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Year : 2018  |  Volume : 2  |  Issue : 2  |  Page : 25-31

Primary spontaneous pneumothorax in children: A literature review

1 Department of Pediatrics, Kaohsiung Veterans General Hospital, Zuoying District, Kaohsiung City, Taiwan
2 Division of Pediatric Allergy, Immunology and Rheumatology/Pulmonology, Department of Pediatrics, Kaohsiung Veterans General Hospital, Zuoying District, Kaohsiung City; Department of Pediatrics, National Defense Medical Center, Tri-Service General Hospital, Taipei, Taiwan
3 Division of Pediatric Allergy, Immunology and Rheumatology/Pulmonology, Department of Pediatrics, Kaohsiung Veterans General Hospital, Zuoying District, Kaohsiung City, Taiwan

Date of Web Publication6-Jul-2018

Correspondence Address:
Bao-Ren Nong
Department of Pediatrics, Kaohsiung Veterans General Hospital, No.386, Dajhong 1st Rd., Zuoying Dist., Kaohsiung City 81362
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/prcm.prcm_3_18

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Studies about primary spontaneous pneumothorax (PSP) in pediatric patients are not as many as in adult patients since the incidence of PSP is lower in children than in adults. There are evidence-based guidelines for the management of PSP in adults, whereas, in children, the approach of PSP is mainly extrapolated from the adult guideline. In this article, aspects of incidence rate, epidemiology, and pathophysiology, diagnosis, management, and recurrence rate about pediatric PSP are discussed.

Keywords: Children, primary spontaneous pneumothorax, recurrence, Video-Assisted Thoracic Surgery(VATS)

How to cite this article:
Kuo PY, Nong BR, Huang YF, Chiou YH. Primary spontaneous pneumothorax in children: A literature review. Pediatr Respirol Crit Care Med 2018;2:25-31

How to cite this URL:
Kuo PY, Nong BR, Huang YF, Chiou YH. Primary spontaneous pneumothorax in children: A literature review. Pediatr Respirol Crit Care Med [serial online] 2018 [cited 2023 Jun 6];2:25-31. Available from: https://www.prccm.org/text.asp?2018/2/2/25/236139

  Introduction Top

Pneumothorax is defined as the accumulation of air in pleural cavity that results in partial or complete collapse of lung. It is an uncommon disorder in children under 18 years of age. The peak age of occurrence in pediatric population is either in neonatal period or late adolescent period. Pneumothorax can be categorized into spontaneous or traumatic. Spontaneous pneumothorax can be further divided into primary and secondary type. Secondary spontaneous pneumothorax arises from preexisting lung disease, such as asthma, cystic fibrosis, or interstitial lung disease [Table 1].[1]
Table 1: Causes of secondary pneumothorax in children

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Compared to adults, studies of primary spontaneous pneumothorax (PSP) in pediatric population are scarce. Furthermore, there is no consensus about the management of PSP in children. The aim of this article is to give a literature review of PSP in children under 18 years of age, mainly focused on its epidemiology, diagnosis, management strategy, and recurrence rate in pediatric population.

  Epidemiology Top

The incidence of PSP in the pediatric population is 3.4/100,000 children.[2] There is a male predominance in this disorder, with a male-to-female ratio ranging from 2:1 to 9:1.[3],[4] In pediatric studies, the peak age of incidence occurs between 14 and 17 years of age, mainly in late teenagers. The affected patients typically showed tall, thin habitus. In some previous studies, the mean Body Mass Index (BMI) was around 18 kg/m 2, which is classified as underweight.[5] It may be explained by that these tall, 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 formation at these regions.[6] In a retrospective study including 171 adolescents, only 34% had underweight BMI.[3] Meanwhile, Noh et al. showed that there was no apparent relationship between BMI and PSP recurrence rate.[7]

  Pathophysiology Top

In spontaneous pneumothorax, the air leaks through visceral pleural, which may be caused by an acute increase in transpulmonary pressure or defects in visceral pleural. Apical bullae and subpleural blebs are found in the majority of PSP patients.[8],[9],[10],[11] In adult studies, subpleural bleb or bullae (usually on the apical portion of the upper lobe) are found in 76%–100% of patients during VATS and in nearly all patients during thoracotomy.[6] In the study of Shih et al., 91.3% (21 of 23) PSP were found to have apical blebs in operation. Lopez et al., 98% (59 of 60) identified blebs/bullae during the operation.[6],[8] While the exact pathogenesis of PSP remains unclear, rupture of blebs/bullae in lung tissue that causing air to leak into pleural space may be the possible reasons.

While many reports revealed that smoking is a major risk factor in adults PSP, the situation is not the same in children. The study of Chiu et al. found that only 22% of adolescent patients with PSP were smokers in their study.[3] This may be explained by the prevalence of smokers in children is low, compared to adults.[3]

  Clinical Features Top

PSP occurred most often when the patient was at rest. A small pneumothorax may be asymptomatic, whereas large pneumothorax may present with acute chest pain, dyspnea, chest tightness, cough, back pain, and ipsilateral shoulder pain. Physical examination usually revealed diminished breath sounds and hyper-resonant percussion over the affected side of the lung. If signs of hemodynamic comprise such as tachycardia, hypotension, and cyanosis were noted, tension pneumothorax showed be considered, and emergent needle decompression may be needed.

  Diagnosis Top

Pneumothorax is mainly diagnosed by symptoms, physical examination, and chest radiography. The size of pneumothorax can be measured from X-ray film. In adults, a large pneumothorax is defined as ≥3 cm of air between the pleural line and apical chest wall (apex-to-cupola distance), or ≥2 cm between the entire lateral lung edge and the chest wall, at the level of hilum.[12],[13]

To calculate the volume of pneumothorax, Light method, Rhea method, and Collins method were used in adult patients.[14],[15],[16] Till date, no standard method had been developed for measuring the size of pneumothorax in the pediatric population. Guideline from the British Thoracic Society (BTS), American College of Chest Physicians (ACCP), and the foresaid methods may be suitable for adolescent patients. For younger children, small or large pneumothorax is usually determined by the relative size of pneumothorax compared to the whole chest cavity.

  Management Top

While the ACCP and the BTS had published guidelines for the management of pneumothorax in adult patients, management of pneumothorax in children has not been standardized. We summarized the management of pneumothorax in children based on several retrospective studies.

Management of pneumothorax includes observation, supplemental oxygen, needle aspiration, thoracostomy tube, and surgical intervention with either video-assisted thoracic surgery (VATS) or open thoracotomy plus pleurodesis.

According to BTS 2010 guideline, observation is the treatment of choice for small PSP without significant breathlessness.[12] Up to 80% of pneumothoraces estimated as smaller than 15% have no persistent air leak.[12] Supplemental oxygen may accelerates the reabsorption of air by the pleura.[17]

PSPs that are large (involving ≥15% of the hemithorax) or progressive may be drained by simple aspiration with a plastic intravenous catheter, thoracentesis catheter, or small-bore (7–14 French) catheter or by the insertion of a chest tube.[17] In a study by Lee et al., the success rates of needle aspiration and chest tube as primary treatments for PSP (small and large) in children were 78% and 67%, respectively, indicating comparable success rates with both interventions. Besides, the overall success rate of conservative treatment (including observation, needle aspiration, and chest tube insertion) for the first episode of PSP was 80% in the foresaid study. Thus, the authors concluded that for most patients with the first episode PSP, conservative treatment with either observation thoracocentesis or tube thoracostomy were suitable.[18]

However, Soccorso et al. suggested that for large PSP in children, initially tube thoracostomy may be better than needle aspiration because 53% initially managed with needle aspiration eventually required chest tube drainage.[19]

Even though most PSP can be initially managed successfully with conservative treatment like needle aspiration or tube thoracostomy, several studies showed that children had a higher recurrence rate than adults after nonoperative treatment of PSP [Table 2]. In adult study, the recurrence rate of PSP was 30%.[17] However, for pediatric PSP, 40%–60% recurrence rate after nonoperative treatment was reported.[18],[13],[26],[27],[28],[29],[30],[31] William et al. showed that for patients initially managed with chest tube, 49.7% ultimately require operative intervention.[24],[32] Soccorso et al. found that most cases with large PSP were identified to have blebs/bulla as their cause for their PSP, and the recurrence rate was high after nonoperative management. The author concluded that recurrent or first episode of large PSP requires computed tomography (CT) evaluation and surgical treatment after initial management with needle aspiration/tube drainage.[19]
Table 2: Data of reported case series of pediatric primary spontaneous pneumothorax

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Furthermore, for patients managed initially with chest tube alone, the probability of subsequent surgery was >50% if they were hospitalized for over 4 days.[25] In the treatment algorithm for PSP in children by Zganjer et al., they started with intercostals tube catheter drainage, aspiration, and observation. If there was a significant air leak that did not stop for 5–6 days, VATS with mechanical pleurodesis was indicated.[9]

The ACCP recommends surgery for adults with air leaks lasting longer than 4 days and for recurrent spontaneous pneumothorax. The optimal timing to shift from tube thoracostomy to VATS in the management of pediatric PSP remains unclear, 3–7 days were reported.[9],[21],[24] Butterworth et al. suggested that air leaks that persist for longer than 3 days are unlikely to close spontaneously, and thus VATS may be indicated in these patients.[21] Noh et al. suggested that if air leaks persisted for 4 days, bullae or blebs were seen on CT scans, or ipsilateral pneumothorax recurred, wedge resection by VATS was performed.[7]

Chiu et al. showed that a large-size pneumothorax with a persistent air leak was the most significant factor for proceeding to VATS surgery. In addition, it was a significant factor for the recurrence of PSP (P = 0.014).[3] Thus, for children with PSP initially managed with tube thoracostomy, early surgical intervention like VATS is needed for persistent air leak. VATS is a safe and effective procedure for PSP in pediatric patients.[25] A retrospective study showed early VATS decreases hospital length of stay, charges, and readmissions.[24]

While CT scan can help to identify the pathology of lung such as blebs/bullae, causes of PSP in most patients, the correlation with intraoperative findings and role in guiding management remains unclear.[8] In a study by Lopez et al., blebs were detected only in 60% of patients who underwent CT scan, whereas 98% of patients who underwent operations were found to have blebs during operation.[8] There was no evidence to support prophylactic VATS in asymptomatic patients with blebs detected during CT scan.[8] For CT scan, radiation exposure and cost also need to be considered. Currently, most studies showed that routine use of HRCT in adolescent patients with PSP was not necessarily.[3] For large PSP or recurrent PSP, CT scan may be indicated to verify possible pathological structure of lung, and help to guide surgical management.[32]

Considering the high recurrence rate of PSP in children managed with conservative treatment, some advocated surgical intervention with VATS as the initial treatment plan, rather than performed after the failure of tube thoracostomy, may bring the benefit of shorter length of stay with lower cost and recurrence rate. However, some studies did not support this point of view.[20]

In the study by Cook et al., the author concluded that a cost-effective treatment strategy for pediatric PSP is tube thoracostomy at first presentation, followed by VATS with thoracoscopic bleb resection. This approach can minimize the number of unnecessary operations.[5]

Qureshi et al. in 2005 revealed that morbidity from recurrent pneumothorax after VATS occurred more frequently after primary VATS (VATS performed as initial treatment) than secondary VATS (VATS performed after nonoperative treatment failure), and the overall cost is higher in primary VATS. The authors concluded that the increased morbidity and cost did not justify a of primary VATS blebectomy/pleurodesis in children with spontaneous pneumothorax.[20]

Lopez et al. also suggested initial management with pleural catheter drainage, and early surgical intervention in the setting of failure of conservative management to achieve full resolution of persistent air leaks in pediatric patients with spontaneous pneumothorax.[8]

The recurrence rate of PSP in children after VATS procedure was reported as 4%–20% [Table 2], and it appeared that recurrence of PSP after surgery was more frequent in children than in adolescents or young adults.[33] Choi et al. reported that the recurrence rate of PSP after VATS was significantly higher in the children's group (<17 years) than the young adult group (10.6 vs. 3.9%, P = 0.032).[33] Noh et al. also showed that the recurrence rate after wedge resection in patients aged ≤16 years was higher than that in older patients, and suggested that wedge resection might be delayed in children.[7] This finding may be due to the fact that children are still growing, and are more likely to have newly formed blebs/bullae in lung tissue, compared to adults.[33]

For PSP management, VATS with either mechanical or chemical pleurodesis is often performed by the surgeon. Pleurodesis can prevent postoperative air leak from staple lines, and help to prevent future pneumothorax by producing adherence of the lung and pleural cavity.[10]

Preventive operation for the contralateral blebs or bulla in asymptomatic patients remains controversial since the risk of development of PSP in these patients was unknown in pediatric practice. Martinez-Ramos et al. did not find an association between the presence or absence of bullae and the recurrence of PSP.[34] Sahn et al. concluded that the presence of bullae should not guide decision-making regarding prevention of recurrence.[17] Ciriaco et al. also suggested that VATS should be considered only for the affected side.[23]

However, in the study of Soccorso et al., 20% (number 10/49) of patients had asymptomatic contralateral blebs/bulla detected on CT scan. Among these children, 40% developed pneumothorax within 6 months.[19] Thus, there was a degree of risk in these patients if contralateral blebs/bulla was detected, and patients should be well-informed about this situation.

  Conclusion Top

The incidence of PSP in pediatric population was 3.4/100,000 children, with male predominance.

In pediatric studies, the peak age of incidence occurs between 14 and 17 years of age. Apical bullae or subpleural blebs are found in the majority of PSP among teenagers and adults. Routine use of HRCT in adolescent patients with PSP is not necessarily. CT should be reserved for large or recurrent PSP.

The recurrence rate of PSP in children after nonoperative treatment is 40%–60%. The optimal timing to shift from tube thoracostomy to VATS in the management of pediatric PSP remains unclear, from 3 to 7 days had been reported. The recurrence rate of PSP in children after VATS was reported as 4%–20%. The recurrence of PSP after surgery is more frequent in children than in adolescents or young adults. Preventive operation for the contralateral blebs or bulla in asymptomatic patients remains controversial. Currently, experts suggest VATS should be considered only for the affected side.

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Conflicts of interest

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  References Top

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