We present the obstetric history of a family of three sisters with Von Willebrand disease, managed in our centre over the course of nine successful pregnancies. The abnormalities result from ...inheritance of an exon 50 skipping mutation in the Von Willebrand factor gene, resulting from consanguinity. Two of the sisters were identified as having a severe phenotype with a Von Willebrand factor level of less than 5 IU/dl, with the other having a mild phenotype. Of the sisters with a severe phenotype, one had a number of prenatal complications and required early onset prophylaxis with Von Willebrand factor concentrate, whilst the other had a less complicated clinical course, only requiring Von Willebrand factor concentrate to cover labour. The sister with mild Von Willebrand disease had a rise in Von Willebrand factor levels during pregnancy and required no specialist treatment. The report highlights the markedly different clinical courses that can occur in patients with Von Willebrand disease and the different approaches to management.
The incidence of congenital heart disease (CHD) accounts for the largest proportion of infant mortality attributable to birth defects. Associations have previously been reported between CHD and low ...birthweight. Low birthweight is independently associated with adverse outcome and has characteristically been calculated using population-based charts. This aim of this study was to determine the incidence of small for gestational age (SGA) in fetuses with CHD utilizing customized birthweight centiles and to determine the effect of SGA on adverse outcome.
A retrospective cohort study was performed between 2006 and 2011. All singleton fetuses with CHD, with no associated karyotype or structural extra-cardiac abnormalities, who delivered at St Thomas's Hospital, London, were included. Population and customized birthweight centiles were calculated and perinatal outcome data were recorded.
A total of 17% of fetuses with CHD had a birthweight centile <10th when population centiles were used, and 25% when customized birthweight centiles were applied. There was no correlation between SGA and increased adverse perinatal outcome.
A high proportion of fetuses with CHD are classified as SGA when both population and customized birthweight centiles are used. SGA does not correlate with adverse outcome in the perinatal period. The cardiac defect therefore appears to be the main determinant of outcome and not the size of the baby at delivery.
The aim of this study was to determine the timing of neonatal cardiac intervention in babies with antenatally diagnosed congenital heart disease and the impact on obstetric management.
A ...retrospective review of all deliveries between January, 2008 and December, 2009 was conducted in a tertiary centre with foetal and paediatric cardiology, maternal-foetal medicine, and obstetric units. All live births with antenatally detected congenital heart disease were included. Data were collected from foetal, paediatric cardiology, and maternity databases and records. Induction, delivery mode, and timing of the first cardiac intervention in the neonate were studied.
205 deliveries were included. Induction and elective Caesarean section rates were 51.2% (105/205) and 14.1% (29/205), respectively. The vaginal delivery rate was 56% (115/205). There was a non-significant trend towards a higher rate of vaginal delivery after spontaneous labour than after induction (75% versus 66%; p = 0.234). The rate of neonatal cardiac intervention during the initial stay was 59.5% (122/205); it was 18.5% (38/205) within 48 hours and 25.8% (53/205) within 72 hours. The median time to first intervention was 4 days (interquartile range 2-8). Babies with hypoplastic left heart syndrome (median 3, interquartile range 2-6), transposition of the great arteries (median 1, interquartile range 0-4.5), and arrhythmia (median 0.5, interquartile range 0-1) had a significantly earlier time to first intervention compared with those with other conditions (p = 0.001).
Vaginal delivery can be achieved in women delivering babies with major congenital heart disease at a tertiary centre. Delivery in or near a tertiary centre is recommended for patients requiring early intervention, of which many can be identified in advance.
To establish the accuracy of prenatal diagnosis in a tertiary referral fetal medicine unit by comparing those diagnoses made prenatally with diagnoses made at birth until discharge, and with ...postmortem information from cases that resulted in termination, intrauterine, or neonatal death.
All cases seen in the Fetal Medicine Unit between 1 June 2004 and 30 November 2005 were collected prospectively and sorted according to diagnosis. Relevant outcome data for these pregnancies were collected including postmortem information.
681 cases seen which accounted for 1219 visits. 198 were classified prenatally as a major abnormality, 46 cases minor, 56 with raised nuchal translucency, and 381 no abnormality. Outcome details were not available for analysis in two cases. Therefore 679 (99.7%) cases were available (711 out of 713 fetuses). Of the liveborns, 93.6% of the prenatal diagnoses were confirmed, 5.1% were resolved (predominantly soft markers), and 1.3% resulted in an additional major abnormality that had a significant clinical effect. Postmortem examinations were performed on 52% fetal or neonatal deaths with a normal or unknown karyotype. There was one new finding at postmortem that changed the fetal medicine diagnosis significantly.
Accuracy of prenatal diagnosis in a tertiary fetal medicine unit is high. Parents and staff need to be aware that not all abnormalities will be detected prenatally, but inaccurate diagnosis is uncommon. Clinical indicators for benchmarking need to be developed.
A national U.K. workshop to discuss practical clinical management issues related to pregnancy in women with myasthenia gravis was held in May 2011. The purpose was to develop recommendations to guide ...general neurologists and obstetricians and facilitate best practice before, during and after pregnancy. The main conclusions were (1) planning should be instituted well in advance of any potential pregnancy to allow time for myasthenic status and drug optimisation; (2) multidisciplinary liaison through the involvement of relevant specialists should occur throughout pregnancy, during delivery and in the neonatal period; (3) provided that their myasthenia is under good control before pregnancy, the majority of women can be reassured that it will remain stable throughout pregnancy and the postpartum months; (4) spontaneous vaginal delivery should be the aim and actively encouraged; (5) those with severe myasthenic weakness need careful, multidisciplinary management with prompt access to specialist advice and facilities; (6) newborn babies born to myasthenic mothers are at risk of transient myasthenic weakness, even if the mother's myasthenia is well-controlled, and should have rapid access to neonatal high-dependency support.
Pre-eclampsia/eclampsia are leading causes of maternal mortality and morbidity, particularly in low- and middle- income countries (LMICs). We developed the miniPIERS risk prediction model to provide ...a simple, evidence-based tool to identify pregnant women in LMICs at increased risk of death or major hypertensive-related complications.
From 1 July 2008 to 31 March 2012, in five LMICs, data were collected prospectively on 2,081 women with any hypertensive disorder of pregnancy admitted to a participating centre. Candidate predictors collected within 24 hours of admission were entered into a step-wise backward elimination logistic regression model to predict a composite adverse maternal outcome within 48 hours of admission. Model internal validation was accomplished by bootstrapping and external validation was completed using data from 1,300 women in the Pre-eclampsia Integrated Estimate of RiSk (fullPIERS) dataset. Predictive performance was assessed for calibration, discrimination, and stratification capacity. The final miniPIERS model included: parity (nulliparous versus multiparous); gestational age on admission; headache/visual disturbances; chest pain/dyspnoea; vaginal bleeding with abdominal pain; systolic blood pressure; and dipstick proteinuria. The miniPIERS model was well-calibrated and had an area under the receiver operating characteristic curve (AUC ROC) of 0.768 (95% CI 0.735-0.801) with an average optimism of 0.037. External validation AUC ROC was 0.713 (95% CI 0.658-0.768). A predicted probability ≥25% to define a positive test classified women with 85.5% accuracy. Limitations of this study include the composite outcome and the broad inclusion criteria of any hypertensive disorder of pregnancy. This broad approach was used to optimize model generalizability.
The miniPIERS model shows reasonable ability to identify women at increased risk of adverse maternal outcomes associated with the hypertensive disorders of pregnancy. It could be used in LMICs to identify women who would benefit most from interventions such as magnesium sulphate, antihypertensives, or transportation to a higher level of care.
Abstract Objective Counselling women where severe growth abnormalities are detected early in the pregnancy is often difficult due to a paucity of outcome data of this specific subset of early onset ...disease. This study therefore aimed to assess the outcome of pregnancies where an estimated fetal weight less than the third centile were detected prior to 24 weeks gestation. Study design A retrospective study in two London teaching hospitals, over an eight year period was performed, analysing all pregnancies with an ultrasound estimated fetal weight less than the third centile prior to 24 weeks gestation. Outcome data: intrauterine death, neonatal death, survival to discharge, gestation at delivery and birthweight were collected. Results Out of 20 pregnancies included in the analysis, six died in utero, two died in the neonatal period and 12 (60%) survived until discharge. Of the livebirths, 67% delivered preterm and 100% percent of livebirths were delivered by Caesarean Section. Conclusion When severe growth abnormalities were detected before 24 weeks, more than half of pregnancies resulted in survival to neonatal discharge. There was an increased incidence of preterm delivery, caesarean section and neonatal unit admission. This information is useful in counselling parents.
Introduction
Thrombocytopenia is well described in pregnancy with an incidence of 6-10%. The majority of data with regards to platelet counts in pregnancy and aetiology of pregnancy related ...thrombocytopenia, however, derives from studies conducted in singletons. There is little information available on this subject in higher order pregnancies.
Aim
This longitudinal study aims to identify the incidence and aetiology of thrombocytopenia in twin pregnancies in order to guide investigation and management. As a reference we also investigate changes in platelet counts in a cohort of uncomplicated twin pregnancies from our population.
Methods
Full blood counts (FBC) and pregnancy outcome data were obtained retrospectively from electronic patient records for 676 twin pregnancies over a five year period at our institution. All women required three FBCs to be performed during their pregnancy (booking, second trimester and delivery) to be included in the study. Women with pre-existing medical co-morbidities or medication know to be associated with thrombocytopenia were excluded. A total of 381/676 women were included in the final analysis. From that original cohort, those women with uncomplicated pregnancies who delivered at term (36/40 onwards) were selected to investigate and report a reference interval for platelet count during pregnancy and compare with known reference intervals in singletons. A total of 301/676 women were included in this sub-analysis.
Results
The mean maternal age was 32.3 years with a mean gestational age of 36.7 weeks at delivery. We defined thrombocytopenia as mild: platelets 100-150 x 109/L, moderate: platelets 50-100 x 109/L and severe: platelets < 50 x 109/L. The table below summarises our results TableFull Blood countMild thrombocytopenia n (%)Moderate thrombocytopenia n (%)Severe thrombocytopenia n (%)1st FBC n=2566 (2.3%)002nd FBC n=38126 (6.8%)1 (0.3%)03rd FBC n=38177 (20.2%)12 (3.2%)1 (0.3%)
The overall rate of thrombocytopenia was 23%. The commonest cause of thrombocytopenia in this population was gestational thrombocytopenia (75%), followed by pre-eclampsia (15%) other hypertensive disorders (5.7%) and the remaining 4.3% included other complications such as sepsis and obstetric cholestasis.
The platelet ranges for our cohort of women with uncomplicated pregnancies were as shown in the table below. These results are in keeping with changes which occur in platelet counts in singleton pregnancies. TableFull Blood countMean plt count (109/L)Range95% interval1st FBC247135-390163-3262nd FBC226115-410147-3443rd FBC18526-427110-317
Conclusion
This study demonstrates that while the incidence of thrombocytopenia is double the rate in twin when compared to singleton pregnancies, the overall distribution in terms of aetiology is very similar. Interestingly this differs somewhat from data in triplet pregnancies where the majority of thrombocytopenia appears to be secondary to pre-eclampsia in one case series. This is an important finding as it indicates that like in most singleton pregnancies the majority of cases are benign and as such investigation and management pathways regarding thrombocytopenia should not differ from investigation and management in singleton pregnancies. However the increased incidence of thrombocytopenia in twin pregnancies overall requires further investigation.
No relevant conflicts of interest to declare.
Key content
There are several obstetric surveillance systems currently used within the National Health Service (NHS).
These systems employ a variety of techniques to monitor a range of important ...health conditions and medical services.
Uses of surveillance data include monitoring disease, clinical audit, research and resource planning.
A surveillance system must be designed appropriately if it is to achieve its desired purpose and remain a justifiable use of public funds.
Learning objectives
To be able to identify the different surveillance system types currently employed within obstetrics and their appropriate uses.
To understand the advantages and limitations of surveillance system data in obstetrics.
To appreciate the importance of appropriate surveillance system design in generating useful data.
Ethical issues
Surveillance systems contain large volumes of personal data. Relevant legal permissions are required to collect, store and permit access to such data that is often collected without explicit consent.
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