Congenital hepatoblastoma in a growing health economy.

A 43-year-old woman, whose pregnancy was complicated by the presence of a large single solid intra-abdominal fetal mass, was referred from the private sector into our fetal maternal unit at the Corniche Hospital, Abu Dhabi at 36 weeks postmenstrual age.Investigations subsequently confirmed that this mass was a congenital hepatoblastoma, one of the very rare embryonic tumours. The baby had chemotherapy and surgical excision of the tumour. Fifteen months later, the alpha feto-protein levels remain normal and follow-on MRI scans do not show recurrence or any residual disease.To our knowledge, this is the first case of congenital hepatoblastoma in the United Arab Emirates (UAE). In the UAE, the interphase between private health insurance schemes and medical (public and private) care within a growing health economy enhances access to unique services such as cancer treatments within specialised centres.

Cardiovascular biomarkers in paired maternal and umbilical cord blood samples at term and near term delivery.

BACKGROUND: Cardiovascular biomarkers might help to identify fetuses or pregnancies at risk. AIM: To examine the umbilical cord neonatal and maternal levels of cardiovascular biomarkers at the time of delivery, and to correlate maternal and fetal biomarker levels to each other, to gestational age and to delivery mode. STUDY DESIGN: In a prospective, observational, cross-sectional, single-center study biomarkers were measured in paired maternal and umbilical venous cord blood samples. SUBJECTS: The sample cohort included 66 sets of fetal and maternal blood samples (11 after multiple gestation, 53 after cesarean section, 17 after exposure to labor). OUTCOME MEASURES: Midregional pro-adrenomedullin (MRproADM), midregional-pro atrial natriuretic peptide (MRproANP), brain natriuretic peptide (BNP), n-terminal-pro brain natriuretic peptide (NTproBNP), copeptin, and high sensitive troponin I (hsTnI) levels were measured. RESULTS: Mean ± SEM for biomarker levels in umbilical venous/maternal blood were: MRproADM [nmol/L] 1.02 ± 0.04/1.24 ± 0.08, MRproANP [pmol/L] 215.53 ± 12.96/54.65 ± 3.41, BNP [pg/mL] 32.02 ± 3.37/19.76 ± 3.29, NTproBNP [pg/mL] 1228.94 ± 91.73/71.48 ± 8.65, copeptin [pmol/L] 103.42 ± 22.89/10.41 ± 1.71, and hsTnI [pg/mL] 13.54 ± 5.17/4.91 ± 2.37. Fetal MRproANP, NTproBNP, and BNP were inversely correlated with gestational age. Maternal and fetal MRproANP (r=0.472, p=0.002) and copeptin (r=0.572, p<0.001) levels were correlated, whereas there was no feto-maternal correlation for the other biomarkers. Fetal copeptin was elevated after exposure to labor. CONCLUSIONS: Biomarker levels appear to be regulated independently in mother and fetus. Fetal biomarkers are influenced by gestational age and delivery mode. In this study on term and near term pregnancies without specific fetal pathology, correlation between paired maternal and fetal biomarker levels was weak or not demonstrable.

Fetal cardiovascular hemodynamics in twin-twin transfusion syndrome.

Twin-twin transfusion syndrome (TTTS) complicates 10-15% of monochorionic-diamniotic (MCDA) pregnancies. It originates from unbalanced transfer of fluid and vasoactive mediators from one twin to its co-twin via placental anastomoses. This results in hypovolemia in the donor and hypervolemia and vasoconstriction in the recipient twin. Consequently, the recipient demonstrates cardiovascular alterations including atrioventricular valve regurgitation, diastolic dysfunction, and pulmonary stenosis/atresia that do not necessarily correlate with Quintero-stages. Selective fetoscopic laser photocoagulation of placental vascular anastomoses disrupts the underlying pathophysiology and usually improves cardiovascular function in the recipient with normalization of systolic and diastolic function within weeks after treatment. Postnatal studies have demonstrated early decreased arterial distensibility in ex-donor twins, but 10-year follow up is encouraging with survivors showing normal cardiovascular function after TTTS. However, prediction and appropriate early management of TTTS remain poor. Assessment of the cardiovascular system provides additional insight into the pathophysiology and severity of TTTS and may permit more targeted early surveillance of MCDA pregnancies in future. It should form an integral part of the diagnostic algorithm.

Twin-twin transfusion syndrome: treatment and outcome.

Twin-twin transfusion syndrome complicates up to 15% of monochorionic pregnancies in the mid-trimester, and results in high perinatal mortality and morbidity if left untreated. Although some humoral factors play a role in the pathogenesis of the disease, an unequal placental sharing and the presence of placental vascular anastomoses at the chorionic plate, allowing blood volume shifts between the twins, are the anatomic prerequisite for this complication unique to monochorionic twins. Within monochorionic pregnancies, it is possible to identify a subgroup of twin pairs at high risk of developing twin-twin transfusion syndrome or selective intrauterine growth restriction during the course of pregnancy as early as in the first or early second trimester. If progressive amniotic fluid discrepancy advances to moderate twin-twin transfusion syndrome, and finally reaches the stages of severe twin-twin transfusion syndrome, accurate classification of the clinical picture and diagnosis of the individual fetal haemodynamic status are crucial for counselling parents on treatment options and possible outcomes. Clear criteria have been established for fetoscopic laser coagulation of placental vascular anastomoses, which is the treatment of choice for severe twin-twin transfusion syndrome, interrupting blood flow between the twins, and relieving uterine over-distension related to severe polyhydramnios.

Intrauterine surgery--choices and limitations.

BACKGROUND: The past decade has seen much progress in intrauterine surgery. Randomized trials have documented the benefit of some procedures of this type for the unborn child. METHOD: Selective literature review RESULTS: Randomized trials have demonstrated the benefit of fetoscopic laser coagulation of placental anastomoses in twin-to-twin transfusion syndrome (TTTS) and of intrauterine surgery via hysterotomy for the repair of spina bifida. Other fetoscopic procedures have yielded promising initial results but are not yet supported by findings from randomized trials. Some intrauterine surgical procedures must still be considered experimental in view of the lack of randomized trials and the rarity of the conditions they are designed to treat. Fetoscopic laser coagulation for TTTS is by far the most common procedure in fetal surgery; TTTS arises in roughly 1 in 2500 pregnancies. The other procedures discussed in this article are performed much less often and for rarer indications. In general, intrauterine surgery is indicated only to treat conditions that would otherwise lead to intrauterine death or irreversible prenatal damage. CONCLUSION: Intrauterine surgery is a rapidly developing field. Prenatal intervention by laser coagulation is indicated to treat severe TTTS, as its benefit has been shown in a randomized trial. Not enough evidence is yet available for the possible benefit of intrauterine surgery to treat myelomeningocele and congenital diaphragmatic hernia. Other indications are experimental. When an indication for intrauterine surgery exists, the parents should be informed and, depending on their wishes, referred to a center where it can be performed.

The combination of intrauterine balloon tamponade and the B-Lynch procedure for the treatment of severe postpartum hemorrhage.

OBJECTIVE: To evaluate intrauterine balloon tamponade with or without B-Lynch sutures in avoiding postpartum hysterectomy in cases with severe postpartum hemorrhage. STUDY DESIGN: Retrospective analysis using all women delivering between January 2005 and July 2010 in our center. Prevention of hysterectomy was the main outcome studied. RESULTS: Twenty-four cases of severe postpartum hemorrhage occurred in which medical treatment alone failed. In 20 cases, the Bakri balloon was the first choice to stop hemorrhage. Sixty percent (n = 12) of these were successfully treated with the balloon alone, 30% (n = 6) with the balloon and the B-Lynch suture. Therefore, 90% (n = 18) were successfully treated with the balloon as part of the treatment. The balloon tamponade was not successful in 2 cases. Four cases were treated with emergency hysterectomy a priori. CONCLUSION: The Bakri balloon with or without B-Lynch sutures in a stepwise approach is an effective option for the treatment of severe PPH.

Stage-related outcome in twin-twin transfusion syndrome treated by fetoscopic laser coagulation.

OBJECTIVE: To assess perinatal outcome in monochorionic twin pregnancies according to different stages of severe mid-trimester twin-twin transfusion syndrome managed by fetoscopic laser coagulation of the placental vascular anastomoses. METHODS: In a prospective study fetoscopic laser therapy was performed in 200 consecutive pregnancies with severe mid-trimester twin-twin transfusion syndrome at a median gestational age of 20.7 weeks (range 15.9-25.3 weeks). Outcome data were analyzed for the whole group and separately for each stage according to the Quintero staging system. RESULTS: The overall survival rate was 71.5% (286/400), with survival of both twins in 59.5% (119/200) and survival of at least one of the twins in 83.5% (167/200). The median gestational age at delivery of liveborn neonates was 34.3 weeks (range 23.1-40.4 weeks). There was a significant trend toward reduced survival rates with increasing stage (P=.038). The percentage of pregnancies with survival of both fetuses was 75.9% (22/29) for stage I, 60.5% (49/81) for stage II, 53.8% (43/80) for stage III, and 50% (5/10) for stage IV. At least one of the twins survived in 93.1% (27/29) at stage I, 82.7% (67/81) at stage II, 82.5% (66/80) at stage III, and 70% (7/10) at stage IV. The overall survival rate for donor fetuses was 70.5% (141/200) and for recipient fetuses, 72.5% (145/200). CONCLUSION: These data show that laser therapy is an effective therapeutic option for all stages of severe twin-twin transfusion syndrome and provide information to counsel patients according to the stage of the syndrome.

Amniotic fluid and maternal blood characteristics in severe mid-trimester twin-twin transfusion syndrome.

OBJECTIVE: To investigate the concentrations of metabolic variables in the amniotic fluid of the recipient twin and maternal blood and to correlate them with clinical features, which are characteristic for the course of pregnancies with twin-twin transfusion syndrome (TTS). MATERIALS AND METHODS: In 109 pregnancies with severe mid-trimester TTS, we measured the concentrations of interleukin-6 (IL-6), alpha-fetoprotein (AFP), sodium, potassium, total protein, beta2-microglobulin and osmolality in the amniotic fluid of the recipient twin prior to laser coagulation of the vascular anastomoses and compared them to a control group of 92 singleton pregnancies. We measured the pulsatility index (PI) of ductus venosus flow velocity waveforms in the recipient twin and performed a retrospective chart analysis for complete maternal blood count before and after the procedure. RESULTS: All variables, except osmolality, IL-6 and AFP were significantly lower in the TTS group. There was a significant positive correlation between PI in the ductus venosus and the amniotic fluid AFP concentrations (r = 0.355; p < 0.001). There was a significant fall in complete maternal blood count after laser therapy (p < 0.001) and a significant correlation between the amount of amniotic fluid drained and the fall of maternal Hb (r = -0.261; p = 0.03) and hematocrit (r = -0.212; p = 0.01). CONCLUSION: Pathophysiologic changes in TTS do not only occur at the inter-twin level of placental vascular anastomoses but also at the materno-fetal level of fluid exchanges. AFP is correlated to the severity of congestive heart failure of the recipient twin.