Generic placeholder image

Current Respiratory Medicine Reviews

Editor-in-Chief

ISSN (Print): 1573-398X
ISSN (Online): 1875-6387

Case Report

Middle Lobe Syndrome in Children: Three Case Reports and a Brief Review

Author(s): Mattia Spatuzzo, Enrica Mancino, Domenico Paolo La Regina, Luigi Matera, Fabrizio Virgili, Sara Ialongo, Elio Iovine, Marra Simona, Raffaella Nenna and Fabio Midulla*

Volume 21, Issue 3, 2025

Published on: 26 August, 2024

Page: [287 - 295] Pages: 9

DOI: 10.2174/011573398X308181240813060833

Price: $65

Abstract

Background: Middle Lobe Syndrome (MLS) is a pediatric respiratory condition characterized by the collapse of the middle lobe of the right lung or the lingula of the left lung. Historically linked with tuberculosis, contemporary cases primarily involve asthma and other causes, like foreign body aspiration or mucous plug.

Case Presentation: We have, herein, analyzed the clinical features and management of three cases of MLS from a single pediatric center. Additionally, we have reviewed 75 articles on pediatric MLS by using common databases, such as PubMed and Medline.

Discussion: MLS often poses a diagnostic challenge, presenting with symptoms, like cough, wheezing, and localized rales. Radiological exams, especially chest X-rays and flexible bronchoscopy, are needed for confirming the diagnosis, particulary in challenging cases. Both obstructive and non-obstructive factors contribute to MLS.

Conclusion: A comprehensive understanding of MLS, its diverse causes, and the significance of early diagnosis is crucial for effective management. Treatment should target the underlying cause. Prompt intervention may offer a positive outcome and help prevent complications, like bronchiectasis.

Keywords: Middle Lobe Syndrome, respiratory diseases, atelectasis, asthma, tuberculosis, foreign body aspiration.

Graphical Abstract
[1]
Graham EA, Burford TH, Mayer JH. Middle lobe syndrome. Postgrad Med 1948; 4(1): 29-34.
[http://dx.doi.org/10.1080/00325481.1948.11693655] [PMID: 18874043]
[2]
Nocerino G. Middle lobe syndrome in children. Medico Bambino 2005; 24: 37-24.
[3]
Brock R. - Tuberculous mediastinal lymphadenitis in chilhood. - Secundary effects of the lungs. Guys Hosp Rep 1937; 87: 295.
[4]
Feng Y, Yu H, Liu X, Chen N, Shang Y, Zhang H. Asthma with recurrent middle lobe syndrome in children: Clinical features and lung function patterns. Front Pediatr 2023; 11: 1113652.
[http://dx.doi.org/10.3389/fped.2023.1113652] [PMID: 36814593]
[5]
Peroni DG, Boner AL. Atelectasis: Mechanisms, diagnosis and management. Paediatr Respir Rev 2000; 1(3): 274-8.
[http://dx.doi.org/10.1053/prrv.2000.0059] [PMID: 12531090]
[6]
Livingston GL, Holinger LD, Luck SR. Right middle lobe syndrome in children. Int J Pediatr Otorhinolaryngol 1987; 13(1): 11-23.
[http://dx.doi.org/10.1016/0165-5876(87)90003-6] [PMID: 3623806]
[7]
Romagnoli V, Priftis KN, de Benedictis FM. Middle lobe syndrome in children today. Paediatr Respir Rev 2014; 15(2): 188-93.
[PMID: 24630779]
[8]
Shaikh U, Heller D. Right Middle Lobe Syndrome. Treasure Island, FL: StatPearls 2022.
[9]
Germann EJ, Metzger H, Stöckle G. Unrecognized foreign body aspiration as the cause of middle lobe syndrome. Rofo 1984; 141(6): 711-3.
[10]
Dees SC, Spock A. Right middle lobe syndrome in children. JAMA 1966; 197(1): 8-14.
[http://dx.doi.org/10.1001/jama.1966.03110010060017] [PMID: 5952545]
[11]
Hoving MFP, Brand PLP. Causes of recurrent pneumonia in children in a general hospital. J Paediatr Child Health 2013; 49(3): E208-12.
[http://dx.doi.org/10.1111/jpc.12114] [PMID: 23438187]
[12]
Ng CSH, Lau RWH, Lau KKW, Underwood MJ, Yim APC. Defeating the pores of Kohn. Asian Cardiovasc Thorac Ann 2014; 22(1): 102-4.
[http://dx.doi.org/10.1177/0218492312474454] [PMID: 24585656]
[13]
Gudbjartsson T, Gudmundsson G. Middle lobe syndrome: A review of clinicopathological features, diagnosis and treatment. Respiration 2012; 84(1): 80-6.
[http://dx.doi.org/10.1159/000336238] [PMID: 22377566]
[14]
Arab A, Voduc N, Moyana T, Mulpuru S. Middle lobe syndrome due to calcified adenopathy. Can Respir J 2014; 21(4): 211-2.
[http://dx.doi.org/10.1155/2014/789542] [PMID: 24712018]
[15]
Raman TS, Mathew S, Ravikumar , Garcha PS. Atelectasis in children. Indian Pediatr 1998; 35(5): 429-35.
[PMID: 10216624]
[16]
Varela P, Pio L, Torre M. Primary tracheobronchial tumors in children. Semin Pediatr Surg 2016; 25(3): 150-5.
[http://dx.doi.org/10.1053/j.sempedsurg.2016.02.013] [PMID: 27301601]
[17]
Remetz MS, Cleman MW, Cabin HS. Pulmonary and pleural complications of cardiac disease. Clin Chest Med 1989; 10(4): 545-92.
[http://dx.doi.org/10.1016/S0272-5231(21)00655-9] [PMID: 2689066]
[18]
M Redondo M, Carpio Segura C, Zamarrón De Lucas E, Álvarez-Sala Walther R, P Sánchez C. Should atelectasis be considered a pulmonary complication and indicator of poor prognosis in cystic fibrosis? J Int Med Res 2024; 52(3): 03000605241233520.
[http://dx.doi.org/10.1177/03000605241233520] [PMID: 38546237]
[19]
Freidkin L, Heching M, Rosengarten D, et al. Bronchoscopy for management and identification of etiology of right middle lobe syndrome: Analysis of 66 cases. Thorac Cancer 2023; 14(32): 3226-31.
[http://dx.doi.org/10.1111/1759-7714.15113] [PMID: 37704575]
[20]
Fabri L, Shanthikumar S, Tadd K, Morgan L, Schultz A, Robinson P. Fissure adjacent partial lobe atelectasis in primary ciliary dyskinesia. J Paediatr Child Health 2022; 58(4): 683-6.
[http://dx.doi.org/10.1111/jpc.15818] [PMID: 34786797]
[21]
Tanahashi M. Tracheobronchial foreign body. Kyobu Geka 2022; 75(10): 851-8.
[PMID: 36155582]
[22]
Wagner RB, Johnston MR. Middle lobe syndrome. Ann Thorac Surg 1983; 35(6): 679-86.
[http://dx.doi.org/10.1016/S0003-4975(10)61085-5] [PMID: 6344820]
[23]
O’Donnell AE, Tsou E, Awh C, Fallat ME, Patterson K. Endobronchial eosinophilic granuloma: A rare cause of total lung atelectasis. Am Rev Respir Dis 1987; 136(6): 1478-80.
[http://dx.doi.org/10.1164/ajrccm/136.6.1478] [PMID: 3688651]
[24]
Kunal S, Jain S, Shah A. Middle lobe syndrome: An exceptional presentation of concomitant lepidic adenocarcinoma and bronchial anthracofibrosis. Monaldi Arch Chest Dis 2017; 87(3): 864.
[http://dx.doi.org/10.4081/monaldi.2017.864] [PMID: 29424200]
[25]
Maranini B, Guzzinati I, Casoni GL, Ballotta M, Lo Monaco A, Govoni M. Case Report: Middle lobe syndrome: A rare presentation in eosinophilic granulomatosis with polyangiitis. Front Immunol 2023; 14: 1222431.
[http://dx.doi.org/10.3389/fimmu.2023.1222431] [PMID: 37638004]
[26]
Abia-Trujillo D, Majid A, Johnson MM, et al. Central airway collapse, an underappreciated cause of respiratory morbidity. Mayo Clin Proc 2020; 95(12): 2747-54.
[http://dx.doi.org/10.1016/j.mayocp.2020.03.004] [PMID: 32829904]
[27]
Wang K, Li M, Wang X, et al. Imaging origins and characteristics analysis of acute and chronic aspiration pneumonia. Zhonghua Yi Xue Za Zhi 2014; 94(41): 3244-7.
[PMID: 25604226]
[28]
Rashid A, Nanjappa S, Greene JN. Infectious causes of right middle lobe syndrome. Cancer Contr 2017; 24(1): 60-5.
[http://dx.doi.org/10.1177/107327481702400110] [PMID: 28178715]
[29]
Gera K, Panjabi C, Shah A. Childhood allergic bronchopulmonary aspergillosis presenting as a middle lobe syndrome. Asia Pac Allergy 2016; 6(1): 67-9.
[http://dx.doi.org/10.5415/apallergy.2016.6.1.67] [PMID: 26844222]
[30]
Garg T, Gera K, Shah A. Middle lobe syndrome: An extraordinary presentation of endobronchial tuberculosis. Pneumonol Alergol Pol 2015; 83(5): 387-91.
[PMID: 26379001]
[31]
Kunal S, Dhawan S, Kumar A, Shah A. Middle lobe syndrome: An intriguing presentation of tracheobronchial amyloidosis. BMJ Case Rep 2017; 2017: 219480.
[http://dx.doi.org/10.1136/bcr-2017-219480] [PMID: 28536221]
[32]
Wohl Gt, Israel Hl, Frobese As. The middle lobe syndrome and histoplasmosis. J Albert Einstein Med Cent 2017; 1(1): 17-23.
[33]
Anderson JJ, Fong KM, Godbolt DB, Leong SC, Marshall HM. Endobronchial silicosis and tuberculosis presenting as the right middle lobe syndrome. J Bronchology Interv Pulmonol 2016; 23(4): e35-7.
[http://dx.doi.org/10.1097/LBR.0000000000000302] [PMID: 27764010]
[34]
Dixit R, Jalutharia J, Goyal M, Chaudhary P, Kasliwal N. Middle lobe syndrome in silicosis. Indian J Occup Environ Med 2021; 25(3): 182-4.
[http://dx.doi.org/10.4103/ijoem.ijoem_26_21] [PMID: 34759607]
[35]
Arkless HA, Chodoff RJ. Middle lobe syndrome due to sarcoidosis. Dis Chest 1956; 30(3): 351-3.
[http://dx.doi.org/10.1378/chest.30.3.351] [PMID: 13356739]
[36]
Abid N, Loukil M, Gader N, Chaabane A, Bouzaidi K, Ghrairi H. Middle lobe syndrome revealing Broncholithiasis: A Case Report. Tunis Med 2023; 101(4): 460-2.
[PMID: 38372529]
[37]
Me A. Surface properties in relation to atelectasis and hyaline membrane disease. AMA J Dis Child 1959; 97(5): 517-23.
[38]
Ho BJ, Crowe JE, Dorfman SR, Camp EA, Yusuf S, Shenoi RP. Correlation of clinical and chest radiograph findings in pediatric submersion cases. Pediatr Radiol 2020; 50(4): 492-500.
[http://dx.doi.org/10.1007/s00247-019-04588-x] [PMID: 31897567]
[39]
Formenti P, Umbrello M. Pleural effusion in ARDS. Minerva Anestesiol 2014; 80(2): 245-53.
[PMID: 24002464]
[40]
Koumbourlis AC. Scoliosis and the respiratory system. Paediatr Respir Rev 2006; 7(2): 152-60.
[http://dx.doi.org/10.1016/j.prrv.2006.04.009] [PMID: 16765303]
[41]
Chacko A, Marshall J, Taylor O, McEniery J, Sly PD, Gauld LM. Dysphagia and lung disease in children with spinal muscular atrophy treated with disease-modifying agents. Neurology 2023; 100(19): 914-20.
[http://dx.doi.org/10.1212/WNL.0000000000206826] [PMID: 36657991]
[42]
Birnkrant DJ, Pope JF, Eiben RM. Management of the respiratory complications of neuromuscular diseases in the pediatric intensive care unit. J Child Neurol 1999; 14(3): 139-43.
[http://dx.doi.org/10.1177/088307389901400301] [PMID: 10190262]
[43]
Orlikowski D, Prigent H, Sharshar T, Lofaso F, C Raphael J. Respiratory dysfunction in guillain-barré syndrome. Neurocrit Care 2004; 1(4): 415-22.
[http://dx.doi.org/10.1385/NCC:1:4:415] [PMID: 16174943]
[44]
Chen HA, Lai SL, Kwang WK, Liu JC, Chen CH, Huang DF. Middle lobe syndrome as the pulmonary manifestation of primary Sjögrenˈs syndrome. Med J Aust 2006; 184(6): 294-5.
[http://dx.doi.org/10.5694/j.1326-5377.2006.tb00242.x] [PMID: 16548837]
[45]
Strollo DC, Donnenberg VS, Madan S, Moran PL, Luketich JD, Sanchez MV. Right middle lobe syndrome after upper lobectomy: Role of the bronchial angle. Asian Cardiovasc Thorac Ann 2023; 31(3): 215-20.
[http://dx.doi.org/10.1177/02184923221144402] [PMID: 36514840]
[46]
Gurney JW. A typical manifestations of pulmonary atelectasis. J Thorac Imaging 1996; 11(3): 165-75.
[http://dx.doi.org/10.1097/00005382-199622000-00001] [PMID: 8784730]
[47]
Patterson J, Graham D, George A, Will M, Sutter D. Right middle lobe collapse and pleural effusion in an 18-year-old man. Chest 2017; 152(2): e33-8.
[http://dx.doi.org/10.1016/j.chest.2017.04.187] [PMID: 28797398]
[48]
Mingoia A, Wittebole X, Gérard L. Diagnosis of middle lobe atelectasis with lung ultrasonography. Intensive Care Med 2021; 47(8): 906-7.
[http://dx.doi.org/10.1007/s00134-021-06421-2] [PMID: 33983465]
[49]
Bhoil R, Ahluwalia A, Chopra R, Surya M, Bhoil S. Signs and lines in lung ultrasound. J Ultrason 2021; 21(86): e225-33.
[http://dx.doi.org/10.15557/JoU.2021.0036] [PMID: 34540277]
[50]
Adler AC, von Ungern-Sternberg BS, Matava CT. Lung ultrasound and atelectasis—The devil is in the details. Paediatr Anaesth 2021; 31(12): 1269-70.
[http://dx.doi.org/10.1111/pan.14286] [PMID: 34750922]
[51]
Molina PL, Hiken JN, Glazer HS. Imaging evaluation of obstructive atelectasis. J Thorac Imaging 1996; 11(3): 176-86.
[http://dx.doi.org/10.1097/00005382-199622000-00002] [PMID: 8784731]
[52]
Kuhn JP, Brody AS. High-resolution CT of pediatric lung disease. Radiol Clin North Am 2002; 40(1): 89-110.
[http://dx.doi.org/10.1016/S0033-8389(03)00111-8] [PMID: 11813822]
[53]
Coren ME, Ng V, Rubens M, Rosenthal M, Bush A. The value of ultrafast computed tomography in the investigation of pediatric chest disease. Pediatr Pulmonol 1998; 26(6): 389-95.
[http://dx.doi.org/10.1002/(SICI)1099-0496(199812)26:6<389::AID-PPUL3>3.0.CO;2-6] [PMID: 9888213]
[54]
Fraioli F, Serra G, Ciarlo G, et al. Chest MR imaging in the follow-up of pulmonary alterations in paediatric patients with middle lobe syndrome: Comparison with chest X-ray. Radiol Med (Torino) 2013; 118(3): 444-55.
[http://dx.doi.org/10.1007/s11547-012-0889-3] [PMID: 23090250]
[55]
Hu HC, Yin BY, Hu JZ. Fibrobronchoscopy in the diagnosis of middle lobe syndrome. Zhonghua Jie He He Hu Xi Za Zhi 1993; 16(4): 211-213, 252.
[PMID: 8174189]
[56]
Midulla F, de Blic J, Barbato A, et al. Flexible endoscopy of paediatric airways. Eur Respir J 2003; 22(4): 698-708.
[http://dx.doi.org/10.1183/09031936.02.00113202] [PMID: 14582925]
[57]
Rock MJ. The diagnostic utility of bronchoalveolar lavage in immunocompetent children with unexplained infiltrates on chest radiograph. Pediatrics 1995; 95(3): 373-7.
[58]
Bertelsen S, Struve-Christensen E, Aasted A, Sparup J. Isolated middle lobe atelectasis: Aetiology, pathogenesis, and treatment of the so-called middle lobe syndrome. Thorax 1980; 35(6): 449-52.
[http://dx.doi.org/10.1136/thx.35.6.449] [PMID: 7434301]
[59]
Redding GJ. Bronchiectasis in Children. Pediatr Clin North Am 2009; 56(1): 157-171, xi.
[http://dx.doi.org/10.1016/j.pcl.2008.10.014] [PMID: 19135586]
[60]
de Benedictis FM, Bush A. Corticosteroids in respiratory diseases in children. Am J Respir Crit Care Med 2012; 185(1): 12-23.
[http://dx.doi.org/10.1164/rccm.201107-1174CI] [PMID: 21920920]
[61]
Shhada E, Nahle AA, Hamdar H, et al. Unveiling the complexity of right middle lobe syndrome: A case series highlighting the association with asthma. Ann Med Surg (Lond) 2024; 86(4): 1836-42.
[http://dx.doi.org/10.1097/MS9.0000000000001848] [PMID: 38576953]
[62]
Oberwaldner B. Physiotherapy for airway clearance in paediatrics. Eur Respir J 2000; 15(1): 196-204.
[http://dx.doi.org/10.1183/09031936.00.15119600] [PMID: 10678646]
[63]
Holmgren NL, Córdova M, Ortúzar P, Sánchez I. Role of flexible bronchoscopy in the re-expansion of persistent atelectasis in children. Arch Bronconeumol 2002; 38(8): 367-71.
[http://dx.doi.org/10.1016/S0300-2896(02)75241-1] [PMID: 12199918]
[64]
Bolliger CT, Mathur PN. Interventional bronchoscopy. Basel: Karger 2000.
[65]
Einarsson JT, Einarsson JG, Isaksson H, Gudbjartsson T, Gudmundsson G. Middle lobe syndrome: A nationwide study on clinicopathological features and surgical treatment. Clin Respir J 2009; 3(2): 77-81.
[http://dx.doi.org/10.1111/j.1752-699X.2008.00109.x] [PMID: 20298381]
[66]
Sehitogullari A, Sayir F, Cobanoglu U, Bilici S. Surgical treatment of right middle lobe syndrome in children. Ann Thorac Med 2012; 7(1): 8-11.
[http://dx.doi.org/10.4103/1817-1737.91554] [PMID: 22347343]
[67]
Ayed AK. Resection of the right middle lobe and lingula in children for middle lobe/lingula syndrome. Chest 2004; 125(1): 38-42.
[http://dx.doi.org/10.1378/chest.125.1.38] [PMID: 14718418]
[68]
Soyer O, Ozen C, Cavkaytar O, Senyücel C, Dallar Y. Right middle lobe atelectasis in children with asthma and prognostic factors. Allergol Int 2016; 65(3): 253-8.
[http://dx.doi.org/10.1016/j.alit.2015.12.002] [PMID: 26806056]
[69]
Cowles RA, Lelli JL Jr, Takayasu J, Coran AG. Lung resection in infants and children with pulmonary infections refractory to medical therapy. J Pediatr Surg 2002; 37(4): 643-7.
[http://dx.doi.org/10.1053/jpsu.2002.31629] [PMID: 11912527]
[70]
De Boeck K, Willems T, Van Gysel D, Corbeel L, Eeckels R. Outcome after right middle lobe syndrome. Chest 1995; 108(1): 150-2.
[http://dx.doi.org/10.1378/chest.108.1.150] [PMID: 7606950]
[71]
Munro KA, Reed PW, Joyce H, et al. Do New Zealand children with non-cystic fibrosis bronchiectasis show disease progression? Pediatr Pulmonol 2011; 46(2): 131-8.
[http://dx.doi.org/10.1002/ppul.21331] [PMID: 20717910]
[72]
Insanov AB. Differential diagnosis of tuberculosis and other diseases affecting the middle lobe of the lung.. Romanian 1981; 30(1): 43-8.
[73]
Rua J, Marques R, Silva R, Gomes B, Fortuna J. Non-obstructive middle lobe syndrome: an unusual cause of recurrent pneumonia in an elderly woman. Eur J Case Rep Intern Med 2018; 5(1): 000737.
[PMID: 30755973]
[74]
Franchini F, Pitta D, Pozzessere A, Cocchi C, Casini T. Middle lobe syndrome in children. Critical review. Pediatr Med Chir 1995; 17(2): 135-8.
[PMID: 7610076]
[75]
Gaston B. Pneumonia. Pediatr Rev 2002; 23(4): 132-40.
[http://dx.doi.org/10.1542/pir.23-4-132] [PMID: 11927745]

Rights & Permissions Print Cite
© 2025 Bentham Science Publishers | Privacy Policy