Congenital lobar emphysema

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Classification according to ICD-10
Q33.8 Other congenital malformations of the lungs
ICD-10 online (WHO version 2019)

A Congenital lobar emphysema denotes a congenital lung malformation with solid compensatory overinflation of a lobe (Latin lobe ) in too small lung lobes of the opposite side and is also called congenital lobar overinflation or Congenital lobar hyperinflation designated.

distribution

The frequency is given as 1–9 in 100,000. The change can already appear prenatal, in the newborn or usually in the first 6 months of life.

root cause

The cause is cartilage dysplasia, anomalies of the bronchi, intra- or extraluminal obstruction due to pathologically enlarged lymph nodes, teratomas or vascular anomalies. However, in 50% no identifiable cause can be proven.

Hypoplasia of the bronchial cartilage is suspected to be the cause of familial autosomal dominant cases.

Clinical manifestations

Depending on the extent of the overinflation, there are signs of shortness of breath, so that newborns with symptoms of shortness of breath should also be considered for this rare disease. An infection of the lower respiratory tract can exacerbate the symptoms.

diagnosis

The left upper lobe is most commonly affected, followed by the right middle and upper lobes. The disease is more common in boys than in girls. In 40% there are also accompanying malformations, in particular heart defects , kidney malformations and anomalies of the skeletal system.

The suspected diagnosis can be made in the womb using fine ultrasound .

Differential diagnosis

In the differential diagnosis, emphysema of obstruction after aspiration , pulmonary aplasia, solitary lung cyst, cyst lung and pneumothorax are to be distinguished.

therapy

Treatment consists of an emergency lobectomy if the symptoms are severe .

history

The clinical picture was first described in 1932 by RL Nelson. The name "Congenital lobar emphysema" comes from R. Robertson and ES James from 1951.

literature

  • D. Wittig, G. Benz-Bohm, E. Rietschel: The congenital lobar emphysema - a radiological visual diagnosis ?. In: RöFo: Advances in the field of X-rays and nuclear medicine. Vol. 176, No. 4, April 2004, pp. 570-573, doi: 10.1055 / s-2004-812949 , PMID 15088183 .
  • DC Cataneo, OR Rodrigues, EN Hasimoto, AF Schmidt Jr, AJ Cataneo: Congenital lobar emphysema: 30-year case series in two university hospitals. In: Jornal brasileiro de pneumologia: publicação oficial da Sociedade Brasileira de Pneumologia e Tisilogia. Vol. 39, No. 4, 2013 Jun – Aug, pp. 418–426, doi: 10.1590 / S1806-37132013000400004 , PMID 24068262 , PMC 4075869 (free full text).

Individual evidence

  1. ↑ a b Congenital Lobar Emphysema. In: Orphanet (Rare Disease Database).
  2. ↑ a b W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. Springer 1996, ISBN 3-540-60224-0 , pp. 65Ff
  3. ↑ a b F. C. Sitzmann: Pediatrics. Diagnostics - therapy - prophylaxis. 6th edition, Hippokrates 1988, ISBN 3-7773-0827-7 , pp. 115f.
  4. ↑ G. Pariente, M. Aviram, D. Landau, R. Hershkovitz: Prenatal diagnosis of congenital lobar emphysema: case report and review of the literature. In: Journal of ultrasound in medicine: official journal of the American Institute of Ultrasound in Medicine. Vol. 28, No. 8, August 2009, pp. 1081-1084, PMID 19643792 (review).
  5. ^ L. Spitz, AG Coran (Editors) Rob & Smith's Operative Surgery. Pediatric Surgery, 5th Edition 1995, Chapman & Hall, ISBN 0-412-59110-3 , p. 178.
  6. ^ RL Nelson: Congenital cystic disease of the lung: Report of a case. In: Journal of Pediatrics . Vol. 1, No. 2, 1932, pp. 233-238. doi: 10.1016 / S0022-3476 (32) 80105-8 .
  7. ↑ R. ROBERTSON, IT JAMES: Congenital lobar emphysema. In: Pediatrics. Vol. 8, Number 6, December 1951, pp. 794-804, PMID 14911250 .