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   Table of Contents      
CASE REPORT
Year : 2022  |  Volume : 8  |  Issue : 2  |  Page : 178-179

Spontaneous Resolution of Type 1 Macrocystic Congenital Cystic Adenomatoid Malformation: A Rare Presentation


Department of Pediatric Surgery, Maulana Azad Medical College (MAMC), New Delhi, India

Date of Submission12-Jul-2021
Date of Decision30-Apr-2022
Date of Acceptance03-May-2022
Date of Web Publication23-Aug-2022

Correspondence Address:
Shasanka Shekhar Panda
Associate Professor, Department of Paediatric Surgery, Maulana Azad Medical College (MAMC), New Delhi-110002
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mamcjms.mamcjms_79_21

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  Abstract 


Abstract We present a case of 6-month-old boy who presented with respiratory distress due to empyema, which was initially managed by the intercostal drain. Computed tomography scan confirmed the diagnosis of type 1 congenital cystic adenomatoid malformation (CCAM). Subsequently, there was the spontaneous resolution of empyema and CCAM prior to surgical intervention. Our case illustrates the postnatal spontaneous resolution of CCAM, which is rare.

Keywords: Congenital cystic adenomatoid malformation, empyema, spontaneous resolution


How to cite this article:
Kumar S, Panda SS, Neogi S, Ratan SK. Spontaneous Resolution of Type 1 Macrocystic Congenital Cystic Adenomatoid Malformation: A Rare Presentation. MAMC J Med Sci 2022;8:178-9

How to cite this URL:
Kumar S, Panda SS, Neogi S, Ratan SK. Spontaneous Resolution of Type 1 Macrocystic Congenital Cystic Adenomatoid Malformation: A Rare Presentation. MAMC J Med Sci [serial online] 2022 [cited 2022 Sep 26];8:178-9. Available from: https://www.mamcjms.in/text.asp?2022/8/2/178/354346




  Introduction Top


Congenital cystic adenomatoid malformation (CCAM) is a developmental anomaly characterized by cystic hamartomatous lesion of the lung, comprising 95% of congenital cystic lung diseases, with an estimated incidence of 1:10000 to 1:35000 live births and with a relatively more common occurrence in males.[1] Antenatal diagnosis can be made in the second trimester by high-resolution ultrasonography.[2] Postnatal radiological diagnosis is done by high-resolution computed tomography, though the confirmatory diagnosis is by histopathological findings.[3]

Types of CCAM are as follows:[4]

Type 0: Acinar atresia

Type 1: Cysts up to 10 cm, lined by pseudostratified ciliated cells, interspersed with mucinous cells

Type 2: Multiple small cysts separated by normal alveoli

Type 3: Solid fetal lung

Type 4: Cysts up to 10 cm, lined by flattened epithelium resting upon loose mesenchymal tissue

The mainstay of treatment is surgical excision (lobectomy)[5] to prevent complications such as recurrent infections, pneumothorax, and malignancies.[6]


  Case Report Top


A 6-month-old male child presented to us with a follow-up case of left-sided empyema, which was initially managed by the intercostal drain. Owing to the nonresolution of empyema in follow-up imaging [Figure 1]a, a computed tomography (CT) chest was done, which revealed large well-defined multiloculated cystic lesions in the left lower lobe (the largest size measured 2.5 × 2.9 cm) [Figure 1]b. There was a 7 mm thick hypodense collection in the left pleural cavity. The patient was planned for thoracotomy and lobectomy but managed conservatively with 6 weeks of antibiotic therapy due to nonconsenting parents. Repeat X-ray chest was normal [Figure 1]c. CT chest was done to confirm the X-ray findings. CT chest showed near-complete resolution of cystic lesion in left lower lobe [Figure 1]d. The decision for surgical intervention was deferred. The patient was kept under follow-up. Now the patient is more than 2 years old and asymptomatic.
Figure 1 (a) Chest X-ray showing unresolved loculated empyema. (b) CT chest (coronal view) showing large well-defined multiloculated cystic lesions in left lower lobe. (c) Normal X-ray chest (resolution of lesion in left lung). (d) CT chest (sagittal view) showing near-complete resolution of cystic lesion in left lower lobe

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  Discussion Top


CCAM arises due to excessive, disorganized proliferation of tubular bronchial structures excluding the alveoli. There is no side predilection and single lobe disease is more commonly seen.[7] The most common mode of presentation in the postnatal period is acute respiratory distress due to cyst expansion that compresses the surrounding structures, or pneumonia, or effusion. The presentation in our case was pleural effusion (empyema). The most common age of presentation is the neonatal period.[8] Presentation beyond the neonatal age group is rare, as was in our case.

Lobectomy is indicated as the main mode of therapy that allows expansion of the normal lung and prevents complications like recurrent infections and malignancies such as bronchioloalveolar carcinoma, adenocarcinoma in aged, and pleuropulmonary blastoma.[9] Early treatment is indicated to prevent consequences of recurrent infections such as chest pain, failure to thrive, pneumothorax, and empyema. Cases complicated by pneumonia are treated by antibiotics and oxygen supplementation and sometimes mechanical ventilation. Spontaneous resolution of CCAM is rare, seen in only 4% of cases.[10]

In our case, the age of presentation was 6 months, and the child responded to nonsurgical treatment and the resolution was confirmed by X-ray and CT chest.


  Conclusion Top


We conclude that CCAM should be kept as a differential diagnosis in cases of recurrent lung infections and nonresolving empyema/pneumothorax. Ideally, surgical intervention should be done; however, spontaneous resolution is a possibility and should be considered in cases where the nonsurgical path is followed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Di Prima FA, Bellia A, Inclimona G, Grasso F, Teresa M, Cassaro MN. Antenatally diagnosed congenital cystic adenomatoid malformations (CCAM): research review. J Prenat Med 2012;6:22–30.  Back to cited text no. 1
    
2.
Hellmund A, Berg C, Geipel A et al. Prenatal diagnosis and evaluation of sonographic predictors for intervention and adverse outcome in congenital pulmonary airway malformation. PLoS One 2016;11:e0150474.  Back to cited text no. 2
    
3.
Strumiłło B, Jóźwiak A, Pałka A, Szaflik K, Piaseczna-Piotrowska A. Congenital cystic adenomatoid malformation - diagnostic and therapeutic procedure: 8-year experience of one medical centre. Kardiochir Torakochirurgia Pol 2018;15:10–7.  Back to cited text no. 3
    
4.
Chikkannaiah P, Kangle R, Hawal M. Congenital cystic adenomatoid malformation of lung: report of two cases with review of literature. Lung India 2013;30:215–8.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Muller CO, Berrebi D, Kheniche A, Bonnard A. Is radical lobectomy required in congenital cystic adenomatoid malformation? J Pediatr Surg 2012;47:642–5.  Back to cited text no. 5
    
6.
Lezmi G, Hadchouel A, Khen-Dunlop N, Vibhushan S, Benachi A, Delacourt C. Congenital cystic adenomatoid malformations of the lung: diagnosis, treatment, pathophysiological hypothesis. Rev Pneumol Clin 2013;69:190–7.  Back to cited text no. 6
    
7.
Giubergia V, Barrenechea M, Siminovich M, Pena HG, Murtagh P. Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases. J Pediatr (Rio J) 2012;88:143–8.  Back to cited text no. 7
    
8.
Liao SL, Lai SH, Hsueh C, Wong KS. Comparing late-onset and neonatally-diagnosed congenital cystic adenomatoid malformation of the lung. Chang Gung Med J 2010;33:36–43.  Back to cited text no. 8
    
9.
Mehta AA, Viswanathan N, Vasudevan AK, Paulose R, Abraham M. Congenital cystic adenomatoid malformation: a tertiary care hospital experience. J Clin Diagn Res 2016;10:SC01–4.  Back to cited text no. 9
    
10.
Shariff M, Singh S, Saxena A, Sharma A, Abusaria P. Spontaneous resolution of CCAM: a rare presentation. Int J Contemp Pediatr 2018;5:251–3.  Back to cited text no. 10
    


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