A 51-year-old man known to be affected by Mounier-Kuhn syndrome (MKS). presented to Accident & Emergecy (A&E) with fever, dyspnea and deterioration of his chronic coughs. Increased diameters of his trachea (39 mm), right (30 mm) and left (26 mm) main bronchi were revealed by chest computerized tomography (CT) scan. CT scan showed also ground-glass opacities (GGO) and bronchiectasis in the mid and lower zones of both lungs. COIVD-19 infection was eventually confirmed by RT-PCR. A severe form of COVID-19 could occur even in the early stages of the disease in presence of underlying co-morbidities including MKS, which increases the susceptibility to more recurrent and severe respiratory infections. Â© 2020 Elsevier Inc

Cegolon, L.

Dehghanpoor, F.

Einollahi, B.

Izadi, M.

Jafari, R.

Javanbakht, M.

Otoukesh, B.

Saadat, S.H.

eprints Iran University of Medical Sciences

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Heart & Lung 49 (2020) 855857Contents lists available at ScienceDirectHeart & Lungjournal homepage: www.heartandlung.comEarly manifestation of ARDS in COVID-19 infection in a 51- year-old manaffected by Mounier-Kuhn syndromeRamezan Jafaria, Luca Cegolonb,c, Fatemeh Dehghanpoora, Mohammad Javanbakhtd,*,Morteza Izadie, Seyed Hassan Saadatd,f, Babak Otoukeshg,h, Behzad EinollahidaDepartment of Radiology, Baqiyatallah University of Medical Sciences, Tehran, Iran and Health Research Center, Baqiyatallah University of Medical Sciences,Tehran, Iranb Local Health Unit N. 2 "Marca Trevigiana" Public Health Department, Treviso, Italyc IRCCS "Burlo Garofolo", Institute for Maternal & Child Health, Trieste, Italyd Nephrology and Urology Research Center, Baqiyatallah University of Medical Sciences, Tehran, IraneHealth Research Center, Baqiyatallah University of Medical Sciences, Tehran, Iranf Behavioral Science Research Center, Lifestyle Institute, Baqiyatallah University of Medical Sciences, Tehran, Irang Bone and Joint Reconstruction Research Center, Iran University of Medical Sciences, Tehran, Iranh Orthopedic Surgery Fellowship in Departement Hospitalo-Universitaire MAMUTH «Maladies Musculo-Squelettiques et Innovations Therapeutiques », Faculte demedecine Pierre et Marie Curie, Sorbonne Universite, Paris, FranceA R T I C L E I N F OArticle History:Received 7 April 2020Revised 14 April 2020Accepted 12 May 2020Available online 16 May 2020* Corresponding author.E-mail address:mhmjvbt81@yahoo.com (M. Javanbahttps://doi.org/10.1016/j.hrtlng.2020.05.0050147-9563/© 2020 Elsevier Inc. All rights reserved.A B S T R A C TA 51-year-old man known to be affected by Mounier-Kuhn syndrome (MKS). presented to Accident & Emerg-ecy (A&E) with fever, dyspnea and deterioration of his chronic coughs. Increased diameters of his trachea (39mm), right (30 mm) and left (26 mm) main bronchi were revealed by chest computerized tomography (CT)scan. CT scan showed also ground-glass opacities (GGO) and bronchiectasis in the mid and lower zones ofboth lungs. COIVD-19 infection was eventually confirmed by RT-PCR. A severe form of COVID-19 could occureven in the early stages of the disease in presence of underlying co-morbidities including MKS, whichincreases the susceptibility to more recurrent and severe respiratory infections.© 2020 Elsevier Inc. All rights reserved.Keywords:COIVD-19Mounier-Kuhn syndromeComputed tomographyRespiratory tract infectionsARDSkht).IntroductionCOVID-19 as an emerging infectious disease of the human respira-tory system, with a broad clinical pattern ranging from asymptomaticto a critical life threatening condition.13 A severe form of COVID-19can develop quickly in a minority of patients, with the onset of acuterespiratory distress syndrome (ARDS), septic shock, pleural effusionand other organ dysfunction/failure.2,46MounierKuhn Syndrome (MKS, a form of tracheo-broncho-megaly), is a clinical idiopatic yet extremely rare disorder, featured byabnormal dilatation of the trachea and main bronchi due to atrophy orlack of elastic fibers and smooth muscle cells. Since it is a significant riskfactor for recurrent lower respiratory infections, MKS needs to bedetected as early as possible to prevent potential complications.710We report a patient affected by MKS admitted to Baqiyatallah hos-pital in Tehran (Iran) for COVID-19, who subsequenty developed aform of the disease with acute respiratory distress syndrome (ARDS).Case presentationA 51-year-old man, known to be affected by MKS was admitted toour accident & emergency (A&E) service of the Baqiyatallah hospitalin Tehran (Iran), with a new onset of fever, dyspnea and deteriorationof his chronic coughs. On physical examination the patient was ill,febrile, tachycardic, hypoxic with O2 saturation of about 80% and dif-fuse rhonchi at chest auscultation. The most significant laboratoryfinding was a notable leukopenia.A chest CT scan revealed increased diameter of his trachea(39mm), right (30mm) and left (26mm) main bronchi, due to MKS.Furthermore, the CT scan showed bilateral ground-glass opacities(GGO) and bronchiectasis in the mid and lower zones of both lungs,with small air cysts in the lower zone of the right lung at coronalbronchiectasis in the mid and lower zones of both lungs, with smallair cysts in the lower zone of the right lung at coronal (Fig 1) and axial(Fig 2) imaging’s, compatible with an early stage ARDS.In a few hours the patient became cyanotic, with altered mentalstatus, shortage of breath and drop in O2 saturation to about 45%. Incompliance with Iran’s treatment protocols, the patient was promptlyFig. 1. Coronal reconstructed ct scan shows trachea-bronchomegaly (Mounier-Kuhnsyndrome) . The diameter of trachea measures 39mm, the right main bronchus 30mmand the left main bronchus 26mm. Ground-glass opacities (GGO) and bronchiectasisin the mid and lower zone of both lungs, with small air cysts in the lower zone of theright lung due to early stage ARDS in COVID-19 pneumonia can be noted.856 R. Jafari et al. / Heart & Lung 49 (2020) 855857intubated and transferred to the intensive care unit (ICU) for furtherdiagnostic and therapeutic procedures, his clinical and radiologicalpattern being compatible with ARDS. He was placed under O2 ther-apy mechanical ventilation, with a tracheostomy also performed. Thepatient health conditions progressively improved over time and afterabout two weeks he was weaned and discharged from hospital.Although the patient conditions was unusual for an early stage ofthe disease, COVID-19 was still our first differential diagnosis, givenwe were in the middle of the COIVD-19 epidemic. COIVD-19 infectionwas eventually confirmed by real time reversed polymerase chainFig. 2. Four axial CT scan show illdefined bilateral ground-glass opacities (GGO) and bronlung lobe, suggestice of an early stage ARDS due to COVID-19 pneumonia subsequently confireaction (RT-PCR). A severe form of COVID-19 could in fact occureven in the early stages of the disease in presence of underlying co-morbidities, including MKS which increases the susceptibility tomore recurrent and severe respiratory infections.DiscussionMKS is a are congenital disorder more common in men and mani-festing with tracheo-bronchial dilation. Although the exact etiologyof MKS is unknown, familial susceptibility and genetic predispositionseem to play a role. MKS is supposedly sustained by lack of musculo-elastic tissue at different levels of the trachea and main bronchi, caus-ing diverticular structures and sacculationar outpouchings especiallyin the posterior membranous trachea.7,912The clinical presentations of MKS are not specific and include awide spectrum of clinical features ranging from asymptomatic orminimal disease to progressive manifestations leading to respiratoryfailure.13 Common presentations are chronic productive cough, dys-pnea and recurrent respiratory infections due to of mucociliary dys-function and accumulation of secretions.11,14,15MKS’ diagnosis relies on detection of enlarged diameter of the tra-chea, right and left main bronchi by CT scan.1013 In the presentedcase the diameter of trachea, right and left bronchi were 39mm,30mm and 26mm, respectively. However, considerable variations inthe enlargement of the lower respiratory tract tree have been docu-mented. For instance, Woodring reported the diameter of right andleft main bronchi being larger than 21mm and 18mm respectively inmen affected by MKS.16 Similar radiological features were confirmedby Nobrega and Menon, with the diameter of trachea, right and leftbrochi being larger than 30mm, 24mm and 23mm, respectively.17,18A remarkable trachea larger than 40mm was reported by Bastos in a33 year old man with MKS.19 Likewise, Abdelghani described a 53 oldman affected by MKS with a diameter of his trachea, right and leftbronchi being of 50 mm, 38 mm and 34 mm respectively.10The above variability in the anatomical presentations of MKS cantranslate into different risk of acquiring respiratory tract infectionsand should be taken into account when managing COVID-19 patients,as they may influence the course of the disease. COVID-19 canchial dilatation within areas of GGO bilaterally and a small air cyst in the right middlermed by PCR test.R. Jafari et al. / Heart & Lung 49 (2020) 855857 857present with mild, moderate and severe symptoms. Whilst the vastmajority of patients develop a mild form of the disease, underlyingco-morbidities such as diabetes, hypertension, cardiovascular disor-ders and malignancies can significantly increase the risk of develop-ing a severe form of COVID-19, especially among patients olderthan 50.20,21ConclusionAll possible underlying co-morbidities, even rare conditions asMKS, should be therefore carefully considered when managing aCOIVD-19 patient, because they can significantly influence the courseof the disease.Authors ContributionsRJ, LC, FD, MJ, MI, SHS,BK,and BE participated in editing through-out the writing process of case report, assessed the images of radiol-ogy and prepared the figures, design and draft of the case report. Allauthors have read and approved the final manuscript.AcknowledgementsThanks to guidance and advice from "Clinical Research Develop-ment Unit of Baqiyatallah Hospital".FundingNoneAvailability of supporting dataAll data are available in the manuscript.Ethics approval and consent to participateThis case report has been described in accordance with the ethicalstandards laid down in the “Declaration of Helsinki 1964”.Consent for publicationWritten consent for publication was obtained from the patient.Declaration of Competing InterestThe authors declare that they have no competing interests.References1. WHO-China Joint Mission. Report of the WHO-China Joint Mission on CoronavirusDisease 2019 (COVID-19). 2020. https://www.who.int/docs/default-source/corona-viruse/who-china-joint-mission-on-covid-19-final-report.pdf. (accessed March 1,2020).2. Lippi G, Plebani M, Henry BM. Thrombocytopenia is associated with severe corona-virus disease 2019 (COVID-19) infections: a meta-analysis. Clinica Chimica Acta.2020;506:145–148.3. Wu F, Zhao S, Yu B, Chen YM, Wang W, Song ZG, et al. A new coronavirus associ-ated with human respiratory disease in China. Nature. 2020;579(7798):265–269.4. Li H, Liu SHM, Yu XH, Tang SL, Tang CHK. Coronavirus disease 2019 (COVID-19): current status and future perspective. Int J Antimicrob Age. 2020 105951..Available online 29 March; https://www.sciencedirect.com/science/article/pii/S0924857920301011.5. Mattiuzzi C, Lippi G. Which lessons shall we learn from the 2019 novel coronavirusoutbreak? Ann Transl Med. 2020;8. accessed March 8, 2020; http://atm.amegroups.com/post/view/which-lessons-shall-we-learn-from-the-2019-novel-coronavirus-outbreak.6. Huang C, Wang Y, Li X, et al. Clinical features of patients infected with 2019 novelcoronavirus in Wuhan, China. Lancet. 2020;395:497–506.7. Lazzarini-de-Oliveira LC, Costa de Barros Franco CA, Gomes de Salles CL. A 38-year-old man with tracheomegaly, tracheal diverticulosis, and bronchiectasis. Chest.2001;120:1018.8. Ghanei M, Peyman MR, Aslani J, Zamel N. Mounier-Kuhn syndrome: a rare cause ofsevere bronchial dilatation with normal pulmonary function test: a case report.Respiratory Med. 2007;101(8):1836–1839.9. Shah SS, Karnak D, Mason D, et al. Pulmonary transplantation in Mounier-Kuhnsyndrome: a case report. J Thorac Cardiovasc Surg. 2006;131(3):757–758.10. Abdelghani A, Bouazra H, Hayouni A, et al. MounierKuhn syndrome: a rare causeof bronchial dilatation: a case report. Respiratory Med CME. 2009;2(4):164–166.11. Gupta P, Gorsi U, Bhalla A, Khandelwal N. Mounier-Kuhn syndrome masqueradingpulmonary thromboembolism in an elderly male. Lung India. 2014;31:76–78.12. Sivanmani K. A case report of Mounier-Kuhn syndrome. J Assoc Chest Physicians.2017;5:39–41.13. Mesiha N., Mogia A., MONA Alipour M., et al. Mounier-Kuhn syndrome: a rarecause of a common disease. Chest154(4):581A582A.14. Fletcher A, Stowell J, amoulis S. 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Early manifestation of ARDS in COVID-19 infection in a 51- year-old man affected by Mounier-Kuhn syndrome

http://eprints.iums.ac.ir/33802/1/Early%20manifestation%20of%20ARDS%20in%20COVID-19%20infection%20in%20a%2051-%20year-old%20man%20affected%20by%20Mounier-Kuhn%20syndrome.pdf

Early manifestation of ARDS in COVID-19 infection in a 51- year-old man affected by Mounier-Kuhn syndrome

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