2021年 · 第03卷第04期

Acute fatty liver of pregnancy (AFLP) is a rare but critical obstetric-specific disease with a high fatality rate, posing a serious threat to the safety of mothers and infants. These guidelines were specially formulated to standardize AFLP clinical pathways and to improve maternal and infant outcomes. Based on a two-round questionnaire survey, the guideline development team identified the following nine clinical issues that clinicians were most concerned about, and developed recommendations for each of them: prenatal outpatient screening for AFLP, diagnosis, preoperative risk assessment, delivery modes and timing, anesthesia methods, perinatal complications, selecting AFLP patients for artificial liver treatment, prognostic assessment, and monitoring during treatment. The guidelines cover the key issues related to AFLP diagnosis and treatment that concern clinicians.

Objective: This study was to supply information of the Duchenne muscular dystrophy (DMD) mutational spectrum in 303 Chinese families and further offer 5-year clinical experience of DMD/Becker muscular dystrophy genetic counseling and prenatal diagnosis. Methods: In this retrospective cohort study, three hundred and five pregnancies in 303 pregnant women who has a birth history of DMD/Becker muscular dystrophy patients underwent prenatal diagnosis using multiplex ligation-dependent probe amplification followed by Sanger sequencing between January 2014 and December 2018 at Peking Union Medical College Hospital. The mean age of pregnant women was (33.0 ± 4.1) years old. Karyotype analysis was performed to exclude fetal abnormal karyotype. Results: The detection rate of DMD gene mutation in 303 probands was (296/303) 97.7% with seven families having a negative genetic diagnosis. The mutational spectrum comprised of large arrangements in 288/303 (95.0%) and small mutations in 8/303 (2.6%). Carrier testing was performed among 204 pregnant women among whom, 108 mothers had the same mutation as family proband. Of the 305 pregnancies underwent prenatal diagnosis, 55 of 173 male fetuses were affected. We also performed karyotype analysis and found three abnormal karyotypes of trisomy 21. We even found a fetus with DMD gene mutation and trisomy 21 in a same fetus by further analysis. We also identified two times of germline mosaicism. Conclusion: This study demonstrated the distribution and mutation profile of 303 probands and 305 fetuses. Furthermore, considering the possbility of maternl germilne mosaicism, prenatal diagnosis should be suggested to mothers with a proband whether they carry the causative mutation in their blood or not.

Objective: To investigate the effects of spontaneous labor before elective repeat cesarean delivery (ERCD) on short-term maternal and neonatal outcomes. Methods: This was a prospective cohort study. All consecutive ERCDs, occurring at ≥37 weeks of gestation between July 1, 2017 and December 31, 2019 in Makassed General Hospital, were evaluated. The maternal and neonatal outcomes of 183 laboring women undergoing unscheduled repeat cesarean delivery (URCD) group were compared with those of 204 women undergoing cesarean delivery (CD) without spontaneous labor (ERCD) group. Primary outcomes were "composite adverse maternal outcome" and "composite adverse neonatal outcome." Fisher’s exact and Student’s t tests were used to assess the significance of differences in dichotomous and continuous variables, respectively. Two logistic regression models were constructed to identify risk factors with most significant influence on the rate of composite adverse maternal and neonatal outcomes. Results: "Composite adverse maternal outcome" was significantly more common in women who underwent spontaneous labor ((40/183) 21.9% vs. (19/204) 9.3%, P= 0.001, relative risk (RR): 2.7, 95% confidence interval (CI): 1.50-4.90). Similarly, "composite adverse neonatal outcome" was significantly increased in the URCD group ((24/183) 13.1% vs. (12/204) 5.9%, P= 0.014, RR: 2.4, 95% CI: 1.18-4.98). These adverse effects persisted after adjustment for confounders. Multivariate regression models revealed that, besides labor, CD-order impacted maternal outcome (RR: 1.5, 95%CI: 1.02-2.30, P= 0.036), while CD-order and teenage pregnancy influenced neonatal outcome (RR: 2.1, 95%CI: 1.29-3.38, P= 0.003, and RR: 16.5, 95%CI: 2.09-129.80, P= 0.008, respectively). Conclusion: In our study, spontaneous labor before ERCD, including deliveries at term, was associated with adverse maternal and neonatal outcomes, indicating that it is preferable to conduct ERCD before the onset of labor. Screening women with MRCD may identify those at increased risk for spontaneous labor for whom CD could be scheduled 1-2 weeks earlier. Further large prospective studies to assess the effects of such an approach on maternal and neonatal outcomes are strongly warranted.

Placenta accreta spectrum is a complication of pregnancy, which poses a great risk on maternal health. Historically, hysterectomy was the modality of treatment of such condition, but an approach towards a more conservative management has been in the light recently. This includes several methods with varying rates of success and complications. Expectant management is effective in up to 78%-80% of the cases. The extirpative method is associated with a high risk of postpartum hemorrhage. The success of the one-step conservative procedure depends on the degree of placental invasion, and the triple-P procedure appears to be successful but requires and interdisciplinary approach. Adjuvant treatment options can be tailored according to individual cases, and these include methotrexate injection, uterine devascularization and hysteroscopic resection of retained placental tissues. Follow up after conservative management is crucial to detect complications early, and it can be done by ultrasound, Doppler examination, and trending β human chorionic gonadotropin levels. Conservative management of placenta accreta spectrum can preserve future fertility but should only be done in hospitals with enough experience as it carries a high risk of maternal complications. In the future, more research should be directed to achieve clear guidelines regarding this topic.

Posterior placenta accreta spectrum (PAS) disorders are infrequent but potentially associated with significant maternal mortality and morbidity, especially if not diagnosed prenatally. Analysis of published literature is problematic since most experiences included only a few cases. Knowledge of the risk factors associated with posterior PAS is crucial to identifying mothers at higher risk and ask for high sensitivity studies. Ultrasound has poor diagnostic accuracy in detecting posterior PAS, while magnetic resonance imaging better delineates the posterior uterine wall. In comparison, prenatal imaging’s diagnostic performance in detecting posterior PAS is significantly lower than anterior placenta invasion. Management of posterior PAS depends on several factors, including maternal hemodynamic status, available resources, clinical presentation, and invasion severity. For accreta or increta cases, a compression suture is habitually enough to perform hemostasis. Nevertheless, organ involvement habitually requires a multidisciplinary team with the assistant of a general or coloproctology surgeon. The present article aims to update the risk factors, prenatal diagnosis, and surgical management of pregnancies complicated by posterior PAS.

Congenital diaphragmatic hernia is a congenital fetal disease, which mainly causes pulmonary hypoplasia and pulmonary hypertension. Effective early prenatal diagnosis can detect and predict the prognosis of congenital diaphragmatic hernia in infants, thus provide a reference for prenatal counseling, early intervention, and potential choices for the child’s family. Ultrasound and magnetic resonance imaging are the most commonly used methods for non-invasive examination of the fetus. This paper discusses evaluation parameters based on these two imaging modalities.

Placenta accreta spectrum (PAS) disorders are severe obstetric complications and can cause life-threatening hemorrhage, hysterectomy, or even death. PAS disorders in one of dichorionic diamniotic twins are rare to be seen in clinical practice and lack of standard management strategies. We reported a case of retained placenta creta after selective fetal reduction in one of dichorionic diamniotic twins. A 32-year-old woman with a history of cesarean section was diagnosed with placenta increta in one of dichorionic diamniotic twins at 16 weeks of gestation. After discussion, the couple required to be hospitalized for selective fetal reduction. She received selective termination by ultrasound-guided single fetal intracardiac injection of potassium chloride. The follow-up prenatal ultrasound examinations detected the placenta of the reduced fetus did not stop invade the myometrium of the uterus but developed from placenta increta to placenta percreta. The patient was diagnosed with placental percreta by prenatal ultrasound signs and surgical findings. The patient underwent scheduled cesarean delivery at 34+5 gestational weeks. The outcome of maternal and fetal was satisfactory for our elaborate operation and optimal management. PAS disorders are severe obstetric complications with an increasing incidence. Combined with twin pregnancy makes PAS disorders more intractable. We should learn that selective fetal reduction probably cannot solve it thoroughly. Intensive and standard management is essential to promote maternal and fetal outcomes.

Placenta percreta with bladder bleeding can occur during gestation or postpartum, posing a great threat to both mother and fetus. But it is rare and lacks standard management strategies. We reported four cases suffering from bladder bleeding caused by placenta percreta even with hemorrhagic shock admitted between January 1st, 2011 and December 31th, 2020 in The First Affiliated Hospital of Zhengzhou University. Clinical information, including age, gravidity and parity, ultrasound and magnetic resonance imaging manifestations, onset gestational age, bladder bleeding volume, clinical manifestations under bleeding, diagnosis, hemostatic methods, hospital stay, treatment cost, and prognosis, are presented. Two cases had bladder bleeding during the second trimester, respectively on the 22+3 and 23+5 weeks. Pregnancy was terminated timely. The other two cases had bladder bleeding on the 2nd day post near-term cesarean section when activity. All the four cases achieved successful hemostasis following angiography and concomitant embolization for iliac vessels, and one of them received electrocoagulation hemostasis under cystoscopy, but failed. They all had favorable clinical outcomes and had no long-term complications. The neonatal outcome in the two cases that bladder hemorrhage occurred after near-term c-section was good. The newborns did not survive in two cases in which bladder hemorrhage occurred at the second trimester of pregnancy. Timely termination of pregnancy is recommended when such a condition develops during gestation. Diagnosis of bladder bleeding is relatively easy, for it is characterized by fast speed and large volume, with concomitant distension of the lower abdomen, blood discharge from the urethral orifice, or the indwelling catheter. Interventional embolization is an effective means to treat bladder bleeding caused by placenta percreta, while electrocoagulation hemostasis under cystoscopy must be applied with great caution. For the pregnant women with a high risk of placenta percreta, timely and accurate diagnosis should be achieved during the gestational age, and bladder bleeding should be concerned when placenta penetrates through the anterior wall of uterus.

Indirect effects of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic are difficult to calculate. Fear of intrahospital infection has led to a decrease in the use of emergency services and the performance of elective procedures. Several low- and middle-income countries have seen the number of institutional deliveries reduced, even in the absence of a follow-up program for home births. We present the case of a patient with adequate prenatal care and an institutional delivery plan who, due to the SARS-CoV-2 pandemic, chose to have a home delivery with unsafe conditions. The lack of supervision by health personnel and the absence of an immediate consultation plan facilitated the presentation of postpartum hemorrhage and poor neonatal results. Little attention has been paid during the pandemic to pregnant women who decide to have their birth at home. A broad discussion is necessary in this regard, to regain the confidence of the population and strengthen institutional births, or to strengthen midwife-assisted home births programs. Patients’ fear to acquiring SARS-CoV-2 infection inside hospitals is a factor that must be taken into account in prenatal care programs.

Umbilical cord (UC) embolism is a rare, life-threatening complication of pregnancy. The exact cause of this condition is not yet known. Women with more than one UC abnormality are at risk of UC obstruction; this condition can lead to stasis, ischemia, and in some cases, thrombosis. However, many women with UC abnormalities remain undetected and may not be recognized until after birth. Here, we present a case involving the prenatal diagnosis and successful treatment of umbilical artery embolism in the third trimester with good maternal and fetal outcomes. The risk of UC embolism increases when more than one UC abnormality is identified in a single case. Ultrasound examination in the third trimester of pregnancy should be able to verify the existence of two arteries and one vein in the UC. If necessary, these results can be compared with ultrasound imaging acquired during the first trimester of pregnancy.