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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 6  |  Issue : 2  |  Page : 112-113
Cystic adenomatoid malformation of the lung: A diagnostic dilemma

1 Department of Paediatric Surgery, Chacha Nehru Bal Chikitsalaya and Maulana Azad Medical College, Delhi, India
2 Department of Anaesthesiology, Chacha Nehru Bal Chikitsalaya and Maulana Azad Medical College, Delhi, India
3 Department of Pathology, Maulana Azad Medical College, New Delhi, India

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Date of Web Publication29-Jul-2009


Congenital cystic adenomatoid malformation (CCAM) of the lung is an uncommon anomaly that arises from excessive disorganised proliferation of tubular bronchial structures excluding the alveoli. These are believed to represent focal pulmonary dysplasia because skeletal muscle may be identified from within the cyst wall. This report describes a case of an infant operated for presumed diagnosis of congenital diaphragmatic hernia. Diaphragm was found to be normal and further investigations revealed cystic mass in the lower lobe of the left lung. Thoracotomy was done to resect the lesion that revealed a type II CCAM on histopathological examination. The case stresses the need for better clinical examination and advanced radiological investigations in doubtful cases.

Keywords: Adenomatoid malformation, congenital diaphragmatic hernia, infant

How to cite this article:
Mohta A, Kanojia RP, Bathla S, Khurana N. Cystic adenomatoid malformation of the lung: A diagnostic dilemma. Afr J Paediatr Surg 2009;6:112-3

How to cite this URL:
Mohta A, Kanojia RP, Bathla S, Khurana N. Cystic adenomatoid malformation of the lung: A diagnostic dilemma. Afr J Paediatr Surg [serial online] 2009 [cited 2021 Oct 27];6:112-3. Available from:

   Introduction Top

Congenital cystic adenomatoid malformations of the lung (CCAM) are uncommon congenital cystic lung lesions that arise from excessive disorganised proliferation of tubular bronchial structures excluding the alveoli. [1] Preoperative diagnosis of this condition can be difficult at times, as it can be confused with other surgical causes of respiratory distress in the infants. [2] This case report describes a case of congenital adenomatoid malformation erroneously diagnosed as congenital diaphragmatic hernia.

   Case Report Top

A two-month-old boy presented with respiratory distress of two months duration. He was born at full term after normal vaginal delivery out of nonconsanguineous marriage. There was no antenatal examination of the mother for any congenital anomalies; and there was no fever. Clinical examination revealed signs of respiratory distress and chest examination revealed decreased air entry on the left side. Plain X-ray chest [Figure 1] was interpreted as that of left congenital diaphragmatic hernia. The patient was referred to a surgeon who operated with a presumptive diagnosis of congenital diaphragmatic hernia (CDH) by abdominal route. At surgery, the diaphragm and abdominal structures were found to be normal, and the incision was closed. In the postoperative period, the child continued to have respiratory distress. The child was referred for further investigations and management. Computed tomography of the chest [Figure 2] suggested a diagnosis of cystic disease of the left lower lobe of the lung. Left lower lobectomy was performed through left thoracotomy. The child improved clinically in the postoperative period. Signs of respiratory distress disappeared and the air entry in the left chest improved. Postoperative chest X-ray improved without any abnormality. Histopathological examination of the excised specimen suggested Type II congenital cystic adenomatoid malformation.

   Discussion Top

Respiratory distress in the infants due to surgical causes is uncommon. Surgical conditions leading to respiratory distress include congenial lobar emphysema (CLE), congenital cystic adenomatoid malformation (CCAM), and pulmonary sequestration. [1],[3] Diaphragmatic defects such as CDH or eventration and bronchogenic cysts; and acquired lung pathology such as empyema or lung abscess can also present with distress.

Usually CDH can be diagnosed on the basis of clinical examination and chest X-ray. The abdominal gas pattern in the abdomen should suggest the clinician, whether the patient is dealing with a case of CDH or cystic lung disease. There are reports which suggest that there may be difficulty in differentiating CCAM from CLE, or CDH may be diagnosed as CCAM. [2],[4] Sometimes, CCAM may coexist with CDH or sequestration. [2] In doubtful cases, a high resolution CT [5] or MRI helps in confirming the diagnosis, demonstrating unsuspected findings, with better depiction of anatomic extent of thoracic anomalies, thus allowing better planning for surgery. It is necessary to confirm the diagnosis before surgery to plan approach.[6],[7]

CCAM is the second most common congenital lung lesion in children. It is more common in males and is the result of an embryologic insult before the 50 th day of gestation causing maldevelopment of the terminal bronchiolar structures. Stocker [8] described different types of CCAM on histopathology. Although, it is said to sometimes disappear before birth, in-utero regression of CCAM on antenatal ultrasound may not represent genuine resolution and need fetal MRI for prenatal diagnosis. [9] Postnatal computed tomography of thorax should be considered in all newborns with antenatally diagnosed congenital cystic adenomatoid malformation; and if confirmed, early operation before first hospital discharge is advised to prevent progressive lung hypoplasia. [10],[11]

   References Top

1.Azizkhan RG, Crombleholme TM. Congenital cystic lung disease: Contemporary antenatal and postnatal management. Pediatr Surg Int 2008;24:643-57.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Chadha R, Singh D, Kathuria P, Sharma S, Roy Choudhury S, Jain M. Congenital cystic adenomatoid malformation associated with ipsilateral eventration of the diaphragm. Indian J Pediatr 2006;73:832-4.  Back to cited text no. 2    
3.Hashemzadeh S, Aslanabadi S, Jafari Rouhi AH, Azhough R, Kaliebar NA. Congenital malformations of the lung. Indian J Pediatr 2007;74:192-4.  Back to cited text no. 3    
4.Pathak D, Sarin YK. Unusual Congenital Cystic Adenomatoid Malformation of the Lung: A Diagnostic Dilemma. Indian Pediatr 2005;42:1255-6.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Battista G, Turci GA, Pisi P, Ghigi G, Zompatori M, Canini R. High-resolution computed tomography in the study of congenital cystic adenomatoid malformation of the lung. Radiol Med (Torino) 1995;89:416-23.  Back to cited text no. 5    
6.Findik G, Gezer S, Sirmali M, Turut H. Aydogdu K, Tastepe I, et al. Thoracotomies in children. Pediatr Surg Int 2008;24:721-5.  Back to cited text no. 6    
7.Guruswamy V, Roberts S, Arnold P, Potter F. Anaesthetic management of a neonate with congenital cyst adenoid malformation Br J Anaesth 2005;95:240-2.   Back to cited text no. 7    
8.Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum. Hum Pathol 1977;8:155-71.  Back to cited text no. 8    
9.Quinn TM, Hubbard AM, Adzick NS. Prenatal magnetic resonance imaging enhances fetal diagnosis. J Pediatr Surg 1998;33:553-8.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.Chow PC, Lee SL, Tang MH, Chan KL, Lee CP, Lam BC, et al. Management and outcome of antenatally diagnosed congenital cystic adenomatoid malformation of the lung. Hong Kong Med J 2007;13:31-9.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]
11.Lo AY, Jones S. Lack of consensus among Canadian paediatric surgeons regarding the management of congenital cystic adenomatoid malformation of the lung. J Pediatr Surg2008;43:797-9.  Back to cited text no. 11  [PUBMED]  [FULLTEXT]

Correspondence Address:
Anup Mohta
28-B, Pocket-C, S.F. S. flats, Mayur Vihar Phase-III, Delhi - 110 096
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0189-6725.54776

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