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Background: Bilateral renal agenesis has uniformly been a lethal diagnosis in the perinatal or neonatal period. With the advent of more advanced renal replacement therapy as a bridge to renal transplantation, amnioinfusion has been explored at our center as a way to prevent lethal pulmonary hypoplasia secondary to oligohydramnios. Case presentation: We describe two cases of bilateral renal agenesis with serial amnioinfusion during pregnancy. The families opted for full neonatal intervention and post-natal renal support. While lethal pulmonary hypoplasia was avoided, early and refractory hypotension limited the ability to perform dialysis in these neonates. Conclusions: Our limited experience shows that barriers to care such as lethal respiratory failure and technical limitations of early dialysis are not the only barriers prohibiting care for this population. [ABSTRACT FROM AUTHOR]

Mid-trimester preterm premature rupture of membranes (PPROM), defined as rupture of fetal membranes prior to 28 weeks of gestation, complicates approximately 0.4%–0.7% of all pregnancies. This condition is associated with a very high neonatal mortality rate as well as an increased risk of long- and short-term severe neonatal morbidity. The causes of the mid-trimester PPROM are multifactorial. Altered membrane morphology including marked swelling and disruption of the collagen network which is seen with PPROM can be triggered by bacterial products or/and pro-inflammatory cytokines. Activation of matrix metalloproteinases (MMP) have been implicated in the mechanism of PPROM. The propagation of bacteria is an important contributing factor not only in PPROM, but also in adverse neonatal and maternal outcomes after PPROM. Inflammatory mediators likely play a causative role in both disruption of fetal membrane integrity and activation of uterine contraction. The “classic PPROM” with oligo/an-hydramnion is associated with a short latency period and worse neonatal outcome compared to similar gestational aged neonates delivered without antecedent PPROM. The “high PPROM” syndrome is defined as a defect of the chorio-amniotic membranes, which is not located over the internal cervical os. It may be associated with either a normal or reduced amount of amniotic fluid. It may explain why sensitive biochemical tests such as the Amniosure (PAMG-1) or IGFBP-1/alpha fetoprotein test can have a positive result without other signs of overt ROM such as fluid leakage with Valsalva. The membrane defect following fetoscopy also fulfils the criteria for “high PPROM” syndrome. In some cases, the rupture of only one membrane – either the chorionic or amniotic membrane, resulting in “pre-PPROM” could precede “classic PPROM” or “high PPROM”. The diagnosis of PPROM is classically established by identification of nitrazine positive, fern positive watery leakage from the cervical canal observed during in specula investigation. Other more recent diagnostic tests include the vaginal swab assay for placental alpha macroglobulin-1 test or AFP and IGFBP1. In some rare cases amniocentesis and infusion of indigo carmine has been used to confirm the diagnosis of PPROM. The management of the PPROM requires balancing the potential neonatal benefits from prolongation of the pregnancy with the risk of intra-amniotic infection and its consequences for the mother and infant. Close monitoring for signs of chorioamnionitis (e.g. body temperature, CTG, CRP, leucocytes, IL-6, procalcitonine, amniotic fluid examinations) is necessary to minimize the risk of neonatal and maternal complications. In addition to delayed delivery, broad spectrum antibiotics of penicillin or cephalosporin group and/or macrolide and corticosteroids have been show to improve neonatal outcome [reducing risk of chorioamnionitis (average risk ratio (RR)=0.66), neonatal infections (RR=0.67) and abnormal ultrasound scan of neonatal brain (RR=0.67)]. The positive effect of continuous amnioinfusion through the subcutaneously implanted perinatal port system with amniotic fluid like hypo-osmotic solution in “classic PPROM” less than 28/0 weeks’ gestation shows promise but must be proved in future prospective randomized studies. Systemic antibiotics administration in “pre-PPROM” without infection and hospitalization are also of questionable benefit and needs to be further evaluated in well-designed randomized prospective studies to evaluate if it is associated with any neonatal benefit as well as the relationship to possible adverse effect of antibiotics on to fetal development and neurological outcome. [ABSTRACT FROM AUTHOR]

Background: Not much data are available on the natural history of bilateral renal agenesis, as the medical community does not typically offer aggressive obstetric or neonatal care asbilateral renal agenesis has been accepted as a lethal condition. Aim: To provide an evidence-based, ethically justified approach to counseling pregnant women about the obstetric management of bilateral renal agenesis. Study design: A systematic literature search was performed using multiple databases. We deploy an ethical analysis of the results of the literature search on the basis of the professional responsibility model of obstetric ethics. Results: Eighteen articles met the inclusion criteria for review. With the exception of a single case study using serial amnioinfusion, there has been no other case of survival following dialysis and transplantation documented. Liveborn babies die during the neonatal period. Counseling pregnant women about management of pregnancies complicated by bilateral renal agenesis should be guided by beneficence-based judgment informed by evidence about outcomes. Conclusions: Based on the ethical analysis of the results from this review, without experimental obstetric intervention, neonatal mortality rates will continue to be 100%. Serial amnioinfusion therefore should not be offered as treatment, but only as approved innovation or research. [ABSTRACT FROM AUTHOR]

Background: On rare occasions, elective iatrogenic reduction of a dichorionic twin is performed due to maternal request and in order to improve the perinatal outcome. Materials and methods: Nine twin-to-singleton reductions were identified retrospectively at the Feto-maternal Unit, University of Szeged, Hungary, between December 1997 and June 2015. A post-procedure, routine weekly sonographic scan revealed severe oligohydramnios in two out of the nine cases (22.2%) and amnioinfusion was performed in the mid-trimester to prolong gestation. The fetus survived in one case (11.1%) and the pregnancy continued until preterm birth. Conclusion: A repeated sonographic follow-up for an early diagnosis of oligohydramnios is feasible to avoid miscarriage after artificial embryo reduction. [ABSTRACT FROM AUTHOR]

Objective: This study aims to treat patients with preterm premature rupture of the membranes (PPROM) and anhydramnion using continuous amnioinfusion through a subcutaneously implanted port system. Methods: An amniotic fluid replacement port system was implanted in seven patients with PPROM and anhydramnion starting at the 20th week of gestation (range, 14-26 weeks) for long-term amnioinfusion. Saline solutions (2 L/day; Jonosteril®, Sterofundin®, isotonic NaCl 0.9% solution, lactated Ringer's solution) and a hypotonic aqueous composition with reduced chloride content similar to the electrolyte concentration of human amniotic fluid were used for the continuous amnioinfusion. Results: The mean duration of the PPROM delivery interval continued for 49 days (range, 9-69 days), with 3 weeks of amnioinfusion via the port system (range, 4-49). The newborns showed no signs of lung hypoplasia. Conclusion: Long-term lavage of the amniotic cavity via a subcutaneously implanted port system in patients with PPROM and anhydramnion may help prolong the pregnancy and avoid fetal lung hypoplasia. A hypotonic aqueous composition with reduced chloride content similar to human amniotic fluid can be safely used for amnioinfusion. Prospective randomized studies are ongoing. [ABSTRACT FROM AUTHOR]