Rubella embryo fetopathy

Classification according to ICD-10
P35.0	Rubella embryopathy
ICD-10 online (WHO version 2019)

Cataract of the newborn after rubella infection

In the congenital rubella syndrome is prenatal damage to the child in the womb as a result of infection of pregnant women with the rubella virus . The rubella virus enters the unborn child through the placenta and causes damage to the inner ear, heart, eye and other organs during embryonic or fetal development . Disability, premature birth or miscarriage are the result. A vaccination before pregnancy protects against congenital rubella syndrome.

Initial description

The Australian ophthalmologist Sir Norman McAlister Gregg (1892–1966) first described a malformation syndrome in newborns in 1941 , which, as he recognized, was due to rubella infection in the mother during pregnancy . The Gregg syndrome named after him is also called embryopathia rubeolosa . Julia Bell then uncovered the entire connection in 1959 .

Cause and route of infection

If the pregnant woman has no immunity (e.g. vaccination protection ) against rubella , rubella infection can occur during pregnancy. In the case of prenatal rubella infection, the pathogen is transmitted to the embryo or fetus via the placenta ( placenta ) during the maternal illness . In the first eight weeks of pregnancy, rubella infection leads to embryo damage in 90% of cases. As the pregnancy progresses, the risk in the middle third of pregnancy drops to 25-30%.

Symptoms

Possible consequences of an infection of the unborn child are spontaneous abortion , premature birth or the classic combination of malformations in the form of heart defects ( open ductus botalli , septal defects and Fallot tetralogy ), clouding of the lens of the eyes ( congenital cataract ) and inner ear hearing loss. This full picture, also called Gregg syndrome , occurs in rubella infections in the fourth week of pregnancy, whereas an infection in the 20th week of pregnancy may only trigger isolated deafness. Other possible symptoms are low birth weight, a tendency to bleed due to reduced blood platelet counts ( thrombocytopenic purpura ), encephalo meningitis , inflammation of the liver, enlargement of the liver and spleen, inflammation of the heart muscle ( myocarditis ) or reduced head circumference ( microcephaly ). Therefore, testing for rubella is part of maternity care .

distribution

In Germany in 1998 the number of seronegative women aged 18-30 was 0.8-3%, so that with an average of 700,000 births per year, up to 20,000 newborns are at risk from prenatal rubella infection. In contrast, only four and five cases of connatal rubella infection were reported in 1999 and 2000, and none at all in 2010 and 2011. However, the Robert Koch Institute assumes that there is considerable under-reporting.

Diagnosis

Since an unprotected pregnant woman can become infected with rubella four to seven days before the rash occurs, initial rubella infections still occur during pregnancy. If a pregnant woman has a questionable or confirmed rubella infection, prenatal diagnosis by means of a chorionic villus sampling , an amniotic fluid test or, from the 22nd week of pregnancy, an umbilical blood test is possible. After birth , all newborns with rubella embryopathy have self- generated IgM antibodies and also IgG antibodies, which are largely from the mother. IgM antibodies cannot be transferred from mother to child via the placenta, so they are always an expression of a congenital infection (offspring are born infected and have formed antibodies themselves). Viruses are present in the blood of the newborn, so the children are contagious.

The retinal dysplasia is to be distinguished .

prevention

Regardless of whether they wish to have children, the Standing Vaccination Commission recommends vaccination for all unvaccinated women of childbearing age and those with an unclear vaccination status, for those with only a single vaccination and for all people without a vaccination or with an unclear vaccination status who are involved in pediatrics, obstetrics and pregnancy care or are employed in communal facilities. However, often pregnancy is not planned. Rubella antibodies are determined in all pregnant women as part of maternity care. Titers of 1: 8 and 1:16 are considered borderline, while 1:32 or higher is assumed to be adequate protection. The titer information is now being replaced by modern analytical methods, e.g. B. by a quantitative ELISA result or a hemolysis-in-gel test (HiG), which indicates sufficient immunity if positive. If no antibodies can be detected (= seronegative ), the mother-to-be should not have any contact with rubella-infected persons. If a pregnant woman who is not sufficiently immune to rubella or who has not been vaccinated has come into contact with rubella, an antibody test should be carried out immediately on her and on the possible source of infection. If the expectant mother is proven to be infected, she should be informed about the risk of abortion, stillbirth and a risk of malformations of up to 85%.

treatment

Since the underlying cause is a viral infection and the damage occurs in the womb, causal therapy is not possible. Only the existing symptoms can be alleviated by means of symptomatic therapy. Existing hearing damage should be supervised by a pediatric audiologist and, if necessary, provided with hearing aids . Lens clouding can be treated surgically with a lens replacement . Heart defects may also need to be corrected surgically. Comprehensive follow-up care and support in developmental neurology, for example in a social pediatric center in Germany, is of decisive importance .

Reporting requirement

Rubella and rubella embryopathy are notifiable diseases in Germany according to Section 6 (1) of the Infection Protection Act (IfSG). There is an obligation to report by name in the event of suspicion, illness or death. Its pathogen, the rubella virus, is also a pathogen that must be reported by name according to Section 7 . Direct or indirect evidence of an acute infection must be reported.

In Austria, rubella is a notifiable disease in accordance with Section 1, Paragraph 1, Number 2 of the 1950 Epidemic Act . Cases of illness and death must be reported.

In Switzerland, rubella (including congenital ) is also a notifiable disease and that by following the Epidemics Act (EpG) in connection with the epidemic Regulation and the (Annex 1) Regulation of EDI on the reporting of observations of communicable diseases of man . A positive laboratory analysis result is mandatory.

literature

Books

Reinhard Marre, Thomas Martens, Matthias Trautmann, Ernst Vaneck: Clinical Infectology. 1st edition. Munich; Jena: Urban & Fischer Verlag 2000
Willibald Pschyrembel, Joachim W. Dudenhausen: Practical obstetrics: with obstetric operations. 17th edition. Berlin; New York: de Gruyter, 1991
Fritz H. Kayser, Kurt A. Bienz, Johannes Eckert, Rolf M. Zinkernagel: Medical microbiology: understand, learn, look up. 9th edition Stuttgart; New York: Thieme, 1997
Wolfgang Miram, Karl-Heinz Scharf: Biology Today SII. 10th edition. Hanover. Schroedel Schulbuchverlag GmbH. 1997
Gert-Horst Schumacher: Embryonic development of humans. 4th edition. Berlin: VEB Verlag Volk und Gesundheit, 1979
Hans W. Ocklitz, Hanspeter Mochmann, Burkhard Schneeweiß: Infectology. 2nd Edition. Berlin: VEB Verlag Volk und Gesundheit, 1977
Berthold Koletzko , Gustav-Adolf von Harnack : Pediatrics. 11th edition. Berlin; Heidelberg: Springer-Verlag, 2000

Magazine articles

JD Schipke, D. Frehen: Gregg phenomenon and garden hose effect. In: Zeitschrift für Kardiologie (2001), 90: 319-326
B. Pustowoit: Rubella in pregnancy and childhood. MMW update Med. (1999) 141: 722-724

Web links

Rubella: RKI guide. Congenital rubella embryo fetopathy (CRS). In: rki.de. Robert Koch Institute, March 26, 2018.; accessed on March 19, 2020

Individual evidence

↑ Sabine Schuchert: Julia Bell, the argumentative, clever "Streamboat Lady" . Deutsches Ärzteblatt 2018, Volume 115, Issue 46 from November 16, 2018, page 64
↑ ^a ^b ^c ^d ^e ^f ^g ^h Robert Koch Institute: Rubella RKI Guide to Infectious Diseases - Leaflets for Doctors.
↑ RKI: Epidemiological Bulletin 16/2012 (full text online, pdf)
↑ German Society for Pediatric Infectious Diseases V. (DGPI) (Ed.): Handbook infections in children and adolescents. 5th edition Georg Thieme, Stuttgart, New York 2009, ISBN 978-3-13-144715-9
↑ Lentze et al .: Pediatrics. Basics and practice. 3rd edition, Springer, Heidelberg 2007
^ Ordinance of the FDHA on the reporting of observations of communicable diseases in humans. from December 1, 2015 (as of February 1, 2020). Federal Chancellery, accessed on March 19, 2020 (Appendix 1).

[1] Sabine Schuchert: Julia Bell, the argumentative, clever "Streamboat Lady" . Deutsches Ärzteblatt 2018, Volume 115, Issue 46 from November 16, 2018, page 64

[rki-2] ↑ ^a ^b ^c ^d ^e ^f ^g ^h Robert Koch Institute: Rubella RKI Guide to Infectious Diseases - Leaflets for Doctors.

[epibull2012-3] RKI: Epidemiological Bulletin 16/2012 (full text online, pdf)

[dgpi-4] German Society for Pediatric Infectious Diseases V. (DGPI) (Ed.): Handbook infections in children and adolescents. 5th edition Georg Thieme, Stuttgart, New York 2009, ISBN 978-3-13-144715-9

[5] Lentze et al .: Pediatrics. Basics and practice. 3rd edition, Springer, Heidelberg 2007

[6] Ordinance of the FDHA on the reporting of observations of communicable diseases in humans. from December 1, 2015 (as of February 1, 2020). Federal Chancellery, accessed on March 19, 2020 (Appendix 1).