Rare presentation of Limb Body Wall Complex in a Neonate: Case report and review of literature

The Limb Body Wall Complex (LBWC) aka. Body Stalk Syndrome is an uncommon congenital disorder characterized by severe malformations of limb, thorax, and abdomen, characterized by the presence of thoracoschisis, abdominoschisis, limb defects, and exencephaly. This condition is extremely rare with an incidence of 1 per 14,000 and 1 per 31,000 pregnancies in large epidemiologic studies. Majority of these malformed fetuses end up with spontaneous abortions. We present this rare case with occurrence in a preterm infant of 35 weeks gestation. Our report highlights majority of the clinical presentations as reported in previous literature, but the significant pathological findings of absent genitalia and malformed genitourinary, anorectal malformations make this case presentation an even more rare occurrence. Infant karyotyping was normal male and there is no specific underlying genetic correlation in this condition which has fatal prognosis.

Postoperative Respiratory Compromise following Cesarean Birth: The Impact of Obesity and Systemic Opioids

Objective The aim of this study was to measure the effect of obesity and systemic opioids on respiratory events within the first 24 hours following cesarean. Methods Opioid-naive women undergoing cesarean between January 2016 and December 2017 were included in this retrospective cohort study. The primary outcome was the proportion of women experiencing at least one composite respiratory outcome (oxygen saturation less than 95% lasting 30+ seconds or need for respiratory support) within 24 hours of cesarean. The impact of obesity and total systemic opioid dose in 24 hours (measured in morphine milligram equivalents [MMEs]) on the composite respiratory compromise outcome were evaluated. Results Of 2,230 cesarean births, 790 women had at least one composite respiratory event. Predictors of the composite respiratory outcome included body mass index (BMI) as a continuous variable (odds ratio = 1.063 for every one unit increase in BMI [95% confidence interval (CI): 1.021–1.108], p = 0.003), and MME (odds ratio = 1.005 [95% CI: 1.002–1.008], p = 0.003), adjusting for magnesium sulfate use. The interaction between obesity and opioid dose demonstrated an odds ratio of 1.000 (95% CI: 0.999–1.000, p = 0.030). Conclusion The proportion of women experiencing respiratory events following cesarean birth increases with the degree of obesity and opioid dose. Key Points

Complete molar pregnancies with a coexisting fetus: Pregnancy outcomes and review of literature

To review obstetric outcomes of complete hydatidiform molar pregnancies with a coexisting fetus (CHMCF), a rare clinical entity, we performed a retrospective case series of pathology-confirmed HMCF. The cases were collected via a private Maternal-Fetal Medicine physician group on social media. Each contributing institution from across the United States obtained informed consent and institutional data transfer agreements as required, then transmitted the data using a HIPAA-compliant modality. Data collected included maternal, fetal/genetic, placental and delivery characteristics. Nine institutions contributed 14 cases. We found that the median gestational age at diagnosis was 12 weeks 2 days (9w0d - 19w4d), and over half were diagnosed in the first trimester. Sixty-four percent of CHMCF cases were a product of assisted reproductive technology. Placental mass size universally enlarged over the surveillance period. When invasive testing was performed, insufficient sample or no growth was noted in 40% of the sampled cases. Antenatal complications occurred in all delivered patients. Four patients developed gestational trophoblastic neoplasia. This is the largest reported series of obstetric outcomes for CHMCF, and highlights the need to counsel patients about the severe maternal and fetal complications in continuing pregnancies, including progression to gestational trophoblastic neoplastic disease.

Respiratory and gastrointestinal management of an infant with a birth weight of 258 grams

Background: Nowadays, more infants weighing ≤ 300 g are born, and they survive because of the improvements in neonatal care and treatment. However, their detailed clinical course and neonatal intensive care unit management remain unknown due to their low survival rate and dearth of reports. Case Presentation: A male infant was born at 24 weeks and 5 days of gestation and weighed 258 g. The infant received 72 days of invasive and 92 days of noninvasive respiratory support, including high-frequency oscillatory ventilation with volume guarantee and noninvasive neurally adjusted ventilatory assist. Meconium-related ileus was safely treated using diatrizoate. Although he was diagnosed with severe bronchopulmonary dysplasia and retinopathy of prematurity requiring laser photocoagulation, he had no other severe complications. He was discharged 201 days post-delivery (3 months of corrected age) with a weight of 3396 g. Conclusions: Although managing infants weighing ≤ 300 g is difficult, our experience shows that it is possible by combining traditional and modern management methods. The management of such infants requires an understanding of the expected difficulties and adaptation of existing methods to their management. The management techniques described here should help improve their survival and long-term prognosis.

Conservative Management of Presumed Fetal Anemia Secondary to Maternal Chemotherapy for Acute Myeloid Leukemia

Acute myeloid leukemia occurs rarely during pregnancy. When it is diagnosed remote from term, treatment in the form of daunorubicin plus cytarabine induction with consolidative cytarabine is typically undertaken after the first trimester. There is little data to guide fetal monitoring, in particular, whether and how often middle cerebral artery peak systolic velocity (MCA PSV) should be measured to screen for fetal anemia. Cytarabine may be particularly myelosuppressive to the fetus, but information pertaining to the management of this complication is also lacking in published literature. To our knowledge, we present the first case of presumed severe fetal anemia related to in utero exposure to chemotherapy that was managed conservatively with close sonographic monitoring, including serial measurement of MCA PSV. This case suggests that in the absence of hydrops fetalis or other signs of fetal decompensation, expectant management with ultrasound twice weekly, including MCA PSV, is appropriate. Ultrasounds may be decreased to weekly when MCA PSV does not suggest fetal anemia. Screening for fetal anemia can provide helpful information to guide the timing of chemotherapy administration and delivery. Key Points

Ethnic Disparities in Coronavirus Disease 2019 after the Implementation of Universal Screening in Hartford, Connecticut

Objective The aim of this article was to estimate the prevalence of coronavirus disease 2019 (COVID-19) in Connecticut, examine racial/ethnic disparities, and assess pregnancy outcomes in pregnant women following the implementation of universal screening for the virus. Materials and methods This is a retrospective cohort study of all obstetric patients admitted to our labor and delivery unit during the first 4 weeks of implementation of universal screening of COVID-19. Viral studies were performed in all neonates born to mothers with severe acute respiratory syndrome coronavirus 2. We calculated the prevalence of COVID-19, compared the baseline characteristics and pregnancy outcomes between those who tested positive and negative for the virus, and determined the factors associated with COVID-19. Results A total of 10 (4.6%) of 220 women screened positive for the virus. All were asymptomatic. Week 1 had the highest prevalence of infection, nearing 8%. No neonates were infected. Hispanics were more likely to test positive (odds ratio: 10.23; confidence interval: [2.71–49.1], p = 0.001). Obstetric and neonatal outcomes were similar between the groups (p > 0.05). Conclusion Although the rate of asymptomatic COVID-19 was low, ethnic disparities were present with Hispanics being more likely to have the infection. Key Points

Neurally Adjusted Ventilatory Assist in Very Prematurely Born Infants with Evolving/Established Bronchopulmonary Dysplasia

Background During neurally adjusted ventilatory assist (NAVA)/noninvasive (NIV) NAVA, a modified nasogastric feeding tube with electrodes monitors the electrical activity of the diaphragm (Edi). The Edi waveform determines the delivered pressure from the ventilator. Objective Our objective was to determine whether NAVA/NIV-NAVA has advantages in infants with evolving/established bronchopulmonary dysplasia (BPD). Methods Each infant who received NAVA/NIV-NAVA and conventional invasive and NIV was matched with two historical controls. Eighteen NAVA/NIV-NAVA infants’ median gestational age, 25.3 (23.6–28.1) weeks, was compared with 36 historical controls’ median gestational age 25.2 (23.1–29.1) weeks. Results Infants on NAVA/NIV-NAVA had lower extubation failure rates (median: 0 [0–2] vs. 1 [0–6] p = 0.002), shorter durations of invasive ventilation (median: 30.5, [1–90] vs. 40.5 [11–199] days, p = 0.046), and total duration of invasive and NIV to the point of discharge to the local hospital (median: 80 [57–140] vs. 103.5 [60–246] days, p = 0.026). The overall length of stay (LOS) was lower in NAVA/NIVNAVA group (111.5 [78–183] vs. 140 [82–266] days, p = 0.019). There were no significant differences in BPD (17/18 [94%] vs. 32/36 [89%] p = 0.511) or home oxygen rates (14/18 [78%] vs. 23/36 [64%] p = 0.305). Conclusion The combination of NAVA/NIV-NAVA compared with conventional invasive and NIV modes may be advantageous for preterm infants with evolving/established BPD.

Factors Associated with Meeting Obstetric Care Consensus Guidelines for Nulliparous, Term, Singleton, Vertex Cesarean Births

Objective This study aimed to identify factors associated with meeting the Obstetric Care Consensus (OCC) guidelines for nulliparous, term, singleton, and vertex (NTSV) cesarean births. Materials and methods This was a retrospective case control study of women with NTSV cesarean births between January 2014 and December 2017 at single tertiary care center. Demographics and clinical characteristics were compared between women with NTSV cesarean births which did or did not meet OCC guidelines. A multivariable logistic regression model was used to evaluate the effect of each variable on the odds of meeting OCC guidelines. Results There were 1,834 women with NTSV cesarean births of which 744 (40.6%) met OCC guidelines for delivery and 1,090 (59.4%) did not. After controlling for confounding factors, the odds of meeting OCC guidelines were increased for in-house providers managing with residents (adjusted odds ratio [aOR] = 2.03, 95% confidence interval [CI]: 1.44–2.87) and without residents (aOR = 1.66, 95% CI: 1.30–2.12), compared with non-in-house providers managing without residents. There was no significant difference in the odds of meeting OCC guidelines for in-house providers managing with or without residents (aOR = 1.23, 95% CI: 0.84–1.79). Conclusion After adjusting for confounding factors, in-house provider coverage, regardless of resident involvement, is associated with increased odds of NTSV cesarean births meeting OCC guidelines. Key Points

Clinical Implications of Second and Third Trimester Surveillance Ultrasounds of Growth-Restricted Fetuses

Objective We sought to investigate the positive predictive value of ultrasound-diagnosed fetal growth restriction (FGR) for estimating small for gestational age (SGA) at birth. Secondary objectives were to describe clinical interventions performed as a result of FGR diagnosis. Study Design This was a retrospective cohort of pregnancies diagnosed with FGR over 3 years at a single institution. Maternal demographics, antenatal and delivery data, and neonatal data were collected. Descriptive statistics and linear regression were conducted. Results We included 406 pregnancies with diagnosis of FGR in second or third trimester. Median birth weight percentile was 17 (interquartile range: 5–50) and only 35.0% of these fetuses were SGA at birth. The positive predictive value of a final growth ultrasound below the 10th percentile for SGA at birth was 56.9%. Patients averaged eight additional growth ultrasounds following FGR diagnosis. One hundred and fourteen (28.1%) received antenatal steroids prior to delivery, and 100% of those delivered after more than 7 days following administration. There were 6 fetal deaths and 14 neonatal deaths. Conclusion In the majority of cases, pregnancies diagnosed with FGR during screening ultrasounds resulted in normally grown neonates and term deliveries. These patients may be receiving unnecessary ultrasounds and premature courses of corticosteroids.

Less Invasive Surfactant Administration in Very Prematurely Born Infants

Background Less invasive surfactant administration (LISA) is the preferred mode of surfactant administration for spontaneously breathing preterm babies supported by noninvasive ventilation (NIV). Objective The aim of this study was to determine whether LISA on the neonatal unit or in the delivery suite was associated with reduced rates of bronchopulmonary dysplasia (BPD) or the need for intubation, or lower durations of invasive ventilation and length of hospital stay (LOS). Methods A historical comparison was undertaken. Each “LISA” infant was matched with two infants (controls) who did not receive LISA. Results The 25 LISA infants had similar gestational ages and birth weights to the 50 controls (28 [25.6–31.7] weeks vs. 28.5 [25.4–31.9] weeks, p = 0.732; 1,120 (580–1,810) g vs. 1,070 [540–1,869] g, p = 0.928), respectively. LISA infants had lower requirement for intubation (52 vs. 90%, p < 0.001), shorter duration of invasive ventilation (median 1 [0–35] days vs. 6 [0–62] days p = 0.001) and a lower incidence of BPD (36 vs. 64%, p = 0.022). There were no significant differences in duration of NIV (median 26 [3–225] vs. 23 [2–85] days, p = 0.831) or the total LOS (median 76 [24–259] vs. 85 [27–221], p = 0.238). Conclusion LISA on the neonatal unit or the delivery suite was associated with a lower BPD incidence, need for intubation, and duration of invasive ventilation.