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Congenital Bilobar Emphysema: A Rare Case

Apoorva Kulkarni, Abhaya Gupta, Vishesh Dikshit, Paras Kothari

Department of Pediatric Surgery, Lokmanya Tilak Municipal Medical College and Government Hospital, Mumbai, India



Apoorva Kulkarni

13 Akshay, KC Road, Bandra Reclamation, Bandra (W)

Mumbai-400050, Maharashtra, India

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A 4-month-old male was referred to our tertiary care hospital with repeated episodes of lower respiratory tract infections and respiratory distress. The patient was operated for congenital lobar emphysema of right lung upper lobe at a private hospital 2 months prior. Patient developed respiratory distress again post operatively and had to be ventilated for 4 days. A high resolution contrast tomography (HRCT) of thorax was done which revealed emphysema of middle lobe of right lung. Patient underwent right middle lobe lobectomy via postero-lateral thoracotomy. Patient was relieved of respiratory distress post operatively and was discharged on post-operative day 7 after removal of intercostal drain.

Keywords: congenital, bilobar, emphysema, thoracotomy



Congenital lobar emphysema is a developmental anomaly of lung causing over-inflation of one or more lung lobes. A rare variety results in emphysema of two lobes either of the same or of both lungs. The patient generally presents with respiratory distress requiring surgery. Both affected lobes can be removed either simultaneously or one after the other [1]. We describe a case where affection of the two lobes was discovered one after the other, both requiring removal.

Case report

A 4-month-old male was referred to our institution with a history of respiratory distress and previous episodes of lower respiratory tract infections. The patient has been previously operated for emphysema of upper lobe of right lung and an upper lobe lobectomy of the right lung was performed 2 months back in a private hospital. After the surgery, the patient was symptomatic with episodes of cough and respiratory distress, so much so that he had to be ventilated for a few days in the same private hospital. On examination, he had subcostal retractions but he was maintaining good oxygen saturation on nasal prongs.

Before surgery at the private hospital, X-ray of the chest revealed a right sided emphysema (Fig. 1). In our institution, we ordered a thoracic HRCT (high resolution contrast tomography) (Fig. 2 and 3) which revealed hyperinflation of the medial segment of right middle lobe with retrosternal extension and hyperinflation of lateral segment of right middle lobe reaching upto apical pleura posterior to the right upper lobe.

Figure 1. Pre- surgery chest radiograph

 11.2 6 1

The patient was scheduled for elective surgery. A posterolateral thoracotomy was done and the middle lobe, found to be emphysematous, was removed. An intercostal drainage was placed. It was removed on post-operative day 5. The patient was kept under observation for respiratory distress for 2 days following the removal of the chest tube and then discharged with no symptoms. The patient had followed up once one month after surgery and had no recurrence of symptoms since surgery.

Figure 2. Transverse section of CT thorax

11.2 6 2

Figure 3. Longitudinal section of CT thorax

11.2 6 3


Congenital lobar emphysema (CLE) is a rare anomaly of lung development in which there is progressive over-inflation of one or more lung lobes [1]. It is usually unilateral and the most commonly affected lobe is the left upper (42%), followed by the right middle (35%) but any lung lobe can be affected. Bilobar involvement can affect the same lung or both lungs. Both are extremely rare and have been reported only in few cases in the literature [2]. There have been 7 cases of bilateral congenital lobar emphysema recorded in literature till date. Surgery was required mainly because of respiratory distress. The symptoms were successfully relieved with surgery. Lobar emphysema involving two lobes of the same lung has been observed only in 5 other cases in addition to ours [3].

The clinical presentation varies from asymptomatic to severe respiratory distress. Symptoms after the neonatal period occur mainly due to recurrent respiratory tract infections, resulting in cough, fever and intermittent dyspnea [4]. When a single lobe is involved, treatment of choice is conservative, if patient is asymptomatic. If symptoms persist, the patient requires resection of the affected lobe. In case of bilobar involvement affecting both the lungs, some authors advocate two-step sequential resection of both lobes [5], although there are authors who also propose simultaneous bilateral thoracotomies to resect both lobes at the same time [6]. In case of both lobes affecting the same lung, it is generally suggested that the worse affected be removed first and the other kept under observation [7]. But in our case, the situation was different. The middle lobe of right lung was found to be affected after the resection of upper lobe had already been done 2 months prior. There have been only 5 cases of bilobar emphysema reported in literature and no other case similar to ours. Our patient has been following up with us and has no more respiratory distress.



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