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Congenital lobar emphysema in an infant with concurrent bilateral pneumothorax

Kongenitales lobäres Emphysem bei einem Säugling mit gleichzeitigem bilateralem Pneumothorax

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Abstract

Congenital lobar emphysema (CLE) is a rare congenital lung disorder characterized by hyperinflation and alveolar distention, mostly associated with bronchial cartilage deficiency. This article describes the case of a 5.5-week-old infant with postnatally diagnosed CLE. The child presented with symptoms of bronchopneumonia and respiratory failure due to a right-sided pneumothorax, and later bilateral pneumothorax. After successful treatment by drainage, a second rupture of emphysematous bullae occurred that was fatal despite resuscitation efforts. Microscopic examination of the lung tissue revealed diffuse distention of alveoli without destruction of alveolar septa and bronchial cartilage deficiency, consistent with CLE. The presented case highlights the importance of clinical awareness of this condition in infants presenting with spontaneous pneumothorax. The importance of a thorough autopsy including histological examination is also emphasized.

Zusammenfassung

Das kongenitale lobäre Emphysem (KLE) ist eine seltene erbliche Lungenfehlbildung mit Hyperinflation und Aufblähung der Alveolen, meist assoziiert mit einem Mangel an Bronchialknorpel. Im vorliegenden Beitrag wird der Fall eines 5,5 Wochen alten Säuglings mit postnatal diagnostiziertem KLE geschildert. Das Kind zeigte Symptome einer Bronchopneumonie und respiratorischen Insuffizienz, bedingt durch einen rechtsseitigen Pneumothorax, und später einen beidseitigen Pneumothorax. Nach erfolgreicher Drainage ereignete sich eine zweite Ruptur emphysematöser Lungenbläschen mit fatalem Ausgang trotz versuchter Reanimation. In der mikroskopischen Untersuchung des Lungengewebes stellten sich im Einklang mit einem KLE eine diffuse Aufblähung der Alveolen ohne Zerstörung der Alveolarsepten und ein Mangel an Bronchialknorpel dar. Der vorgestellte Fall unterstreicht, wie wichtig es klinisch ist, bei Säuglingen mit Spontanpneumothorax an diese Störung zu denken. Betont wird auch die Bedeutung einer gründlichen Autopsie einschließlich der histologischen Untersuchung.

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References

  1. Noppen M (2010) Spontaneous pneumothorax: epidemiology, pathophysiology and cause. Eur Respir Rev 19:217–219

    Article  CAS  Google Scholar 

  2. Bintcliffe O, Maskell N (2014) Spontaneous pneumothorax. BMJ 348:g2928

    Article  Google Scholar 

  3. Weidner N, Cote RJ, Suster S, Weiss LM (2009) Modern surgical pathology, 2nd edn. Saunders, Philadelphia

    Google Scholar 

  4. Simonsen K (1990) Sudden death due to bilateral spontaneous pneumothorax caused by rupture of congenital lung cysts. Z Rechtsmed 103:379–383

    Article  CAS  Google Scholar 

  5. Gardikis S, Didilis V, Polychronidis A, Mikroulis D, Sivridis E, Bougioukas G, Simopoulos C (2002) Spontaneous pneumothorax resulting from congenital cystic adenomatoid malformation in a pre-term infant: case report and literature review. Eur J Pediatr Surg 12:195–198

    Article  CAS  Google Scholar 

  6. Suárez-Peñaranda JM, Rodríguez-Calvo MS, Muñoz JI, Pumarega-Vergara M, Sánchez-Herrero MJ, Concheiro-Carro L (2001) Sudden neonatal death from congenital cystic adenomatoid malformation. Int J Legal Med 115:76–78

    Article  Google Scholar 

  7. Slade M (2014) Management of pneumothorax and prolonged air leak. Semin Respir Crit Care Med 35:706–714

    Article  Google Scholar 

  8. Wall MA, Eisenberg JD, Campbell JR (1982) Congenital lobar emphysema in a mother and a daughter. Pediatrics 70:131–133

    CAS  PubMed  Google Scholar 

  9. Wolf SJ, Bebarta VS, Bonnett CJ, Pons PT, Cantrill SV (2009) Blast injuries. Lancet 374:405–415

    Article  Google Scholar 

  10. Lima M, Ugolini S, Di Salvo N, Libri M, Gargano T, Ruggeri G (2017) A unique case of foreign body aspiration and recurrent pneumothorax. Pediatr Med Chir 39:171

    PubMed  Google Scholar 

  11. Husain AN, Stocker JT (2011) Color atlas of pediatric pathology. Demos Medical Publishing, New York

    Google Scholar 

  12. Andrade CF, Ferreira HP, Fischer GB (2011) Congenital lung malformations. J Bras Pneumol 37:259–271

    Article  Google Scholar 

  13. Gilbert-Barness E, Debich-Spicer D (2004) Embryo and fetal pathology. Cambridge University Press, New York

    Google Scholar 

  14. Roberts PA, Holland AJ, Halliday RJ, Arbuckle SM, Cass DT (2002) Congenital lobar emphysema: like father, like son. J Pediatr Surg 37:799–801

    Article  CAS  Google Scholar 

  15. Chapdelaine J, Beaunoyer M, St-Vil D, Oligny LL, Garel L, Bütter A, Di Lorenzo M (2004) Unilobar congenital pulmonary lymphangiectasis mimicking congenital lobar emphysema: an underestimated presentation? J Pediatr Surg 39:677–680

    Article  Google Scholar 

  16. Gorostidi F, de Buys Roessingh A, Gonzalez M, Simon C, Monnier Y (2015) Congenital lobar emphysema presenting as an airway foreign body. Int J Pediatr Otorhinolaryngol 79:2450–2452

    Article  Google Scholar 

  17. Perea L, Blinman T, Piccione J, Laje P (2017) Bilateral congenital lobar emphysema: staged management. J Pediatr Surg 52:1442–1445

    Article  Google Scholar 

  18. Babu R, Kyle P, Spicer RD (2001) Prenatal sonographic features of congenital lobar emphysema. Fetal Diagn Ther 16:200–202

    Article  CAS  Google Scholar 

  19. Oliver ER, DeBari SE, Horii SC, Pogoriler JE, Victoria T, Khalek N, Howell LJ, Adzick NS, Coleman BG (2019) Congenital lobar overinflation: a rare enigmatic lung lesion on prenatal ultrasound and magnetic resonance imaging. J Ultrasound Med 38:1229–1239

    Article  Google Scholar 

  20. King N, Ramesh SS, Essandoh M, Merritt RE (2017) Near complete obliteration of the left hemithorax by congenital lobar emphysema in an adult. Ann Thorac Surg 104:e367–e369

    Article  Google Scholar 

  21. Demir M, Çobanoğlu N (2016) An 18-year-old man with recurrent pneumothorax since he was 10-year-old. Pediatr Pulmonol 51:E41–E43

    Article  Google Scholar 

  22. Ochs M, Nyengaard JR, Jung A, Knudsen L, Voigt M, Wahlers T, Richter J, Gundersen HJ (2004) The number of alveoli in the human lung. Am J Respir Crit Care Med 169:120–124

    Article  Google Scholar 

  23. Ernst LM, Ruchelli ED, Huff DS (2011) Color atlas of fetal and neonatal histology. Springer, New York

    Book  Google Scholar 

  24. Wilson RD, Hedrick HL, Liechty KW, Flake AW, Johnson MP, Bebbington M, Adzick NS (2006) Cystic adenomatoid malformation of the lung: review of genetics, prenatal diagnosis, and in utero treatment. Am J Med Genet A 140:151–155

    Article  Google Scholar 

  25. Ursini WP, Ponce CC (2018) Congenital pulmonary airway malformation. Autops Case Rep 8:e2018022

    Article  Google Scholar 

  26. Thakral CL, Maji DC, Sajwani MJ (2001) Congenital lobar emphysema: experience with 21 cases. Pediatr Surg Int 17:88–91

    Article  CAS  Google Scholar 

  27. Kovacevic A, Schmidt KG, Nicolai T, Wisbauer M, Schuster A (2009) Two further cases supporting nonsurgical management in congenital lobar emphysema. Klin Padiatr 221:232–236

    Article  CAS  Google Scholar 

  28. Cataneo DC, Rodrigues OR, Hasimoto EN, Schmidt AF Jr, Cataneo AJ (2013) Congenital lobar emphysema: 30-year case series in two university hospitals. J bras pneumol 39:418–426

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Forensic Medicine, Faculty of Medicine, Pavol Jozef Šafárik University, Trieda SNP 1, 04011, Košice, Slovakia

    Dorota Sopková MD & Silvia Farkašová Iannaccone

  2. Children’s Faculty Hospital, Trieda SNP 1, 04011, Košice, Slovakia

    Jana Šprláková

  3. Department of Pathology and Molecular Medicine, Thomayer Hospital, Vídeňská 800, 14059, Prague, Czech Republic

    Radoslav Matěj

  4. Department of Pathology, Louis Pasteur University Hospital, Rastislavova 43, 04190, Košice, Slovakia

    Lucia Fröhlichová

  5. Medico-Legal and Pathological-Anatomical Department of Health Care Surveillance Authority, Ipeľská 1, 04374, Košice, Slovakia

    Alžbeta Ginelliová & Daniel Farkaš

  6. Institute of Forensic Medicine, Justus Liebig University, Frankfurter Straße 58, 35392, Gießen, Germany

    Reinhard Dettmeyer

Authors
  1. Dorota Sopková MD
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  2. Jana Šprláková
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  3. Silvia Farkašová Iannaccone
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  4. Radoslav Matěj
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  8. Daniel Farkaš
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Correspondence to Dorota Sopková MD.

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Conflict of interest

D. Sopková, J. Šprláková, S. Farkašová Iannaccone, R. Matěj, L. Fröhlichová, A. Ginelliová, R. Dettmeyer and D. Farkaš declare that they have no competing interests.

For this article no studies with human participants or animals were performed by any of the authors. All studies performed were in accordance with the ethical standards indicated in each case. Additional written informed consent was obtained from all individual participants or their legal representatives for whom identifying information is included in this article.

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Sopková, D., Šprláková, J., Farkašová Iannaccone, S. et al. Congenital lobar emphysema in an infant with concurrent bilateral pneumothorax. Rechtsmedizin 30, 190–193 (2020). https://doi.org/10.1007/s00194-020-00386-0

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  • DOI: https://doi.org/10.1007/s00194-020-00386-0

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