A rare case of congenital lobar emphysema diagnosed in a 13-year-old boy

Congenital lobar emphysema (CLE) is an innate malformation of the lungs, diagnosed in 1 in 20,000 live births [1]. It was described for the first time by Nelson in 1932, and later by Robertson and James in 1951 [1]. Erroneous development of the respiratory system in the third week of gestation leads to malformation of the lungs [1]. It is rarely diagnosed prenatally with the help of ultrasonography (USG) [2]. The pathology is due to partial obstruction of the bronchus causing hyperinflation of the pulmonary lobe [1, 2]. Most commonly absent or hypoplastic cartilage leads to the collapse of the bronchus, and during expiration to trapping of air [2]. Causes of acquired CLE are aspiration of the meconium, plaques of mucous, hypertrophy of the mucous membrane, aspiration of a foreign body, bronchogenic cyst and mediastinal tumor [1, 2]. Rare abnormalities of the vessels, e.g. abnormal pulmonary venous return, may also be the cause of CLE [2, 3]. According to some authors CLE can be hereditary [4]. CLE is accompanied by heart defects in 10% of cases, so all patients should have echocardiography diagnostics [1–4]. Other possible concomitant malformations are double superior vena cava and horseshoe kidney [1–4]. Most cases are diagnosed in neonates, with male preponderance (boy to girl ratio 3 : 1) [1–4]. Typically the left upper lobe is involved (43%), then the right middle lobe (32%) and the right upper lobe (21%) [3, 4]. CLE localized in the lower lobes is rather unique (2%) [3, 4]. Bilateral localization was also observed [1, 2]. Histological examination reveals alveolar overgrowth and normal structure of the pulmonary acinus [1–3]. CLE may be the cause of respiratory distress and cardiac arrest. Children with severe symptoms are treated with thoracotomy and lobectomy [1, 4]. It is postulated that asymptomatic CLE should be treated nonoperatively, because infections and malign malformations are rare [1, 4].
A 13-year old boy, with uneventful past medical history, presented to his GP with the symptoms of breathing difficulty, pain in the chest, shortness of breath, and a dry, nonproductive cough. According to the medical interview, the symptoms had developed over the last 4 days, along with the infection of the respiratory tract, and had progressively worsened. Family history was not significant, and personal pathological history included few upper airway infection episodes, which were treated by the family doctor with symptomatic drugs. Physical...


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