Fetal intestinal volvulus is a rare life-threatening condition usually manifesting after birth. It appears on prenatal ultrasound imaging as a twisting of the bowel loops
CASE REPORTA 26-year-old woman, gravida 3, para 1, with a history of one termination of pregnancy and one normal delivery underwent a fetal anatomy scan at 20 weeks' gestation, which was normal. At 31 weeks' gestation, the woman presented with reduced fetal movements and abdominal pain, which had been present for the preceding 48 h. Cardiotocography (CTG) showed no episodes of high variation, a short-term variation of 3.2 and reduced baseline variability of < 5 beats per min without accelerations. Fetal head circumference and femur length were on the 50 th percentile, but abdominal circumference was > 95 th percentile for gestational age and polyhydramnios was noted. Fetal ascites and dilated bowel loops were observed (Figures 1 and 2), but there was no evidence of pleural or pericardial effusion. No other fetal abnormalities were noted. Umbilical artery Doppler imaging showed positive end-diastolic flow. However, fetal movements were absent and the peak systolic velocity in the middle cerebral artery was increased, suggesting fetal anemia as a possible cause of the abnormal fetal heart rate patterns. A live 1860-g male was delivered by emergency Cesarean section, with Apgar scores of 3 and 6 at 1 min and 5 min, respectively. Umbilical artery pH was 6.9 and the base excess was −12.0. The neonate was anemic (hemoglobin 10.8 g/dL), and was resuscitated with endotracheal intubation and blood transfusion. The abdomen was distended and tense with dark discoloration of the skin (Figure 3).Ultrasound examination at 2 h after birth showed a large amount of free fluid mixed with echogenic particles
Ehlers-Danlos syndromes are a clinically and genetically heterogeneous group of rare inherited connective tissue disorders. Hypermobile Ehlers-Danlos syndrome is one of the common types and not infrequently encountered in pregnancy. While, in the majority of women with hypermobile Ehlers-Danlos syndrome, the pregnancy is uncomplicated, it is important to be aware of the condition in view of potential complications such as recurrent joint dislocations and history of surgical joint stabilization procedures, secondary autonomic pain and postural orthostatic tachycardia syndrome. Increased awareness of the condition and a multi-disciplinary approach to the management of these women in pregnancy result in good outcome for the mother and the baby. We report the clinical characteristics and outcome of pregnancies in eight women with hypermobile Ehlers-Danlos syndrome and present a review of the literature with particular reference to management in a pregnant woman with joint hypermobility syndrome.
Haemochromatosis should be considered in the differential diagnosis of hydrops fetalis. The recurrence risk is high, and immunomodulation with intravenous immunoglobulin treatment appears to alter the course of the disease with better infant survival.
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