Pulmonary hypoplasia

from Wikipedia, the free encyclopedia
Classification according to ICD-10
Q33.6 Pulmonary hypoplasia
including congenital pulmonary dysplasia; Lung underdevelopment; Pulmonary hypoplasia; Imperfect congenital lung development (not associated with short gestation period); Insufficient lung development
ICD-10 online (WHO version 2019)

The pulmonary hypoplasia is a lack of development ( hypoplasia ) of the fetal lung with (partial) size and volume reduction of one or both lungs .

The English term pulmonary hypoplasia should not be confused with “pulmonary hypoplasia”, ie hypoplasia of the pulmonary artery , especially in older literature.

distribution

The frequency is given as 9-11 in 10,000 live births or 14 in 10,000 births . According to other sources, lung hypoplasia occurs in 1.4% of all births.

With oligohydramnios before the 24th week of pregnancy, the likelihood of pulmonary hypoplasia is about 24 to 40%.

The incidence of premature rupture of the bladder should be 9–28%.

Pulmonary hypoplasia is a common cause of stillbirth or perinatal mortality .

Pathomechanism

Lung development can be disrupted during any of its four developmental stages:

  • Embryonic stage up to about 5th week
  • Pseudoglandular stage up to about 17th week
  • Canalicular stage up to about 24th week, a common cause here is oligohydramnios
  • Alveolar stage from the 24th week

to form

Lung hypoplasia can occur on one or both sides, affect only one lobe of the lung or an entire lung.

Classification

Based on systematic considerations, it can be divided into:

  • Primary pulmonary hypoplasia
    • Lobar agenesis, mostly absent right upper and middle lobes, e. B. combined with scimitar syndrome or accessory diaphragm
    • Horseshoe lung
  • Secondary hypoplasia due to compression of the lungs, more common form, see below

causes

Proper lung development requires:

As a cause of secondary pulmonary hypoplasia , numerous factors can therefore be considered, such as:

In the context of syndromes

Pulmonary hypoplasia can also appear as a main feature in the context of syndromes :

Clinical manifestations

Depending on its severity, pulmonary hypoplasia manifests itself as a respiratory distress syndrome in the newborn .

diagnosis

The diagnosis can be made by x-ray after the birth , usually the mentioned underlying abnormalities already allow a presumption diagnosis in the intrauterine sonography , usually with fine ultrasound .

Fetal magnetic resonance imaging can be used to estimate the lung volume .

Differential diagnosis

In adults with unilateral pulmonary hypoplasia, the Swyer-James syndrome , Poland syndrome and Scimitar syndrome must also be considered in the differential diagnosis :

therapy

Treatment depends on the underlying cause. There are attempts to improve lung development in the fetus , e.g. B. through the administration of corticosteroids , through amniotic fluid replenishment or more invasive interventions.

During fetoscopic closure of the child's trachea in the womb, a small balloon is inserted into the trachea of ​​the fetus through tiny incisions on the mother's abdomen and uterus. This minimally invasive treatment method for diaphragmatic hernias has been established for over 15 years. With this method, good growth of the lungs and a significant improvement in their blood flow can be achieved in a short time.

Prospect of healing

The prognosis correlates with the severity and severity of associated comorbidities.

literature

  • YP Delgado-Peña, A. Torrent-Vernetta, G. Sacoto, I. de Mir-Messa, S. Rovira-Amigo, S. Gartner, A. Moreno-Galdó, JA Molino-Gahete, F. Castillo-Salinas: [ Pulmonary hypoplasia: An analysis of cases over a 20-year period]. In: Anales de pediatria (Barcelona, ​​Spain: 2003). [Electronic publication before printing] November 2015, doi: 10.1016 / j.anpedi.2015.10.008 , PMID 26625967 .
  • CA Barros, G. d. Rezende, E. Araujo Júnior, G. Tonni, AK Pereira: Prediction of lethal pulmonary hypoplasia by means fetal lung volume in skeletal dysplasias: a three-dimensional ultrasound assessment. In: The journal of maternal-fetal & neonatal medicine: the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians. Vol. 29, No. 11, June 2016, pp. 1725-1730, doi: 10.3109 / 14767058.2015.1064887 , PMID 26135769 .

Web links

Individual evidence

  1. ↑ a b c Entry on pulmonary hypoplasia in Flexikon , a wiki from DocCheck
  2. ↑ a b c d e f Radiopaedia
  3. ^ ME Abbott, W. Moffatt: Mirror-Picture Dextrocardia, Complicated by Mitral Aplasia and Pulmonary Hypoplasia, with Great Hypertrophy of the Transposed "Right" Chambers. In: Canadian Medical Association journal. Vol. 20, No. 6, June 1929, pp. 611-616, PMID 20317366 , PMC 1710330 (free full text).
  4. ↑ A. FONTAN, JJ BATTIN, G. BILDSTEIN: [RIGHT PULMONARY HYPOPLASIA AND COMPLEX CONGENITAL HEART DISEASE]. In: Archives françaises de pédiatrie. Vol. 22, Jan 1965, pp. 123-126, PMID 14302476 .
  5. ↑ SB Cadichon, Sandra: "Chapter 22: Pulmonary hypoplasia", in P. Kumar, B. Burton (eds.): Congenital malformations: evidence-based evaluation and management , Mcgraw-Hill Education, 2007, ISBN 978-0-07 -147189-3 , p. 143
  6. ↑ WF Knox, AJ Barson: Pulmonary hypoplasia in a regional perinatal unit. In: Early human development. Vol. 14, No. 1, July 1986, pp. 33-42, PMID 3732117 .
  7. ↑ S. Yoshimura, H. Masuzaki, K. Miura et al .: "The effects of Oligohydramnios and cervical cord transection on lung growth in experimental pulmonary hypoplasia in rabbits". In: American Journal of Obstetrics and Gynecology Vol. 177, No. 1, 1997, pp. 72-7. [doi]: 10.1016 / s0002-9378 (97) 70440-x. PMID 9240585
  8. ↑ T. Berrocal, C. Madrid, S. Novo, J. Gutiérrez, A. Arjonilla, N. Gómez-León: Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. In: Radiographics: a review publication of the Radiological Society of North America, Inc. Volume 24, No. 1, 2004 Jan-Feb, pp. E17, doi: 10.1148 / rg.e17 , PMID 14610245 (review).
  9. ^ H. Pinar: Postmortem findings in term neonates. In: Seminars in neonatology: SN. Vol. 9, No. 4, August 2004, pp. 289-302, doi: 10.1016 / j.siny.2003.11.003 , PMID 15251146 (review).
  10. ↑ a b c d W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. Springer 1996, Vol. II, p. 63, ISBN 3-540-60224-0
  11. ↑ P. Moerman, C. Vanhole, H. Devlieger, JP Fryns: Severe primary pulmonary hypoplasia ("acinar dysplasia") in sibs: a genetically determined mesodermal defect? In: Journal of medical genetics. Vol. 35, No. 11, November 1998, pp. 964-965, PMID 9832050 , PMC 1051499 (free full text)
  12. ↑ JL Gwinn, FA Lee, CJ Fagan, LE Swischuk: Radiological case of the month. Right upper and middle lobar agenesis with accessory diaphragm. In: American journal of diseases of children (1960). Vol. 128, No. 3, September 1974, pp. 367-368, PMID 4415461
  13. Jump up ↑ Y. Bando, M. Nakagawa, K. Ito, Y. Ozawa, K. Sakurai, M. Shimohira, Y. Shibamoto: Horseshoe Lung Associated with Left Lung Hypoplasia: Case Report and Systematic Review of the Literature. In: Polish journal of radiology / Polish Medical Society of Radiology. Vol. 80, 2015, pp. 464-469, doi: 10.12659 / PJR.894445 , PMID 26516390 , PMC 4603592 (free full text) (review).
  14. ^ ND Hawass, MG Badawi, AM al-Muzrakchi, AI al-Sammarai, AJ Jawad, MA Abdullah, H. Bahakim: Horseshoe lung: differential diagnosis. In: Pediatric radiology. Vol. 20, No. 8, 1990, pp. 580-584, PMID 2251000 (review).
  15. ↑ Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
  16. ↑ Growth retardation - hydrocephalus - pulmonary hypoplasia. In: Orphanet (Rare Disease Database).
  17. ↑ Pagod syndrome. In: Orphanet (Rare Disease Database).
  18. ^ R. Langer, HJ Kaufmann, M. Vogel, M. Vocks: X-ray findings in primary bilateral pulmonary hypoplasia - correlation with pathological-anatomical findings. In: Clinical Pediatrics. Vol. 197, No. 5, 1985 Sep-Oct, pp. 427-430, doi: 10.1055 / s-2008-1034016 , PMID 4068602 .
  19. ^ A. Beyersdorff, K. Linnemann, V. Bredow, PC Krüger, M. Heckmann: Fetale MRT - Prenatal representation of a pulmonary hypoplasia. In: Journal of Obstetrics and Neonatology. Vol. 219, No. 2, April 2015, pp. 102-103, PMID 26086051 .
  20. ↑ Emedicine
  21. ↑ J. Al-Maary, MP Eastwood, FM Russo, JA Deprest, R. Keijzer: Fetal Tracheal Occlusion for Severe Pulmonary Hypoplasia in Isolated Congenital Diaphragmatic Hernia: A Systematic Review and Meta-analysis of Survival. In: Annals of surgery. [Electronic publication before printing] February 2016, doi: 10.1097 / SLA.0000000000001675 , PMID 26910202 .
  22. ↑ UMM: Diaphragmatic hernia: University Hospital Mannheim. Retrieved August 1, 2018 .
  23. ↑ UMM: Premature rupture of the bladder: University Hospital Mannheim. Retrieved August 1, 2018 .