[Congenital lobular emphysema. Eight case reports]

We have conducted a retrospective study about 8 infants having CLE and who were hospitalised for 11 years in the Pediatric department of Sfax university hospital (1989-1999). The average age of these patients having revealing symptoms ranges from birth to 8 months, with an average age of 2 months and 3 weeks. During the neo-natal period (< 1 month), the disease was found among 35.5% of the patients. The discovery circumstances are represented by a permanent dyspnea in 4 cases, repetitive bronchopneumopathies with paroxystic dyspnea in 2 cases, a prolonged bronchopneumopathy in one case and a whooping cough in one case. The pre-operatory diagnosis was suspected on the chest-radiography in all cases and on the chest scanner in 7 cases. All patients have undergone a surgical treatment. The anatomy-pathological exam has confirmed the diagnosis in all cases. The immediate post-operatory results were simple in all the 8 cases and the long-term evolution has shown minor respiratory and orthopedic defects only in one patient aged 8 at present. The CLE is a lung-malformation often responsible for serious respiratory problems. The symptomatic forms should be operated very early because the ulterior "prognosis" depends on the patient's age at the moment of the surgery.