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Year : 2021  |  Volume : 64  |  Issue : 1  |  Page : 202-203
Congenital pulmonary airway malformation with coexistent cytomegalovirus infection in an infant: An unusual presentation

Department of Pathology and Paedssurgery, Maulana Azad Medical College and CNBC, New Delhi, India

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Date of Submission30-May-2019
Date of Decision19-Oct-2019
Date of Acceptance27-Feb-2020
Date of Web Publication8-Jan-2021

How to cite this article:
Khuraijam B, Udayakumar D S, Mandal S, Khurana N, Manchana V. Congenital pulmonary airway malformation with coexistent cytomegalovirus infection in an infant: An unusual presentation. Indian J Pathol Microbiol 2021;64:202-3

How to cite this URL:
Khuraijam B, Udayakumar D S, Mandal S, Khurana N, Manchana V. Congenital pulmonary airway malformation with coexistent cytomegalovirus infection in an infant: An unusual presentation. Indian J Pathol Microbiol [serial online] 2021 [cited 2022 Aug 16];64:202-3. Available from: https://www.ijpmonline.org/text.asp?2021/64/1/202/306516

Cytomegalovirus (CMV) is a group of herpes virus that can produce a variety of disease manifestation depending on the age of the host and most importantly on the immune status of the host.[1] Extensive search of the literature has shown no case with coexistent CPAM in an immunocompetent patient. We report the first case of CMV with coexistent CPAM in an immunocompetent patient.

A 7-month-old male child presented to the pediatric OPD with history of high-grade fever with respiratory distress for 15 days. The antenatal check-up of the mother was uneventful. The mother had no history of immunosuppression. The baby was born full term and by normal vaginal delivery. The baby failed to cry immediately after birth and APGAR score was low.

On examination, there were decreased breath sounds on the left side. Hemoglobin was low and PO4 was high. Chest X-Ray showed left-sided pleural effusion with pyothorax. CECT showed a multicystic lesion in the left lower lobe of the lung and was suggestive of C-PAM [Figure 1]a. The patient was operated and intraoperatively, the left lobe was found to be cystic.
Figure 1: (a) CECT shows a multicystic lesion in the left lower lobe. (b) Grossly many cysts ranging in size from 0.6 to 1.5 cm were identified in the left lower lobe. (c) Sections from the cysts showed a cyst lined by flattened epithelium with the surrounding lung parenchyma showing hemorrhage and chronic inflammatory infiltrate. (d) Section from the cyst showing CMV inclusion (arrow). (e) Inset: cytomegalic cell

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A left lobe of the lung was received, which measured 4 × 4 × 3.5 cm; on cut section, multiple cysts ranging in size from 0.1 to 0.6 cm in diameter was noted [Figure 1]b. lymphohistiocytic sections examined from the cyst's wall showed fibrocollagenous cyst wall lined by flattened epithelium. Also seen within the cyst epithelium were large cells with prominent intranuclear inclusions and clear halo indicative of CMV inclusion [Figure 1]c,[Figure 1]d,[Figure 1]e. The rest of the lung showed alveolar congestion, mild interstitial fibrosis with focal areas showing the presence of sheets of histiocytes [Figure 2]a. There was mild focal chronic inflammatory infiltrate around the bronchiole with the presence of focal lymphoid aggregates within the interstitium. CMV inclusion was also seen within the epithelium of alveoli, blood vessels, and bronchiolar epithelium [Figure 2]b and [Figure 2]c. A final diagnosis of CPAM type IV with CMV was made.
Figure 2: (a) Dense lymphohistiocytic cells in the surrounding lung parenchyma [HE × 100]. (b) Section from the terminal bronchiole showing cytomegalic cells within it [HE × 200]. (c) Cells showing characteristic owl-eye inclusions. [HE × 400]

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CMV causes an asymptomatic or mononucleosis-like infection in healthy individuals but devastating systemic infections in neonates and in immunocompromised people. Transmission can be transplacental, neonatal, through saliva, genital route, or iatrogenically.[1] It is usually asymptomatic.

Among neonates, pneumonitis is a recognized manifestation of CMV infection.[2] Children usually have afebrile pneumonitis with tachypnoea and hyperinflation.[2] The most common clinical manifestation of CMV infection in immunocompetent hosts beyond the neonatal period is an infectious mononucleosis-like illness. The infected infants may rarely develop interstitial pneumonitis, failure to thrive, rash, or hepatitis.[1] Many recover without any sequelae. The diagnosis is usually made by serology.[1] Immunocompromised individuals are susceptible to severe CMV infection. It can be primary infections or reactivation of a latent CMV. The present case, the child, was immunocompetent.

The cytopathic effect of CMV produces enlarged cells with intranuclear and cytoplasmic inclusions, which may give the cells an “owl's eye” appearance. Infected lung sections show a multifocal distribution in the alveolar walls, alveolar spaces, and interstitium.[3]

Congenital adenomatoid malformation (CAM) was originally described by Chin and Tang[4] as rare lesions occurring in premature or stillborn infants. Stocker proposed an expanded classification of CPAM based on clinical, radiological, and pathological features and renamed it as congenital pulmonary airway malformation (CPAM).[5] CPAM are common hamartomatous lesions and separated into five types (types 0, 1, 2, 3, and 4). Histologically there are multiple, irregular, and variably sized acinar structures which can be cystic or solid lined by cuboidal, ciliated pseudostratified columnar or alveolar epithelium. CMV infections are treated with antiviral ganciclovir and is instituted in patients with serious end-organ diseases like CNS, liver, and lungs involvement. In this patient also ganciclovir was administered.

Extensive literature search showed two reports of cystic or emphysematous lung disease with CMV infection. Bradshaw and Moore described postnatally acquired CMV infection in an infant.[6] The baby developed on the 19th day multicystic lung disease in the left upper lobe and right perihilar region. He was not given any antiviral therapy. The infant showed gradual clinical improvement with substantial resolution of the cystic changes. Carrol et al. reported a case of congenital lobar emphysema in an antenatally diagnosed patient. The excised specimen showed features of congenital lobar emphysema along with mild interstitial inflammation and CMV inclusion bodies.[7] We hereby report the first case of CMV with CPAM in an immunocompetent patient. Extensive search has failed to show any reported case.

This case is being presented for its rarity and to make the pathologist and the clinician aware of such a coexistence so that they are not missed. Timely medical and surgical management should be done in these young patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Alexander JM, Danny AM, Arlene HS. Infectious disease. In: Kumar V, Abul KA, Jon CA, editors. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Philadelphia: Elsevier; 2015. p. 359-60.  Back to cited text no. 1
Whitley RJ, Brasfield D, Reynolds DW, Stagno S, Tiller RE, Alford CA, et al. Protracted pneumonitis in young infants associated with perinatally acquired cytomegaloviral infection. J Pediatr 1976;89:16-22.  Back to cited text no. 2
Sinzger C, Grefte A, Plachter B, Gouw AS, The TH, Jahn G, et al. Fibroblasts, epithelial cells, endothelial cells and smooth muscle cells are major targets of human cytomegalovirus infection in lung and gastrointestinal tissues. J Gen Virol 1995;76:741-50.  Back to cited text no. 3
Chin KY, Tang MY. Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Pathol (Chic) 1949;48:221-9.  Back to cited text no. 4
Stocker JT. Congenital pulmonary airway malformation- a new name for and an expanded classification of con- genital cystic adenomatoid malformation of the lung. Histopathology 2002;41(Suppl 2):424-30.  Back to cited text no. 5
Bradshaw JH, Moore PP. Perinatal cytomegalovirus infection associated with lung cysts. J Paediatr Child Health 2003;39:5636.  Back to cited text no. 6
Carrol ED, Campbell ME, Shaw BN, Pilling DW. Congenital lobar emphysema in congenital cytomegalovirus infection. Pediatr Radiol 1996;26:900-2.  Back to cited text no. 7

Correspondence Address:
Shramana Mandal
Assistant Professor, Department of Pathology, 642. Pocket E, Mayur Vihar Phase II, Delhi - 110 091
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_425_19

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