Pulmonary lymphangiectasia
| Classification according to ICD-10 | |
|---|---|
| Q33.8 | Other congenital malformations of the lungs |
| ICD-10 online (WHO version 2019) | |
The congenital pulmonary Lymphangiectasia is a very rare congenital disease with a Lymphangiectasia in the lungs . It is a developmental disorder of the lungs in which the lymphatic vessels under the pleura , between the lobes of the lungs, around the vessels and around the bronchi are dilated.
Synonyms are: Njolstad syndrome; English Lymphangiectasia, pulmonary, congenital; CPL; Pulmonary cystic lymphangiectasis
The name refers to the first author of a description from 1998 by PR Njølstad and colleagues.
Spread and cause
The frequency is not known.
As a cause of development defects (incomplete regression of the lymph channels to the 20th week of pregnancy around) or a laying suspected of lymphatic vessels.
A heart defect can cause pulmonary lymphangiectasia.
Associations with Noonan syndrome , Turner syndrome , Hennekam syndrome, and Fryns syndrome have been described.
Clinical manifestations
Clinical criteria are:
- Severe shortness of breath , tachypnea and cyanosis already at birth.
- High mortality in the first hours of life.
diagnosis
In uterine hydrops fetalis , fine ultrasound has an essential role in prenatal diagnosis.
literature
- M. Gray, KZ Kovatis, T. Stuart, E. Enlow, M. Itkin, MS Keller, HM French: Treatment of congenital pulmonary lymphangiectasia using ethiodized oil lymphangiography. In: Journal of perinatology: official journal of the California Perinatal Association. Vol. 34, No. 9, September 2014, pp. 720-722, doi: 10.1038 / jp.2014.71 , PMID 25179384 .
- HS Toru, CY Sanhal, GT Yilmaz, IH Ozbudak, I. Mendilcioglu, G. Ozbilim: Rare congenital pulmonary malformation with diagnostic challenging: congenital pulmonary lymphangiectasia, report of four autopsy cases and review of literature. 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. 28, No. 12, August 2015, pp. 1457-1460, doi: 10.3109 / 14767058.2014.956719 , PMID 25142108 (review).
- C. Bellini, F. Boccardo, C. Campisi, E. Bonioli: Congenital pulmonary lymphangiectasia. In: Orphanet Journal of Rare Diseases. Vol. 1, October 2006, p. 43, doi: 10.1186 / 1750-1172-1-43 , PMID 17074089 , PMC 1637094 (free full text) (review).
Individual evidence
- ↑ a b c d Lymphangiectasia, pulmonary, congenital. In: Orphanet (Rare Disease Database).
- ↑ PR Njølstad, H. Reigstad, J. Westby, A. Espeland: Familial non-immune hydrops fetalis and congenital pulmonary lymphangiectasia. In: European Journal of Pediatrics. Vol. 157, No. 6, June 1998, pp. 498-501, PMID 9667408 .
- ^ Rare Diseases
