Background

congenital lobar emphysema is a rare cause of respiratory distress in neonates and infants. It was treated by lobectomy since 1943.

Objectives

The aim of this study was to present our experience in the diagnosis and surgical treatment of congenital lobar emphysema cases and highlight the effect of multi-displinary team work on the outcome.

Methods

We retrospectively analyzed data of patients operated for congenital lobar emphysema at cardiothoracic surgery department, Sohag university hospital over the period of 15 years starting from January 2000 and ending in December 2015.

Results

Total number of cases was fifty three. Males were 39 (74%) while females were 14 (26%). Mean age was 12 weeks. Six patients (11%) had associated cardiac anomalies. Main clinical presentations were acute respiratory distress and recurrent chest infection. Left upper lobe affected in 35 cases (66%), right upper lobe in 10 cases (19%) and right middle lobe in 8 cases (15%). All cases subjected to anatomical lobectomy by thoracotomy. One patient subjected to patent ductus arteriosus ligation simultaneously with Left upper lobectomy at the same operative setting. Post operative complications reported in 8 cases (15%) and one patient of them died from sepsis and respiratory failure (1.9%). Postoperatively, all surviving patients were followed up for one year in the outpatient clinic.

Conclusion

congenital lobar emphysema is a life threatening disease. Early diagnosis and surgical management by a multi-displinary team is the golden key for favorable and

safe outcome.

 Introduction

Congenital lobar emphysema (CLE) is a rare developmental malformation of the lung. Its incidence being nearly 1 per 25000 births [1]. This disease entity was precisely described for the first time in 1932 by Doctor Nelson [2]. This lung abnormality often presents in the neonatal period and early infancy, so sometimes termed infantile lobar emphysema [3,4]. It is characterized by over-inflation of one or more lung lobes which is due to localized air trapping that compresses the ipsilateral and contralateral normal lungs. The cause of CLE is not yet fully understood; bronchial abnormalities [5] or alveolar defects [6] have been reported as etiological factors. The most frequently affected lobe is the left upper lobe (LUL), followed by the middle and then the right upper lobe (RUL) [7]. Males are more commonly affected with a male to female ratio is 3:1 [8]. The most common clinical presentation is neonatal acute respiratory distress. Dyspnea, tachypnea, wheezing, cough, and cyanosis are among the most common presenting symptoms. Symptoms may be exaggerated by feeding or crying [9,10]. The time of onset and severity of symptoms varies depending on the degree of hyperinflation. In some infants, Progressive respiratory distress develops rapidly, while others have a more gradual and insidious onset, 50% presenting by one month of age [11,12]. On the other hand there is a rare adult presentation [13]. Some authors reported CLE as a rare cause of hypertension [14]. Physical findings include hyper-resonant percussion, diminished breath sounds on the affected side and mediastinal shift which manifested by displacement of the cardiac point of maximal impulse. The chest may appear hype-expanded with limited respiratory excursion. The infants may present with recurrent respiratory infections or poor feeding with failure to thrive [10]. Other congenital anomalies may be associated with CLE. Cardiovascular anomalies are the most common which represent up to 20% of the associated anomalies [15,16]. The diagnosis is suspected by clinical examination and chest X-ray imaging which can be confirmed by computed tomography (CT) of chest [17]. The traditional treatment of CLE is lobectomy of the involved lobe or lobes [1,[17], [18], [19]]. Some authors reported conservative treatment for patients with mild symptoms [10,20,21]. The first curative lobectomy for CLE were performed on a four-year-old girl in 1943 by Gross and Lewis [12], then many reports of successful surgical treatment have been published [1,19,22].

The aim of this study was to present our experience in the surgical treatment of Children with CLE at Cardiothoracic surgery department, Sohag university hospital over 15- year period. In addition to review the clinical presentation, radiographic findings, the surgical approach and the post operative outcomes of these children. Also to highlight the effect of multi-displinary team works on the outcome.