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 Table of Contents  
RADIOLOGY ROUNDS
Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 220-223

An infant with hyperinflated lungs: What's the secret?


1 Department of Pediatrics, PGIMER, Chandigarh, India
2 Department of Radiodiagnosis and Imaging, PGIMER, Chandigarh, India
3 Department of Pediatric Surgery, PGIMER, Chandigarh, India
4 Department of Histopathology, PGIMER, Chandigarh, India

Date of Submission26-Jul-2021
Date of Decision07-Aug-2021
Date of Acceptance09-Aug-2021
Date of Web Publication31-Aug-2021

Correspondence Address:
Prof. Joseph L Mathew
Department of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ipcares.ipcares_239_21

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How to cite this article:
Singh S, Mathew JL, Saxena AK, Menon P, Chatterjee D, Singh M. An infant with hyperinflated lungs: What's the secret?. Indian Pediatr Case Rep 2021;1:220-3

How to cite this URL:
Singh S, Mathew JL, Saxena AK, Menon P, Chatterjee D, Singh M. An infant with hyperinflated lungs: What's the secret?. Indian Pediatr Case Rep [serial online] 2021 [cited 2021 Sep 26];1:220-3. Available from: http://www.ipcares.org/text.asp?2021/1/3/220/325092

Congenital lobar emphysema (CLE) is a rare developmental abnormality of the lung, and is usually unilateral. Bilateral CLE is very rare and has been reported only in a small number of cases in the literature. CLE can result in progressive respiratory distress during the neonatal period and early infancy, and it often presents a diagnostic and therapeutic dilemma. We report a case of bilateral CLE in a 6-week male infant who presented with acute respiratory distress.


  Clinical Description Top


A 6-week-old male infant presented to us with a history of fast breathimg since birth, and recent chest indrawing. Delivery was at term by cesarean section in a different health facility, the indication being a previous cesarean. The birth weight was 3.2 kg and Apgar scores 8 and 9, at 1 and 5 min, respectively. The infant had developed respiratory distress immediately and was admitted in the neonatal intensive care unit. Although there was no history suggesting maternal sepsis, or prolonged rupture of membranes, infectious pneumonia was presumed and he was treated with antibiotics, besides supportive management with intravenous fluids and oxygen delivered by nasal prongs. The baby was the second born of a nonconsanguineous marriage. The pregnancy was booked and supervised. No abnormalities were reported on antenatal ultrasonography. He improved and was discharged after 5 days. The discharge slip chest radiograph and reports were not available for review. Breastfeeding had been established.

At the age of 12 days, the infant developed cough and fast breathing, which worsened over the next 2 days. He also developed episodes of bluish discoloration while crying. There was no history of choking or coughing while breastfeeding, regurgitation of feeds, noisy breathing, or presence of suck-rest-suck cycles. The infant was admitted to another hospital and managed as pneumonia with antibiotics, oxygen, and fluids, for 1 week, the details of which were unavailable. Postdischarge, the baby remained well at home except for a slightly fast rate of breathing that the parents did not consider significant as he was active, alert, and breastfed well.

At 6 weeks of age, the infant developed a runny nose, worsening of the fast breathing and chest indrawing. After being admitted in another hospital for 3 days, he was referred to us for ascertaining the underlying etiology.

At admission, the temperature was 101°F, heart rate 150/min, respiratory rate 60/min with intercostal, subcostal retractions, and nasal flaring, SpO2 86% in room air, and blood pressure 92/57 mmHg. The weight was 4.4 kg (−1.85 Z score), length 57 cm (−0.67 Z score), and head circumference 39 cm (−0.07 Z score). There was pallor, but no cyanosis, or significant lymphadenopathy. The throat, ear, and nasal cavity examinations were normal. No stridor was audible. The baby had a hyper-inflated chest with symmetric chest expansion, centrally positioned trachea, absence of mediastinal shift, normal percussion note in all areas, and diminished breath sounds over the right mammary area. There were no crackles or wheeze. There was no abdominal organomegaly and the examination of the other systems was unremarkable.

Since the clinical impression was of an infant with recurrent pneumonia, the differentials that were considered included aspiration syndromes (though there was absence of feeding-related symptoms, episodic regurgitation, posturing during feeding, or cough/wheezing during or immediately after feeds); primary ciliary dyskinesia (although there was no history of ear discharge or sinusitis) and cystic fibrosis (despite absence of history of delayed meconium passage, saltiness when kissed, failure to thrive, or persistent cough). Differentials that were excluded were acyanotic congenital heart disease with left-to-right shunts (due to absence of supportive clinical findings); primary or secondary immune deficiency (not supported by multi-site infections, poor weight gain, or suggestive maternal history) and congenital TORCH infections (given the absence of low birth weight and organomegaly). A chest X-ray was done at the age of 6 weeks [Figure 1].
Figure 1: X-ray chest performed at the age of 6 weeks. reveals hyperlucency of the right middle and lower zones, as well as almost the entire left hemithorax

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  What are the Salient Findings in the Chest X-ray? Top


The rotated radiograph makes it difficult to comment on the chest symmetry, however, the thymic shadow is prominent on the right side. The lungs seem well-inflated and eight intercostal spaces can be counted bilaterally. The ninth intercostal space is just visible on the right side. There is hyperlucency of the right middle and lower zones, and also almost the entire left hemithorax [Figure 1]. The right upper lobe area is relatively hazy compared to the other lung fields, suggesting consolidation. Similarly, the right lower zone also shows haziness in the paracardial area. The cardiac silhouette seems normal, although the pulmonary bay appears to be fuller. The pleural spaces, trachea, bones, and soft tissues appear normal.


  What are the Differential Diagnoses Based on the Clinical History and Radiograph? Top


Congenital airway or thoracic malformations, perinatal tuberculosis, and the aforementioned conditions associated with recurrent pneumonia (such as primary ciliary dyskinesia). In addition to the causes excluded before the radiograph, gastro-esophageal reflux disease was also considered less likely.


  What should be the Next Line of Investigations? Top


This was planned according to the differentials. Hemoglobin, total and differential leukocyte count, inflammatory markers, and blood culture were all normal. Sweat chloride level was within the normal range. Gastric lavage specimens did not show acid-fast bacilli on smear examination and were negative on GeneXpert. A screening electrocardiogram and echocardiogram were normal. Since everything was still inconclusive, we planned a computed tomography (CT) scan of the thorax to rule out congenital thoracic malformations. Two noncontiguous representative sections of the CT scan are shown in [Figure 2].
Figure 2: Coronal reconstructed image of the CECT scan shows hyperinflation of the right middle lobe (blue arrow) and left upper lobe (red arrow)

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  What are the Salient Findings in the Computed Tomography Scan? Top


[Figure 2]a shows hyperinflation in the left upper lobe with attenuated vascularity (blue arrow). The left upper lobe appears to herniate across the midline. [Figure 2]b shows similar findings The contrast enhanced CT scan showed hyperinflation of the right middle and left upper lobes, with mass effect on the remaining lung parenchyma [Figure 2]. There is no obvious cardiomegaly, mediastinal or hilar lymphadenopathy, or pleural or pericardial effusions. The radiological diagnosis was bilateral lobar emphysema.


  Management and Outcome Top


The family was counseled regarding the disease, its prognosis, potential complications, and the need for surgery. Bilateral thoracotomy was planned in a staged manner as the surgery carries high intra-operative and postoperative risk. The left upper lobe was removed through a left posterolateral thoracotomy. The infant recovered uneventfully and 3 weeks later, the procedure was repeated to remove the right middle lobe. The infant remained stable and was discharged 5 days later. Histopathological examination of both excised lobes revealed changes consistent with CLE. The infant was discharged with the absence of symptoms, normal respiratory rate, no chest indrawing and a SpO2 of 98% in room air. The infant was followed-up regularly. At the last visit at 8 months of age, he is growing well, has had no respiratory symptoms, and is acquiring milestones appropriately. Age-appropriate immunizations were administered as per the National Immunization Schedule.


  Discussion Top


This case highlights the fact that despite a step-wise clinical approach and relevant investigations, a clinical diagnosis may remain elusive. In the index case, the onset of symptoms since birth, the bilateral hyperinflation, and complete absence of wheezing could have led us to consider CLE. An infectious etiology was considered due to the history of the first two episodes being managed as pneumonia. It may be conjectured that had the X-rays taken during both these events been available to us, a diagnosis of CLE may have been made earlier.

CLE is a rare developmental anomaly of the lung characterized by progressive over-inflation of one or more lung lobes due to partial obstruction of the developing airway leading to ball valve obstruction and air trapping. This results in hyperinflation and consequent compression or displacement of the adjacent normal lung tissue and progressively increasing respiratory distress.[1],[2] It was first reported by Gross and Lewis in 1954. The precise etiology is unknown, but a quarter of cases have deficient bronchial cartilage leading to the inappropriate airway collapse and air trapping, creating over-inflation of the lobe. Prevalence ranges from 1 in 20,000 to 30,000 infants. Males are affected more than females with a ratio of 3:1.

The designation of CLE has been revised to the following terms: congenital lobar hyperinflation, congenital lobar over-inflation, infantile lobar emphysema, congenital large hyperlucent lobe, and congenital alveolar overdistension. These names better describe the condition and to some extent overcome the limitations of the misnomer “emphysema” which is histopathologically a different entity. Infants commonly present with progressive tachypnea, respiratory distress, wheeze, recurrent respiratory infections, and failure to thrive.[1],[2] However, delayed diagnosis is known in 5% cases with presentation occurring as late as 6 years of age. CLE is typically unilateral, most commonly affecting the left upper lobe (42%), followed by the right middle lobe (35%), and right upper lobe (21%). Lower lobes are affected in only about 2% cases.[1] Bilateral involvement has been reported in very few cases.[3]

The chest radiograph may show a radiolucent lobe, atelectasis of the other lobe(s), and mediastinal shift. The challenge is to determine which side of the X-ray is abnormal, as hyperinflated lobes may be misinterpreted as normal, and the focus may be on the atelectatic or consolidated portions alone. This may be the reason for the infant having been diagnosed as pneumonia twice during the first two hospital admissions. Unilateral lesions can be distinguished from radiological differentials of hyperlucency such as pneumatocele or pneumothorax by the lobar pattern, absence of cysts/cavities in the lung, and absence of pleural involvement. Bilateral lesions are more challenging. CT scans may not be required for the diagnosis, but once a diagnosis is made it helps to delineate the extent of involvement and plan the surgical approach. We strongly discourage the temptation of performing a CT scan in lieu of a meticulous clinical approach and chest radiography. Antenatal detection of CLE is possible using fetal ultrasonography or fetal magnetic resonance imaging.[4] This can help to plan delivery in an institution with facilities for neonatal intensive care and early postnatal surgery. CLE is associated with congenital heart disease in about 20% of cases, including left to right shunt defects and tetralogy of Fallot. Therefore, thorough clinical examination followed by echocardiography is essential.[5],[6] Management depends upon the severity.[1],[2],[3],[4] Severely symptomatic patients, with progressive worsening, require early lobectomy, whereas conservative approach with close follow-up may be tried in mild or asymptomatic cases. In bilateral lobar emphysema, surgery done in a staged manner can reduce the surgical complication as well as pain to infant.[7],[8]

To conclude, infants who are symptomatic from birth with hyperinflated lungs in the absence of wheezing should trigger a search for a congenital malformation. Bilateral lobar over-inflation is very rare, but early diagnosis and management do yield gratifying results.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Bush A, Chitty L, Harcourt J, et al. Congenital Lung Disease. 9th ed. Kendig's Disorders of the Respiratory Tract in Children. Philadelphia; Elsevier Inc; 2019. p. 289-337.  Back to cited text no. 1
    
2.
Demir OF, Hangul M, Kose M. Congenital lobar emphysema: Diagnosis and treatment options. Int J Chron Obstruct Pulmon Dis 2019;14:921-8.  Back to cited text no. 2
    
3.
Lei Q, Zeng W, Ju R. Congenital lobar emphysema in bilateral lung lobes: A case report. Transl Pediatr 2020;9:266-71.  Back to cited text no. 3
    
4.
Bulas D. Fetal magnetic resonance imaging as a complement to fetal ultrasonography. Ultrasound Q 2007;23:3-22.  Back to cited text no. 4
    
5.
Abdel-Bary M, Abdel-Naser M, Okasha A, et al. Clinical and surgical aspects of congenital lobar over-inflation: A single center retrospective study. J Cardiothorac Surg 2020;15:102.  Back to cited text no. 5
    
6.
Cataneo DC, Rodrigues OR, Hasimoto EN, et al. Congenital lobar emphysema: 30-year case series in two university hospitals. J Bras Pneumol 2013;39:418-26.  Back to cited text no. 6
    
7.
Maiya S, Clarke JR, More B, et al. Bilateral congenital lobar emphysema: How should we proceed? Pediatr Surg Int 2005;21:659-61.  Back to cited text no. 7
    
8.
Perea L, Blinman T, Piccione J, et al. Bilateral congenital lobar emphysema: Staged management. J Pediatr Surg 2017;52:1442-5.  Back to cited text no. 8
    


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