Ronald Jaekle

Late gestation fetal magnetic resonance imaging–derived total lung volume predicts postnatal survival and need for extracorporeal membrane oxygenation support in isolated congenital diaphragmatic hernia

PURPOSE Magnetic resonance imaging (MRI) has been used as an imaging modality to assess pulmonary hypoplasia in congenital diaphragmatic hernias (CDHs). The objective of this study was to determine if there is a correlation between late gestational fetal MRI-derived total lung volumes (TLVs) and CDH outcomes. METHODS From 2006 to 2009, 44 patients met criteria of an isolated CDH with a late gestational MRI evaluation. The prenatal TLV (in milliliters) was obtained between 32 and 34 weeks gestation. The measured study outcomes included survival, need for extracorporeal membrane oxygenation (ECMO), and length of stay. RESULTS There were 39 left and 5 right CDH patients. The average TLV was significantly lower for nonsurvivors (P = .01), and there was a significant association between lower TLV and the need for ECMO (P = .0001). When stratified by TLV, patients with a TLV of greater than 40 mL had a 90% survival vs 35% survival for a TLV of less than 20 mL. Furthermore, patients with a TLV greater than 40 mL had a lower rate of ECMO use (10%) than patients with a TLV of less than 20 mL (86%). Shorter length of stay was found to correlate with increasing TLV (P = .022). CONCLUSION Late gestation fetal MRI-derived TLV significantly correlates with postnatal survival and need for ECMO. Fetal MRI may be useful for the evaluation of patients who present late in gestation with a CDH.

Severe micrognathia: indications for EXIT-to-Airway.

The ex utero intrapartum treatment (EXIT) procedure has become an important management option in cases of fetal airway obstruction. Select cases of severe micrognathia may be candidates for EXIT-to-Airway due to high-risk of airway obstruction at birth. Here we present three successful EXIT-to-Airway procedures for the management of congenital micrognathia in its most severe manifestations. Case 1: A 23-year-old G3P1011 with a pregnancy complicated by severe micorgnathia, jaw index <5th percentile, as well as polyhydramnios. At 36 weeks EXIT-to-Airway was performed utilizing a bronchoscopically positioned laryngeal mask airway (LMA) during 23 min of uteroplacental support followed by tracheostomy. Case 2: A 26-year-old G4P0120 with a pregnancy complicated by severe micrognathia, jaw index <5th percentile, and an obstructed oropharynx associated with polyhydramnios. At 37 weeks EXIT-to-Airway was performed with placement of tracheostomy. Case 3: A 36-year-old G6P3023 with fetal magnetic resonance imaging (MRI) revealing esophageal atresia, polyhydramnios, and severe micrognathia with a jaw index <5th percentile. At 35 weeks the patient underwent EXIT-to-Airway with formal tracheostomy during 35 min of uteroplacental bypass. In the most severe cases of fetal micrognathia, EXIT-to-Airway provides time to evaluate and secure the fetal airway prior to delivery. We propose indications for EXIT-to-Airway in micrognathia to include a jaw index <5%, with indirect evidence of aerodigestive tract obstruction such as polyhydramnios, glossoptosis or an absent stomach bubble.

Improved recipient survival with maternal nifedipine in twin-twin transfusion syndrome complicated by TTTS cardiomyopathy undergoing selective fetoscopic laser photocoagulation

OBJECTIVE The purpose of this study was to evaluate the effect of maternal nifedipine on fetal survival when started 24-48 hours before selective fetoscopic laser photocoagulation (SFLP). STUDY DESIGN We conducted a case control study of consecutive cases of twin-twin transfusion syndrome (TTTS) in which TTTS cardiomyopathy was treated with maternal nifedipine 24-48 hours before SFLP, compared with gestational age and stage-matched control cases. The primary outcome was recipient and donor survival. RESULTS One hundred forty-one cases of TTTS were treated with nifedipine, and 152 gestational age- and stage-matched control cases were analyzed. There was a significant increase in overall fetal survival in nifedipine-treated cases compared with control cases (237/284 [83%] vs 232/308 [75%]; P = .015). There is an increase in survival of recipients who were treated with nifedipine in stage IIIA (100% vs 81%; P = .021) and IIIB (93% vs 71%; P = .014); however, there was no difference in donor survival. CONCLUSION Maternal nifedipine is associated with improved recipient survival in TTTS that undergoes SFLP. This is the first study to suggest a benefit of adjunctive maternal medical therapy in patients with TTTS who undergo SFLP.

Giant chorioangiomas: perinatal outcomes and techniques in fetoscopic devascularization.

Objective: To evaluate the intrauterine management and perinatal outcome of pregnancies complicated by giant placental chorioangioma (>4 cm) and elaborate on various devascularization techniques. Materials and Methods: Retrospective review of 10 cases of giant placental chorioangioma evaluated between January 2005 and August 2012. Maternal demographics, prenatal imaging, response to fetoscopic treatment, obstetrical complications, and perinatal outcomes were evaluated. Results: Overall survival was 80%. Seven (70%) cases were associated with obstetrical complications, including polyhydramnios (n = 7), non-immune hydrops (n = 3), and high cardiac output state (n = 5). Five patients underwent fetoscopic devascularization at a mean gestational age of 24.03 weeks with 80% survival. The tumors were devascularized by bipolar coagulation (n = 1), combination of bipolar and diode laser (n = 2), bipolar and radiofrequency ablation (n = 1), and surgical clip application (n = 1). Postoperatively, all survivors had resolution of hydrops and high cardiac output states with survival beyond 60 days of life. Discussion: Fetoscopic devascularization is indicated for high cardiac output states or non-immune hydrops and may require multiple techniques including bipolar coagulation, clip application, and/or laser to interrupt arterial inflow and devascularize the mass.

Fetal Cervical Teratoma: What Is the Role of Fetal MRI in Predicting Pulmonary Hypoplasia?

Objective: To determine whether total fetal lung volumes estimated by MRI could predict lethal pulmonary hypoplasia in a cohort of fetuses with cervical teratomas. Methods: We performed a retrospective cohort study of fetal cervical teratomas from January 1, 2005, through April 1, 2012. The primary outcome was the ability of total lung volumes measured by MRI to predict neonatal mortality specifically due to pulmonary hypoplasia. Measured lung volumes were compared to previously reported normal values. The percent of observed-to-expected lung volume and the percent predicted lung volume were calculated. The positive and negative predictive values were calculated for each variable. Results: Fetal MRI-derived total lung volumes 1 standard deviation below the median for gestational age had a positive predictive value of 100% in predicting lethal pulmonary hypoplasia. Conversely, total lung volumes above this level were uniformly associated with pulmonary survival (100% negative predictive value). Additionally, percent predicted lung volume ≤75.7 and observed-to-expected lung volume ≤68.3 were associated with lethal pulmonary hypoplasia. Conclusion: In this small cohort, MRI-estimated lung volumes were helpful in predicting the presence of pulmonary hypoplasia complicating fetal cervical teratoma.

Use of an Amnioport to Maintain Amniotic Fluid Volume in Fetuses with Oligohydramnios Secondary to Lower Urinary Tract Obstruction or Fetal Renal Anomalies.

Objective: We describe a technique to maintain amniotic fluid in fetuses with severe oligo-/anhydramnios secondary to lower urinary tract obstruction or fetal renal disease when urine production is inadequate to maintain a normal amniotic fluid volume (AFV). Methods: An amnioport was inserted into the amniotic space. The catheter was secured to prevent dislodgment and tunneled to a subcutaneous reservoir. The reservoir was accessed as necessary, infusing normal saline to maintain AFV. Pregnancy continued until term or indicated delivery. Results: Since 2010, 15 patients in this category were considered for an amnioport. Six chose comfort care and one elected percutaneous amnioinfusions. Nine amnioport procedures were performed in eight patients. There were no fetal deaths. All eight had successful restoration and maintenance of amniotic fluid. Delivery ranged from 9 to 96 days after placement (mean 63.7 days). One died due to unrecognized laryngeal web and another one died of pulmonary hypoplasia after preterm premature rupture of membranes. None of the remaining six had pulmonary hypoplasia. Three remain alive. Discussion: Severe oligo-/anhydramnios in the second trimester secondary to fetal anomalies is almost uniformly lethal due to pulmonary hypoplasia without restoration of amniotic fluid. The amnioport procedure may allow pulmonary survival but commits families to postnatal care decisions regarding pulmonary and renal complications.

Intrauterine fetal demise after prenatal diagnosis of congenital heart disease: assessment of risk

Elective deliveries in fetal congenital heart disease (CHD) attempt to balance fetal and neonatal risk with the goal of optimizing overall outcome. However, the magnitude of the risk for intrauterine fetal demise (IUFD) is unclear. This study aimed to (1) determine the rate of IUFD and (2) identify fetal risk factors associated with IUFD.

Accuracy of portable glucose meters for rapid determination of amniotic fluid glucose levels

This study was designed to evaluate the accuracy and feasibility of use of three commercially available portable blood glucose meters to measure amniotic fluid glucose(AFG) levels as compared to an accepted laboratory standard. A prospective study of amniotic fluid from 101 consecutive amniocenteses was performed. Glucose concentration in the amniotic fluid was assessed by hexokinase method in our hospital laboratory (control) and by using three portable meters: Advantage (ADV) (Boehringer Mannheim), Glucometer Elite (ELT) (Bayer), and One Touch II Hospital (T-2) (Lifescan). Twenty consecutive amniotic fluid samples were sent to the laboratory in two vials, the first without additive and the second with potassium oxalate to prevent metabolic activity, to assess the effect of cellular metabolism and time delay on amniotic fluid glucose concentrations. Data are reported as mean +/-SE and were assessed by one-way ANOVA. Of the 101 patients studied, 29 were of gestational age > or = 20 wks. The remaining 72 patients were < 20 wks. All three ambulatory meters demonstrated a linear relationship with control (all P < 0.001). Given a slope of almost 1.0 (m = 0.94) and a y-intercept approaching zero (b = 4.3), the OT2 proved to correlate best with control. ELT: (r2 = 0.55, m = 0.79, b = 22.2) and ADV: (r2 = 0.74, m = 1.45, b = 16.9) both overestimated amniotic fluid glucose. When AFG was < 30 mg/dl via laboratory standard, OT2: (r2 = 0.78, m = 1.05, and b = -2.20, P < 0.001), ADV: (m = 1.02, b = 24.1, r2 = 0.12, P = 0.133). The One Touch II Hospital accurately predicted amniotic fluid glucose at the bedside with excellent correlation including with laboratory standard glucose levels < 30 mg/dl. ADV and ELT proved too inaccurate for clinical use. Control samples were not affected by additives or time delay. These findings confirm that AFG determinations can be obtained rapidly with the OT2 meter at the bedside.

Fetal MRI of Torsed Bronchopulmonary Sequestration with Tension Hydrothorax and Hydrops in a Twin Gestation

While bronchopulmonary sequestration typically has a benign course, this congenital lung malformation has a high mortality rate when associated with untreated in utero tension hydrothorax and hydrops. Hydrops related to bronchopulmonary sequestration is believed to result from torsion of the mass with compromise of the associated blood supply. The impaired venous return of the mass then leads to tension hydrothorax with compression of the heart and mediastinal vessels, impairing global venous return. To our knowledge, this scenario has only been described prenatally by ultrasound. We present the imaging findings of a dichorionic, diamniotic twin gestation with one fetus developing tension hydrothorax and hydrops from presumed intermittent torsion of a bronchopulmonary sequestration. This diagnosis was only able to be confirmed by MRI prior to the use of ultrasound-guided interstitial laser photocoagulation for the treatment of this anomaly.

The use of a Google search in the diagnosis of a rare condition.

Background This study aims to describe the use of the Internet in aiding the diagnosis of a rare lower urinary tract finding in a primiparous woman. Methods A case of bladder schistosomiasis was reviewed. Results After the referral from the obstetrics service with a bladder lesion noted on dating ultrasound, an asymptomatic primipara with 9 weeks of gestational age presented for cystoscopy. A polypoid lesion was noted, and suspected diagnosis of schistosomiasis was made using a Google image search. Urine cytologic evaluation confirmed the diagnosis, and the patient was treated without need for further invasive testing or bladder biopsy. Conclusions The usefulness of Internet searches and other electronic resources should be appreciated in the diagnosis of rare conditions.