Summary: Blue rubber bleb naevus syndrome (BRBNS) is a rare vascular disorder characterized by rubbery blue -purple cutaneous nodules that are histologically thin-walled dilated vascular spaces. The exact inheritance of the disease in unknown but in cases of familial recurrence, there appears to be a pattern of autosomal dominant inheritance. The vascular lesions may manifest in any organ system but tend to predominate in the gastrointestinal tract (GI). There are only a handful of cases reported in the literature, but reported complications arising from the naevi include sponatenous GI bleeding requiring laparotomy and blood transfusion and the development of large naevi in the cervix thus preventing vaginal delivery. In this case we describe a patient with known BRBNS who developed symptomatic anaemia during her pregnancy which required antenatal admission and blood transfusion. She was managed expectantly in a multidisciplinary setting by obstetricians, gastroenterologists and an obstetric physician with the aim of a vaginal delivery. Nevertheless, she had an elective caesarean section at term for breech presentation. Surgery was complicated by the unexpected finding of venous malformations within the abdominal wall musculature and subcutaneous fat that resulted in a primary haemorrhage and required urgent blood transfusion. The patient made a good postoperative recovery and had a healthy male infant who at birth displayed no external features of BRBNS. This report demonstrates for the first time the appearance of naevi in the abdominal wall and the important considerations that need to be made regarding mode of delivery and future pregnancies.Keywords: maternal-fetal medicine, high-risk pregnancy, clinical genetics CASE REPORTBlue rubber bleb naevus syndrome (BRBNS) is a rare genetic disorder of which familial recurrences consistent with autosomal dominant inheritance have been reported. The condition is characterized by multifocal, rubbery, blue -purple cutaneous nodules whose histology appears as thin-walled dilated vascular spaces. These slow-flow vascular malformations can occur in other organs, particularly the small bowel where they predispose to gastrointestinal (GI) bleeding. Lesions can also occur in other organs and tissues, including brain and muscle, and patients are at risk of thrombocytopenia and consumptive coagulopathy.1,2 We report the management of a patient with known BRBNS in whom vascular lesions in the anterior abdominal wall resulted in unexpected blood loss during delivery by caesarean section.Our patient was diagnosed with BRBNS in childhood when she presented with increasing numbers of small, blue cutaneous naevi at the age of four months (Figure 1). Prior to adulthood she underwent abdominal surgery for GI bleeding from small-bowel vascular malformations on two occasions and had further surgery for bowel obstruction secondary to abdominal adhesions. At the age of 18 years, she had a magnetic resonance imaging (MRI) brain scan that was normal. Thereafter, she remained well until age...
Summary A new and specific method is used for the measurement of plasma free fatty acids (FFA) in pregnancy. Fasting plasma FFA and glucose concentrations were measured serially in twenty normal and twelve overweight women in pregnancy and related to infant birth weight. Large variation between individuals was noted for FFA and no change was found with advancing gestation. Studies of the day‐to‐day variation in the same women at 20 and 36 weeks gestation showed wide variations in concentrations. No significant change in glucose concentration was noted with advancing gestation, but postnatal values were significantly higher. Small variability of glucose concentrations was noted in both the serial and day‐to‐day studies. In normal‐weight women a positive correlation between the observed birth weight of their infants and the fasting plasma glucose levels was found at 20 and 40 weeks and also with the mean of 5 levels measured in pregnancy. No similar correlation was found for the group of overweight women.
their right to choose and obstetricians are losing out on their rewarding and fulfilling role, which is the reason why they chose the specialty in the first place.Midwives and obstetricians are two wheels of the maternity services and we must move towards more integrated and truly woman-centred care, rather than drawing battle lines. We must reclaim and restore patient and professional satisfaction and bring back obstetricians into normal pregnancy and delivery, which, incidentally, is essential for their training in order to recognise and deal with the abnormal. &
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