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  Table of Contents 
Year : 2019  |  Volume : 22  |  Issue : 11  |  Page : 1621-1625

Congenital pulmonary airway malformation: A case report of a rare cause of neonatal respiratory distress and review of the literature

1 Department of Paediatrics, Lagos State University Teaching Hospital; Department of Paediatrics and Child Health, Lagos State University College of Medicine, Ikeja, Lagos, Nigeria
2 Department of Paediatrics and Child Health, Lagos State University College of Medicine, Ikeja, Lagos, Nigeria
3 Department of Surgery, Lagos State University College of Medicine, Ikeja, Lagos, Nigeria

Date of Submission11-Jan-2019
Date of Acceptance28-Jun-2019
Date of Web Publication13-Nov-2019

Correspondence Address:
Dr. P O Ubuane
Department of Paediatrics, Lagos State University Teaching Hospital, Ikeja
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/njcp.njcp_20_19

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Congenital pulmonary airway malformation (CPAM), formerly known as congenital cystic adenomatoid malformation (CCAM), is a rare developmental dysplastic lesion of the fetal tracheobronchial tree. CPAM is a rare cause of neonatal respiratory distress; however, its presence may span fetal to adult period. In two previous case-reports from Nigeria, CPAM was present in post-neonatal infants. We report the case of a neonate, who presented with increasing respiratory distress and an abnormal chest radiograph, initially assumed as pneumonic changes. A revised diagnosis of CPAM was made after a chest computed tomography (CT) scan. The neonate subsequently had a successful excision of the affected lobe with remarkable clinical improvement. The case highlights the need to utilize superior imaging studies such as CT when plain radiographs are inconclusive.

Keywords: Case report, congenital cystic adenomatoid malformation, respiratory distress, congenital pulmonary airway malformation, neonate, Nigeria

How to cite this article:
Disu E A, Kehinde O A, Anga A L, Ubuane P O, Itiola A, Akinola I J, Falase B. Congenital pulmonary airway malformation: A case report of a rare cause of neonatal respiratory distress and review of the literature. Niger J Clin Pract 2019;22:1621-5

How to cite this URL:
Disu E A, Kehinde O A, Anga A L, Ubuane P O, Itiola A, Akinola I J, Falase B. Congenital pulmonary airway malformation: A case report of a rare cause of neonatal respiratory distress and review of the literature. Niger J Clin Pract [serial online] 2019 [cited 2022 Jun 27];22:1621-5. Available from:

   Introduction Top

Congenital lung malformations (CLM), a heterogeneous group of embryologically-related lung malformations, include congenital pulmonary airway malformation, pulmonary sequestrations, bronchial atresia, lobar agenesis, bronchogenic cyst, congenital lobar emphysema, and polyalveolar lobe.[1],[2] The commonest of these are congenital pulmonary airway malformation (CPAM), formerly known as congenital cystic adenomatoid malformation (CCAM), constituting over half of CLM.[3],[4],[5] Since, the first case report of CPAM by Ch'in and Tang [6] in 1949, several cases have been published worldwide with an estimated prevalence of CPAM in 1-4/100,000 births.[7] Although, almost all reported cases are from whites there is no evidence for racial predilection, neither is it associated with sex, weight, gestational age, parity or intrauterine maternal exposures.[8],[9],[10]

Two previous reports of CPAM from Nigeria were in postneonatal infants. Atalabi et al.[11] in Ibadan in 2006, reported an incidental finding of bilateral CPAM while evaluating a six-week-old female infant who presented with scalp ulcer and fever. An initial diagnosis of congenital diaphragmatic hernia was suspected on chest radiograph (CXR) but a chest CT diagnosed CPAM type -2. The baby died before surgery. Adeniyi et al.[12] in Lagos in 2012, reported a child who was initially diagnosed with hydro-pneumothorax, but CPAM was diagnosed after a CT was done. We report a case of a Nigerian neonate, who presented with respiratory distress secondary to CPAM, with the aim of highlighting the need for a high index of suspicion and the need for superior imaging studies in infants with doubtful radiographic images; this will enhance the prompt diagnosis and appropriate treatment of this rare but potentially fatal condition.

   Case Report Top

A 13-day-old male neonate was referred to our center with tachypnoea noticed since the third day of life. There was no history of fever, cough, cyanosis, forced feeding, choking or difficulty at breastfeeding. A chest X-ray (CXR) was done on the 9th day of life at the referring private hospital and it showed uniform opacities in the right upper and middle lobes, reported as consolidation. The baby was referred as tachypnoea persisted despite being on antibiotics therapy for pneumonia. The mother, a 34-year old primigravida registered nurse, had normal antenatal ultrasonography and pregnancy was carried to term uneventfully. She had her labor induced on account of post-datism and was vaginally delivered of a 3,100g healthy baby. She established breastfeeding within the first hour after birth and the immediate neonatal period was uneventful until the third day of life.

An examination at presentation in our center showed a vigorous baby in severe respiratory distress. He was not pale, jaundiced, cyanosed or febrile. Respiratory examination revealed respiratory rate of 100 cycles/minute, inter-costal and sub-costal recessions, bulging anterior chest wall, hyper-resonant percussion notes in the right hemithorax, and broncho-vesicular breath sounds which were reduced in the lower half of the right hemithorax posteriorly. The cardiovascular and other systems were essentially normal.

A review of the CXR done at the referring hospital with our Radiology unit showed leftward shift of the mediastinum, hyper-luscency of the right lower lobe with reduced lung markings, and haziness of the right upper and middle lobes. On this account, a diagnosis of congenital lobar emphysema or congenital pneumonia was made and a chest CT advised. The CT, performed on the same day of presentation, showed a multi-cystic mass in the right lower lobe suggestive of CCAM. A gaseous distension of the abdomen was also seen [Figure 1] and [Figure 2]. The neonate's complete blood count, abdominal ultrasound scan and barium meal with follow-through (requested due to stomach distension) were essentially normal.
Figure 1: Coronal CT slice showing large cystic lesion in the right lower lobe

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Figure 2: Transverse CT slice showing large multi-loculated cyst in the right lung

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He was commenced on oxygen and prophylactic antibiotic, reviewed by the Cardiothoracic Surgery Unit and prepared for surgery. At surgery (8th day after admission, 21st day of life), the lesion was resected and histology confirmed “lung tissue with multi-loculated cystic lesion diagnostic of type 3 to type 4 CCAM” [Figure 3]. Post-operatively, he had an uneventful course with resolution of respiratory distress by 4th-day post-surgery and was discharged home 15 days after admission. He had three scheduled clinic visits following discharge wherein parents relocated out of Lagos; his clinical condition was unremarkable for respiratory morbidities at these visits. A recent phone call to mother (April 2019) showed that he is now 5 years old, symptom-free with no hospitalization since discharge.
Figure 3: Photograph of the excised right lower lobe showing a large cystic cavity (ARROW) surrounded by smaller cystic cavities

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   Discussion Top

Our patient had CPAM which was symptomatic in the early neonatal period but was successfully resected followed by remarkable postoperative recovery till date. CPAM, a very rare cause of respiratory distress in the neonatal period, is a dysplastic malformation of the tracheobronchial tree resulting from disruptive morphogenesis of the tracheobronchial tree at about the 35th day of life.[2],[7] Although the exact pathogenesis is unknown, studies in rats suggested the role of overexpressed mesenchymal growth factors involved in lung branching embyogenesis.[13],[14] Recently, some authors observed mutations in germline genes implicated in the causation of lung cancers, suggesting a predisposition to malignancy.[15]

Following the first report of CPAM by Chin and Tang [6] in 1949, Kwittken and Reiner [16] provided a more detailed microscopic description of CPAM in 1962 while Stocker et al.[17],[18] published clinico-pathological classification consisting of five types, 0-4. Type 0 involves all the lobes and is incompatible with life. Type 1, the most common type (60-65% of cases), consists of a single or multiple large cysts lined by ciliated, pseudostratified or columnar epithelium. Type 2 (20%) consists of multiple small cysts lined by cuboidal or columnar epithelium without the presence of mucous cells and cartilage; it is associated with other congenital anomalies in 60% of cases.[2] Type 3 consists of large, gland-like and non-cystic lesion usually involving a lobe which may expand to cause hypoplasia of contralateral lung.[2] Type 4 consists of thin-walled large cysts which have the potential for malignant transformation. While the prognosis for type 1 lesion is excellent, that for types 2 and 3 is poor.[17]

Postnatally, CPAM may be symptomatic or asymptomatic.[2],[7] Respiratory distress is the commonest presentation of symptomatic CPAM in the neonatal period, which was the case in our patient.[2] Common causes of neonatal respiratory distress like congenital pneumonia, congenital heart disease (CHD) or congenital diaphragmatic hernia (CDH) were excluded in the child: tachypnoea with bulgy chest in an otherwise afebrile, apparently healthy neonate reduced the likelihood of congenital pneumonia, while a normal echocardiography and intact diaphragm on chest CT excluded CHD and CDH, respectively.[2],[7] Beyond infancy, CPAMs rarely present with respiratory distress; most are asymptomatic even into adulthood while few present with recurrent chest infections in infancy, childhood or adulthood.[2]

CPAM may be associated with renal, cardiac, gastrointestinal or central nervous disorders.[2],[9],[18] In our patient, no other anomaly was detected with echocardiography, barium enema or chest CT; the gastric distension seen on chest CT was attributed to aerophagy from persistent crying as oral feeds were initially withheld on account of respiratory distress.

In developed countries, most cases of CCAM are diagnosed during routine mid-trimester anomaly sonography, occasionally supplemented with fetal MRI.[2],[7] It is not immediately clear why our patient's lesion was not detected during antenatal sonography, but possible factors may include scan machine resolution, sonographer's skill, the timing of scan or size of the lesion.[2],[19] The sensitivity of ultrasonographic detection of congenital cystic lung malformations reduces as pregnancy advances due to loss of fluid-tissue interface.[2],[14] Antenatal detection provides an opportunity to monitor the lesion serially as pregnancy progresses, assess the possible need for postnatal surgical intervention even before symptoms develop, and help in planning delivery in a facility with advanced respiratory and surgical expertise.[2]

CPAM may appear as homogenous opacity on chest radiograph, as in our patient, or as multiple air- or fluid-filled cysts. However, the diagnostic accuracy of radiography, or even MRI, is inferior to that of CT.[2],[14] CXR could not diagnose CPAM in our patient and also in two previous Nigerian reports.[11],[12] Although about half of CPAM may regress spontaneously in utero, CT in the postnatal period is recommended, as apparently resolved lesions on sonography may become visible on CT.[2],[7],[10],[14]

Antenatally, persistent CPAM complicated by hydrops or mediastinal shift have been successfully treated with thoraco-amniotic shunting/drainage, percutaneous sclerotherapy or open fetal surgery.[2],[9],[20],[21] Postnatally, the natural history of CPAM is largely unknown because of its rarity, however, spontaneous resolution is extremely rare but most of CPAMs remain asymptomatic till at least the fifth birthday; less than 10% of them present with symptoms in the neonatal period like the index case.[2],[21],[22] The general consensus is that such symptomatic CPAM should be promptly resected (lobectomy, segmentectomy or wedge-resection) via thoracotomy or thoracoscopy.[2],[3],[4],[7],[20],[21] CPAM, especially large lesions, is best managed in centers with thoracic surgery and intensive care expertise. Thus, early surgical intervention of symptomatic cases would yield better chances of survival.[2],[9]

Currently, the postnatal management of asymptomatic CPAM is controversial: elective surgery versus conservative management.[3],[23] Advocates of elective postnatal surgery cite the risk of recurrent pneumonia, impaired lung growth from mass effect of the lesion and malignant transformation.[2],[7],[15],[23],[24] The contrary opinion is that the risk of surgery outweighs the risk of these complications.[4],[20],[25],[26]

Postoperative complications such as pneumonia, pneumothorax, and pectus excavatum are about four-fold higher in children with symptomatic CPAM compared with asymptomatic lesions.[3],[4],[5],[23] The risk of recurrent pneumonia may be persistent postsurgery, hence adequate vaccination, prompt antibiotics treatment, and lung function monitoring are beneficial for long-term care.[4],[5],[27] Other long-term postsurgical sequelae are not well known. However, Farolfi et al.[28] observed that a small cohort of children with CPAM had normal lung function at 4 to 9 years of age. The risk of long-term malignant transformation is not known, but CPAM type 3/4, as in our patient, is associated with a small risk of malignancy, hence, the index case requires follow-up into adulthood.[4],[20]

In conclusion, neonates with persistent respiratory distress (or symptoms) and inconclusive chest radiographs that do not correlate with clinical presentation, should be offered thoracic CT scan which is superior to CXR in the diagnosis of congenital lung malformations.[10],[11],[12] Such children will benefit from prompt referral to facilities with thoracic surgery and intensive care expertise. We also recommend improved antenatal sonographic expertise and equipment to enable early diagnosis, monitoring, prognostication and prompt treatment with better outcomes in the management of CPAM.[19]

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.


The authors express gratitude to the parents of the patient for allowing this report and providing additional information on the current state. We also thank all clinical staff who participated in the care of the baby.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

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