BACKGROUND: Congenital emphysema is a rare lung malformation characterized by overinflation of lung segments or lobe, together with ventilation and lung perfusion disorders. The lesion frequently causes respiratory distress in infants and may require urgent surgery. The pathogenesis is still controversial, and the usual treatment recommended is a lobectomy. AIM: The aims of the study were to clarify the pathogenesis and relationship to vascular abnormalities and to assess the safety and efficacy of lung-sparing segmental resections. MATERIALS AND METHODS: Retrospective review of hospital records, chest radiograph, digital subtraction angiography, and contrast-enhanced chest CT were used for diagnosis. RESULTS: Forty-three (median age 4 months, range 10 days to 10 years) children were diagnosed with congenital emphysema. Most were <1 year of age at presentation (n=33; 77%). The predominant side affected was the left (n=24; 56%), and of these all but one had segmental (not lobar) lesions. Most of these then underwent segmental lung resection (S(1)-S(3)) with preservation of the uninvolved lingual (S(4-5)) segments preserved. A standard lobectomy was used in right-sided lesions. There was a high incidence of vascular abnormalities in association with the emphysematous segments, which may be important in its etiology. One child, referred in extremis and died before surgery could be undertaken. Otherwise, early and late results of surgery were rated as good or excellent in all patients. Longer-term follow-up of the preserved left lingual segments did not suggest any recurrence of emphysema. CONCLUSION: Segmental resection in cases of left-sided congenital emphysema appears effective and merits further use as a lung-sparing operation.
BACKGROUND:Congenital emphysema is a rare lung malformation characterized by overinflation of lung segments or lobe, together with ventilation and lung perfusion disorders. The lesion frequently causes respiratory distress in infants and may require urgent surgery. The pathogenesis is still controversial, and the usual treatment recommended is a lobectomy. AIM: The aims of the study were to clarify the pathogenesis and relationship to vascular abnormalities and to assess the safety and efficacy of lung-sparing segmental resections. MATERIALS AND METHODS: Retrospective review of hospital records, chest radiograph, digital subtraction angiography, and contrast-enhanced chest CT were used for diagnosis. RESULTS: Forty-three (median age 4 months, range 10 days to 10 years) children were diagnosed with congenital emphysema. Most were <1 year of age at presentation (n=33; 77%). The predominant side affected was the left (n=24; 56%), and of these all but one had segmental (not lobar) lesions. Most of these then underwent segmental lung resection (S(1)-S(3)) with preservation of the uninvolved lingual (S(4-5)) segments preserved. A standard lobectomy was used in right-sided lesions. There was a high incidence of vascular abnormalities in association with the emphysematous segments, which may be important in its etiology. One child, referred in extremis and died before surgery could be undertaken. Otherwise, early and late results of surgery were rated as good or excellent in all patients. Longer-term follow-up of the preserved left lingual segments did not suggest any recurrence of emphysema. CONCLUSION: Segmental resection in cases of left-sided congenital emphysema appears effective and merits further use as a lung-sparing operation.