Clinical Picture of Diaphragmatic Hernia Resize

Description

Background
The contract for the Maternal Neonatal and Infant (MNI)CORP was awarded to MBRRACE-UK by the Healthcare
Quality Improvement Partnership (HQIP) on 30th May 2012. MBRRACE-UK is a collaboration led from the
National Perinatal Epidemiology Unit in Oxford with members from the Universities of Leicester, who lead the
perinatal aspects of the work, Liverpool and Birmingham and University College London, as well a general
practitioner from Oxford, and Sands, the stillbirth and neonatal death charity.
This report is based on the findings of first perinatal Confidential Enquiry process run by the MBRRACE-UK
team and focusses on the care of fetuses and babies with congenital diaphragmatic hernia.
Congenital diaphragmatic hernia
Congenital diaphragmatic hernia (CDH) occurs in approximately 1 in 3000–4000 live births, and is associated
with a high overall mortality and a high rate of morbidity amongst survivors. Estimates of the number of cases
in the UK vary but it is likely that there are between 200 and 300 new cases annually. Of these up to 70% are
likely to be diagnosed antenatally as part of routine screening. Up to a third of all UK cases end in either a
spontaneous loss during the pregnancy or an elective termination.
The Confidential Enquiry Process
A stratified random sample of cases was selected by UK country, timing of diagnosis (antenatal or postnatal) and
pregnancy outcome (classified as: termination of pregnancy, stillbirth, neonatal death or survival to discharge).
Cases were reviewed by confidential enquiry panels using a standardised methodology. Cases for review were
identified from an existing epidemiological study of CDH cases born in UK during 2009 and 2010. As there
were no national or international standards that related to the whole care pathway for CDH, criteria to judge
the quality of care were established by consensus with the help of a Topic Expert Group (TEG). This was a
multidisciplinary group comprising experts from the relevant clinical specialties and a patient representative
from the charity CDH UK. The group utilised a consensus approach to identify components of good practice in
relation to each of the following aspects of the care pathway:
• Diagnosis Screening and Delivery Issues
• Resuscitation
• Early after-care
• Surgery and post-operative care
• Palliative care issues – either antenatal or postnatal where appropriate
• Long-term outcome