Implementation and Assessment of a Laparotomy-Assisted Three-Port Fetoscopic Spina Bifida Repair Program.

Open spina bifida (OSB) is a congenital, non-lethal malformation with multifactorial etiology. Fetal therapy can be offered under certain conditions to parents after accurate prenatal diagnostic and interdisciplinary counseling. Since the advent of prenatal OSB surgery, various modifications of the original surgical techniques have evolved, including laparotomy-assisted fetoscopic repair. After a two-year preparation time, the team at the University of Giessen and Marburg (UKGM) became the first center to provide a three-port, three-layer fetoscopic repair of OSB via a laparotomy-assisted approach in the German-speaking area. We point out that under the guidance of experienced centers and by intensive multidisciplinary preparation and training, a previously described and applied technique could be transferred to a different setting.

Non-invasive risk-assessment and bleeding prophylaxis with IVIG in pregnant women with a history of fetal and neonatal alloimmune thrombocytopenia: management to minimize adverse events.

INTRODUCTION: In pregnant women with a history of fetal and neonatal alloimmune thrombocytopenia (FNAIT), prenatal intervention in subsequent pregnancies may be required to prevent fetal bleeding. Several invasive and non-invasive protocols have been published: amniocentesis for fetal genotyping, fetal blood sampling for the determination of fetal platelet count, intrauterine platelet transfusions, and weekly maternal i.v. immunoglobulin (IVIG) infusion with or without additional corticosteroid therapy. This is the first retrospective study that report the experience with a non-invasive protocol focused on side effects of maternal IVIG treatment and neonatal outcome. METHODS: Pregnant women with proven FNAIT in history and an antigen positive fetus were treated with IVIG (1 g/kg/bw) every week. To identify potential IVIG-related hemolytic reactions isoagglutinin titer of each IVIG lot and maternal blood count were controlled. IVIG-related side effects were prospectively documented and evaluated. Furthermore, ultrasound examination of the fetus was performed before starting IVIG administration and continued regularly during treatment. Outcome of the index and subsequent pregnancy was compared. Corresponding data of the newborns were analyzed simultaneously. RESULTS: IVIG was started at 20 weeks of gestation (median). Compared to the index pregnancy, platelet counts of the newborns were higher in all cases. No intracranial hemorrhage occurred (Index pregnancies: 1 case). Platelet counts were 187 × 109/l (median, range 22-239, 95% CI) and one newborn had mild bleeding. No severe hemolytic reaction was observed and side effects were moderate. CONCLUSION: Among pregnant women with FNAIT history, the use of non-invasive fetal risk determination and maternal IVIG resulted in favorite outcome of all newborns. Invasive diagnostic or therapeutic procedures in women with a history of FNAIT should be abandoned.

Fetal micrognathia: objective assessment and associated anomalies on prenatal sonogram.

OBJECTIVE: To determine the accuracy and characteristics of prenatally detected fetal micrognathia. METHODS: A retrospective analysis of all pregnancies with the suspicion of fetal micrognathia was performed. The affected fetuses were reassessed by estimation of the inferior facial angle (IFA) and the frontal nasomental angle on stored gray scale images to objectively establish the diagnosis. RESULTS: Of the 28.935 ultrasounds (USs) reviewed, 58 cases were eligible and 4 were excluded because of inconclusive data. The mean values for IFA and frontal nasomental angle were 44.8° and 123.3°, respectively. In 33 cases, the pregnancy was terminated. Four fetuses died sub partu or immediately after birth, five were stillborn. Invasive testing in 40/54 cases revealed aneuploidies in 35%. Associated anomalies comprised musculoskeletal disorders (43%) and non-skeletal anomalies (15%). Less than one fifth (9/54) were alive beyond postnatal period. Four fetuses had an isolated micrognathia, one of which was found to have a cleft palate postnatally. CONCLUSION: The diagnosis of micrognathia has a crucial impact on both prenatal and postnatal outcomes of affected individuals due to its association with additional abnormalities. A detailed sonographic survey using objective criteria for defining micrognathia is mandatory. Once the diagnosis is confirmed, an intensive interdisciplinary counseling of the parents is needed.