Neonatal outcome using ultrathin fetoscope for laser coagulation in twin-to-twin-transfusion syndrome.

OBJECTIVE: To improve neonatal outcome using ultrathin fetoscope for laser treatment of twin-to-twin transfusion syndrome. METHODS: Retrospective cohort study of a series of 80 cases of twin-to-twin-transfusion syndrome prior to 26-weeks' gestation subjected to laser coagulation by means of a 1.0/1.2 mm fiber fetoscope with a sheath sectional area 2.65 mm(2)/3.34 mm(2) (n=27) and a 2.0 mm classic lens fetoscope with a sheath sectional area: 6.63 mm(2)/11.27 mm(2) (n=53). RESULTS: The survival rates of at least one twin in the compared groups were 94.4% (classic optic) and 100% (ultrathin optic), for both twins: 75.5% and 83.3%, respectively. By decreasing sheath diameter a pregnancy was prolonged by an average of 21.3 days (P=0.0045), with a resulting increase in the recipient's weight of 389 g (P=0.0049) and an increase in the donor's Apgar score. However, the intervention with ultrathin optic took 11 min longer (P=0.031). CONCLUSION: The reduction of the iatrogenic damage of the amniotic membrane using ultrathin fetoscope with a small sheath, significantly improves the neonatal outcome after laser treatment of twin-to-twin-transfusion syndrome. The operator should only commence working with the 1 mm fetoscope after the learning curve has been accomplished.

Pregnancy management in women within the bladder-exstrophy-epispadias complex (BEEC) after continent urinary diversion.

BACKGROUND: Management of operative delivery in pregnant women after reconstruction of the bladder-exstrophy-epispadias complex (BEEC) using bowel segments remains a challenge. PATIENTS AND METHODS: We report urological history, pregnancy and delivery course of two BEEC patients after previous abdominal bowel surgeries. One had an ileocecal pouch after previously failed reconstruction, and the other had an ileum augmentation and a catheterizable Mitrofanoff stoma after functional reconstruction of the exstrophic bladder. RESULTS: Frequent bacteriuria and hydronephrosis warranted low-dose prophylaxis throughout pregnancy in one female, bilateral mild upper tract dilatation sonographic monitoring in both patients. Both were successfully delivered by cesarean section. No complications or clinical and sonographic signs for prolapse occurred. However, our operative experience revealed the importance of the abdominal incision type after different reconstructed reservoirs. CONCLUSION: Though care should be intense in pregnant BEEC individuals, patients should not be discouraged to have own children. To facilitate successful pregnancy outcome operative delivery should be done as a interdisciplinary team work and emergency situations should be avoided by meticulous planning and counseling of the BEEC patients.

Pelvic-floor imaging using three-dimensional ultrasonography and magnetic resonance imaging in the long term follow-up of the bladder-exstrophy-epispadias complex.

OBJECTIVE: To investigate whether established pelvic floor variables can be used to predict the risk of uterine prolapse after surgery and to compare two different operative strategies, pelvic adaptation with functional reconstruction and an open pelvis with urinary diversion, as a congenital bony pelvis and pelvic floor defect predisposes females with bladder-exstrophy-epispadias complex (BEEC) to uterine prolapse. PATIENTS, SUBJECTS AND METHODS: We conducted a cross-sectional study using perineal three-dimensional ultrasonography (3D-US) and magnetic resonance imaging (MRI) to describe pelvic floor anatomy in 19 women with BEEC (mean age 27.3 years) and five controls, with the outcome evaluated by a semi-structured interview. The analysis of 3D-US and MRI was conducted by two independent investigators. RESULTS: Of the 19 women with BEEC, 13 initially had a functional bladder reconstruction, six a urinary diversion with removal of the exstrophic bladder, and 12 women had closure of the pelvis either by traction bandage or osteotomy, and in the other seven the symphysis was not approximated. Four patients had a complete and one a mild uterine prolapse. After a mean follow-up of 24 years, the mean symphyseal diastasis was 4.5 cm after symphyseal approximation, 9.0 cm without symphyseal approximation in BEEC and 0.49 cm in controls. The mean levator hiatus was 4.9 cm on 3D-US and 4.1 cm on MRI after symphyseal approximation, 5.9 and 7.6 cm without symphyseal approximation and 4.2 and 3.2 cm in controls, respectively. The respective mean levator angle was 86.6 degrees and 87.3 degrees after symphyseal approximation, 104.1 degrees and 101.3 degrees without and 71.3 degrees and 45.5 in controls. Prolapse was statistically significantly more common in patients with no symphyseal approximation. CONCLUSION: This is the first study showing that perineal 3D-US is useful for pelvic floor imaging in BEEC. Established pelvic floor variables might be useful for predicting the risk of pelvic organ prolapse in BEEC.

HIF-1beta determines ABCA1 expression under hypoxia in human macrophages.

ATP-binding cassette transporter A1 plays (ABCA1) a major role in reverse cholesterol transport, a process closely related to atherogenesis. In the thickening atherosclerotic lesions lipid loaded macrophages are exposed to regions of local hypoxia that may influence reverse cholesterol transport. Here we studied the effect of hypoxia on ABCA1 regulation and cholesterol efflux in human macrophages. We found that the hypoxia-inducible factor 1 (HIF-1) specifically binds to the HIF-1 response element of the ABCA1 promoter and the HIF-1 complex increases ABCA1 promoter activity along with ABCA1 expression. Primary human macrophages exposed to hypoxia or expressing constitutively active HIF-1alpha responded with a potent change in ABCA1 expression, which showed a strong correlation with HIF-1beta expression (r: 0.95-0.91). Moreover, ABCA1-mediated cholesterol efflux was also found to be regulated by HIF-1beta under hypoxia. In vivo, in macrophages prepared from human atherosclerotic lesions ABCA1 levels showed a strong correlation with HIF-1beta expression. This in vivo regulatory mechanism was confirmed in human pre-eclamptic placentas, a clinical condition with severe local hypoxia. These results demonstrate that HIF-1beta availability determines ABCA1 expression and cholesterol efflux in macrophages under hypoxia and may contribute to the interpersonal variability of atherosclerotic lesion progression.

Partial mole with fetal triploidy as a cause for imminent HELLP-syndrome at 16 weeks of gestation.

A woman at 16 weeks of gestation was admitted to our perinatal center with unspecific abdominal pain. The results from blood samples 12 h after admission revealed a fulminant HELLP-syndrome. After starting i.v. corticosteroid therapy, the woman recovered quickly. CVS was performed because of abnormal findings by ultrasound and a fetal triploidy (69, XXX) was diagnosed. Pregnancy was terminated and histopathological examination of the placental tissue confirmed a partial mole.

Silver-Russell syndrome as a cause for early intrauterine growth restriction.

The diagnosis of Silver-Russell syndrome is based on the characteristic growth restriction and the presence of typical dysmorphic features. We present the prenatal and postnatal findings of a case that was treated at our perinatal center. The suspected diagnosis Silver-Russell syndrome was confirmed after delivery by our medical genetic and neonatology services. The authors want to point out that SRS should be considered in the differential diagnosis of early asymmetric intrauterine growth restriction.