large pleural defects and collapse of entire lung and compromised ventilation.

Pneumothorax ranks second to rib fracture, as the most common manifestation of traumatic chest injury and is noted in 40–50% of patients with chest trauma [3]. Weissberg et al. in a study of 1199 cases of pneumothoraces found secondary

spontaneous pneumothorax (505 patients) to be most common, followed by primary spontaneous pneumothorax (218 patients), traumatic pneumothorax (403 patients), and iatrogenic pneumothorax (73 patients) [4].

#### **2. Pathophysiology**

Normally, the pressure in pleural space is negative compared to the alveolar pressure during the entire respiratory cycle, due to the inherent elastic recoil of the lung. The pleural pressure is also negative with respect to atmospheric pressure. Development of communication between alveolus or atmosphere and the pleural space allows air to flow into the pleural space until there is no longer a pressure difference or until the communication is sealed [5].

Tension pneumothorax is a condition where there is continuous increase in the air trapped in the pleural space, due to formation of a one-way valve by the injured tissues. This trapped air builds up pressure on the affected side, causing collapse of the ipsilateral lung and shift of mediastinum into the contralateral hemithorax. This causes respiratory distress. Also, there is reduced venous return and thus decreased cardiac output. Further, hypoxia leads to increased pulmonary vascular resistance via vasoconstriction. Cardiopulmonary arrest becomes imminent. Tension pneumothorax, thus, culminates in a life-threatening condition.

Spontaneous rupture of blebs may result in pneumothorax. The rupture may be a consequence of pressure change, as seen in airplane crew members or scuba divers [6]. The volume of given mass of gas at a constant temperature is inversely proportional to its pressure. A given volume of air at an altitude of 3050 m, saturated at body temperature, expands to 1.5 times the volume at sea level. Scuba divers breathe the compressed air delivered by a regulator and during ascent, as ambient pressure falls rapidly, gas in the lungs expands and may rupture blebs [7].

Secondary spontaneous pneumothorax may be due to rupture of pre-existing blebs or due to areas of increased porosity. These are areas of disrupted mesothelial cells on the visceral pleura, replaced by an inflammatory elastofibrotic layer with increased porosity, allowing air leak into the pleural space [8]. Pneumothorax has, also, been reported to be the presenting sign of peripheral necrotic tumour or centrally located tumour.

Catamenial pneumothorax is defined as two episodes of pneumothorax temporally related to the onset of menses, usually within 72 hours. Catamenial pneumothorax is the presentation of thoracic endometriosis and thorax is the most common site of extra pelvic endometriosis. An older age at diagnosis (34.2 ± 6.9 years), and right sided lesions predominate the clinical picture. Thirty-nine percent of patients have associated diaphragmatic lesions. Diverse hypothesis have been advanced to explain the pathogenesis of endometriosis related pneumothorax: spontaneous rupture of blebs, shedding of endometrial implants of visceral pleura, and the transdiaphragmatic crossing of air from the genital tract during menses. Known risk factors associated with thoracic endometriosis include previous gynaecologic surgery (such as curettage for miscarriage, hysteroscopy for endometrial biopsy, or revision of the uterine cavity after caesarean section), primary or secondary infertility, and the history of pelvic endometriosis.

Iatrogenic pneumothorax may be caused during transthoracic needle aspiration or biopsy, subclavian or jugular vein catheterization, thoracocentesis, mechanical ventilation, cardiopulmonary resuscitation, tracheobronchial biopsy, among the commonly reported causes. Rarer reported causes are liposuction of axilla fat, liver biopsy, colonoscopy and gastroscopy [9]. Surgeries with operative fields far removed from thorax, have been reported to be associated with pneumothorax,

such as orthognathic surgery [10]. Iatrogenic pneumothorax related to mechanical ventilation has been reported in up to 15% of ventilated patients [11].

Communication between a bronchus (main stem, lobar or sublobar bronchus) and pleural space, called bronchopleural fistula, usually results as a complication of lung-resection surgery. The incidence of bronchopleural fistula is up to 1% after lobectomy and about 4–20% after pneumonectomy [12].

#### **3. Diagnosis**

#### **3.1 Clinical examination**

On inspection, tachypnea, increased work of breathing and respiratory distress may be seen. Cyanosis, drowsiness and decreased oxygen saturation may be found in tension pneumothorax. On palpation, tachycardia, chest wall tenderness, subcutaneous emphysema, decreased chest wall expansion, decreased tactile fremitus and tracheal shift are noted.

Hyper-resonant notes on percussion over the affected lung fields and decreased air entry perceived on auscultation are indicative of pneumothorax. There may be absent breath sounds on the ipsilateral side with contralateral reduced air entry in tension pneumothorax. Iatrogenic pneumothorax should be suspected in any patient who becomes more dyspneic after a medical or a surgical procedure that is known to be associated with the development of the pneumothorax. Sudden increase in peak airway pressure and sudden decline in oxygen saturation in a patient on mechanical ventilation should ring warning bells for the intensivist.

#### **3.2 Investigation**

A chest X-ray may reveal free air around the periphery of the lung fields and decreased lung volume. It may demonstrate the aetiology of the pneumothorax, such as rib or sternal fractures or presence of emphysematous lungs. Films should be taken in erect position, because in supine position, air spreads out in whole of pleural cavity, and films may appear normal, even in the presence of significant air. In patients who cannot be positioned erect and need to be supine, a deep sulcus sign (deep lateral costophrenic angle) should be looked for [13].

Methods to determine the size of pneumothorax on chest X-ray give approximate idea only. There are currently two methods described in adults. If the lateral edge of the lung is >2 cm from the thoracic cage, then, it implies air is occupying at least 50% of thoracic volume and hence, pneumothorax is large in size. Another method is measuring the fractional change in linear dimension of lung, and that multiplied by a factor of three, gives the fractional volume of pneumothorax [14].

Computed tomography (CT) chest provides more accurate information regarding volume of pneumothorax and associated pathology. Obtaining X-ray or CT images may be problematic and time-consuming in poly-trauma patients. Nowadays, in many trauma centres, pneumothorax is detected by sonography and has been included as a part of focused abdominal sonography for trauma (FAST) examination [15]. Ultrasound plays a important role in patients who are not stable enough for chest X-ray and CT. Also, ultrasound is not invasive and the patient is not exposed to radiation. According to a study of Blaivas et al., chest X-ray and ultrasound have a sensitivity of 75.5 and 98.1%, respectively and a specificity of 100 and 99.2%, respectively [16].

Bronchopleural fistula should be suspected in a lung resection patient with large continuous air leak and signs of empyema (leukocytosis, fever, purulent fluid on

thoracocentesis, and pleural fluid on chest X-ray or CT scan). Large pneumothorax developing days or weeks after resection is strongly indicative of a bronchopleural fistula. There is often a persistent and worsening cough. Since these patients have high mortality rates, of 11–18% for early fistula (within 30 days of surgery) and 0–7% for late fistula (beyond 30 days of surgery), they should be evaluated thoroughly by CT scan and flexible bronchoscopy. Bronchopleural fistula is separately discussed thoroughly elsewhere.

#### **4. Management**

#### **4.1 Initial management**

Initial management of pneumothorax patients involves ensuring adequate airway, providing supplemental oxygen, securing an intravenous line, looking for signs of compromised breathing and deciding on the need of tube thoracostomy. Tension pneumothorax should be diagnosed by clinical assessment and a tube thoracostomy/needle thoracocentesis should be performed immediately. Scant data exists in literature proving the efficacy of needle thoracocentesis procedure. However, when tube thoracostomy is anticipated to take time, a needle thoracocentesis may be done immediately, to save life.

Tube thoracostomy is an emergency procedure and is mandatory where pneumothorax is large, or patient has respiratory compromise. Some centres practice drainage of all traumatic pneumothoraces irrespective of symptoms [11]. This line of management in simple pneumothorax is considered invasive by other centres, who recommend observation and oxygen supplementation for small pneumothoraces.

Sucking chest wounds require immediate sealed-cover with an occlusive, air-tight, clean plastic sheet. The sterile inside of gloves-packet can be used in an emergency situation. No patient with penetrating chest wound should be neglected, as tension pneumothorax or life-threatening respiratory emergency can arise.

Upright positioning is beneficial unless contraindicated, like in spinal injury. In a patient with pneumothorax who requires air transport, it is essential that an intercostal tube with Heimlich valve be placed prior to transfer, as pressure changes during flight will cause progression in the severity of the injury and may potentially lead to development of tension pneumothorax.

Pain impairs the ability of the person to breathe, further compromising lung mechanics, in inflammed and contused lungs. In addition, it causes the retention of pulmonary secretions which further suppresses the patient's cough reflex, finally leading to atelectasis and increasing morbidity, Nonsteroidal anti-inflammatory drugs, systemic opioids or regional analgesia methods such as epidural analgesia, intrapleural analgesia, intercostal nerve block, and thoracic paravertebral block have been used for pain control.

Supplemental oxygen therapy, instead of room air, accelerates the resorption of air in pleural cavity by four-fold. By breathing 100% oxygen instead of air, alveolar pressure of nitrogen falls, and nitrogen is gradually washed out of tissue and oxygen is taken up by vascular system. This builds substantial gradient of nitrogen between tissue capillary and the pneumothorax space, resulting in multifold increase in absorption from pleural space. About 1.25% of the volume of pleural air is absorbed in 1 day; hence 25% of the volume is absorbed in 20 days [17]. Small pneumothoraces are often managed with oxygen administration and monitoring via chest X-rays.

#### **4.2 Tube thoracostomy**

Correct placement of the tube is seen as the stream of the bubbles during expiration and coughing and the rise on the level of fluid in the underwater seal during inspiration. Complications of tube thoracostomy include injury to lung or mediastinum, haemorrhage (usually from intercostal artery injury), neurovascular bundle injury, infection, bronchopleural fistula, and subcutaneous or intraperitoneal tube placement.

Heimlich valve or the Vycon self-sucking chest drainage valve are applied directly to the chest tube and reduce or eliminate the underwater drainage period. The Heimlich flutter valve is quite inconspicuous under clothes and makes ambulatory treatment possible. The valve is made of latex rubber that acts as one-way valve, letting air out and preventing reentry. The Vycon device is a double selfsucking valve. It has a soft plastic casing that allows application of manual pressure to aspirate air or fluid [18].

If the lung remains unexpanded or if there is a persistent air leak 72 hours after tube thoracostomy, thoracoscopy or thoracotomy should be considered. Presence of hemopneumothorax, bilateral pneumothorax, first contralateral pneumothorax and pregnancy may be considered for early invasive treatment [19].

Tube thoracostomy is usually sufficient to treat primary spontaneous pneumothorax. However, Schramel et al. reviewed 11 studies over 32 years, involving 1242 patients with primary spontaneous pneumothorax, treated with needle aspiration or tube drainage, and concluded that about 30% patients have recurrence of pneumothorax [20]. Risk factors for recurrence are radiographic evidence of pulmonary fibrosis, smoking, asthenic habitus and younger age [21]. Presence of blebs or bullae was not found to be significantly associated with recurrence [21]. Of patients with recurrent pneumothorax after initial spontaneous pneumothorax, 72% will develop a subsequent pneumothorax within a 2-year period [21]. Recurrent spontaneous pneumothorax or persistent air leaks at initial presentation are indications for operative treatment. Patients in occupations with excessive pressure changes (pilots and divers) or those residing in remote areas, are candidates for operative intervention after a single episode of spontaneous pneumothorax to prevent a potentially life-threatening recurrence.

Clinical picture in secondary spontaneous pneumothorax (SSP) is complicated by the presence of underlying diffuse lung disease, such as chronic obstructive pulmonary disease (COPD), cystic fibrosis, tuberculosis, fibrotic lung diseases such as idiopathic pulmonary fibrosis, and autoimmune diseases involving pleura such as rheumatoid arthritis, ankylosing spondylitis, systemic sclerosis, and Sjogren's syndrome. Observation without evacuation of the pneumothorax, is usually not possible because these patients usually are very symptomatic. Simple aspiration is less likely to be successful in SSP than in primary pneumothorax [22]. It is attempted as an initial treatment in small (air space <2 cm) pneumothoraces in minimally breathless patients under the age of 50 years. If the patient with SSP is 50 years or older, and if the rim of intrathoracic air is larger than 2 cm on a chest X-ray, intercostal tube drainage is advocated. Clinically unstable patients should have a chest tube inserted, notwithstanding the size of the pneumothorax. Sixty-one to seventy percent of leaks resolve by day seven of tube drainage. Further drainage is unlikely to improve success. If air leak does not stop after 48 hours of continuous drainage, consultation for surgical intervention is recommended because of significantly lower healing rate of pleura in cases of SSP compared with primary spontaneous pneumothorax [23].

Indications for operative treatment include persistent air leak, recurrent pneumothorax, pneumothorax after pneumonectomy or intolerance of the prolonged effects of pneumothorax, not relieved by more conservative approaches.

#### **4.3 Surgical method**

Prevention of persistent air leak or future recurrence requires initial identification of the source of the air leak, that is, macroscopic blebs or bullae. Bullae are air filled spaces within the lung parenchyma resulting from the progressive destruction of alveolar tissue. Typically they have relatively thick fibrous walls, grow progressively larger, and are poorly ventilated and with poor perfusion. A giant bulla is defined as one which occupies more than one third of the chest cavity. Complete intrathoracic inspection requires division of all pleural adhesions since these often conceal the culprit lesion. The source of air leak is then controlled by stapling, or suturing.

After completion of the bleb resection, pleurodesis is performed to decrease risk of recurrence. Horio et al. has shown, in a comparative study, that recurrence rate diminished from 16 to 1.9%, when pleurodesis is added to bullectomy [24]. Areas of pleural porosity are potential sources of recurrence, and may be too widespread to be resected. Hence, pleural symphysis is important.

There are three basic approaches to achieve the principles above: thoracostomy, thoracoscopy or thoracotomy. Video-assisted thoracoscopic surgery (VATS) is enabled by the insertion of a 5- to 10-mm videothoracoscope via a 1- to 2-cm incision in the lateral sixth intercostal space. Two more similar incisions are placed anteriorly and inferiorly in the fourth and seventh intercostal space (**Figure 1**). Instruments are introduced via rigid or flexible ports. Adhesions are taken down with sharp dissection. Bleb excision or bullectomy is carried out with an endoscopic linear cutter. The bullae are deliberately opened by cautery or scissors and allowed to deflate. An endoscopic lung clamp is used to grasp the bulla and is then rotated repeatedly as if winding a clock. This action collapses the bulla onto itself and the demarcation between bulla and normal lung parenchyma is revealed. Small ventilated breaths to the ipsilateral lung can also highlight this transition zone. The endoscopic linear cutter stapler is then used to amputate the base of the bulla (**Figure 2**). Alternatively, bleb may be ligated using a pre-tied Roeder slip knot, introduced by an external applicator.

When excising the emphysematous bulla, the staple lines must be reinforced to reduce chance of postoperative air leak. Application of buttress material in the staple line distributes tension throughout the staple line, seals off the staple holes and narrows the spaces between each staple, thus reducing tearing at the staple line. Additionally, the buttress provides a broader pressure profile around each

**Figure 1.** *Port placement for blebectomy [25].*

**Figure 2.** *Apical bullectomy using ring forceps and endoscopic stapler [25].*

individual staple across the staple line, leading to potentially improved haemostasis. Material such as fibrin glue, bovine pericardium, poly-glycolic acid, polydioxane ribbon, Teflon felt, collagen patches and polytetrafluoroethylene (PTFE) sheets have been used to reinforce staple line. Nonabsorbable synthetic materials carry the potential hazard of inflammation and/or bacterial colonisation. Biomaterials originating from animal tissues have a risk of cross-species transmission of infection.

Accessory ports are removed under direct thoracoscopic guidance and the sites inspected for haemostasis. A 24 or 28 F drain is placed to the apex of the hemithorax. This is brought out of one of the port sites and connected to underwater seal drainage and suction. The lung is inflated under direct vision by the scope to verify complete inflation, locate additional blebs, and insure proper placement of the chest tube to the apex of the hemithorax. An inflated lung can displace a tube 2–3 cm caudally. If not corrected, this will frequently lead to a loculated pneumothorax at the apex and thwart the pleurodesis. The sites are closed in two layers with an absorbable suture.

Open surgery is usually performed via muscle-sparing thoracotomy. A lateral or axillary thoracotomy via the fourth intercostal space preserving the fibres of latissimus dorsi and with minimal rib retraction is the approach of choice. Bullae are opened, bronchial edges are oversewed, edges of the bullae are unfolded and stapled. Exogenous materials are buttressed to minimise postoperative air leak.

Randomised prospective study comparing VATS with axillary thoracotomy found no significant difference in postoperative blood loss, lung function, postoperative pain, use of analgesics, postoperative complications, duration of hospital stay and resumption of normal activities. However, with a minimum follow-up of 2 years the recurrence rate after VATS was 4.3% and after a limited thoracotomy, was 0% [26].

However, for recurrent pneumothorax, a randomised study found significantly longer operative time with VATS. Complication rate, chest tube duration, hospital stay, and incidence of chronic pain were not significantly different [27].

In multiple studies, VATS was found to be associated with higher recurrence rate compared to open thoracotomy [28–30]. Barker and colleagues performed a metaanalysis by comparing the reported recurrence rates in patients undergoing VATS with those having open surgery. Results showed a four-fold increase when a similar pleurodesis procedure is performed with a video-assisted approach compared with an open approach [28]. One of the reasons attributed to it was insufficient visualisation of bullae or blebs on the lung by thoracoscopy. Another reason quoted

was less adhesion between the lungs and the chest wall postoperatively when VATS is performed compared with open thoracotomy. Inspite of this, many thoracic surgeons prefer the VATS approach as it is less invasive, less painful, and associated with a shorter hospital stay [31]. VATS is, thus, now considered approach of choice for elderly patients or those with multiple comorbidities [32, 33].

Migliore et al. approached pneumothorax through single port, using handcrafted 20 mm flexible trocar [31]. Jutley et al. compared the standard three-port VATS and uniVATS for surgical management of spontaneous pneumothorax and demonstrated safety and effectiveness with the latter technique [34]. Reduction of intraoperative blood loss and postoperative pain with a higher patient's satisfaction score in uniVATS emerged from a propensity matched comparative analysis by Dai et al. [35]. However, retrospective comparison of uniport versus multiport VATS lobectomies by Chang et al. revealed no difference in operative time, postoperative 30-day mortality, chest tube permanence, hospital stay and reoperation rates [36].

More recent advance in the field of thoracic surgery is robotic-assisted surgery. The surgeon sits at a console, away from the patient in operating room and controls the instruments, including camera, on the robotic surgical system. A small 3D high-definition camera is placed through one of the incisions to provide a good view of the chest cavity, while wristed robotic instruments are inserted through the other small incisions.

For bilateral bullous disease, staging the operations is preferred, to minimise morbidity as well as to allow the ipsilateral lung to re-expand completely, optimising the patient's functional status before tackling the contralateral lesion.

Catamenial pneumothorax with mild symptoms is usually managed with simple rest and thoracocentesis or chest tube for symptomatic relief. The surgical aspects include removal of blebs and bullae, wedge resection, and pleurodesis (abrasion or talc). Most surgical treatment is performed by thoracoscopy, and pleurodesis has been advocated to reduce recurrences. Endometrial deposits on diaphragm are removed as conservatively as possible to spare the diaphragmatic function. Multiple small defects are repaired by titanium clips. The diaphragm is finally reinforced by Prolene or Gore-Tex® mesh. Spiral clips are placed radially at the border of the prosthesis [37]. There is still no agreement regarding whether a prosthetic repair should be recommended. Bagan et al. reported fewer recurrences after diaphragm reinforcement with polyglactin mesh [38]. Concern exists about the use of VATS for large diaphragm defects. Minimally invasive approach is not fully supported by evidence. Both sides of the diaphragm need to be evaluated if one side is noted to have endometrial implants. Superficial diaphragmatic endometriosis can be treated with cold scissors, monopolar energy, bipolar energy, CO2 laser, or a plasma energy source [39]. Bagan et al. suggested application of surgical treatment during menses, for better visualisation of the endometriotic lesions [38].

Postoperative treatment with GnRH agonists or oral contraceptives for 6–12 months is suggested for all patients with proven catamenial pneumothorax for symptomatic relief and to reduce recurrences. The goal of early GnRH analogues administration is to prevent cyclic hormonal changes and induce suppression of ectopic endometrium activity, until accomplishment of effective pleurodesis, since formation of effective pleural adhesions require time [40]. Longer period of hormonal treatment (median 17.5 months) has been required after reoperations for catamenial pneumothorax. Recurrence rate varied from 14.3 to 55%.

#### **4.4 Pleurodesis (mechanical and chemical) and parietal pleurectomy**

Pleural symphysis is used to obliterate the potential space between pleural surfaces to prevent recurrent pneumothorax. This is accomplished by inducing an *Pneumothorax: A Concise Review and Surgical Perspective DOI: http://dx.doi.org/10.5772/intechopen.101049*

inflammatory reaction between the visceral and parietal surfaces with a chemical agent, mechanical abrasion or by stripping the parietal pleura which results in fusion of the visceral surface to the denuded thoracic wall. Chemical agents include talc, doxycycline, tetracycline, bleomycin, iodopovidone, Corynebacterium parvum and silver nitrate. Mechanical pleurodesis is done by vigorously abrading the parietal pleural surface with tightly rolled gauze, held by ringed forceps or a Bovie scratch pad (**Figure 3**).

Parietal pleurectomy involves sacrifice of the parietal pleura. With the help of saline infusion in sub-pleural space, the parietal pleura can be bluntly dissected with a end-forceps. Alternatively, electrocautery can be used. Ayed and Chandrasekran suggested that in apical region, pleurectomy might be a more effective procedure than pleural abrasion [41].

In a randomised prospective study of 96 patients, pleurodesis by talc slurry resulted in the lowest recurrence rate of 8%, compared to 13% with tetracycline and 36% with simple tube drainage [42]. Talc is insufflated into the chest so that complete dispersion throughout the hemithorax is accomplished. This is typically accomplished with an atomizer. Alternatively, talc can be blown into the chest from a LUKI tube in front of a 6 L/minute oxygen flow rate. Alternatively, talc slurry can be instilled through a chest tube in patients who are not surgical candidates.

Talc is cheap. Talc instillation carries a low risk. However, complications such as pulmonary edema, acute respiratory distress syndrome, and hypotension have been reported [43, 44]. In an experimental study in rats, rapid absorption of talc from the pleural space was seen and systemic distribution might explain the complications [45]. Thus, size of the talc particles seems important, smaller particles inducing more systemic complications. In a recent prospective European multicentre study, thoracoscopic pleurodesis with 2 g of graded talc consisting of large particles, was found to be safe after a 30 day observation period [46].

Talc induces a painful inflammatory reaction on the pleural surfaces, which requires adequate analgesia. Aggressive pleurodesis methods should be avoided in chronic obstructive pulmonary disease patients who are suitable for lung transplantation, to reduce graft implantation complications.

In a comparative, randomised study including 73 patients with pleural effusion or spontaneous pneumothorax, talc and iodopovidone were found to be equally efficient and safe [47]. Pleurodesis by autologous blood has been initially used by Robinson, for treatment of persistent air leak in spontaneous

**Figure 3.** *Method of mechanical pleurodesis [25].*

#### *Pleura - A Surgical Perspective*

pneumothorax patients [48]. This method is being widely used as a treatment of choice for air leaks, since pain and fever, which have been reported with other chemical pleurodesis agents, are rarely encountered with this agent [49, 50]. Development of empyema and tension pneumothorax have been reported, which had occurred due to clotting of the blood in the chest tube and care must be taken to prevent it [50].

In children, the management protocols of pneumothorax remain almost the same. In children too, surgery reduces ipsilateral primary spontaneous pneumothorax recurrence. But, surgery is shown to be predictive for contralateral recurrence in them [51]. Perhaps the positive pressure ventilation required during surgery leads to formation of new blebs contralaterally, or to over-distension of already existing contralateral blebs [52].

#### **5. Anaesthesia**

VATS is commonly performed under general anaesthesia with split-lung ventilation. The COPD patient's baseline pulmonary functions are often suboptimal and they may represent a relative contraindication to split-lung ventilation, thus conferring axillary thoracotomy an advantage over VATS. However, postoperative exacerbation of respiratory function or postoperative chest pain has been more effectively avoided with thoracoscopic surgery [53, 54]. To prevent hypoxemia during one-lung ventilation for thoracoscopic surgery, application of continuous positive airway pressure to the non-ventilated lung is performed [55]. More sophisticated techniques using fiberoptic bronchoscopic segmental oxygen insufflation and recruitment have been reported [56].

Awake surgery under epidural anaesthesia might be advocated in case with several thoracic diseases [57, 58]. Though the efficacy and safety of awake surgery are still controversial, and definitive criteria for indications for awake surgery do not exist, studies have shown that the mean time for chest tube drainage, hospital stay, and operative time were shorter in epidural anaesthesia group than in general anaesthesia group. The postoperative pain score was significantly lower in the epidural anaesthesia group. The study proved that well-maintained breathing and hemodynamics during the awake thoracoscopic surgery attenuated the surgical stress responses and had a smaller impact on the postoperative lymphocyte responses when compared with conventional thoracoscopic surgery under general anaesthesia with single-lung ventilation [59, 60].

Another alternative to general anaesthesia with split-lung ventilation is total intravenous anaesthesia, using propofol and sufentanil, with local anaesthesia, using lignocaine, at incision sites and pleural surface. This has been described to have comparable results, while doing away with the adverse effects of epidural anaesthesia, such as epidural hematoma, spinal cord injury and phrenic nerve palsy. Total intravenous anaesthesia is technically demanding, and anaesthesia-related phenomena, such as hypotension and bradycardia, may arise. Anaesthetists have used laryngeal masks to secure the patients' airway during the procedure, and provided deep sedation without compromising patient safety [61].

In contrast to secondary spontaneous pneumothorax due to COPD, that caused by lung fibrotic disease shows different characteristics—lungs with fibrotic disease are very fragile and shrunken. The postoperative mortality rate is high (three of 14 patients in one study) due to the exacerbation of basic lung disease and also because full expansion of lung is not achieved by applying negative intrathoracic pressure due to low respiratory compliance [62]. Such a pulmonary fibrotic disease that has taken the centre stage among all diseases, is the COVID-19 disease.

#### **6. Pneumothorax in COVID-19 patients**

Lungs of patients with COVID-19 who have significant interstitial involvement seem physiologically small, with low compliance and reduced elastance. The thickened, stiff tissue makes it difficult for lungs to expand properly, and sustained-pressure ventilation may be necessary to obtain acceptable gas exchanges. In this setting, fibrotic parenchyma and preexisting emphysematous blebs are prone to rupture, with consequent risk of pneumothorax. Overinflation and high positive end-expiratory pressure in such fibrotic and hypoelastic lungs may cause alveolar or preexisting bleb rupture.

Furthermore, pneumothorax and bulla have been reported in COVID-19 patients who did not have any risk factors for pneumothorax, including mechanical ventilation, history of smoking, or pulmonary comorbidities [63]. The alveolar damage, and bronchiolar distortion and narrowing, caused by fibrosis following resolution of COVID-19 pneumonia, led to pulmonary bullae formation. Moreover, the severe cough associated with viral infections increases the intrapulmonary pressure. This, in turn, may precipitate bullae rupture and pneumothorax formation [64].

Chest tube placement should be considered first-line treatment. Persistence of air leak may constitute an indication for low-tidal volume two-lung ventilation thoracoscopy. Because of stiffer parenchyma, black cartridge staplers are needed for bulla resection. Ideal timing for surgical procedure is unclear. It may be better to do the procedures early in the disease when the interstitial tissues are less traumatised, less fibrotic, and less inflamed [65].

Extra-corporeal membrane oxygenation (ECMO) as a treatment option for pneumothorax with severe ventilator settings has been tried successfully, to reduce ventilator settings and thus, allowing the lungs to rest. This reduced the lung inflation, and avoided over distension of the lungs, while reducing the air leak and allowing the pleura to heal [66, 67].

#### **7. Conclusion**

Pneumothorax is a relatively common malady, both in traumatic and nontraumatic setting. The management is initiated by tube thoracostomy and other supportive measures. Presence of underlying lung disease warrants a more aggressive approach. Prevention of recurrence is also crucial, as recurrences are associated with poorer outcome. VATS is an attractive surgical option due to smaller incision and faster recovery. Innovative procedures continue to be described and many will achieve wide acceptability.

*Pleura - A Surgical Perspective*

### **Author details**

Shilpi Karmakar Department of Burns and Plastic Surgery, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

\*Address all correspondence to: drshilpikarmakar@rediffmail.com

© 2022 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

*Pneumothorax: A Concise Review and Surgical Perspective DOI: http://dx.doi.org/10.5772/intechopen.101049*

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#### **Chapter 8**

## Secondary Pneumothorax from a Surgical Perspective

*Simona Sobrero, Francesco Leo and Alberto Sandri*

#### **Abstract**

Although less frequent than the primary spontaneous pneumothorax (PSP), secondary pneumothoraces (SP) are a common clinical problem with a wide range of severity, depending on the triggering cause(s) and patient clinical condition. By definition, an SP occurs in those patients with an underlying condition that alters the normal lung parenchyma and/or the visceral pleura and determines air entry in the pleural space (e.g., COPD) or, eventually, following trauma or invasive procedures (i.e., iatrogenic pneumothorax). Less frequent, yet described, is SP occurring in neoplastic patients or infectious ones. The gravity of an SP is directly correlated to the underlying cause and patients' clinical conditions. For example, it may be a lifethreatening condition in an end-stage COPD but less severe in a catamenial related syndrome. In this chapter, we are providing a surgical overview of the most relevant and updated information on etiology, incidence, pathophysiology, and management of secondary pneumothoraces.

**Keywords:** secondary pneumothorax, COPD, malignant pneumothorax, post-traumatic pneumothorax, iatrogenic pneumothorax, catamenial pneumothorax

#### **1. Introduction**

Pneumothorax (PNX) is an abnormal collection of air in the pleural space. It is defined as primary spontaneous pneumothorax (PSP) or secondary, based on age, causes, and the requirement of different management [1].

A PSP usually occurs following the rupture of subvisceral pleural blebs and its cause is unknown. Most frequently, patients are males, healthy teenagers or young adults, and smokers [2].

Instead, a spontaneous secondary pneumothorax (SPS) is often associated with a known lung disease and the main cause is a chronic obstructive pulmonary disease (COPD). Other causes are, for instance, idiopathic fibrosis, acquired immunodeficiency syndrome (AIDS), and neoplastic disease. Secondary pneumothorax (SP) could also occur after a chest trauma (post-traumatic pneumothorax) or after invasive procedures (iatrogenic). A specific subgroup is represented by the catamenial pneumothorax, as discussed further on.

The annual incidence of SPS is in general 26 cases per 100.000 and it is more common in men with a 3:1 ratio. It is mainly observed in patients aged 50–60 years old [3, 4].

In general, symptoms include chest pain and shortness of breath, but symptoms could differ according to the cause of pneumothorax [5].

In the following chapter, all the aspects related to a secondary pneumothorax are described.

### **2. Secondary pneumothorax**

The annual incidence of an SP is approximately 6.3 cases per 100.000 in men and 2 cases per 100.000 in women, every year [3].

A SP may occur:


Moreover, compared to a primary pneumothorax, SP afflicts patients with a known history of lung disease. For such reasons, an SP:


Generally, the management of such patients is complex and the treatment requires a chest tube insertion, prolonged hospitalization, and consideration for a surgical procedure to induce pleurodesis.

#### **2.1 Secondary pneumothorax in nonneoplastic lung disease**

#### *2.1.1 Definition and incidence*

The main cause of SP is COPD, with an incidence of 26 cases per 100.000 patients per year [7]; COPD is one of the three main causes of death in the world and one of the main causes of chronic disease [8].

Based on computed tomography (CT) images, COPD may be divided into:


*Secondary Pneumothorax from a Surgical Perspective DOI: http://dx.doi.org/10.5772/intechopen.105414*

According to the GOLD guideline [9], COPD severity depends on pulmonary function, evaluated by means of a spirometry. The risk of an SP is higher in patients affected by severe COPD. Patients with FEV1 < 1 L and/or a FEV1/FVC < 40% are deemed at high-risk [10], with a common observance of severe hypoxemia associated with hypercapnia in case of pneumothorax [8].

The development of a secondary pneumothorax in COPD patients is a parameter of high mortality and, in fact, it has been demonstrated that each SP event increases fourfold the chances of death in such patients [11].

#### *2.1.2 Pathophysiology and clinical features*

When a pneumothorax occurs in a COPD patient with a low respiratory function, it is common to observe severe hypoxemia caused by a lower ventilation-perfusion (V/Q ) rate, which is capable to determine an incremented shunt that is directly proportional to the PNX size. Compensatory hypercapnia is often associated [8].

For such reasons, an SP onset in COPD patients is generally associated with a rapidly progressive dyspnea and pleuritic chest pain where a prompt management is mandatory.

#### *2.1.3 Diagnosis*

Generally, COPD patients present with hyperinflated lungs and abnormal lung auscultation. A reduced or absent vesicular murmur associated with symptoms such as shortness of breath, low saturation, and hyperventilation should easily guide to diagnose a PNX.

A chest X-ray is mandatory to assess the presence of a PNX, which usually appears as a complete collapse of the lung. Seldomly, a subcutaneous emphysema may occur, concealing the PNX at the chest X-ray (**Figure 2**).

Generally, at chest X-ray, bullous lesions have a concave appearance while a pneumothorax has a concave profile (**Figure 3**).

**Figure 2.** *Massive subcutaneous emphysema. Red arrow to indicate the small size SP.*

#### **Figure 3.**

*The different appearance between an SP and bullae. Image a: a secondary pneumothorax in a COPD patient. At the chest X-ray, a pneumothorax has a concave profile. Image b: bulls lesion has a concave appearance.*

In COPD patients, however, a CT scan is useful to better analyze the pneumothorax size and the severity of the lung disease. Moreover, The CT scan can help in distinguishing a big bulla from a true PNX. This may not be an easy task; however, if a bulla is detected, a chest tube is not always indicated, or if it is, it should be carefully placed under CT guidance, in order to avoid an iatrogenic rupture of the bulla determining a complex PNX, which potentially requires a surgical treatment.

It is important to remember that due to the underlying pathology and the low functional reserve, these patients could become suddenly critical in case of a PNX and should therefore be treated accordingly. For the same reason, even if the PNX is correctly treated by means of a chest drain, these patients still remain at a higher risk of death. Death causes are, in fact, associated with the onset of an acute or late

#### *Secondary Pneumothorax from a Surgical Perspective DOI: http://dx.doi.org/10.5772/intechopen.105414*

respiratory failure [12, 13], or with a higher risk of developing sepsis as a consequence of pneumonia or empyema due to the pneumothorax management (i.e., chest tube and/or pleurodesis) [14].

#### *2.1.4 Other causes of SP in non-COPD patients*

Any noxa capable to affect the integrity of the visceral pleura and reduce the lung elasticity could be a cause of a secondary pneumothorax and therefore, a secondary pneumothorax may be diagnosed in the process of:


Acquired immunodeficiency syndrome (AIDS) could be another cause of SP, likely related to an increased risk of developing cystic lesions in the sub-pleural space [16].

#### *2.1.5 Management*

As in the case of primary pneumothorax, the treatment goals should aim at:

• evacuating the pleural cavity from the air to restore the normal intrapleural negative pressure;

**Figure 4.** *Pneumothorax caused by tubercular infection.*


According to the BTS guidelines, a secondary pneumothorax that occurred in a known diseased lung requires the insertion of a small-bore chest tube to drain the air in the pleural space [17].

Pneumothorax aspiration, which finds a treatment indication in the primary spontaneous pneumothorax, has a high-risk of failure, but it may be taken into account in the case of symptomatic patients with a small pneumothorax. A persistent air leak can be managed conservatively obtaining a complete resolution [17].

A talc slurry pleurodesis could be a nonsurgical therapeutic management option to be considered for persistent air leak in patients deemed unfit for surgery. According to the American College of Chest Physicians (ACCP) consensus, a talc slurry pleurodesis through the chest tube is indicated to avoid recurrence after the first episode [14].

By contrast, the BTS guidelines suggest pleurodesis in case of a recurrent SP or in case of a persistent air leak, which in COPD patients may resolve in a long time compared to non-COPD patients [18]. Surgery is an option in SP, but COPD patients may not be deemed fit enough for surgery because of their clinical status. A prerequisite for talc pleurodesis is a complete or major re-expansion of the lung. In case of partially expanded or nonexpandable lungs, other options should be advocated, such as permanent drains connected to a Heimlich valve. Furthermore, prior to proceeding to talc pleurodesis, the increased risk of a pulmonary restrictive dysfunction secondary to talc insufflation should be well pondered and discussed interdisciplinary.

Contrarily to the ACCP guidelines that suggest medical thoracoscopy or VATS as the first choice to perform talc pleurodesis, because of their lower morbidity, the BTS guidelines consider an open approach (thoracotomy) as the procedure of choice, limiting VATS procedures to unfit patients [17].

Furthermore, in nonsurgical patients with a persistent air leak a talc slurry via chest tube should be taken into consideration.

#### *2.1.5.1 Focus on: management of emphysema-dominant COPD lung volume reduction surgery (LVRS)*

The 2021 GOLD guidelines consider as the main surgical option in high-grade COPD [9]:


#### **Table 1.**

*Results in the four groups studied in the NETT trial.*

*Secondary Pneumothorax from a Surgical Perspective DOI: http://dx.doi.org/10.5772/intechopen.105414*


LVRS can improve survival in severe lung emphysema, mainly in the upper lobes localized emphysema, and low-ability exercise patients [17]. The National Emphysema Treatment Trial (NETT) identified four groups of patients [19] on the basis of their postoperative exercise tolerance and their emphysema pattern at the CT scan (**Table 1**).

The NETT demonstrated that the effects of LVRS are durable and that it is strongly recommended in upper lobe-predominant emphysema with low exercise capacity and should be considered for palliation in patients with upper lobe emphysema and high exercise capacity (**Figure 5**).

#### *2.1.5.2 Endobronchial valves*

Predominantly, the following endobronchial procedures find an indication in reducing the end-expiratory lung volume and to improve exercise tolerance:


Endobronchial valves are placed in segmental or lobar bronchi through rigid bronchoscopy, allowing peripheral lung deflation, lung volume reduction, and improvement of symptoms with an accepted mortality rate equal to 5–10% [20]. The valve mechanism does not permit air to go through the segmental or lobar bronchus during the inspiration but, instead, it allows it passage during the expiratory phase. The valves are available in multiple diameters, ranging from 4 to 8.5 mm.

#### **Figure 5.**

*Results of NETT. Image D: Upper lobe–predominant emphysema with low exercise capacity group. The surgical group has a lower probability of death than the medical group.*

In general, two main devices are available:


Both devices are inserted in the operating theater with the patient sedated and in spontaneous assisted (jet-) ventilation [21].

The VENT trial (Endobronchial Valve for Emphysema Palliation Trial) [22] is a two-arm, randomized, controlled, multi-center trial that showed a 4.3% FEV1 increase in the EBV group compared to the 2.5% in the control medical group.

However, the study shows that complications following the procedure may counterbalance the advantages of the procedure itself. In fact, on the one hand, patients may obtain an improvement in their respiratory function, and on the other hand, it may be associated with hemoptysis, pneumothorax with persistent air leak, and COPD exacerbations, which may occur more frequently in advanced, hyperinflated emphysema patients. Moreover, as evidenced in the VENT trial, the 2-yrs mortality rate in the EBV group was 2.8% compared to no deaths in the control group, but the difference wasn't statistically significant (p = 0.19).

#### **2.2 Secondary pneumothorax in concomitant neoplastic disease**

Although rare findings, the main causes of SP from malignant diseases are primary cancers of the lung and pleura (e.g., mesothelioma) followed by infiltrative/ metastatic pleural diseases, such as in the case of germ cell tumors, breast cancer, or osteogenic and soft tissue sarcomas metastasis [21–23].

#### *2.2.1 Incidence*

The occurrence of an SP as the first manifestation of a lung cancer ranges between 0.03% and 0.05% and usually allows to detect the unknown presence of a lung tumor or metastasis [24] without affecting the prognosis [25, 26].

By contrast, a review published in 2010 analyzed data available in the literature concerning pneumothorax secondary to sarcoma, highlighting increased mortality in such patients compared to those without such complications [26].

#### *2.2.2 Pathophysiology*

Several hypotheses have been taken into account to explain the pathogenesis of pneumothorax secondary to malignant disease, which include (i) an alteration of the pleural surface following tumoral pleural infiltration; (ii) rupture of a necrotic tumoral nodule; and (iii) necrosis of subpleural metastases [27].

Moreover, adjuvant or neoadjuvant chemotherapy and/or radiotherapy treatments may alter the lung parenchymal structure. In these cases, the high-risk of infection associated with a reduced functional repair mechanism could enhance the risk of pleural alterations, possibly leading to a secondary pneumothorax. Also, a

tumoral invasion of the small airways could be responsible for a distal alveolar space dilatation determining air-trapping, which may lead to rupture and pneumothorax.

#### *2.2.3 Clinical features and diagnosis*

The clinical presentation depends on the patients' performance status according to their functional status and disease stage. Usually, clinical signs and symptoms are chest pain and shortness of breath.

A chest X-ray is mandatory to assess the presence of a pneumothorax, which usually appears as a complete collapse of the normal areas of the lung. A CT scan is useful to better analyze the pneumothorax size and to investigate a possible progression of disease.

Pneumothorax can be the first sign of neoplastic disease, especially when it assumes a recurrent nature in high-risk patients (heavy smokers, COPD), who will therefore undergo further investigations, which will lead to eventually diagnose the tumor.

#### *2.2.4 Management*

Diagnosis and management of a secondary pneumothorax concomitant to a pulmonary neoplastic disease are the same for a pneumothorax happening in a pre-existing neoplastic condition. A chest tube is recommended according to its size and the clinical features of patients.

In case of a persistent air leak or in a recurrent pneumothorax, surgery is an option in order to investigate the causes and proceed to talc pleurodesis in case of nonoperable tumors or unfit for surgery patients. If surgery is contemplated, the surgeon may proceed to obtain a diagnosis in case this was not achieved previously, by means of pleural biopsy/wedge resection/lymph node sampling. Cases of recurrent pneumothorax as the first sign of pleural mesothelioma or sarcoma metastasis have been reported, identifying the thoracoscopic bullectomy as the key to the best diagnosis and treatment [28, 29].

#### **2.3 Secondary pneumothorax in chest trauma**

#### *2.3.1 Incidence*

Thoracic trauma is the third leading cause of death following abdominal injury and head trauma in polytrauma patients [30]. Management of chest trauma patients is complex and requires an interdisciplinary team with experience in anesthesia, critical care, and surgical disciplines, especially neurosurgery, trauma surgery, abdominal surgery, and thoracic surgery.

Blunt and penetrating chest traumas can be the cause of pneumothorax and trauma should be taken into account when discussing secondary pneumothoraces.

#### *2.3.2 Definition*

Thoracic trauma can be differentiated into blunt or penetrating.

Penetrating injuries, such as blade wounds and firearm injuries, are disruptive to tissue integrity. Gunshot and stabbing account for 10% and 9.5% of penetrating chest injuries, making these the most common etiology of penetrating trauma.

A blunt trauma (**Figure 6**) is a nonpenetrating injury of the chest. Blunt thoracic injuries are more common than penetrating injuries [30].

#### **Figure 6.**

*Pneumothorax after a barotrauma.*

Blunt injuries can cause damage to organs and structures without disrupting the integrity of the tissue. Falls from a great height, motor vehicle accidents, and occupational accidents are the main mechanisms of blunt injuries [31–33].

#### *2.3.3 Diagnosis*

Depending on the mechanism of injury (e.g., acceleration-deceleration and direct impacts on the chest) an SP may be detected and should be treated and investigated on the cause of its onset (rib fractures, tracheal/bronchial or esophageal disruptions, lung contusion, and lung laceration).

Particular attention is reserved for a tension pneumothorax, in which air enters the pleural space at each inspiration, while the air in the pleural space cannot escape from the pleural space due to the one-way valve mechanism. The continuous accumulation of air in the pleural space determines a lung collapse, hypoxia, tachypnea, and tachycardia, a mediastinal shift with compression of the contralateral lung and the superior vena cava (SVC), leading to respiratory distress and rapidly to respiratory failure with cardiovascular collapse.

#### *2.3.4 Management*

Management of patients following major trauma should follow the standardized protocol of emergency and resuscitation advanced trauma life support guidelines (ATLS), and a primary survey of the airway, breathing, circulation, disability, and exposure (ABCDE approach) should be performed.

Particular attention is required in a tension pneumothorax because, if a prompt intervention is not carried out, can rapidly lead to death [34]. Immediate evacuation through a chest tube is mandatory as recommended by the ATLS [35].

In general, a chest tube has to be positioned in a post-traumatic pneumothorax, in order to stabilize the patient.

Once the patient is stabilized, radiological investigations, such as chest X-ray and chest CT scan are mandatory to identify the cause of pneumothorax and eventually determine the need for further treatments (surgery).

At the same time, minor blunt traumas may be cause of small pneumothoraces, which may not require a chest tube and can be treated conservatively with high flow oxygen, pain control, and repeated CXR [36].

Penetrating injuries are disruptive to tissue integrity, with direct communication between the pleural space and the external environment and they can be acutely life-threatening.

It is mandatory to know the mechanism of injury as the management may vary. For example, stab versus gunshot injury to the chest can result in different patterns of injury.

Depending on the penetrating trauma, immediate surgery may be deemed necessary and a chest tube is required to stabilize the patient for surgery (e.g., during patient transportation from the trauma site to the hospital). Frequently, when a post-traumatic pneumothorax is present it can be associated with a hemothorax [37].

#### **2.4 Secondary pneumothorax after invasive procedures (iatrogenic pneumothorax)**

Iatrogenic pneumothorax is a possible complication of several invasive procedures, such as a central venous line insertion (0.5–5%), thoracentesis (1, 5–7%), and CT-guided lung biopsy (1–6%); at times they can be associated with hemothorax (1% of all procedures) [38, 39].

In some cases, it may occur as a complication following bronchoscopic positioning of unidirectional valves in emphysematous patients [21].

#### **2.5 Catamenial pneumothorax**

The term "catamenial" derives from two Greek words meaning "pertaining to" and "monthly."

Catamenial pneumothorax is defined as a recurrent accumulation of air in the pleural cavity in reproductive-age women.

#### *2.5.1 Incidence and etiology*

A catamenial pneumothorax (CP) arises within 48–72 h from menstruation. It occurs in 3–6% of spontaneous pneumothoraces and most frequently involves the right side [40].

This kind of pneumothorax is, mainly, included in two syndromes:


#### *2.5.2 Diagnosis*

The cancer antigen 125 (CA-125), a gynecological serum marker, has assumed a role in the diagnostic work-up for TES. In fact, high levels of CA-125 have been associated with recurrent SP with evidence of thoracic endometriosis, such as focal thoracic endometrial implants, during VATS procedures. On the contrary, patients without thoracoscopic evidence of endometrial disease have normal CA-125 serum level [44].

The presence of a CP is not always related to pelvic endometriosis.

In a prospective study, 32 women had a CP diagnosis, but only 2 had pelvic endometriosis associated [41].

#### **2.6 Pneumothorax and pregnancy**

The occurrence of PSP in women of childbearing age is not unusual and there appears to be an increased risk of recurrence during pregnancy and during parturition with potential risks to the mother and fetus.

A more recent case series and literature review have recommended the use of more modern conservative management methods for which favorable outcomes have now been experienced [17, 45]. Pneumothorax in pregnancy can be managed by simple observation, if the mother is not symptomatic, there is no fetal distress and the pneumothorax is small (<2 cm). Otherwise, aspiration can be performed, chest drain insertion being reserved for those with a persistent air leak or a greater pneumothorax.

To avoid spontaneous delivery or cesarean section, both of which have been associated with an increased risk of recurrence, the safest approach will usually be that of elective assisted delivery at or near term. Less maternal effort is required with forceps delivery, which is therefore preferable. Because of the risk of recurrence in subsequent pregnancies, a minimally invasive VATS surgical procedure should be considered few weeks from delivery. Successful pregnancies and spontaneous deliveries without pneumothorax recurrence have been reported after a VATS procedure [17, 45].

#### **3. Conclusions**

In contrast to the benign clinical course of a PSP, SP is associated with preexisting underlying lung disease, trauma, or invasive procedures and can be a lifethreatening event. Patients with pre-existing lung disease tolerate a pneumothorax less well, and the distinction between PSP and SSP should be made at the time of diagnosis to guide appropriate management. The consequences of a pneumothorax in patients with a pre-existing lung disease are significantly greater, and the management is potentially more complex than in PSP patients. Furthermore, SP is correlated to a higher morbidity and mortality compared to PSP. Differently from a PSP, where the management is well defined by different international guidelines, SP treatment may vary because of the clinical conditions, previous episodes, severity and duration of symptoms, and the presence of an underlying pulmonary disease. Its treatment will therefore vary accordingly, from observation to needle aspiration or thoracostomy tube drainage with or without pleurodesis and potentially escalating to an open thoracotomy or VATS procedures, as described in this chapter. In case of surgery in high-risk patients (e.g., COPD patients, frail patients), the risk of proceeding to surgery with the aim of resolving the underlying cause and securing the lung with adequate pleurodesis should be well pondered both from the surgical and anesthesiological point of view based onto the patients' clinical status and consent.

*Secondary Pneumothorax from a Surgical Perspective DOI: http://dx.doi.org/10.5772/intechopen.105414*

#### **Author details**

Simona Sobrero, Francesco Leo and Alberto Sandri\* Thoracic Surgery Division, San Luigi Hospital, University of Torino, Orbassano, TO, Italy

\*Address all correspondence to: alberto.sandri@icloud.com

© 2022 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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#### **Chapter 9**

## Pneumothorax in Children

*Hatice Sonay Yalçın Cömert*

#### **Abstract**

Pneumothorax is a common pleural disease worldwide and is defined as the free accumulation of air between visceral and parietal pleura. Pneumothorax can be spontaneous, iatrogenic, and traumatic. Although it is less common than adults, it is seen in about 1.1–4 per 100,000 per year in the childhood age group. In patients presenting with variable clinic according to the cause of etiology, diagnosis is confirmed on a PA chest radiograph, sometimes a computed tomography may be required. The management of pneumothorax is varying from conservative, over intermediate (chest tube drainage) to invasive methods (video-assisted thoracoscopic surgery—VATS, thoracotomy). Here, we planned to write a chapter that includes a text containing general information about pediatric pneumothorax, algorithms, and visual and clinical cases of the causes of pneumothorax in children, including age, etiology, and treatment approach of pneumothorax in children.

**Keywords:** pneumothorax, children

#### **1. Introduction**

Although pneumothorax has been known in medical history since the times of Hippocrates and Galen, it was the first time that Itard named the term pneumothorax in 1803 [1]. Spontaneous pneumothorax due to bullae rupture was defined for the first time in 1926, and in 1932, Kjaergaard reported that pneumothorax may occur in completely healthy individuals due to isolated lung blebs [2]. In the treatment of pneumothorax, which was tried to be corrected with long bed rest, Noble has used a cannula, plastic drain, and underwater drainage system for the first time in 1873 [3]. The first thoracotomy and bulla resection was performed by Bigger in 1937, pleural abrasion by Churchill in 1941, subtotal parietal pleurectomy by Gaensler in 1956, and the first axillary thoracotomy and bulla excision and apical parietal pleurectomy by Deslauriers in 1980 [3].

#### **2. Definition**

Pneumothorax is defined as the free accumulation of air between visceral and parietal pleural space for various reasons. Pneumothorax can be spontaneous, iatrogenic, and traumatic in both neonatal and juvenile patients. Spontaneous pneumothorax is divided into two as primary and secondary. Primary spontaneous pneumothorax occurs secondary to apical blebs or bullae without evidence of other lung pathologies. Secondary spontaneous pneumothorax happens in the context of underlying lung diseases such as cystic fibrosis, asthma, connective tissue disorders, or pneumonia [4, 5].

Apart from these, if we define pneumothorax according to age, we should also mention neonatal and catamenial pneumothorax. Neonatal pneumothorax is the most common pneumothorax in childhood. It is reported that the cause is most likely the high transpulmonary pressure with the onset of breathing [6]. Catamenial pneumothorax is often associated with thoracic endometriosis syndrome.

#### **3. Physiopathology of pneumothorax**

Pressure in the pleural space is negative throughout the entire respiratory cycle, as the chest wall tends to expand and collapse in the lung. The pressure of −2 to −5 cm H2O in expiration decreases to −25 to −30 cm H2O in inspiration, and this pressure increases approximately 0.25 cm H2O per cm from the lung basal to the apex [7]. Alveolar pressure is always greater than intrapleural pressure. Therefore, due to the high alveolar pressure and tension in the apical region, existing bleps and bullae in the apex may rupture. Thus, it causes air entry from the alveoli to the pleural space. Airflow continues until the pressure in the pleural space is equalized or until air leakage from the alveoli into the pleural space stops. This condition is called pneumothorax. Pneumothorax physiology includes a reduction in vital capacity and a decrease in oxygen partial pressure.

#### **4. Types of pneumothoraces**

Pneumothoraces can be classified as spontaneous (primary and secondary), iatrogenic, traumatic, neonatal, and catamenial pneumothorax. The types of pneumothoraces are shown in **Table 1**.

#### **4.1 Spontaneous pneumothorax**

Spontaneous pneumothorax (SP) is a comparatively rare condition in children. The peak age of occurrence in children is either in the neonatal period or in the late adolescent period [8]. Air enters the pleural space without any evident traumatic or iatrogenic mechanism. The incidence of pediatric SP is 4 per 100,000 in males and 1.1 per 100,000 in females with most occurring in patients 16–24 years of age [5, 9, 10]. SP is generally categorized into primary and secondary. In primary spontaneous pneumothorax (PSP), there is no underlying pathology and occurs unknown etiology. PSP refers to a pneumothorax from apical blebs or bullae [10]. However, secondary spontaneous pneumothoraces occur in children with underlying lung problems.


#### **Table 1.** *Types of pneumothoraces.*

#### *4.1.1 Primary spontaneous pneumothorax*

A primary spontaneous pneumothorax (PSP) occurs without a precipitating event and in the absence of clinical lung disease and has an estimated incidence of 3.4 per 100,000 children with 4:1 male predilection [11]. In pediatric studies, the peak age of incidence occurs between 14 and 17 years of age, mainly in late teenagers [8]. The risk factors of PSP include tall and thin stature with low body weight [8]. Smoking is also the primary environmental risk factor for primary spontaneous pneumothorax, especially in teenage patients [12]. Some studies have shown that familial and genetic forms of PSP are related to mutations in the folliculin gene on chromosome 17 in the literatüre [5, 12].

It has been recommended that subpleural blebs and bullae are causally related to the development of primary SP and may be clarified by that these tall and slim children tend to have higher transpulmonary pressure at lung apex, and their rapid growth relative to pulmonary vasculature may result in ischemia and thus blebs evolution at these regions [5, 8].

Most patients are clinically stable on initial evaluation and small cases may present in fulminant distress [1]. Chest pain and shortness of breath are common presenting symptoms of PSP and may be developed at rest or accelerated by any maneuver that increases intrathoracic pressure (Valsalva) [5, 13]. Other clinical findings in patients with pneumothorax include cough, ipsilateral hypoventilation, and nonspecific respiratory distress [4, 5].

Sample chest X-ray and thorax-computed tomography of our patients admitted with primary spontaneous pneumothorax from our archive are shown in **Figures 1** and **2**.

#### *4.1.2 Secondary spontaneous pneumothorax*

Commonly known situations predisposing individuals to a secondary spontaneous pneumothorax (SSP) include primary lung disease as asthma, cystic fibrosis, interstitial emphysema, inflammatory/connective tissue diseases such as Marfan syndrome, Ehlers-Danlos syndrome, juvenile idiopathic arthritis, systemic lupus erythematosus, polymyositis, dermatomyositis, sarcoidosis, Langerhans cell histiocytosis, α1-antitrypsin deficiency, Birt–Hogg–Dube syndrome, infections such

#### **Figure 1.**

*(a) A 16-year-old male patient presented to the emergency department with a sudden onset of chest pain and was diagnosed with spontaneous pneumothorax on the right side of his chest X-ray (free air in the thorax marked with a red arrow). (b) Film of the same patient after right side chest tube placement (inserted chest tube marked with blue arrow).*

#### **Figure 2.**

*A 17-year-old male patient presented to the emergency department with the complaint of sudden onset of chest pain. (a) There was no pneumothorax in the anterior–posterior chest X-ray of the patient. (b) Minimal pneumothorax image on the left side in the thorax-computed tomography of the patient (free air in the thorax marked with red arrows).*


#### **Table 2.**

*Causes of pediatric secondary spontaneous pneumothorax.*

as *Pneumocystis jirovecii*, tuberculosis, necrotizing pneumonia/abscess, measles, human immunodeficiency virus/acquired immunodeficiency syndrome, parasitic, malignancy (lymphoma, metastases), foreign body aspiration, and congenital malformations such as congenital cystic adenomatoid malformation and congenital lobar emphysema [5, 14]. SSP causes are summarized in **Table 2** [5].

The theorized mechanism is chronic airway inflammation that causes small airway obstructions and creates the pressure needed for air to escape into the pleural space. These conditions can make the lung pleura more susceptible to rupture and subsequent development of pneumothorax [15]. The most important symptom of SSP is dyspnea, tachypnea, and tachycardia.

Cystic fibrosis (CF) is a severe obstructive airways disease and one of the most common causes of secondary spontaneous pneumothorax. Pneumothorax is seen approximately 3.4% of all patients will suffer from CF during their lifetime and mostly occurs in adult patients [16, 17]. Cysts, blebs, and bullae are all commonly found in the lungs of CF patients, and these cause gas to accumulate in the small airways, resulting in a cystic appearance. The typical presentation is acute onset of chest pain and

#### **Figure 3.**

*A 13-year-old male patient followed up with the diagnosis of cystic fibrosis was admitted to the emergency department with respiratory distress. Upon the presence of pneumothorax in the right upper lobe in the thorax in computed tomography (a, b) and the chest X-ray (c), a pig-tail catheter was placed in the right thorax (d). The child's clinical condition did not improve and thoracotomy with pleurectomy was performed. The child was followed up with a chest tube after the operation (e), and has been covered and discharged (f).*

breathlessness, and the treatment decisions include the size of the pneumothorax, severity of disease, stability of the patient, and whether this is the first or a recurrent pneumothorax [16]. Pneumothorax due to cystic fibrosis can also be seen in the childhood age group and invasive surgeries may be required. A spontaneous pneumothorax chest X-ray film of a CF patient from our archive is shown in **Figure 3a**–**f**.

#### **4.2 Iatrogenic pneumothorax**

The most frequent cause of iatrogenic pneumothorax is a transthoracic pulmonary biopsy, but it also may appear as a complication of many other procedures and caused by barotrauma secondary to mechanical ventilation [18, 19]. Iatrogenic pneumothorax is related to underlying lung disease along with high ventilatory settings [19]. The most common cause of iatrogenic pneumothorax is invasive diagnostic and therapeutic procedures, such as central venous access, thoracocentesis,

thoracic surgery, or intubation [1]. Iatrogenic pneumothorax may also develop during cardiopulmonary resuscitation and tracheostomy.

#### **4.3 Traumatic pneumothorax**

Although thoracic injuries occur less frequently in children than adults, thoracic trauma in children carries a 5% mortality [20, 21]. The most causes of trauma in pediatric patients are traffic accidents, followed by falling from heights, and bicycle accidents [22]. The greater flexibility of the thoracic cage in young children permits the anterior ribs to be compressed to meet the posterior ribs [23]. Because of the flexibility, pulmonary contusions are more common than rib fractures in children [23].

The most common injury in children with blunt thoracic trauma is pulmonary contusion and pneumothorax, which is observed as isolated injury in 30% of the cases [22].

Traumatic pneumothorax can be classified as small occult, tension, and open (**Table 3**). A small pneumothorax from blunt torso trauma is often asymptomatic, with more than half identified as being occult (defined as a pneumothorax observed on computed tomography scan of the chest, but not on chest radiograph) [22]. However, a large pneumothorax may cause clinical symptoms that overlap with those produced by lung parenchymal damage—tachypnea, distress, and decreased saturation [22]. A traumatic pneumothorax and contusion chest X-ray film of a patient from our archive is shown in **Figure 4**.


#### **Table 3.**

*Characteristics of traumatic pneumothorax [14].*

#### **Figure 4.**

*An 8-year-old male patient applied to the emergency department due to a traffic accident. (a) Pneumothorax in the right thorax and contusion in the left lung were detected in the chest X-ray (free air in the right thorax marked with red arrows and contusion has shown with yellow arrow). (b) Chest X-ray after tube placement in the patient's right thorax (inserted chest tube marked with blue arrow).*

#### *Pneumothorax in Children DOI: http://dx.doi.org/10.5772/intechopen.100329*

When the mediastinum is displaced to the contralateral side with impairment of the venous return, the tension pneumothorax occurs and is more common in children [22]. The symptoms of tension pneumothorax are tachycardia, severe respiratory distress, and hypoxemia, with hypotension and tracheal deviation. Heartbeat is heard on the opposite side and the neck veins become dilated and severe cyanosis occurs. No chest X-ray is required to insert a chest tube in children with tension pneumothorax. The child's symptoms improve dramatically with chest tube insertion.

Open pneumothorax is usually seen after penetrating injuries. This causes a collapse in the lung on the side of the trauma and ventilation failure in the other lung. The patient who develops open pneumothorax is cyanosed and has serious respiratory distress is present. In the treatment, the defect should be closed with a sterile gas.

#### **4.4 Neonatal pneumothorax**

Neonatal pneumothorax, with an incidence of 1–2% in newborns, is symptomatic in 0.08% of all live births and is reported as 5–7% in those with a birth weight of less than 1500 g, although it can reach 30% in those with an underlying lung problem and those who need mechanical ventilation comes out [10, 11]. The most common cause of this condition is barotrauma [13]. In addition, male gender and cesarean delivery are also considered among risk factors [11].

In order to inflate the lungs of a newborn baby when he is not breathing himself, mechanical ventilation with an average pressure of 50–80 cm H2O is required to overcome the high transpleural pressure. During this resuscitation, the air given into the lungs is distributed with an uneven pressure inside the lungs. As a result, some alveoli are ruptured and air passes from the peribronchial area to the mediastinum and pneumothorax develops [24]. A chest X-ray visualization from a newborn from our archive who needed resuscitation at 41-week postpartum and had pneumothorax on the right in the chest X-ray has been shown in **Figure 5**.

It most commonly occurs in the first three days and should be suspected in cases of sudden respiratory distress, decrease in oxygen saturation, inability to listen to breath sounds, or when ventilator parameters have to be increased.

#### **Figure 5.**

*(a) Film of newborn who needed resuscitation at 41-week postpartum and had pneumothorax on the right in the chest X-ray (free air in the thorax marked with red arrows). (b) Film of the same newborn after right-side chest tube placement (inserted chest tube marked with blue arrow).*

#### *Pleura - A Surgical Perspective*

It causes high mortality and morbidity, especially in premature babies and newborns with underlying lung parenchyma disease. Whatever the cause, neonatal pneumothorax needs to be treated very quickly because pneumothorax in neonates will lead to serious complications, including lung perforation, phrenic nerve palsy, chylothorax, and hemopericardium [11].

#### **4.5 Catamenial pneumothorax**

Catamenial pneumothorax (CP) is a form of thoracic endometriosis syndrome, which also includes catamenial hemothorax, catamenial hemoptysis, catamenial hemopneumothorax, and endometriosis lung nodules, as well as some exceptional presentations [25]. The most common extrapelvic manifestation of endometriosis is thoracic endometriosis and often presents as catamenial pneumothorax [10]. Most commonly occurs in women aged 30–40 years, but has been diagnosed in young girls as early as 10 years of age and postmenopausal women (exclusively in women of menstrual age) most with a history of pelvic endometriosis [25].

CP is a rare and important condition of recurrent pneumothoraces, which occurs within 48–72 h from the onset of menses [11]. The pathophysiology is not completely understood but it is treated with hormonal therapies [11].

#### **5. Diagnosis**

The diagnosis of pneumothorax can be made by physical examination or imaging studies including chest X-ray, ultrasonography, and computed tomography (CT) scan [19]. A conventional chest X-ray is a typical imaging examination used to confirm the diagnosis of pneumothorax and a CT scan may be validated to show smaller pneumothoraces. CT scan is commonly accepted as the gold standard in pneumothorax diagnosis [1]. Dotson et al. proposed that detection of blebs/bullae on the CT scan may be predictive of recurrence of PSP, especially bilaterally pneumothoraces [5]. There are multiple methods for calculating pneumothorax sizes like Light, Rhea, and Collins for adults, but these methods are not appropriate for the childhood age group [5, 26, 27].

Dahmarde et al. suggested that ultrasound is accurate and reliable for newborn pneumothoraces [28]. Ultrasound can result in timely diagnoses specifically in neonatal pneumothorax and facilitates the therapy process; lack of ionizing radiation and easy operation are the benefits of this imaging technique.

#### **6. Treatment**

The treatment options are changing by age, size, and the type of pneumothorax in childhood. There are no standardized guidelines for therapeutic interventions for children with pneumothorax; however, early identification and appropriate management can reduce morbidity and mortality.

While the size of the pneumothorax can be calculated by various methods in adults, there is no method that can be applied to children yet. Although minimal pneumothoraxes that do not cause clinical problems can be followed conservatively, most patients require drainage, and a thorax tube is inserted. Nonoperative treatment methods are monitoring with supplemental oxygen (100% high-flow) or needle aspiration. Surgical treatment methods range from the insertion of a chest tube to more invasive interventions such as video-assisted thoracoscopic surgery (VATS) or thoracotomy, including resections, pleurodesis, or bullectomy [10]. Surgical

#### *Pneumothorax in Children DOI: http://dx.doi.org/10.5772/intechopen.100329*


**Table 4.**

*Guide for chest tube selection for pneumothorax [4].*

indications for pneumothorax are resistant and prolonged air leak (>4 days), persistent and recurrent pneumothorax, large pneumothorax, first pneumothorax with a history of pneumothorax in the other lung, and bilateral pneumothorax [10, 11]. Chest tube placement should be the first choice in patients with surgical indication, and then, open or closed surgical techniques should be planned according to the child's clinic. Although the VATS procedure is easily used in the childhood age group, thoracotomy with resections, pleurodesis, or bullectomy may be preferred or needed in cases with severe air leak and recurrent pneumothorax [29].

Pleural catheters are tools that are placed in the fourth, fifth, or sixth intercostal space in generally anterior or midaxillary line with Seldinger technique and placed to water seal in children. In newborns, the catheters are usually placed from the second or third midclavicular line with again Seldinger technique and placed to water seal. The chest tube sizes are changing from the patient's size and age. The guide for chest tube selection for pneumothorax for children patients is summarized in **Table 4** [4].

The aim of surgical treatment is to resect of blebs and bullae and pleurodesis to prevent recurrences. VATS procedure is performed with good results in children with PSP and as the gold standard for surgical management of PSP by using various surgical instruments from 1 to 3 incisions of approximately 1.5–2 cm, which are opened on the chest with the help of a video [29, 30]. Blebs and bullae due to pneumothorax are removed with VATS with the help of staples. Pleurodesis ensures that the parietal and visceral pleura sheets stick together. Pleurodesis can be performed by using pleurectomy, pleural abrasion, or chemicals [31].

Lewit et al. suggested that nonoperative methods are not suitable for the treatment of pneumothorax and mentioned a decreased recurrence rate in those undergoing surgical treatment at initial presentation in the childhood age group [32]. Also, Lopez et al. have observed decreased median total length of stay and decreased recurrence rate in the surgical group compared with the initial non-VATS group in children [11].

On the other hand, Brown et al. discussed whether conservative management is an acceptable alternative to nonconservative procedures and found that conservative management of primary spontaneous pneumothorax was similar to interventional management, with a lower risk of significant adverse events [33].

The general approach is chest X-ray negative and CT scan positive pneumothoraces do not require invasive methods and they can be followed conservatively [23]. However, since childhood is a wide range, a pneumothorax that looks small may even be mortal for a newborn premature baby. Although thoracic tube insertion is a minor surgical procedure, every procedure has surgical stress, especially for neonatal intensive care patients. Therefore, every child with pneumothorax for whom a follow-up decision is made requires very special close follow-up. Likewise, close follow-up of a child patient with a chest tube placed should be very important in terms of possible complications.

#### **7. Complications**

The most common complications of pneumothorax seen in childhood are air leak, tension pneumothorax, pneumomediastinum, subcutaneous emphysema, hemothorax, and very rarely Horner's syndrome. If the air leak is continued within 48 hours of pneumothorax treatment, it may become resistant. Therefore, a second chest tube or even VATS or thoracotomy may be required for persistent air leakage, depending on the age of the child or the etiology of the pneumothorax [3].

#### **8. Conclusion**

In conclusion, the etiology and management vary according to age and type of pneumothorax in the childhood age group, and this is a life-threatening special condition that requires urgent intervention and special follow-up.

#### **Author details**

Hatice Sonay Yalçın Cömert Faculty of Medicine, Department of Pediatric Surgery, Karadeniz Technical University, Trabzon, Turkey

\*Address all correspondence to: sonayyalcin@hotmail.com

© 2021 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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*Pneumothorax in Children DOI: http://dx.doi.org/10.5772/intechopen.100329*

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### *Edited by Alberto Sandri*

This book provides the thoracic community and pleura experts with an up-to-date surgical vision of pleura pathology. It provides in-depth knowledge and a better understanding of the indications, positioning techniques, and management of chest drains and indwelling catheters, which are commonly utilized in the management of the pleural disease. The book addresses complex topics such as bronchopleural fistula and postoperative empyema as well as pneumothorax.

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Pleura - A Surgical Perspective

Pleura

A Surgical Perspective

*Edited by Alberto Sandri*