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A novel non-surgical treatment of symptomatic congenital lobar emphysema

Mareike Price, Christoph Happel, Folke Brinkmann, Georg Hülskamp, Timothy Price, Gesine Hansen, Harald Bertram, Nicolaus Schwerk
European Respiratory Journal 2016 48: PA3847; DOI: 10.1183/13993003.congress-2016.PA3847
Mareike Price
1Paed. Pulmonology, Allergology and Neonatology, University Children's Hospital, Hannover, Lower SaxonyGermany
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Christoph Happel
2Paed. Cardiology and Intensive Care Medicine, University Children's Hospital, Hannover, Lower SaxonyGermany
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Folke Brinkmann
3Paed. Pulmonology, Ruhr University Children's Hospital, Bochum, North Rhine-WestphaliaGermany
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Georg Hülskamp
4Neonatology and Paed. Pulmonology, Clemens Hospital, Münster, North Rhine-WestphaliaGermany
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Timothy Price
1Paed. Pulmonology, Allergology and Neonatology, University Children's Hospital, Hannover, Lower SaxonyGermany
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Gesine Hansen
1Paed. Pulmonology, Allergology and Neonatology, University Children's Hospital, Hannover, Lower SaxonyGermany
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Harald Bertram
2Paed. Cardiology and Intensive Care Medicine, University Children's Hospital, Hannover, Lower SaxonyGermany
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Nicolaus Schwerk
1Paed. Pulmonology, Allergology and Neonatology, University Children's Hospital, Hannover, Lower SaxonyGermany
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Abstract

Introduction: Congenital lobar emphysema or congenital large hyperlucent lobe (CLHL) is a rare lung malformation. Afflicted children typically develop progressive tachydyspnoea in the first 3 months of life and radiologically have hyperlucency of one or more lung segments. The current treatment recommendation in symptomatic infants is surgical resection of the affected lung lobe.

Objective: To present preliminary results of a novel interventional technique of endobronchial lobar or segmental occlusion as a less invasive treatment option in symptomatic infants with CLHL.

Material and Methods: Retrospective analysis of clinical parameters in 3 infants with symptomatic CLHL after bronchoscopically and fluoroscopically controlled placement of self-expanding occluders.

Results: Symptoms started on average at 4 weeks (3-5) of life. Two patients had left upper lobe (UL) bronchus malacia and one patient malacia of segment 1 in the right UL bronchus. Self-expanding occluders were deployed in the affected segment bronchus. One patient developed a pneumothorax during the procedure. All profited within 24 hours with a decrease in tachypnoea and radiological appearance. In 2 patients hyperlucency was incompletely resolved: inserting a second device full occlusion was achieved in 1 patient at 9 months. Because of the third infant's size, a second occluder could not yet be inserted. No significant complications were noted to date.

Conclusion: Endobronchial occlusion of a malacious lobar or segment bronchus in symptomatic infants with CLHL represents a minimally invasive alternative to surgical lobe resection; however it requires ongoing technical refinement with longer term follow-up to fully assess the clinical benefit.

  • Congenital lesion/malformation
  • Treatments
  • Bronchoscopy
  • Copyright ©the authors 2016
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A novel non-surgical treatment of symptomatic congenital lobar emphysema
Mareike Price, Christoph Happel, Folke Brinkmann, Georg Hülskamp, Timothy Price, Gesine Hansen, Harald Bertram, Nicolaus Schwerk
European Respiratory Journal Sep 2016, 48 (suppl 60) PA3847; DOI: 10.1183/13993003.congress-2016.PA3847

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A novel non-surgical treatment of symptomatic congenital lobar emphysema
Mareike Price, Christoph Happel, Folke Brinkmann, Georg Hülskamp, Timothy Price, Gesine Hansen, Harald Bertram, Nicolaus Schwerk
European Respiratory Journal Sep 2016, 48 (suppl 60) PA3847; DOI: 10.1183/13993003.congress-2016.PA3847
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