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 Table of Contents  
Year : 2019  |  Volume : 3  |  Issue : 4  |  Page : 72-75

Chronic right middle lobe atelectasis in ambulatory children

Department of Pediatrics, Division of Pediatric Pulmonology, Chang Gung Memorial Hospital at Linkou and Chang Gung University, Taoyuan, Taiwan

Date of Submission28-Aug-2019
Date of Acceptance21-Jan-2020
Date of Web Publication28-Sep-2020

Correspondence Address:
Kin-Sun Wong
Department of Pediatrics, Division of Pediatric Pulmonology, Chang Gung Memorial Hospital at Linkou and Chang Gung University, 5 Fu Hsin Street, Taoyuan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/prcm.prcm_12_19

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Background: Intrinsic obstruction and extrinsic compression of the airway are the main causes of pulmonary atelectasis. The differential diagnoses of right middle lobe atelectasis (MLA) in children are lengthy, and practicing pediatricians usually are frustrated by the diagnostic possibilities in the clinic. Objective: The aim of our study is to present the experience of a children facility with chronic MLA in ambulatory children and guide a pragmatic approach. Materials and Methods: Retrospective chart analysis was performed by a computer search for discharge diagnosis of right MLA or MLA between January 2006 and December 2017 in a pediatric department in Northern Taiwan. Demographic data, underlying diseases, clinical symptoms, radiographic features, and course of treatment were collected and analyzed by descriptive statistics. Results: A total of 30 pediatric patients with chronic MLA were recruited in this study. Isolated MLA was identified in four (13.3%) patients. The remaining 26 patients had associated atelectasis or bronchiectasis in other parts of the lung. The most common causes of chronic MLA identified in our patients were postinfectious bronchiectasis (40%) and immunodeficiency (23.3%). Asthma was an uncommon cause of MLA in this study. Conclusions: Tumors, tuberculosis, retained foreign body, and asthma were all uncommon in the children identified with MLA. While chronic cough was common in the children studied, most were associated with bronchiectasis in other pulmonary segments. When faced with evidence of right MLA, one should consider a chronic suppurative lung disease with or without bronchiectasis, either postinfectious or related with recurrent aspiration.

Keywords: Ambulatory children, middle lobe atelectasis, middle lobe syndrome

How to cite this article:
Wong KS, Chiu CY. Chronic right middle lobe atelectasis in ambulatory children. Pediatr Respirol Crit Care Med 2019;3:72-5

How to cite this URL:
Wong KS, Chiu CY. Chronic right middle lobe atelectasis in ambulatory children. Pediatr Respirol Crit Care Med [serial online] 2019 [cited 2023 Apr 1];3:72-5. Available from: https://www.prccm.org/text.asp?2019/3/4/72/296481

  Introduction Top

Atelectasis occurs in three ways: (i) airway obstruction; (ii) compression of the lung by extrathoracic, intrathoracic, or chest wall processes; and (iii) increased surface tension in alveoli.[1] Children are more prone to atelectasis due to smaller airway caliber, increased collapsibility, a greater number of mucous glands in the airway, more compliant chest wall, and less well-developed collateral ventilation.[1],[2]

Middle lobe syndrome (MLS) refers to recurrent and chronic atelectasis of the right middle lobe atelectasis (MLA) of the left lingual lobe that may be associated with the MLS.[3] Reported predisposing causes of MLS in children include stenosis of the right middle bronchus and poor development of the interalveolar pores of Kohn and bronchoalveolar canals of Lambert.[4],[5],[6],[7]

Our previous experience with chronic atelectasis in children demonstrated various etiologies.[8] Our objective for this report was to summarize our experience of children with MLA in a pediatric tertiary facility in Northern Taiwan after the year 2000.

  Materials And Methods Top

We performed a computer search for discharge diagnosis of right MLS or MLA of pediatric patients below 18 years of age from Chang Gung Memorial Hospital between January 2006 and December 2017. A retrospective chart review was performed for data collection and analysis. Patients with right MLA for <1 month of duration were excluded from the study. Other excluding criteria included patients with chronic debilitating diseases, bedridden neuromuscular disorders with a tracheotomy, and obvious swallowing disturbance, and patients with primary immunodeficiency who had undergone stem-cell transplantation.

Demographic data obtained from the medical record of patients identified with MLA included gender and age at diagnosis. Clinical data included symptoms, associated diseases, radiographic features, bronchoscopic findings, and bacteria isolated from bronchoalveolar lavage fluid. Clinical course and treatment were collected. Data were presented as descriptive statistics. The study was approved by the Institutional Review Board of Chang Gung Memorial Hospital. Informed consent from the parents was waived because of retrospective chart analysis (201701857BO).

  Results Top

A total of 30 pediatric patients with chronic MLA below 18 years of age were identified in this study. The median age of the patients was 10 years (range 2–18 years). Seventeen (56.7%) were male patients. Fifty percent presented with chronic cough, 30% with recurrent pulmonary infections, 13% with hemoptysis, and 7% with wheezing.

Isolated MLA was identified in only four (13.3%) patients. The remaining 26 patients had other associated radiographic finding including segmental or lobar collapse or bronchiectasis in other areas of the lung, either ipsilaterally or contralaterally. The frontal radiographs commonly showed blurring of the right heart border, irregular infiltrates, triangular shadow, mass-like lesion, or dilated bronchi.

In this series of 30 patients with MLA, 18 patients had bronchiectasis. Seven patients had a primary immunodeficiency disorder. Three had common variable immunodeficiency. One had low levels of IgG1 and IgG2. Wiskott–Aldrich and hyper-IgE syndromes and T-cell immunodeficiency were each identified in 1 patient [Table 1]. Two patients had gastroesophageal reflux disease (GERD), two had surgically corrected tracheoesophageal fistula (TEF), and two had chronic suppurative lung disease (CSLD). Single cases of Kartagener syndrome, bronchial asthma, recurrent aspiration, postinfectious bronchiolitis obliterans, and connective tissue disorder were identified. Various causes of bronchiectasis in our patient are presented in [Figure 1].
Table 1: The underlying diseases of patients with chronic middle lobe atelectasis

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Figure 1: Etiologies of patients with bronchiectasis.

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Fifteen patients underwent diagnostic fiberoptic bronchoscopy. Bacterial growth from bronchoalveolare lavage included five isolates of either mixed flora or colony counts of <10 × 104 colony forming units/ml. Ten specimens of bronchoalveolar lavage fluids that were available showed single growths of Viridans streptococcus in 2, Haemophilus influenzae in 2, Streptococcus pneumoniae in 2, and one patient with Streptococcus mitis and Moraxella catarrhalis; one patient with V. streptococcus and Streptococcus salivarius; one patient with Stenotrophomonas maltophilia, and one patient with Pseudomonas aeruginosa and Serratia marcescens.

  Discussion Top

Our data demonstrate that chronic MLS in these patients differed from past observations and current reports elsewhere. Right MLS was originally reported in the 1940s associated with a high incidence of external compression due to tuberculous lymph nodes. In the adult population, the term was meant to be a potential red flag for lung cancer caused by compression of the bronchus from enlarged lymph nodes of the neoplasm.[3] The most common abnormality associated with MLA in our study was bronchiectasis [Table 1]. Further analysis of the group showed that many patients who had other abnormalities also had bronchiectasis and only four (13.3%) patients who had MLA lasting for 6 months had no bronchiectasis [Figure 2]. We found only one case of MLA due to asthma.
Figure 2: Outcome of patients with chronic middle lobe atelectasis.

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Pulmonary atelectasis caused by obstructive lesions in children was uncommonly observed in previous reports.[8],[9] Asthma was reported to be the chief reason for isolated MLS in children.[5],[10],[11],[12],[13] The prevalence of MLS was examined in a study of 1126 children with asthma by Waldbott; only 6 cases of segmental or lobar collapse were found in that study.[12] In another study, Sekerel and Nakipoglu experienced 56 (1.62%) cases of right MLS among 3528 asthmatic children.[13] Springer et al. performed bronchoalveolar lavage in 21 children with asthma and right middle lobe or lingular collapse. Differential cell counts of the lavage fluid revealed predominance of neutrophils in 12 of the 21 patients (57%). Nine of these patient's cultures grew pathogenic bacteria, mainly H. influenzae and S. pneumoniae. There was no correlation between the severity of asthma and a positive bacterial culture. There was also no correlation between the duration of the right middle lobe collapse and a positive culture. The authors concluded that long-standing right middle lobe collapse in asthmatic children is often associated with a bacterial infection.[14]

Chronic pulmonary atelectasis was commonly seen in children with bronchiectasis.[15] Einarsson et al. reported a series of 18 children with MLS where bronchiectasis was common.[16] Studies in adults found that 25%–50% of the patients with MLS had bronchiectasis.[17],[18]

Pulmonary infection was the major cause of non-cystic fibrosis (CF) bronchiectasis in Taiwanese children.[19] In a systematic review of 989 patients with bronchiectasis, the prevalent disease categories were infectious (17%), primary immunodeficiency (16%), aspiration (10%), and ciliary dyskinesia (9%).[20] Li et al. studied 136 non-CF children with bronchiectasis, and they found that immunodeficiency, aspiration, and primary ciliary dyskinesia were responsible 67% of the cases.[21] Seven children with MLA in this study had primary immunodeficiency which revealed a similar prevalence.[22] Literature reviews depicted that children with primary immunodeficiency usually are manifested as bronchitis, pneumonia, interstitial lung disease, chronic inflammatory airways, or atelectasis.[22] The causes of bronchiectasis in our studied group are presented in [Figure 2].

Chronic lung aspiration had been an important cause of progressive lung disease, bronchiectasis, and respiratory failure.[23] Five patients in this study were due to pulmonary aspiration. For patients with esophagus atresia (EA), despite a surgical repair, they often are associated with esophageal dysmotility, residual esophageal stenosis, gastroesophageal reflux, or pulmonary aspiration.[24] Porcaro et al. studied 105 children with EA and TEF, 29 patients who had undergone chest computed tomography scans, 41% showed localized atelectasis, and 31% revealed bronchiectasis.[25],[26] In contrast, Mirra et al. did not find GERD increased the risk of recurrent pneumonia.[27] Nonetheless, early detection and management of aspiration in this group of patients should be crucial to prevent long-term complications of atelectasis and bronchiectasis.

In the past decade, increasing numbers of articles highlighted the importance of persistent bacterial infection in cohorts of children with refractory cough and wheeze.[28],[29],[30] Lower airway infections with non-typeable H. influenzae cause localized damage and disruption of cilia, recurrent protracted bacterial bronchitis (PBB) predisposed to future bronchiectasis in children.[30],[31],[32],[33],[34],[35] In our study, we saw a high percentage of patients with bronchiectasis, which may be due to delayed diagnosis of PBB, progressing to CSLD, and finally, resulting in the irreversible bronchiectasis. Increasing evidence supports the intensive treatment of CSLD that prevents poor lung function in recent years.[36],[37] Therefore, more aggressive and prolonged antibiotics' use should be encouraged to decrease bronchiectasis in patients with PBB or CSLD.

The outcome of MLA depends on the presence of coexisting bronchiectasis. As shown in [Figure 1], among the nine patients who had pulmonary re-expansion of the right middle lobe, only one patient had bronchiectasis, whereas in 21 patients with nonre-expanded middle lobe collapse, 17 patients had concomitant bronchiectasis. Treatments have been conservative and are directed to the cause of middle lobe collapse. Three patients in this study received lobectomy at other pulmonary segments instead of the middle lobe. In another study of 17 children with MLS, about one-third had persistent respiratory symptoms, mostly mild obstructive airway disease.[38] In a complicated situation such as bronchiectasis associated with persistent atelectasis over 6 months, aggressive medical treatment may require surgical lobectomy.[39],[40]

  Conclusions Top

Atelectasis of the right middle lobe in ambulatory children we studied was commonly associated with diseases in other lobes. Tumors, tuberculosis, retained foreign body, and asthma were all uncommon. When faced with evidence of right MLA, one should consider a more general process such as a chronic endobronchial bacterial infection, PBB, and CSLD with or without bronchiectasis. More aggressive and prolonged courses of antibiotics for PBB and CSLD may obviate the progression to bronchiectasis in such instances.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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  [Figure 1], [Figure 2]

  [Table 1]

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