A Novel VACTERL Assessment Tool to Facilitate Counseling for Expectant Families.

INTRODUCTION: VACTERL is defined as 3 or more of the following congenital defects: vertebral, anorectal, cardiac, tracheoesophageal (TE), renal, and limb. The purpose of this study was to create an easy-to-use assessment tool to help providers counsel expecting families regarding the likelihood of additional anomalies and postnatal outcomes. METHODS: Employing the Kids' Inpatient Database from 2003-2016, neonates (<29 days old) with VACTERL were identified using ICD-9-CM and ICD-10-CM codes. For each unique combination of VACTERL, multivariable logistic regression was used to estimate inpatient mortality, and Poisson regression was used to estimate length-of-stay during the initial hospitalization. RESULTS: The assessment tool used in this study is available at https://choc-trauma.shinyapps.io/VACTERL. 1,886 of 11,813,782 (0.016%) neonates presented with VACTERL. 32% weighed <1,750 g, and 239 (12.7%) died prior to discharge. Associated with mortality were limb anomaly (1.8 [1.01-3.22], p < 0.05), prematurity (1.99 [1.14-3.47], p < 0.02), and weight <1,750 g (2.19 [1.25-3.82], p < 0.01). Median length-of-stay was 14 days (IQR: 7-32). Associated with increased length-of-stay were cardiac defect (1.47 [1.37-1.56], p < 0.001), vertebral anomaly (1.1 [1.05-1.14], p < 0.001), TE fistula (1.73 [1.66-1.81], p < 0.001), anorectal malformation (1.12 [1.07-1.16], p < 0.001), and weight <1,750 g (1.65 [1.57-1.73], p < 0.001). CONCLUSION: This novel assessment tool may help providers counsel families confronting a VACTERL diagnosis.

Risk of donor demise after laser therapy for twin-twin transfusion when complicated by growth discordance and abnormal umbilical artery Doppler findings.

OBJECTIVE: To investigate the natural course of abnormal umbilical artery Doppler (UAD) findings in donor fetuses after laser surgery and the prognostic significance of resolution, persistence, or new onset UAD abnormalities with and without the presence of preoperative growth discordance. STUDY DESIGN: Retrospective cohort study of all monochorionic-diamniotic multi-fetal gestations diagnosed with twin-twin transfusion syndrome (TTTS) undergoing laser surgery at a single large metropolitan referral center from 2010-2016. The estimated fetal weight was measured preoperatively and the UAD were measured both pre- and postoperatively (median = 8 days). Patients were grouped according to the presence of abnormal UAD with or without growth discordance pre- and postoperatively. Risk ratios (RR) were calculated for each group for risk of intrauterine fetal demise compared to the pre- and postoperative normal UAD groups as the referents. RESULTS: There were eighty-one women who met inclusion criteria throughout the study period. Forty-three (53.1%) patients had abnormal donor twin UAD preoperatively and 33 (40.7%) were diagnosed with growth discordance. However, 13 (44.8%) had normalization of the UAD postoperatively (median = 8 days) with a similar rate of donor fetal demise as the non-discordant preoperative normal UAD group (7.7% versus 10.3%, p = .79). Abnormal preoperative UAD was associated with an increased risk of donor demise (RR 3.6, CI 1.1-12.1), which was further elevated in the presence of growth discordance (RR 5.2, CI 1.7-16.3). The greatest risk for donor demise was seen if the UAD remained abnormal postoperatively with concomitant growth discordance (RR 10.3, CI 2.5-41.6). CONCLUSIONS: Preoperative abnormal donor UAD is a significant risk for donor demise post-laser therapy for TTTS. Persistent postoperative abnormal UAD with concomitant growth discordance confers the greatest risk for donor demise. However, resolution of abnormal UAD has a similar rate of donor demise when compared to patients with normal UAD preoperatively.

Impact of recipient twin preoperative myocardial performance index in twin-twin transfusion syndrome treated with laser.

OBJECTIVE: To validate the efficacy of laser therapy for twin-twin transfusion syndrome (TTTS) in the treatment of recipient twin cardiomyopathy and investigate whether severity of preoperative cardiomyopathy can predict fetal survival postoperatively. STUDY DESIGN: Retrospective study of monochorionic-diamniotic (MCDA) pregnancies complicated by TTTS treated by selective fetoscopic laser photocoagulation (SFLP) performed between March 2010 and October 2014 at a single center. The recipient right ventricular (RV) and left ventricular (LV) myocardial performance index (MPI) were measured both pre- and postoperatively. The data were analyzed with the Wilcoxon signed rank and parametric t-tests. RESULTS: Forty-three women met inclusion criteria during the study period. There was a substantial improvement in recipient LV (0.57 ± 0.13 versus 0.43 ± 0.13, p ≤ 0.0001) and RV (0.60 ± 0.16 versus 0.49 ± 0.18, p ≤ 0.0001) MPI postoperatively (median = 8 days). Recipient preoperative LV and RV MPI did not correlate with recipient or donor survival at 24 hours, 7 days or at birth. Thirty-seven recipients (95%) showed improvement in either LV or RV MPI and 22 (56%) showed complete resolution of cardiac dysfunction. CONCLUSION: Laser treatment for TTTS causes rapid improvement in the cardiac function of recipient fetuses. The severity of recipient preoperative MPI does not correlate with survival of either twin postoperatively.

Microwave Ablation for Twin-Reversed Arterial Perfusion Sequence: A Novel Application of Technology.

INTRODUCTION: Twin-reversed arterial perfusion sequence is a rare complication of monochorionic pregnancies that is characterized by the presence of an acardiac mass perfused by an apparently normal pump twin. The risk of death to the pump twin has led to a range of therapeutic interventions aimed at separating their vascular connection. We report a novel application of microwave ablation for vessel coagulation in the treatment of twin-reversed arterial perfusion sequence. MATERIAL AND METHODS: Microwave ablation has been adopted by surgical subspecialties as a superior energy source for vessel and tissue ablation as it creates heat without a circuit and has less thermal spread. We describe the use of a 2.45-GHz microwave system using a 1.8-mm antenna to coagulate the intra-abdominal portion of umbilical vessels of the acardiac mass. RESULTS: We report 6 cases of twin-reversed arterial perfusion sequence treated by microwave ablation. All patients were treated with microwave ablation with successful coagulation of intra-abdominal umbilical cord vessels of the acardiac mass with cessation of flow. DISCUSSION: Microwave ablation is an excellent energy source for vessel coagulation due to its thermal properties and can be used effectively in the treatment of twin-reversed arterial perfusion sequence.

Liver transplantation for acute liver failure at 11-week gestation with successful maternal and fetal outcome.

Acute liver failure (ALF) during pregnancy is very uncommon. Pregnancy-specific liver conditions like hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome and acute fatty liver of pregnancy can cause ALF at term or postpartum, but, typically occur during the third trimester. Most of these patients recover spontaneously after delivery, but, on occasion, they require liver transplantation in the postpartum period. However, ALF during the first and second trimester of pregnancy requiring antepartum liver transplantation is rare. Only fifteen cases of liver transplantation during pregnancy have been reported, and very few occurred during the first trimester. We report a Woman who developed acute liver failure during the first trimester of pregnancy and underwent successful liver transplantation at 11-week gestation, followed by successful delivery of the fetus at 30 weeks. To our knowledge, this is the earliest case of successful liver transplantation during pregnancy followed by successful fetal outcome. We discuss management of the patient and fetus before, during, and after liver transplantation and review the literature on antepartum liver transplant in pregnancy.

Combined sonographic and endoscopic umbilical cord occlusion in twin and triplet gestations.

OBJECTIVE: To review our experience with a minimally invasive technique for umbilical cord occlusion as a method of selective feticide in monochorionic sets. STUDY DESIGN: Umbilical cord occlusion was completed using 3 mm bipolar cautery under ultrasound guidance (3D/4D, n = 6; 2D, n = 6) with pre and post ligation endoscopic evaluation. RESULTS: 12 cases of monochorionic sets where selective feticide was performed were identified during the period 2000 to present. There were four triplet and eight twin gestations in the study cohort. Median interval from intervention to delivery was 16 weeks (range, 5-19). All co-twins survived the procedure and 15 of 16 were delivered after 33 weeks. CONCLUSIONS: Umbilical cord occlusion in monochorionic sets can be accomplished in a minimally invasive manner with combined endoscopic and ultrasound guidance in both twin and triplet pregnancies.

Thrombin-dependent regulation of matrix metalloproteinase (MMP)-9 levels in human fetal membranes.

OBJECTIVE: Amniochorion matrix metalloproteinase (MMP)-9 levels increase during labor, reaching a maximum in patients with preterm premature rupture of membranes (PPROM). Bleeding is a major risk factor for PPROM. Since such hemorrhage into the tissue factor-enriched decidua induces intense thrombin formation, we determined whether thrombin stimulates MMP levels in amniochorionic membranes. STUDY DESIGN: Fetal membrane (amniochorion) cultures were maintained in media with and without thrombin, lipopolysaccharide (LPS), thrombin receptor agonist peptide (TRAP)-14, and the anti-inflammatory steroid, dexamethasone (DEX). Concentrations of MMP-9, MMP-1, and tissue inhibitor of metalloproteinase (TIMP)-1 in culture media were measured by ELISA and normalized to total cell protein. RESULTS: The presence of thrombin induced MMP-9 levels. TRAP-14, a thrombin receptor agonist, also significantly increased MMP-9 levels, suggesting that thrombin-induced changes in MMP-9 expression were mediated through the thrombin receptor. Conversely, levels of MMP-1 and TIMP-1 were not affected by thrombin treatment, indicative of specificity of its action. The presence of LPS increased the concentration of MMP-9 and MMP-1. In contrast, DEX treatment significantly reduced MMP-9 levels. CONCLUSION: Our findings clearly demonstrated that thrombin treatment selectively increased the concentration of MMP-9 in culture media of amniochorionic membranes. Our results provide a potential mechanism through which alterations in hemostasis promote PPROM through thrombin-dependent stimulation of MMP-9.

Three-dimensional ultrasound to differentiate epigastric heteropagus conjoined twins from a TRAP sequence.

Twin reversed arterial perfusion sequence and epigastric heteropagus conjoined twins may appear similar antenatally. Three-dimensional ultrasound evaluated the relationship of a completely formed fetus and an adjacent second body consisting of a pelvis with 2 lower extremities, confirming the final diagnosis when two-dimensional ultrasound was unsuccessful. Three-dimensional ultrasound is useful in diagnosing epigastric heteropagus conjoined twins.

The closure of iatrogenic membrane defects after amniocentesis and endoscopic intrauterine procedures.

OBJECTIVE: To describe a new technique for wound closure after endoscopic intrauterine procedures which prevents amniotic fluid leakage after the procedure. STUDY DESIGN: This is an observational study which reviews a new technique under an IRB-approved protocol. The rationale for this study was the increasing frequency of intrauterine endoscopic procedures. The most common complication of these procedures is persistent leakage of amniotic fluid from puncture sites, which can result in preterm labor and preterm delivery. Thus, these procedures carry a high morbidity rate that may overcome the benefit of the intervention. We have employed a new technique, which has successfully prevented amniotic fluid leakage following the procedure. The instruments used for the endoscopic procedures were no larger than 3.5 mm for all cases. A sealant of platelets was rapidly injected followed by injection of fibrin glue and powdered collagen slurry at each puncture site. Sonography for modified AFI, clinical examination for nitrazine and ferning, and pad count were performed after each procedure at three intervals: immediately after the procedure, 24 h and 48 h. RESULTS: Eight patients undergoing an endoscopic intrauterine procedure (either cord ligation for twin-twin transfusion syndrome or sealing of ruptured membranes after amniocentesis) were included. All patients were treated between 18 and 24 weeks of gestation. Sonography, clinical examination and pad count revealed no evidence of amniotic fluid leakage either intra-abdominally or vaginally in any of the patients. There was 1 patient who ruptured membranes 12 h after the procedure due to severe vomiting. Another patient elected to terminate the pregnancy 48 h after the procedure without evidence of leakage. The remaining patients continued for 8 weeks or more without fluid leakage. CONCLUSION: The technique described, immediate sealing of puncture wounds following endoscopic intrauterine procedures, is effective in preventing amniotic fluid loss after the procedure.